KSAP 2021

When should maintenance HD be initiated in CKD5?

(KDOQI) Hemodialysis Adequacy Work Group recommends that the decision to initiate maintenance dialysis therapy should be based primarily on the presence of symptomatic uremia, protein energy wasting, metabolic abnormalities, and volume overload rather than a specific level of kidney function.

What is the recommendation regarding pregnancy planning in a lupus nephritis patient?

- advised to wait until the patient achieved a stable remission from lupus nephritis for at least 6 months before attempting pregnancy There is an increased risk of adverse maternal and fetal outcome in women with active disease at the time of conception. Medications changes: - Discontinuation of MMF (increased risk of first trimester fetal loss and congenital malformations) -> change to Azathioprine - ACEi avoid in childbearing age who are trying to conceive

Although there are no absolute normal values for arterial and venous pressures on the hemodialysis machine, pressures within the range of -___ to ___ mmHg are generally viewed as "safe"; however, the pressures at a blood flow rate of 250 ml/min are typically much lower.

-350 to 350 venous pressures are outside these parameters and suggest significant resistance to inflow and outflow, which could increase the risk of hemolysis Anything that obstructs the flow of blood, including thrombi at the catheter tip, kinking of blood tubing or catheter extension tubes, small needle size relative to blood pump speed, or defects in the blood flow circuit related to manufacturing problems, can result in increased shear stress and subsequent damage to red blood cells.

When using regional citrate solution, the dose should be adjusted to provide a post-filter ionized calcium level of < __ mmol/L to provide adequate anticoagulation.

0.35 < 0.35 means adequate anticoagulation with current citrate and calcium chloride

Granulomatous inflammation induced by the silicone breast implants leading to overproduction of ____ is the most likely cause of hypercalcemia, hypercalciuria, and recurrent kidney stones.

1,25-(OH)2 vitamin D3 (calcitriol) Activated macrophages in the granulomata may generate calcitriol via extrarenal 1α-hydroxylase, which drives increased intestinal absorption and renal reabsorption of calcium. Normally, 25-(OH) vitamin D (calcidiol) is converted to the physiologically active 1,25-(OH)2 vitamin D (calcitriol) in the proximal tubule of the kidney by 1α-hydroxylase under the direct control of PTH. Hypercalcemia, via negative feedback, suppresses the release of PTH and thereby suppresses the production of calcitriol. However, in granulomatous diseases, 1,25-(OH)2 vitamin D production in mononuclear cells occurs independently of PTH via extrarenal 1α-hydroxylase. Thus, patients with granuloma-mediated hypercalcemia have elevated 1,25-(OH)2 vitamin and suppressed PTH. The formation of granulomatous plaques on the surface of the silicone breast and buttock implants, as in this patient, reflects a chronic inflammatory reaction to the foreign body. Therapy includes glucocorticoids, which inhibits macrophage 1α-hydroxylase. Some patients may require surgical removal of implants to alleviate the problem.

Guidelines advise that patient who want to become pregnant should wait at least __after transplant.

1-2 years In patients without hypertension, impaired graft function (defined as creatinine >1.5 mg/dL), or proteinuria, pregnancy does not generally lead to a decline in kidney function or allograft failure. Pregnancy within the first year of transplant clearly increases the risk of graft failure, and this risk may continue into the second transplant year.

Minimal eligibility criteria for simultaneous liver-kidney transplant are defined as?

1. AKI: eGFR < 25 or the patient must be on dialysis (or a combination of dialysis and eGFR < 25 for 6 consecutive weeks) 2. CKD criteria; eGFR must be < 60 for the previous 90 days and at the time of listing eGFR must be < 30 or the patient must be on dialysis 3. Selected metabolic conditions are also considered

Causes of hemoperitoneum in PD.

1. Benign - retrograde menstruation and ovulation 2. Minor - PCKD with intracyst bleeding or cholecystitis 3. Significant - rupture ectopic pregnancy - colonic perforation, splenic rupture/infarction The initial evaluation of hemoperitoneum in a peritoneal dialysis patient should include assessment of hemodynamic stability. Unstable patients should be hospitalized emergently. If the patient is hemodynamically stable, performing several rapid peritoneal dialysis exchanges can help determine whether the bleeding is persistent. Failure of the effluent to clear would warrant further evaluation, including serial determinations of blood count and imaging studies, as well as hospitalization.

The three major types of replacement fluids?

1. Crystalloid solutions (this includes saline solutions and buffered solutions such as Ringer's lactate or sodium bicarbonate-containing solutions) 2. Colloid solutions (this includes albumin-containing solutions, which may also have significant sodium chloride content, and fluids with hyperoncotic synthetic starches, such as hydroxyethyl starch, gelatin, or dextrans) 3. Blood products or substitutes When crystalloid fluids are infused, the fluid distributes in the extracellular space between the vasculature and the interstitium as governed by capillary Starling forces. There has been growing interest in balanced solutions, such as lactated Ringers, which have lower and more physiologic concentrations of chloride. Hyperchloremic solutions including 0.9% saline have been shown to cause or worsen metabolic acidosis and are associated with a reduction in renal perfusion. Balanced solutions have a neutral or favorable effect on acid-base equilibrium and recent trials have suggested a reduction in mortality, need for renal replacement therapy, and persistent renal impairment with the use of balanced solutions in critically ill patients. Although balanced solutions contain physiologic amounts of potassium (for example 3mEq/L), they have not been shown to cause or worsen hyperkalemia.

Which factors in the nonneoplastic portion of the nephrectomy specimen correlated with progressive loss of renal function after nephrectomy for renal cell cancer?

1. Diabetic nephropathy 2. >10% tubulointersititial fibrosis or atrophy 3. > 5% glomerulosclerosis 4. Severe arterial or arteriosclerosis In patients with symmetric renal function, radical nephrectomy results in an immediate reduction in renal function by 50%, but compensation through hypertrophy and hyperplasia results in recovery such that the final GFR is often 80% of the prior baseline, which may be above the GFR threshold for a diagnosis of CKD. Compared with partial nephrectomy, radical nephrectomy has been shown through retrospective studies, population-based studies, and meta-analysis to be an independent risk factor for CKD after cancer nephrectomy.

The Banff criteria for the diagnosis of chronic active AMR include all of the following?

1. Evidence of chronic tissue injury including transplant glomerulopathy (glomerulitis and glomerular basement membrane double contours). 2. Evidence of current or recent antibody interaction with vascular endothelium including C4d staining in the peritubular capillaries, microvascular inflammation including glomerulitis and peritubular capillaritis, or increased expression of gene transcripts associated with AMR 3. Presence of HLA or non-HLA DSAs

Initial treatment of catheter dysfunction in the HD unit?

1. Forceful saline flush to dislodge a possible small thrombus 2. Instillation of alteplase or similar thrombolytic should be, dwell times 30-60 minutes, versus infusion over 3 hours 3. Diagnostic angiography and invasive tx such as catheter exchange or mechanical distortion of the sheath (sheath stripping)

Name 3 cause of asymptomatic persistent and isolated hematuria of glomerular origin is?

1. IgA nephropathy 2. Hereditary nephritis (Alport syndrome) 3. Thin basement membrane nephropathy

Treatment for Polyarteritis nodosa (PAN) in mild versus severe disease.

1. Mild disease - (for only skin involvement, arthritis, anemia, normal renal function, no cardiac, neuro, GI manifestations: - Corticosteroids alone 2. Moderate to severe presentations - AKI, new or worsening HTN, aneurysm, ischemic disease in limb or GI or cardiac or CNS - combo cyclophosphamide and corticosteroids Relapse is relatively uncommon after remission, but maintenance therapy is often provided for up to 18 months. Untreated PAN is associated with a high mortality, with AKI and gastrointestinal, cardiac, and cerebrovascular complications being among the most common causes of death in these patients. Azathioprine and methotrexate have both been used for maintenance of remission, but neither is preferred for induction therapy; in addition, methotrexate is contraindicated in AKI.

What are two ways to determine the risk of rapid progression in ADPKD?

1. Predicting Renal Outcomes in Polycystic Kidney Disease (PROPKD) score, in which male sex (1 point), hypertension (2 points), first urologic event before age 35 years (2 points), and genotype (truncating PKD1 mutation [4 points], nontruncating PKD1 mutation [2 points], or PKD2 mutation [0 points]) are considered. - A score >6 is consistent with a high risk of progression. 2. Mayo imaging calculator (more commonly use), in which age, height, and total kidney volume are used to determine risk. - Patients with 1C, 1D, or 1E classification are at risk of rapid progression. More generally, and based on an analysis of data from the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP), the risk of progression to stage 3 CKD is highest if total kidney volume >1000 mL (height-corrected total kidney volume >600 mL/m by MRI) or kidney length >17 cm.

The diagnosis and treatment of AMR.

1. Presence of circulating anti-HLA donor specific antibodies (DSA) 2. Allograft biopsy with evidence of peritubular capillaritis and/or glomerulitis in association with c4d deposition by immunofluorescence After confirmation of AMR recommend treatment with plasmapheresis and IVIG to remove donor-specific antibodies

Intervention with renal artery stenting is appropriate in selected patients including patients who have renal artery stenosis?

1. Unable to tolerate antihypertensive medications 2. Significant HTN despite max medical therapy 3. Progressive decline in renal function 4. Recurrent heart failure exacerbations/flash pulmonary edema despite medical management The Cardiovascular Outcomes in Renal Atherosclerotic Lesions (CORAL) trial was the largest study of renal artery stenting. This study found that the addition of renal artery stenting to medical management provided no benefit for the primary composite end point of death, myocardial infarction, stroke, hospitalization for congestive heart failure, progressive renal failure, or need for renal replacement therapy.

The 2015 Update to the Kidney Dialysis Outcome Quality Initiatives (KDOQI) Clinical Practice Guidelines and Clinical Practice Recommendations for Hemodialysis Adequacy is unchanged from prior guidelines. The Update recommends a target spKt/V of ___ and a minimally adequate dose of hemodialysis measured by spKt/V of 1.2, delivered three times per week to patients with residual native urea clearance of <2 ml/min per 1.73 m2.

1.4

There are limited guidelines on helping patients address drug-intolerant hypertension (MDI-HTN), but the literature suggests some success with a stepwise approach?

1.Prescribe fractional doses of conventional antihypertensives, use liquid formulations of conventional antihypertensives, or both. 2.Use transdermal preparations such as clonidine. 3.Consider unconventional antihypertensives such as 5-phosphodiesterase inhibitors. 4.Consider device therapy such as renal denervation. - Renal denervation should be considered only when medical therapy options have been exhausted.

According to the International Practice Guidelines from the Infectious Diseases Society of America (IDSA), a catheter-associated urinary tract infection (CAUTI) is defined by the presence of ≥ ___ CFU/mL of pathogenic bacteria and symptoms that are compatible with a UTI.

10^3 CFU/mL a higher threshold of 10^5 CFU/mL is used to diagnose catheter-associated asymptomatic bacteriuria The presence or degree of pyuria does not help distinguish CAUTI from asymptomatic bacteriuria in a catheterized patient. Hematuria is commonly observed in patients with indwelling urinary catheters and does not distinguish UTI from asymptomatic bacteriuria or other causes of hematuria such as trauma associated with the catheter. Urine appearance or odor is not helpful in diagnosing UTI in patients with catheters.

Dx: Young age at onset of hypertension and hypokalemia with elevated bicarbonate with a state of mineralcorticoid excess. Renin is low. Aldosterone is low. Urinary free cortisol to cortisone ratio is elevated.

11β-hydroxysteroid dehydrogenase deficiency - leads to persistence of cortisol which activates the mineralocorticoid receptor, causing HTN, hypokalemia, and metabolic alkalosis These clinical findings are identical to the presentation of primary hyperaldosteronism, except the aldosterone level is undetectable and the urinary free cortisol to cortisone ratio is elevated. rare autosomal recessive disorder, but more than 35 mutations in the gene for 11β-hydroxysteroid dehydrogenase have been identified. Most affected individuals present in the first two decades, but occasionally, presentation with a milder phenotype may occur at an older age. Patients who ingest glycyrrhizic acid (found in licorice) can also present with apparent mineralocorticoid excess, as glycyrrhizic acid inhibits 11β-hydroxysteroid dehydrogenase.

Given a lack of clear evidence of benefit from any modality, no additional specific treatment for hyponatremia related to chronic liver disease is recommended in asymptomatic patients with serum sodium levels >___ mmol/L. Rather, in the absence of severe hypotension or AKI, patients with evidence of extracellular fluid (ECF) overload such as ascites and edema should be treated with __ therapy, coupled with moderate fluid restriction.

120 diuretic *demeclocycline can lead to AKI in patients with cirrhosis and is not recommended

The normal expected creatinine generation ratio is ___ mg/kg per day with rates in women and elderly people toward the lower end of this range.

15-25 mg/kg per day low creatinine generation is consistent with advanced age, malnutrition, and severe cachexia

The combination of asymptomatic hypercalcemia and PTH level that is normal but not suppressed can be seen in both primary hyperparathyroidism (PHPTH) and familial hypocalciuric hypercalcemia (FHH). A ___ is useful in this setting.

24-hour urine collection Urine calcium excretion of >200 mg/d is consistent with PHPTH, whereas normal or low urinary calcium excretion favors FHH. Although PHPTH can be masked by concomitant vitamin D deficiency or poor dietary calcium intake, which can result in a relatively low urine calcium excretion of <200 mg/d, this patient has normal a 25-hydroxyvitamin D concentration. The calcium-to-creatinine clearance ratio is often considered rather than the 24-hour urine calcium excretion. This calculation is based on the urinary clearance of calcium in a 24-hour urine collection. Ratios of >0.02 are highly suggestive of PHPTH, ratios from 0.01-0.02 are indeterminate, and those <0.01 are suggestive of FHH.

Controlled correction of hyponatremia with a combination of ___ and ___ is indicated for this patient with severe hyponatremia of uncertain duration and a risk for over-rapid correction. Patient comes in with Na 114 and was given 1 L NS bolus and comes in hypovolemic with n/v and on a thiazide at home.

3% saline and IV desmopressin Desmopressin should be given every 8 hours until the serum sodium concentration is >125 mEq/L. The rate of administration of 3% saline can be adjusted to achieve the goal increase in sodium of 6-8 mEq/L each 24 hours until the sodium concentration approaches normal.

The approach to the treatment of idiopathic (or primary) membranous nephropathy (MN) involves an observation period of ___ months, with shorter observation recommended for patients at high risk of CKD progression. This approach is based on the observation that some patients (approximately 30%) will spontaneously remit without immunosuppression.

3-6 months The decision to treat with immunosuppression is therefore based on an estimation of the patient's risk to develop progressive kidney disease. High levels of proteinuria (>8 g/d), older age at onset (>50 years), baseline CKD, and male sex are independent risk factors for progression to advanced CKD.

Which is strongly associated with biopsy related bleeding?

3-6% rate of kidney biopsy complications AKI and CKD have both been independently associated with an increased risk of bleeding after kidney biopsy. The risk increases with higher stages of CKD. This increased risk has been attributed to increased interstitial fibrosis and cortical thinning. In more advanced disease, uremic platelet dysfunction may also play a role. Desmopressin is recommended by some authorities to prevent bleeding for patients with an eGFR <30 mL/min/1.73 m Smaller studies have shown that a BP >160/100 mm Hg is associated with an increased risk of bleeding. Most guidelines continue to recommend cessation of anti-platelet agents 5-7 days prior to the biopsy. Amyloidosis was not shown to be associated with an increased risk of post-biopsy bleeding complications in a large retrospective study.

The target phosphorus goal for patients with ESKD?

3.5-5.5 mg/dL high phosphorus foods: processed food, meats, cheeses, dressings, beverages, bakery products, dark cola A typical goal for phosphate restriction for a dialysis patient is 900 mg/d.

A ___ is needed to determine whether additional antihypertensive therapy is indicated in this patient with pre-dialysis hypertension and intradialytic hypotension.

44-hour ambulatory BP Thus, before changing therapy, interdialytic ABPM is helpful to assess the CV risk and avoid overtreatment.

The kidney biopsy in this patient with AKI in the setting of treatment for Crohn's disease demonstrates acute interstitial nephritis, which can be provoked by treatment with ____.

5-Amicosalicylic acid (5-ASA) aka Mesalamine Kidney biopsy reveals diffuse lymphocytic interstitial inflammation with tubulitis -> AIN

CMV infection is the most common opportunistic infection following solid organ transplantation. The highest-risk recipients are those who are seronegative before transplantation and receive an organ from a seropositive donor as in this patient. In general, guidelines recommend prophylaxis with valganciclovir for __ months in this high-risk group, based on available data suggesting better graft survival and clinical outcomes.

6 months

Mycophenolate can increase the risk of fetal birth defects. It is recommended that mycophenolate be discontinued at least __ __ prior to becoming pregnant.

6 weeks

What is the typical survival time after discontinuation of long-term dialysis?

7-10 days Predictors of rapid decline among patients who discontinued dialysis included male sex, supplemental oxygen requirement, presence of peripheral edema, poor functional status (Palliative Prognostic Score <20), and admission to hospice from an inpatient setting. A systematic review of several studies suggests that median survival after electing to forgo dialysis in a CKD5 is at least 6 months but may range widely between 6-23 months depending on factors such as age, functional status, and comorbid conditions.

Target BP for adults with CKD and hypertension should be?

< 130/90 mmHg These updated guidelines acknowledge that treatment with an ACE inhibitor or an angiotensin receptor blocker (ARB) is reasonable in those with albuminuria of >300 mg/day to slow progression of kidney disease. The evidence supporting the use of renin-angiotensin-aldosterone blockade in patients without proteinuria is less clear.

More recently, the DOPPS investigators have reported an association between higher dialysate bicarbonate levels and mortality, irrespective of pre-dialysis serum bicarbonate levels. Dialysate bicarbonate levels > __mEq/L were associated with a higher mortality than lower dialysate levels, and the hazard ratio was 1.08 for every 4-mEq/L increase in the dialysate bicarbonate concentration (95% confidence interval, 1.01 to 1.15). In this study, there was a strong association between high dialysate bicarbonate concentrations and risk of death from infection.

> 38

Population studies have shown increased mortality in patients receiving HD with serum potassium levels >___ mEq/L.

> 5.8 The risk is especially pronounced in the long intradialytic window that occurs in thrice-weekly HD. The lowest risk for sudden cardiac arrest was observed at a serum potassium level of 5.1 mEq/L.

High-output heart failure is a well-known complication of arteriovenous fistulas or grafts, especially those in the upper arm, with blood flow >___.

>2 L/min

How does obstruction cause hyperkalemia?

A common finding in patients with acute or chronic urinary tract obstruction, or retention is hyperkalemic, hyperchloremic metabolic acidosis disproportionate to the severity of AKI. Hyperkalemia may persist even after the obstruction is relieved. Mechanisms invoked to explain obstruction-associated hyperkalemia include a defect in sodium absorption in the cortical collecting duct (CCD), resulting in impairment in generating a lumen-negative electrical potential with consequent defective hydrogen and potassium secretion (voltage defect). Another proposed mechanism is a generalized defect in hydrogen and potassium secretion due to obstructive damage to the CCD. * Hyperkalemia and NAGMA due to type 4 RTA

Patient with hypomagnesemia, CKD, and early onset DM2. A defect in ____ is the cause.

A defect in hepatocyte nuclear factor-1β (HNF1β) is most likely cause of this patient's presentation characterized by hypomagnesemia, CKD, and early onset of type 2 diabetes mellitus. NF1β is a transcription factor that is involved in the development of the kidney, urogenital tract, pancreas, parathyroid glands, brain, and liver; therefore, mutations in this transcription factor have a variable phenotype and may include multiple abnormalities. Early-onset type 2 diabetes along with pancreatic atrophy, hypokalemia, and hypomagnesemia. This disorder was previously called maturity-onset diabetes of youth (MODY) type 5 or "renal cyst and diabetes syndrome." This condition may mimic Gitelman syndrome because of hypomagnesemia and hypokalemia. Mutation in HNF1β is one of several disorders recognized recently that are characterized by autosomal dominant nonglomerular kidney disease, which results in progressive tubulointerstitial fibrosis and progression to ESRD. Histopathology usually features interstitial fibrosis, tubular atrophy, tubule microcysts, and basement membrane lamellation; immunofluorescence is negative for immunoglobulins and complement. ADTKD subtypes are further classified by the causative gene mutation.

Side effects of hypophosphatemia.

A phosphorus level <1.5 mg/dL can result in encephalopathy, weakness, loss of appetite, seizures, paralysis, and rhabdomyolysis. With phosphorus levels <1.0 mg/dL, respiratory failure may ensue, because phosphorus is essential for respiratory muscle function (as part of adenosine triphosphate).

In a patient with rheumatoid arthritis the most likely diagnosis is ___, characterized by diffuse, amorphous, weakly periodic acid-Schiff (PAS)-positive material in the glomerulus and 10-nm randomly oriented, non branching fibrils on EM.

AA amyloidosis AA amyloidosis is a systemic disease characterized by extracellular deposition of amyloid fibrils derived from the acute-phase reactant, serum amyloid A protein. It is typically the result of a longstanding chronic inflammatory condition such as rheumatoid arthritis or Crohn's disease, although increasingly, patients with unusual causes or no known inflammatory stimulus are being identified. Clinical findings include macroglossia, peripheral neuropathy, hepatomegaly, cardiac wall thickening, and orthostatic hypotension. Unlike AL (primary) amyloidosis, there is no significant underlying plasma cell dyscrasia. Both AL and AA amyloid are weakly PAS-positive and demonstrate birefringence on Congo red staining (as do other forms of amyloidosis). Immunohistochemistry can be used to specifically type amyloid as AA versus AL or one of the hereditary amyloidosis syndromes.

Patient is s/p renal transplant (negative DSA) developing allograft dysfunction with renal biopsy showing: A kidney biopsy revealed minimal interstitial inflammation, no tubulitis, and mild acute tubular injury. Fibrin thrombi and fragmented red blood cells were noted in arterioles and glomeruli, along with endothelial injury and subendothelial thickening. These features were concerning for acute thrombotic microangiopathy. Glomerulitis and peritubular capillaritis were noted. Mild acute tubular injury was noted. C4d staining was positive in the peritubular capillaries. There was faint glomerular IgA staining. Dx.

ABMR causes by de novo donor-specific HLA antibodies this patient presents with acute allograft dysfunction with findings of thrombotic microangiopathy (TMA) on her biopsy. A number of conditions can lead to posttransplant de novo TMA; however, based on this patient's biopsy results, acute antibody-mediated rejection (ABMR) is the most likely cause of the TMA in this case. The features of microvascular inflammation (glomerulitis and peritubular capillaritis) in addition to acute tubular injury, TMA, and peritubular C4d staining are highly suggestive of ABMR. The diagnosis of ABMR is confirmed with detection of donor-specific HLA antibodies, which are expected to be positive based on the biopsy findings. ABMR should be considered in patients who develop posttransplant TMA. She was sensitized prior to transplantation (likely from prior pregnancies), which may have increased her risk of developing ABMR.

Treatment Alport syndrome.

ACEi and ARB reduce proteinuria and may slow disease progression Of note: Kidney transplantation is preferred in patients with ESRD, but recipients may develop anti-GBM disease on de novo exposure to normal type IV collagen in the allograft.

First line therapy for patients with proteinuric CKD and HTN. What is another agent if contraindication (hyperkalemia) to first line therapy?

ACEi/ARB first line Nondihydropyridine CCB shown to have antiproteinuric effect (diltiazem, verapamil) Spironolactone also has antiproteinuric activity when added to an ACEi/ARB but contraindicated in hyperkalemia The KDIGO 2012 Clinical Practice Guideline for the Evaluation and Management of CKD suggests treating to a goal of ≤130/80 mmHg when albuminuria is ≥30 mg/day.

Treatment for hypertension with proteinuric renal disease?

ACEi/ARB most appropriate first line

Patient with stage 5 CKD with fatigue, malaise, dyspnea, and elevated cardiac troponin levels should be considered to have an ?

ACS Many patients with CKD, and a majority of patients with ESRD, have elevated levels of CTN but usually < 0.5 ng/mL at baseline. The relative contribution of reduced renal clearance is minor. Autopsy studies demonstrate that most patients with elevated troponins and kidney disease have some form of myocardial injury. Serial measurements of troponin are as effective in diagnosing an ACS in patients with CKD as in those with normal kidney function. Stable elevation of CTN in kidney disease can be seen with uremic cardiomyopathy, left ventricular hypertrophy, and scarring. Dynamic changes in CTN can occur with myocardial infarction, unstable angina, small-vessel coronary artery disease, hypotension, or decompensated congestive heart failure (CHF).

___ includes multiple conditions that are characterized by progressive loss of kidney function, bland urine sediment, minimal proteinuria, normal blood pressure, normal to small kidneys by ultrasound, and an autosomal dominant pattern of inheritance. These conditions are caused by mutations in the genes for uromodulin (UMOD), hepatocyte nuclear factor-1β (HNF1B), renin (REN), and mucin-1 (MUC1). In this case, the clinical findings, family history, and imaging are all consistent with the diagnosis of __.

ADTKD

Per the Systolic Blood Pressure Intervention Trial (SPRINT) compared primary outcomes of CV morbidity and mortality between non-diabetic pt with intense SBP < 120 versus standard < 140. In the intensive-treatment group had higher likelihood of ___ compared to the standard-treatment group.

AKI The trial was stopped early when a 25% lower risk of cardiovascular morbidity or mortality was noted in patients with more intense BP control.

HUS triad

AKI Thrombocytopenia Microangiopathic hemolytic anemia

Amyloidosis, fibrillary, and cryoglobulinemic glomerulopathy also present with fibrils on electron microscopy, but the size of fibrils and their structure are different. What are the sizes of each?

AL, or light chain-mediated, amyloidosis demonstrates 10-nm fibrils Fibrillary GN shows 20-nm fibrils Cryoglobulinemic glomerulopathy shows 40-nm fibrils (frequently with a "fingerprint" pattern).

Patient with arthralgias, mouth sores, and palpable purpura after cocaine use. Kidney biopsy with crescents with fibrinoid necrosis. IF shows minimal staining. Causes?

ANCA-associated vasculitis - > vasculitis caused by levamisole, which is an anti-helminthic agent added as an adulterant to cocaine. Levamisole has been implicated in numerous cases of ANCA vasculitis among cocaine users. Levamisole activates the subtype 3 muscarinic receptor on neutrophils, causing necrosis, release of intracellular contents, and formation of neutrophil extracellular traps (NETs). Of note, proteinase-3 and myeloperoxidase, the major autoantigens in ANCA vasculitis, are among the proteins externalized in these NETs. Neutropenia is sometimes observed.

__ is a systemic autoimmune disease characterized by arterial, venous, or microvascular thrombosis and recurrent fetal loss.

APS (antiphospholipid antibody syndrome) - APS may be primary or may occur in association with other autoimmune syndromes including systemic lupus erythematosus (SLE). - A paradoxical elevation of the activated partial thromboplastin time (aPTT) is often observed, which reflects in vitro interference with phospholipid-dependent clotting, whereas the in vivo effect is prothrombotic. - venous and arterial clots - thrombocytopenia Dx: The diagnosis is established by a clinical thrombotic event and the identification of a lupus anticoagulant or immunoglobulin G (IgG) or immunoglobulin M (IgM) antibodies directed against cardiolipin or β2-glycoprotein. - Because nonpathogenic, transient elevations of these antibodies may occur, diagnosis requires their presence on at least two occasions 12 weeks apart. Renal: HTN, RAS, renal artery occlusion, TMA, renal vein thrombosis Management consists of anticoagulation with or without immunosuppression, depending on course and the presence of other autoimmune diseases.

___ caused by any of a number of mutations in the PKHD1 gene, which encodes fibrocystin or polyductin. ___ is diagnosed by demonstrating enlarged kidneys with poor corticomedullary differentiation, increased echogenicity, and multiple bilateral microcysts (<2 mm in diameter) within the cortex and medulla. Liver imaging usually shows an enlarged left lobe with common bile duct or intrahepatic ductal dilation and portal hypertension.

ARPKD autosomal recessive polycystic kidney disease Although most patients are diagnosed in utero or in infancy, 25-30% are diagnosed as young adults when they present with signs and symptoms of portal hypertension. Hepatic dysfunction is due to biliary duct malformation that eventually leads to hepatic fibrosis, referred to as Caroli syndrome. Sixty percent of patients with ARPKD will develop ESRD by 20 years of age.

After kidney biopsy a bruit is heard in the kidney and doppler US shows a blue-red flow pattern within hypoechoic area in the lower pole of the left kidney characteristic of ___.

AVF Most AVFs are asymptomatic, do not require intervention, and resolve spontaneously. In some cases, however, they may become aneurysmal and present years later with hematuria and hypertension as noted in this patient. High-output cardiac failure, rupture, infection, mass effect, and impaired kidney function have also been reported. An abdominal or flank bruit is an important diagnostic clue. Computed tomography (CT) angiography and magnetic resonance angiography serve as complimentary diagnostic studies.

Patient with hematuria 5 days after gastroenteritis what is the most likely cause of the hematuria?

Abnormal galactosylation of immunoglobulin A (IgA) IgA nephropathy consisting of visible hematuria appearing within a few days of a mucosal infection Alternatively: ** poststreptococcal GN manifests 1-4 weeks following group A strep, results from deposition of bacterial antigens in the glomerular BM, immune complexes can form in the subepithelial space and may form the characteristic "humps" that can be seen in postinfectious glomerulonephritis.

AKI and nephritic syndrome with positive test for ANA, ANCA, and anti-histone antibodies the most likely diagnosis is drug induced vasculitis which is typically characterized by ___ deposition.

Absent or scant immunoglobulin deposition ("pauci-immune") The trichome stain light micrographic image demonstrates a cellular crescent (largest arrow) and fibrinoid necrosis of the capillary tuft and arteriole wall (small arrows). Hydralazine is a direct-acting vascular smooth muscle potassium channel opener leading to vasodilation. It is used in the treatment of hypertension and, in combination with isosorbide, congestive heart failure. It is known to cause 2 drug-induced rheumatic syndromes: lupus and ANCA-associated vasculitis (AAV). Drug-induced lupus generally presents with constitutional symptoms and serositis; kidney involvement is relatively uncommon. It resolves with cessation of the drug. Hydralazine-induced AAV can present with hemoptysis and pauci-immune glomerulonephritis (GN). Cessation of the drug is necessary, but patients often need systemic immunosuppression as well. ANA and anti-histone antibodies are often positive in both hydralazine-induced lupus and AAV.

The more effective strategy for reducing sxs in patient with hypokalemic periodic paralysis (HPP) is ___.

Acetazolamide HPP is a rare disorder in which patients develop proximal muscle weakness accompanied by hypokalemia precipitated by exercise, fasting, or carbohydrate loading. The condition may be familial or acquired in association with thyrotoxicosis. Hypokalemia results from rapid movement of potassium into cells, and excessive repletion can lead to rebound hyperkalemia because these individuals do not have a total body potassium deficit. Verapamil may reduce the frequency of attacks in a subset of patients refractory to other therapies but is not a first- or second-line treatment.

Patient with staghorn calculi but is a poor surgical candidate with an EF 15%, angina, and is unable to get nephrolithotomy due to surgical risk. What is the most appropriate management?

Acetohydroxamic acid - urease inhibitor that has been shown to reduce stone growth and progression in patients with residual or recurrent struvite stone disease after surgery, as well as those in whom stone removal is not feasible. - SE: palpitations, edema, nausea, vomiting, and diarrhea Prolonged therapy, sometimes coupled with antibiotics for active infection, is necessary, as is dose adjustment for CKD.

The most likely diagnosis in a patient who has been HD-dependent for 10 years and has atrophic kidneys with multiple cysts is ___.

Acquire cystic kidney disease-associated RCC (ACKD-RC) Clinical risk factors include longer duration of dialysis (>10 years) and male sex. Histologically, the tumors can have a cribriform, microcystic, or sieve-like architecture, with eosinophilic or clear cytoplasm or both. Though calcium oxalate deposition is not required for diagnosis, it is often present, both in the tumor as well as the surrounding non-neoplastic kidney.

T-lymphocytic interstitial infiltration is observed in cases of ___ associated with NSAID use.

Acute interstitial nephritis (AIN) Typically, the onset of symptoms is weeks to months after initial exposure to NSAIDs. Although some cases feature pyuria and systemic manifestations such as eosinophilia, fever and rash, many cases present with bland urine sediment and an absence of systemic signs and symptoms. Although AIN cannot be excluded and many studies have confirmed that AIN cannot be diagnosed reliably by any technique other than kidney biopsy

Patient develops AKI after treatment with oral phosphate laxative and multiple phosphate-containing enemas which should prompt suspicion for ?

Acute phosphate nephropathy - Patient with volume depletion and those on RAAS inhibitors are at risk of APN when using oral and rectal phosphate APN has also been reported in transplant patient treated with oral phosphate supplements for hypophosphatemia Labs: AKI, hyperphosphatemia, bland UA, mild proteinuria Renal path: diffuse tubular injury and interstitial fibrosis, abundant calcium-phosphate deposits in the distal tubules and collecting duct, demonstrated by von Kossa stain

What can cause intracellular shift of phosphorus leading to hypophosphatemia?

Acute respiratory alkalosis, refeeding of malnourished individuals, and rapid replication of tumor cells are examples of conditions in which intracellular phosphorus redistribution may result in hypophosphatemia. In acute respiratory alkalosis, the sudden decline in intracellular CO2 leads to a rise in intracellular pH. This is felt to stimulate the glycolytic pathway and specifically phosphofructokinase, the rate-limiting enzyme of glycolysis. The resultant enhanced production of sugar phosphates consumes intracellular phosphate, leading to movement of phosphate into the cell, and causing an abrupt decline in the serum phosphate.

___ is excreted in the urine via glomerular filtration and tubular secretion, but it is relatively insoluble. The doses of __ that are given with IV therapy for serious viral infections can lead to precipitation of crystals in the kidney, particularly in the setting of low urine flow. These crystals are often small and may appear to be engulfed by leukocytes. Their appearance may resemble that of gout crystals in synovial fluid samples. The precipitation of ___ is a well-described cause of AKI related to tubular obstruction. Fortunately, it is usually reversible with volume expansion and improved urine flow rate.

Acyclovir

Infection with ___ is most likely in this kidney transplant recipient with fever, hematuria, bladder inflammation, and interstitial nephritis with viral inclusions.

Adenovirus Adenovirus infection presents fever + gross hematuria within the first 3 months following transplantation. Hemorrhagic cystitis is a well-described complication. Rarely, adenovirus can cause pneumonitis, orchitis, gastroenteritis, and lymphopenia. Dx is made by serum or urine PCR, but kidney biopsy is often performed to evaluate for other causes of allograft dysfunction. Histologic features of adenovirus nephropathy are tubular cell necrosis, nuclear enlargement, basophilic nuclear inclusions, and granulomatous tubulointerstitial nephritis. Immunohistochemical stains confirm the presence of adenovirus antigens. EM shows adenovirus particles 70-80 nm in diameter within tubular epithelial cells. Features of tubular inflammation may be difficult to distinguish from those of cellular rejection. Tx often includes IV immunoglobulin as well as reducing immunosuppression. Cidofovir has been used with inconsistent response. Treatment response can be monitored through serial PCR testing.

The most likely bone pathology is __ in a patient with normal serum calcium and phosphorus and relatively suppressed PTH and alkaline phosphate levels.

Adynamic bone disease - associated with significant vascular calcifications - gold standard dx: bone biopsy, rarely done Patients with adynamic bone disease may present with fracture or complain of bone pain. Serum calcium may be normal or elevated because the bone is unable to take up calcium. High PTH and alkaline phosphatase would exclude adynamic bone disease; in this disorder, both are typically normal. Treatment is targeted at factors that allow PTH secretion to rise. This includes avoiding calcium-based binders, conservative use of vitamin D, and decreasing the dialysate calcium concentration.

Treatment for uremic pruritus (UP).

After optimizing dialysis adequacy (if the patient receives dialysis) and calcium-phosphorus-PTH parameters, the progression of interventions includes a trial of (1) Skin moisturization using emollients and lotions, for which there are limited data but little downside (2) Gabapentin or pregabalin - Gunal et al. showed that administering gabapentin 300 mg three times weekly after dialysis for 4 weeks resulted in a statistically and clinically significant decrease in pruritus. (3) Opioid receptor modulators, ultraviolet (UV) B phototherapy, or acupuncture.

_____ has been identified as a negative prognostic factor in patients with IgA vasculitis for CKD progression.

Age > 65 A recent study of Japanese adults with IgAV showed that age >65 years was an independent risk factor for CKD. Other risk factors for progressive kidney disease include hypertension, proteinuria >1 g/d, and adverse biopsy findings such as crescentic glomerulonephritis and interstitial fibrosis. IgAV, formerly known as Henoch-Schönlein purpura, is a small-vessel vasculitis characterized by acute flares that classically involve the skin, gastrointestinal tract, joints, and kidneys

Air embolism symptoms during HD in a seated versus recumbent patient.

Air embolism occurring in a seated patient may result in migration of air into the venous cerebral circulation, causing loss of consciousness or seizure. In a recumbent patient, air may enter the cardiopulmonary circulation, resulting in chest pain, dyspnea, cough, and hypotension. Air embolism is rare, in part due to protective technology that includes venous air traps and air alarms. Reported cases most frequently are associated with the use of central venous catheters or the administration of anticoagulation or saline during dialysis. Systemic embolization may also occur with consequent ischemia. Rapid recognition can be life-saving. Foam in the venous blood line may suggest the diagnosis. If air embolism is suspected, 100% oxygen should be administered and the patient should be placed in the left lateral decubitus position to confine the air to the apex of the right ventricle and reduce the risk of pulmonary or systemic air embolism. The venous line must be clamped and the blood pump stopped

When is allograft nephrectomy indicated?

Allograft nephrectomy is indicated in the presence of signs and symptoms of rejection in the failed allograft, including graft tenderness, fever, and gross hematuria. Additional indications may include weight loss, anemia, poor responsiveness to erythropoietic-stimulating agents, and fatigue. Also, in those with early allograft failure, preemptive nephrectomy and immediate withdrawal of immunosuppression is recommended.

Biopsy showing splitting of the GBM on EM, with alternating thick and thin regions, as well as lamellated or "basket weave" appearance. Light microscopy findings are nonspecific, but glomerulosclerosis and interstitial fibrosis would indicate advanced disease. Immunofluorescence may demonstrate minor positivity for immunoglobulin or complement, but electron-dense deposits will be absent by electron microscopy dx.

Alport syndrome Because the dermal basement membrane contains type IV α-5 collagen, skin biopsy may be diagnostic in cases of X-linked COL4A5 mutations but is not useful in cases due to COL4A3/4mutations.

___ is caused by a defect in the synthesis of the α-3, α-4, or α-5 chains of type IV collagen, which is a constituent of the glomerular basement membrane (GBM). Most cases of ___ are X-linked mutations in the COL4A5 gene, but autosomal recessive or dominant mutations in COL4A3 and COL4A4 account for a sizable minority of cases. ____ may also present with sensorineural deafness and anterior lenticonus.

Alport syndrome Renal manifestations include proteinuria and hematuria, which typically progress. Onset of ESRD may occur by age 20-40 years.

Patient with hypomagnesemia associated with Gitelman syndrome should be treated with ___.

Amiloride - selective blocker of ENaC The first step in the evaluation of a dysmagnesemia is to calculate the fractional excretion of magnesium (FEMg) to determine renal magnesium (Mg) handling: FEMg = (urine Mg ÷ urine creatinine)/([serum Mg × 0.7] ÷ serum creatinine) × 100% FEMg >2% indicates renal Mg wasting Inappropriate renal magnesium excretion in the setting of hypomagnesemia, in addition to hypokalemia, metabolic alkalosis, and undetectably low urine calcium, is consistent with a diagnosis of Gitelman syndrome. Clinical manifestations commonly include fatigue, weakness, and a tendency to cramps.

Which antibiotic has been reported to cause a Bartter-like syndrome characterized by hypokalemia and metabolic alkalosis hypomagnesemia, and hypocalcemia with or without AKI?

Aminoglycosides (gentamicin and tobramycin) Aminoglycosides such as gentamicin and tobramycin are polyvalent, cationic molecules that are thought to activate the calcium-sensing receptor (CASR) in the thick ascending limb of the loop of Henle. Thus, aminoglycoside therapy can lead to abnormalities that resemble those seen in patients with type V Bartter syndrome, which is caused by gain-of-function mutations in the CASR gene. Gentamicin administration in rats was associated with increased renal excretion of Na, K, Ca, and Mg. At a dose of 40 mg/kg in these animals, gentamicin decreased the expression of NKCC1 in the thick ascending limb of the loop of Henle and induced a Bartter-like syndrome. Clinical symptoms typically resolve after the aminoglycoside is discontinued, although this may take several weeks

The most appropriate next step in the management a pregnant patient with asymptomatic bacteriuria is treatment with ___ for 3-7 days.

Amoxicillin Resistant organisms can use cefpodoxime 100 mg BID for 3-7 days or fosfomycin 3 g single dose *nitrofurantoin should be avoided in patients with CKD and in the first trimester, bactrim safe mid-pregnancy but avoid first trimester and near term

___ is a progressive form of interstitial nephritis due to years of consumption of analgesics. It is most strongly associated with products that included phenacetin (which is metabolized to acetaminophen) in combination with aspirin, codeine, and/or caffeine. The incidence appears to have fallen since phenacetin containing compounds were withdrawn from the market. It is usually associated with some degree of hematuria and sometimes papillary necrosis.

Analgesic nephropathy

Differential diagnosis of chest pain and dyspnea during HD?

Angina Hemolysis Dialyzer reaction Allergy Thromboembolism

A patient with ADPKD and persistent gross hematuria accompanied by hypotension and progressive anemia should undergo which procedure?

Angiography and embolization of the vessel supplying the bleeding cyst Most cases of hematuria may be managed conservatively with intravenous hydration, analgesics, rest, and abdominal compression. However, brisk or persistent bleeding may warrant further intervention.

Progressive decline in renal function due to renal artery stenosis is an indication for ____.

Angiography with intervention with angioplasty (and likely stenting) RAS occurs in about 5% of the general population with hypertension, although prevalence increases with age >65 years. The most common cause is atherosclerotic disease. Clinical trials such as ASTRAL and CORAL have shown no benefit to angioplasty or stenting atherosclerotic renal artery stenosis compared with medical therapy. However, other studies have suggested that there are still certain scenarios where intervention is warranted, including worsening or uncontrolled HTN, progressive loss of kidney function without other cause (>50% within 12 months), decompensated heart failure, or recurrent pulmonary edema. For patients who do not have an indication for intervention - > BP control with a RAAS antagonist, in addition to statin and aspirin therapy. - > Kidney function must be monitored after the initiation of these agents, because RAAS blockade may lead to worsening kidney function in the setting of a hemodynamically significant stenosis (>75%) of a solitary kidney or bilateral stenoses.

A Uk:Ucr < 15 mmol/g suggests ___ in the setting of hypokalemia?

Appropriate renal K conservation in the presence of hypokalemia

A patient with AKI and HIT should be treated with ____ (drug).

Argatroban, agent is metabolized by the liver and is a preferred alternative for patients with AKI or advanced CKD. Patients with HIT are hypercoaguable and require systemic anticoagulation with a non-heparin alternative to prevent or treat thrombosis. Patients with HIT but without a thrombotic complication should receive anticoagulation for 4 weeks. Patients with HIT who experience a thrombotic event should receive anticoagulation for 12 weeks. *** Recurrent thrombosis of the extracorporeal circuit and/or the dialysis access can be an important clue in patients receiving HD or continuous renal replacement. Lepirudin, is renally cleared and has a prolonged half-life in AKI, making it less attractive for patients with severe renal impairment, though it has been used successfully. Danaparoid and fondaparinux are factor Xa inhibitors that are additional non-heparin options.

__ stenosis should be suspected when there is difficulty with cannulation, high negative arterial pressures during dialysis, and a discontinuous bruit and thrill, notably during systole.

Arterial Additional maneuvers on physical examination can also be used to assess the fistula for inflow stenosis These include the following: 1) The "arm elevation test": When the arm is elevated, there will be excessive collapse of the fistula if there is an inflow stenosis. 2) The "pulse augmentation test": Normally, when the access is occluded beyond the anastomosis, the pulse of the fistula is augmented, but if there is an inflow stenosis, then this augmentation is limited. A fistulagram or ultrasound should be ordered for further evaluation. **A venous stenosis might also cause a discontinuous bruit and thrill, but may also cause prolonged bleeding following needle removal or high venous pressure

Imaging and treatment for angiomyolipomas (AML) in the kidneys in a patient with tuberous sclerosis?

At the time of TSC diagnosis, abdominal imaging should be obtained to evaluate for AML. Although most AML are fat-rich, up to 5% are fat poor and these can be difficult to distinguish from renal malignancies. MRI is the preferred imaging modality because of its superior ability to detect and correctly classify fat-poor lesions. Surveillance MRI should be done every 1-3 years thereafter for asymptomatic patients with small, stable AMLs. mTOR inhibitors, such as Everolimus, are recommended for AML >3 cm and/or those increasing in size or in symptomatic patients. Because AML express estrogen and progestin receptors, the risk of acute hemorrhage increases during pregnancy or with use of oral contraceptives. Embolization is first line therapy for AMLs >4 cm, with acute hemorrhage, or with intra-AML aneurysm ≥5 mm. Corticosteroids are used in addition to reduce symptoms related to the inflammatory response of the postembolization syndrome. Partial or radical nephrectomy should be reserved for patients with a nonfunctioning kidney, severe hypertension, or renal malignancy.

Adult-onset distal (type 1) RTA can be a manifestation of ___.

Autoimmune disorders such as Sjögren syndrome, SLE, RA, autoimmune hepatitis, PBC

Costicomedullary kidney cysts, mild hypertension, progressive CKD with bland urine sediment and minimal proteinuria. ESRD occurs in an autosomal dominant, early-onset pattern. Dx.

Autosomal dominant tubulointerstitial kidney disease (ADTKD) ADTKD is usually diagnosed clinically, although genetic testing may be done in certain circumstances. Biopsy may be performed if family history is unavailable or inconclusive for ADTKD. - additional finding would be interstitial fibrosis and tubular atrophy on kidney biopsy

___, regardless of dose, should be avoided in this patient with low TPMT activity. ___ is a purine analog, and its metabolite, 6-mercaptopurine, interferes with DNA synthesis to inhibit cell proliferation. TPMT inactivates 6-mercaptopurine during normal metabolism. Patients with low TPMT activity are more likely to develop drug toxicity, including myelosuppression, due to accumulation of 6-mercaptopurine.

Azathioprine (Imuran)

An asymptomatic elevation of the plasma creatinine level that occurs 3-6 months after transplantation is concerning for ___ (condition) that almost exclusively affects a kidney allograft in the setting of immunosuppression. Prevalence varies according to the immunosuppression protocol used, but rates as high as 10% have been reported.

BK nephropathy, an infection mediated by a polyomavirus The combination of BK viremia of >10,000 copies/mL and allograft dysfunction is highly suspicious for this diagnosis. There may be pyuria, hematuria, and inflammatory cells, or the urinalysis may be normal. Urine cytology may reveal BK-infected cells, which have been called "decoy" cells because of their resemblance to renal carcinoma cells, but this finding is neither sensitive nor specific A kidney biopsy with specific staining for the virus, using an antibody targeting the related SV40 virus, is usually performed to confirm the diagnosis and exclude any concurrent process such as rejection. There are no proven strategies to treat BK virus infection. Recent guidelines based on non-randomized data recommend reducing immunosuppression as the main treatment strategy.

___ toxicity causes central nervous system depression, including somnolence, hypotonia, and coma, occasionally complicated by seizures. Because it is a small molecule and minimally protein bound, ___ is easily dialyzable. Dialysis is recommended for patients with acute or chronic kidney disease with neurotoxicity from __.

Baclofen Baclofen is a synthetic derivative of the neurotransmitter γ-aminobutyric acid (GABA) and is used as a muscle relaxant. Baclofen is primarily renally excreted, with 75-80% cleared by the kidney and 15-20% metabolized by the liver. With normal kidney function, the half-life of baclofen is 2-4 hours. However, with decreased kidney function, the half-life is proportionally increased.

___ is a chronic, progressive tubulointerstitial kidney disease, strongly associated with urothelial cancer, that is prevalent in the southeastern region of Europe along the Danube River. It is thought to relate to chronic, low-level exposure to aristolochic acid (AA), although genetic and other predisposing factors may also play a role. The clinical presentation can be similar to ADTKD, and it can affect multiple family members with similar exposures. The kidneys typically appear atrophic by ultrasound by the time CKD is advanced.

Balkan endemic nephropathy

___ is caused by chronic aristolochic acid exposure and also causes chronic interstitial nephritis.

Balkan endemic nephropathy

What is eosinophilic peritonitis in a PD patient?

Benign condition that usually resolves spontaneously Eosinophilic peritonitis, also known as peritoneal eosinophilic serositis Sxs: abdominal pain, cloud peritoneal fluid It is characterized by an eosinophilic pleocytosis in the peritoneal dialysate fluid, may have peripheral eosinophilia The condition is thought to relate to an allergic reaction to some component of the dialysis process: the dialysate fluid, air, blood, catheter, plasticizers, and sterilants have all been postulated as the causative agent in various cases. Rarely, a PD infection can present with an eosinophilic predominant pleocytosis, so the PD fluid should also be sent for bacterial culture. Typically, eosinophilic peritonitis resolves spontaneously, but rarely it can persist for months. If this occurs, drug reactions, parasitic infections, vasculitis, and intraperitoneal malignancy should all be considered. Both oral antihistamines and systemic corticosteroids have been used in persistent cases.

AKI following a history of recent exposure to an undisclosed liquid laxative is highly suggestive of acute phosphate nephropathy. Kidney biopsy findings?

Biopsy findings include von Kossa-positive deposits of calcium phosphate in the distal tubule and interstitium. Risk factors for acute phosphate nephropathy including age, female sex, CKD, the use of an angiotensin converting enzyme inhibitor, and diuretics.

Urinary leak or urinoma is usually due to ureteral or bladder injury resulting in collection of urine outside the bladder. The frequency of this complication is 0.8-9.3%, usually occurring in the first month following transplantation. The most frequent causes include trauma to the ureter during dissection or ischemia of the distal ureter resulting in necrosis or acute rejection. Initial step?

Bladder decompression with a catheter is a critical initial step in the management of suspected leaks - The catheter reduces intravesical pressure and in some cases may resolve the leak completely. Urine leaks can also occur at the ureterovesical anastomosis, particularly in patients with diabetes mellitus who may have a friable and thin bladder mucosa. The increased creatinine concentration in the fluid aspirate relative to the serum creatinine is indicative of a urine leak. Dx confirmed by demonstration of contrast extravasation on cystogram or antegrade nephrostogram, or by radioactive tracer accumulation outside the collecting system and bladder on nuclear medicine scanning. Definitive treatment depends on the etiology and may often require surgical intervention.

MOA ACEi

Block angiotensin-mediated aldosterone release by preventing conversion of angiotensin I to angiotensin II (constricts efferent arterioles > afferent), which normally stimulates the cells of the zona glomerulosa of the adrenal gland to secrete aldosterone. ACEi => lead to increase serum creatine caused by reduction in intraglomerular pressure => increase in serum creatinine up to 20-30% is physiologic -> greater increase look for Renal artery stenosis or another cause decline GFR Hyperkalemia is an uncommon side effect of ACE inhibitors (or angiotensin receptor blockers) in the absence of additional risk factors such as diabetes, decreased kidney function, decompensated heart failure, or co-administration of potassium-sparing diuretics.

SIADH versus CSW based on fractional excretion of uric acid and fractional excretion of phosphate

Both SIADH and CSW have hypo-osmolar hyponatremia, manifested by excretion of an inappropriately concentrated urine with respect to plasma osmolality. Hyperosmolar urine, hypouricemia, and high (>10%) fractional excretion of urate (FE urate) are features common to both disorders Both syndromes are characterized by elevated levels of antidiuretic hormone. Volume status can be the most important means of distinguishing these disorders, as patients with SIADH are, by definition, clinically euvolemic, whereas those with CSW appear to be hypovolemic. Other potentially distinguishing features include resolution of the high FE urate with resolution of hyponatremia in SIADH, but a persistently high FE urate in CSW. In the setting of hypophosphatemia, a high FE phosphate (>20%) is reported to be a feature of CSW but not of SIADH. Furthermore, patients with CSW demonstrate improvement in plasma sodium and dilution of urine after correction of volume contraction, whereas those with SIADH do not improve. This divergent response is the basis for treatment of CSW with isotonic volume expansion, a strategy that typically results in progressive hyponatremia in SIADH.

How does nephrocalcinosis appear on renal US?

Bright, hyperechoic medullary interstitium with acoustic shadowing characteristic of nephrocalcinosis Nephrocalcinosis is defined as the parenchymal deposition of either calcium oxalate or calcium phosphate The distribution of nephrocalcinosis is most often medullary. Cortical nephrocalcinosis is unusual and may develop from severe AKI such as cortical necrosis **Nephrolithiasis appears most often as a bright, echogenic focus with shadowing. Hydronephrosis would appear as echo-lucent dilatation of the collecting system

Treatment of infected AVG?

Broad-spectrum antibiotics for a minimum of 3 weeks (adjusted depending on culture results) Immediate surgical debridement because of the risk of progressive sepsis as well as the high likelihood that the graft will require varying degrees of surgical resection and debridement **Prosthetic arteriovenous grafts are at increased risk for infectious complications compared with native arteriovenous fistulas. This is in part consequent to repetitive cannulation of the foreign material.

___ or ___, are manifestations of severe hyperparathyroidism. ___, which are collections of active osteoclasts, may present in several locations, including long bones, clavicles, and digits. ___ appear as well-defined, radiolucent areas within the bone, whereas the lesion seen here is extra-osseous with increased calcification.

Brown tumors, or osteitis fibrosa cystica

In AVF cannulation which technique can be used if AVF is only a short segment or aneurysm is present in other areas?

Buttonhole technique - track is created with repetitive cannulation of the same path by sharp needles, followed by blunt needle cannulation - reduce pain, unsuccessful attempts, bleeding, hematoma, and aneurysm formation - higher incidence of infection (poor cannulation technique) - preferred technique for self cannulation

An inactivating mutation of ___ results in the syndrome of familial hypocalciuric hypercalcemia.

CASR gene

__ and ___ appear to be beneficial to reduce BP variability.

CCB and thiazide diuretics In a cohort of patients with CKD, those in the lowest quartile of visit-to-visit BP variability were more likely to be taking a calcium channel blocker than those in the higher quartiles. Similarly, the participants in the ALLHAT trial randomized to receive chlorthalidone and amlodipine had lower visit-to-visit BP variability 6-28 months after randomization, whereas those randomized to receive lisinopril had higher variability. Similar patterns were observed in the SPRINT trial, with lower BP variability seen in participants receiving thiazide-type diuretics and calcium channel blockers. Short-term BP variation (BPV) is defined as fluctuations over 24 hours as demonstrated with an ambulatory BP monitor, whereas long-term BPV is defined as changes over days to months, such as those identified at office visits. A "normal" degree of BPV has not been established, but higher levels of BPV compared with a population mean are associated with increased CV risk as well as an increased prevalence of CKD.

As many as 60% of patients with childhood renal malignancies and 33% of those with nonrenal malignancies will develop ___ after 18 years.

CKD Risk factors for CKD include nephrectomy, total body irradiation, and cisplatin and ifosfamide exposure. Significant albuminuria is uncommon. CKD is usually attributable to chronic tubulointerstitial disease.

__ syndrome typically presents with fever, malaise, and leukopenia. Other potential end-organ __ manifestations include esophageal ulcers, gastritis, colitis, hepatitis, and meningoencephalitis.

CMV

__ (drug) cause vasoconstriction and acute tubular injury with vacuolization. Thrombotic microangiopathy can also occur. Chronic injury appears as atrophy and fibrosis of the interstitium in a striped pattern ("striped fibrosis"), often with prominent medial hyalinosis of the arterioles. The initial management strategy consists of dose reduction of the ___ (drug). .

CNIs If this is unsuccessful, conversion to an alternative immunosuppressive is indicated. Note that trough levels provide reasonable targets for CNI dosing, but patients remain at risk for both rejection and toxicity even when levels are maintained within target range. Conversion to belatacept is an attractive strategy for patients with CNI toxicity, but patients who are EBV-negative are not considered candidates because of the higher risk of post-transplant lymphoproliferative disorders. Conversion to sirolimus, a mammalian target of rapamycin inhibitor, could be a reasonable strategy as well, although recent trials have reduced the enthusiasm for the use of this class of drugs based on the higher risk of rejection and alloantibody generation after conversion. In particular, patients with >500 mg/d of proteinuria or estimated GFR <40 mL/min/1.73 m2 are considered poor candidates for conversion based on published trials.

Causes of de novo TMA after kidney transplant?

CNIs AMR Rapamycin Viral infections (CMV) hemolytic anemia, thrombocytopenia, and AKI, may be lacking or subtle in patients with de novo TMA after transplant Although not always successful, discontinuation of CNI is the mandatory first treatment strategy for patients with de novo TMA post-transplant. If TMA resolves, it is sometimes possible to reintroduce a CNI at a lower dose or alternative form without recurrent TMA. Can also replace CNI with belatacept. If TMA persists, complement inhibition with eculizumab may be helpful, as demonstrated in a small study in which 29% of patients with de novo TMA post-transplant were found to have complement mutations. Note that many patients will have normal C3 levels despite abnormalities in the alternative pathway.

___is the gene encoding the α-5 chain of type 4 collagen and is mutated in individuals with X-linked Alport syndrome. Patients with Alport syndrome present with hematuria, progressive kidney disease, and deafness.

COL4A5

Patient with intracranial bleed and brain edema with elevated ICP. He also has oligo-anuric AKI. Hypernatremia is induced with 3% saline. What is the best RRT modality and what prescription should be used to maintain a serum sodium of 150 mEq/L?

CVVHDF with a slow blood flow rate (150 mL/min) and relatively low dialysate flow rate (20 mL/kg per hour), while receiving a post-filter infusion of 3% saline titrated to maintain his serum sodium concentration at the current level. iHD can increase ICP and increase risk for herniation; not recommended To determine an initial rate of post-filter replacement solution, a simple strategy is to assume that there is equilibration of the serum sodium with the dialysate sodium and no net ultrafiltration. The sodium lost in the effluent will equal the difference in the [Na+]; here, 152-140 mEq/L multiplied by the volume of the effluent, in this case, 1.5 L/h (the rate is 20 mL/kg per hour × 75 kg). This results in a negative balance of Na+ of 18 mEq/h. Because 3% NaCl contains 0.512 mEq/mL of Na+, a rate of roughly 35 mL/h would be needed. This rate can be adjusted based on the measured serum sodium concentration during CRRT.

Which immunosuppressive agents have been associated with the development of post-transplant diabetes mellitus (PTDM)?

Calcineurin inhibitors (tacrolimus > cyclosporine) Mammalian target of rapamycin (mTOR) inhibitors (everolimus, sirolumus) Corticosteroids * MMF and azathioprine have NOT been shown to be diabetogenic * Belatacept has lower incidence of PTDM, can avoid CNIs if patient high risk for diabetes

This condition has been reported in 1% of patients with ESRD and rarely in patients with CKD or without kidney disease. The disease is characterized by extremely painful, violaceous plaques or nodules involving the skin and subcutaneous fat, which typically ulcerate and form eschars. There may be purpuric lesions that radiate from the areas of the nodules. Poor healing after biopsy is also a notorious feature. what is the name of the condition and what are risk factors?

Calciphylaxis is also known as calcific uremic arteriolopathy (CUA) Distal calcification of the toes and fingers leading to digital gangrene or acral involvement of the nose or penis was commonly observed. In recent years, it is more common to observe involvement of more central areas of adiposity including the abdomen, buttocks, breasts, and thighs. Often clinical dx. When performed, biopsies of the plaques show calcification (calcium deposition, usually composed of calcium and phosphorus) in the media and intima of small- + medium-sized blood vessels that lie within the subcutaneous fat and dermis. Inflammation of the surrounding fat (panniculitis) and fat necrosis can also be seen. Risk factors - Female - Long dialysis vintage - DM, Insulin injection - Hypercoagulable state (warfarin use) - Autoimmune disease - Liver disease - Malignancy - High levels of calcium, phos, PTH - elevated calcium-phos product - low serum albumin - elevated alkaline phosphatase Medications associated with CUA: - Corticosteroids - Warfarin - Calcitriol - Calcium containing supplements - Phosphate binders - IV iron

How does calcipotriene (calcipotriol) a topical vitamin D analog lead to kidney stones?

Calcipotriene (calcipotriol), a topical vitamin D analog, is an established therapy for psoriasis. It may be used as an alternative or adjunct to topical corticosteroid therapy but can lead to hypercalcemia, particularly if used excessively. Calcipotriene increases intestinal absorption of calcium and phosphorus while suppressing endogenous production of active vitamin D (calcitriol) and PTH. It is noteworthy that hypercalcemia is absent in most patients with nephrolithiasis and vitamin D deficiency. Small clinical trials and large cohort studies of such patients do not show increased risk of stone formation with oral vitamin D supplementation.

___ IV should be given immediately in cases of symptomatic hypermagnesemia because __ blocks most of the toxic effects of magnesium.

Calcium gluconate Alternatively, saline (if hypovolemic) or furosemide (if hypervolemic) is recommended for nonoliguric patients, but calcium gluconate has immediate benefit Effects of acute hypermagnesemia: diminished DTR, quadriplegia, somnolence, and respiratory arrest, hypotension, bradycardia, complete heart block, and cardiac arrest, can lead to hypocalcemia by inhibiting PTH release, hyperkalemia can occur

How does topiramate cause calcium phosphate stones?

Calcium phosphate stones precipitate in elevated urine pH Topiramate can inhibit carbonic anhydrase activity of isoforms in both the proximal and distal tubule of the nephron, which can lead to urinary bicarbonate loss, and this can result in alkaline urine and a metabolic acidosis.

Hypercalcemia, metabolic alkalosis, and AKI consistent with? Low PTH

Calcium-alkali syndrome - enteric calcium load or a non-PTH-mediated release of calcium from bone. Metabolic alkalosis with alkaline urine is consistent with an alkali load - co-ingestion of large amounts of calcium and alkali. - mechanisms of calcium-alkali syndrome are calcium-mediated vasoconstriction, which reduces GFR and the ability to excrete calcium in the urine, and volume depletion from impaired tubular absorption of sodium in the thick ascending limb of the loop of Henle secondary to the inhibition of sodium chloride transport mediated by the calcium-sensing receptor.

A patient develops myoclonus while taking vanco and cefepime with a reduction in renal function, BUN 66, calcium 9, bicarb 24. What is the most likely cause of the neurologic findings?

Cefepime-induced neurotoxicity. Cefepime is predominately renally cleared by filtration and excreted unchanged in the urine. Dose reduction is necessary in the setting of impaired kidney function. The pathogenesis of cephalosporin-induced neurotoxicity is believed to be related to antagonism of inhibitory γ-aminobutyric acid (GABA), which leads to an increase in excitatory response. Altered consciousness, seizures, or myoclonus. Cessation of cefepime usually results in an improvement in symptoms. Because symptoms vary, many believe that this adverse effect is more common than recognized. Uremia, hypocalcemia, and metabolic alkalosis can all cause myoclonus, but the recent use of cefepime should raise the possibility of cefepime toxicity.

The differential diagnosis for polyuria?

Central DI Nephrogenic DI Primary polydipsia Solute diuresis When water diuresis is present and Uosmx24 hour urine volume = total daily osmoses < 600 mOsm = water diuresis Need to distinguish primary polydipsia from DI, when plasma sodium concentration upper range of normal thinking DI to differentiate central from nephrogenic order water restriction/deprivation test

Patient is on CRRT and hypophosphatemic, patient intubated and sedated on tube feeds. Tx options.

Change the enteral nutrition to a formulation with more phosphorus Can change the CRRT replacement solution with one that contains phosphorus IV phosphorus administration when phos < 1

Treatment of lithium-induced hyperparathyroidism.

Cinacalcet - calcimimetic drug that can suppress parathyroid hormone - Lithium may increase absorption of calcium from renal tubules and the gastrointestinal tract. - lithium appears to decrease parathyroid gland sensitivity to calcium by inhibiting the calcium sensing receptor (CaSR), which results in an increase in PTH, a response analogous to that seen with familial hypocalciuric hypercalcemia - PHPT-mediated hypercalcemia may also be aggravated by lithium inhibition of CaSR activity in the loop of Henle

___ is a calcimimetic agent that acts by allosteric activation of the calcium-sensing receptor on parathyroid tissue. It dramatically lowers levels of PTH, and this beneficial effect has been demonstrated in multiple randomized controlled trials beginning with a high-profile study published in 2004. Small studies and a combined analysis of similarly designed trials have also demonstrated a marked reduction in the need for parathyroidectomy to control severe hyperparathyroidism. In addition, the risk of fracture is decreased by 16%-29%.

Cinacalcet - use is complicated by hypocalcemia - if hypocalcemia develops then it must be discontinued, and vitamin D or vitamin D analogue should be used to control secondary hyperparathyroidism

The antibiotic of choice for a patient with UTI with systemic signs and symptoms complicating underlying ADPKD is?

Ciprofloxacin Instead, lipid-soluble antibiotics, such as quinolones and trimethoprim-sulfamethoxazole, should be considered first-line treatments, because these drugs appear to penetrate kidney cysts and achieve adequate concentrations in cyst fluid. Both have good oral bioavailability but IV administration is reasonable in a patient with significant nausea. Although trimethoprim-sulfamethoxazole can penetrate kidney cysts, potassium retention and hyperkalemia can occur. This effect is usually mild but life-threatening cases have been reported. Risk factors appear to include dose, age, kidney function, and other medications or conditions that predispose to hyperkalemia. Therefore, this drug would not be the most appropriate initial therapy in this patient with preexisting hyperkalemia and CKD. ***third-generation cephalosporins do appear to be efficacious for the treatment of infected cysts in ADPKD, but not for initial therapy

Bosniak renal cyst classification

Class I is a simple cyst that is thin walled. This is a normal finding and has no risk of malignancy. Class II is a thin-walled cyst that is minimally complex. It may have a few thin-walled septations. This is also normal and has no risk of malignancy. Class IIF is also thin walled but has features that suggest follow-up is indicated such as calcifications, a large number of septations, or some nodularity. Class III shows indeterminate cystic masses that have thickened walls and some enhancement by CT scan. Roughly 40%-50% of these lesions are malignant. Class IV lesions are nearly all malignant. These cysts have radiologic enhancement and may appear as a solid mass with a cystic or necrotic component.

___ is dysfunctional in familial hypomagnesemia with hypercalciuria and nephrocalcinosis.

Claudin-16/paracellin-1

How is effluent dose calculated for CRRT?

Clearance in CRRT is conveniently measured as the effluent flow rate. This is the sum of the diffusive and convective clearance that takes place with the treatment. For every milliliter of fluid that the machine runs as replacement solution or dialysate, a milliliter of ultrafiltrate of plasma is removed by the machine into the effluent bag. Any additional volume removal that is prescribed also contributes to the effluent flow rate. In this case, the rate of infusion of citrate and calcium, replacement solution, and dialysate all contribute to the effluent dose Total effluent then is divided by the patient's weight to estimate the effluent dose (mL/kg/hour)

___ ___ is a rare glomerulopathy with organized deposits. These deposits are made of type 3 collagen, and the pathognomonic electron microscopic feature is fibers in a transverse band pattern approximately 60 nm in diameter. Light microscopy shows a lobular appearance of glomeruli that can be confused with Kimmelstiel-Wilson nodules typically seen in diabetic nephropathy. Immunofluorescence is generally unremarkable. Pathogenesis of this disease is unknown.

Collagenofibrotic glomerulopathy

___ is a rare glomerulopathy with organized deposits. These deposits are composed of type III collagen. The pathognomonic finding is a transverse band pattern of fibrils approximately 60 nm in diameter on electron microscopy. Light microscopy shows a lobular appearance of glomeruli that can be confused with Kimmelstiel-Wilson nodules. Immunofluorescence is generally unremarkable. Pathogenesis of this disease is unknown.

Collagenofibrotic glomerulopathy

Treatment for hematuria in a patient with ADPKD. Patient presents with flank pain and hematuria after exercise. CT showing multiple hepatic cysts and bilaterally enlarged polycystic kidneys.

Conservative management - bedrest and hydration - sxs resolved 2-7 days with conservative tx - avoid NSAIDs as analgesics - persistent pain or severe bleeding, then percutaneous arterial embolization/nephrectomy is required **Recurrent visible hematuria is a poor prognostic sign in ADPKD and is usually associated with more rapid decline in renal function.

Treatment for secondary FSGS in a patient born prematurely.

Conservative tx: ACEi When proteinuria is in the nephrotic range, secondary FSGS can be difficult to differentiate from primary FSGS. Those with secondary FSGS typically present with indolent onset of proteinuria without nephrotic syndrome, whereas primary FSGS typically presents with nephrotic syndrome. Kidney biopsy of primary FSGS shows diffuse effacement of podocytes, whereas in secondary FSGS, the foot processes may be preserved in regions that are less affected; however, this distinction is controversial. Because IgM may be trapped in areas of podocyte injury, IgM and C3 may be present by immunofluorescent microscopy in either condition. Glomerulomegaly is more consistent with secondary FSGS. Patient has a secondary form of focal segmental glomerulosclerosis (FSGS). In this case, the pathogenesis of secondary FSGS is likely to be the result of adaptive responses to a reduction in nephron number. This leads to glomerular hypertrophy and hyperfiltration and subsequent glomerular injury and fibrosis. The mechanisms of this process are poorly understood but may be related to direct effects from glomerular hypertension or to cytokines and other mediators of inflammation or fibrosis.

The most common cause of peritoneal dialysate outflow failure?

Constipation - trial of laxative is the first step in management Constipation can lead to outflow obstruction because the lumen of the catheter can be obstructed or compressed by the bowel, or the catheter may migrate from the optimal position in the pelvis. Use of laxatives can induce peristalsis, relieve constipation, and improve catheter function. Indeed, patients who perform peritoneal dialysis are encouraged to have at least one bowel movement daily, which often requires regular use of laxatives such as senna, polycarbophil, lactulose, or polyethylene glycol 3350. A plain film of the abdomen may suggest fecal impaction, but even when imaging is normal, a trial of laxatives should still be considered.

Patient with chronic NAGMA that persists despite high-dose oral bicarbonate therapy. Patient has exocrine drainage of the pancreas allograft to the bladder. Bladder-drained pancreas are associated with high risk recurrent UTI, dysuria, hematuria, and bladder stones. Excessive bicarbonate loss is common. Tx?

Convert pancreatic allograft from bladder to enteric drainage - Successful conversion from bladder to enteric drainage results in complete resolution of the metabolic acidosis.

MOA steroids in treatment sarcoidosis associated hypercalcemia

Corticosteroids reduce the 1-alpha hydroxylation that leads to 1,25-(OH)2D formation. By reducing 1,25-(OH)2D production, less calcium is absorbed or reabsorbed from the gastrointestinal tract, kidneys, and bone. Hypercalcemia, in this setting, is mediated by extra-renal production of 1,25-dihydroxyvitamin D3 [1,25-(OH)2D] in granulomatous tissue. Corticosteroids are currently considered the best initial management for sarcoidosis and associated hypercalcemia. Prednisone can be initiated at 0.3-0.5 mg/kg/day, with higher doses for more significant organ impairment. Generally, a starting dose is maintained for at least 4 weeks with a gradual taper to a 5-10 mg maintenance dose for up to 12 months. Other therapies that can reduce conversion of 25-(OH)D to 1,25-(OH)2D include ketoconazole and hydroxychloroquine. Azathioprine, mycophenolate mofetil, and tumor necrosis factor-α inhibitors such as infliximab can also be used in addition to, or in place of, corticosteroids for those patients who are intolerant of or do not respond to corticosteroids.

Nephritic sediment in the presence of systemic manifestations, HTN, AKI, low complement C4, and high RF in a patient with a history of HCV infection makes ___ most likely cause of hematuria and proteinuria.

Cryoglobulinemic glomerulonephritis Cryoglobulinemia can be asymptomatic or result in tissue damage from hyperviscosity (mainly with type I) or immune-complex mediated inflammation (mainly type II, strongly associated with HCV infection). Other associated conditions include infections, such as hepatitis B virus and HIV, and autoimmune disorders, such as Sjögren syndrome, systemic lupus erythematosus, and rheumatoid arthritis. The LM appearance of a kidney biopsy specimen most commonly shows a membranoproliferative pattern, but may also show vasculitis, hyaline thrombi, and crescents. IF reveals deposition of immune complexes in the mesangium and subendothelium that are often IgM-dominant; EM may reveal substructure to the electron-dense deposits with a microtubular or fibrillary pattern. It is important to note that because of independent B-cell proliferation, cryoglobulins can persist even after HCV has been eradicated by antiviral therapy. Treatment for cryoglobulinemia that persists or appears after antiviral therapy may include steroids, rituximab, and plasma exchange in severe cases.

Hexagonal crystals which stone type?

Cystinuria Cystinuria is diagnosed by identification of hexagonal urinary crystals, stone analysis, or increased urinary cystine levels on a 24-hour urine collection. Patients with cystinuria have a mutation in the gene that codes for the proximal tubule transporter for dibasic amino acids: cystine, ornithine, lysine, and arginine ("COLA"). The only clinical manifestation of this disorder is related to the fact that cystine is not soluble in an acid pH thus resulting in nephrolithiasis. should be suspected in patients who develop a kidney stone in adolescence

Which of the following anti-DM drugs can be used with a GFR < 30? A. Glyburide B. Metformin C. Glimepiride D. Glipizide E. SGLT-2

D. Glipizide = only 10% is renally cleared, can be used in a patient with CKD, caution still needed can cause hypoglycemia and can accumulate in a patient GFR < 30 Metformin can accumulate in patients with CKD and, rarely, can cause lactic acidosis. The Food and Drug Administration (FDA) recommends that clinicians reduce the dosage of metformin for those with eGFR in the 30-45 mL/min per 1.73 m2 range; when eGFR is <30 ml/min per 1.73 m2, it should be discontinued altogether. Both glyburide and glimepiride are primarily cleared by the kidney and use of these agents when the eGFR is <60 mL/min per 1.73 m2 is not recommended. SGLT-2 is approved for individuals with GFR not less than 30

Kidney biopsy shows lobular accentuation with increased mesangial cellularity, matrix expansion, and basement membrane thickening. Immunofluorescence microscopy demonstrates linear C3 deposition in the basement membrane. Electron microscopy demonstrates electron dense deposits in a similar distribution. Young patient with persistent c3 hypocomplementemia. dx.

DDD - tend to flare with infection, proteinuria and hematuria at a young age patients with DDD have electron-dense intra-membranous deposits of C3 in a linear or ribbon-like fashion Further evaluation of the alternate pathway is indicated, especially measurement of factor H. Identification of a specific complement abnormality may facilitate treatment. DDD is associated with drusen deposition in Bruch membranes, which may occasionally lead to loss of vision. Patients require serial examinations, and although there is no definitive treatment, close follow-up can address related complications and potentially provide emerging treatments.

Which is a significant risk factor for uric acid stone disease?

DM2 The predominant metabolic abnormality in uric acid nephrolithiasis is low urine pH. Patients who form uric acid stones demonstrate features of insulin resistance, which is associated with defects in renal ammonium production and lower 24-hour urinary pH. However, a trial examining the influence of type 2 diabetes, BMI, age, and sex on uric acid stone formation found that type 2 diabetes was the strongest risk factor independently associated with uric acid kidney stone disease.

The most likely finding on renal US in a patient with nephrotic syndrome and new onset flank pain is ?

Decreased flow in the left renal vein from renal vein thrombosis (may be isolated elevation of LDH) Renal vein thrombosis in the setting of the nephrotic syndrome is poorly understood, but urinary loss of anticoagulant factors such as antithrombin and plasminogen may play a role. Among the causes of nephrotic syndrome, membranous nephropathy is the most highly associated with venous thromboembolism. the risk appears to be greatest in the renal veins where blood may be relatively hemoconcentrated, and the left renal vein is more susceptible, possibly because of its longer length before reaching the inferior vena cava. Risk also appears to increase with greater proteinuria and lower serum albumin levels.

What is multiple drug-intolerant hypertension (MDI-HTN)?

Defined as intolerance to ≥3 classes of antihypertensive drug MDI-HTN is an important cause of pseudoresistance and medication nonadherence in patients with hypertension. It is a relatively common phenomenon, especially in women and older patients. It has been associated with psychiatric morbidity (depression and anxiety).

Water testing at a dialysis facility shows high fluoride levels using reverse osmosis? What measures should be used to prevent fluoride exposure?

Deionization (DI) of the reverse osmosis (RO) product water should be performed to remove fluoride and meet standards for water quality and patient safety. High fluoride levels in dialysate have been associated with nausea, pain, pruritis, and arrhythmia. The AAMI standard for dialysis water is to maintain the fluoride concentration at <0.2 PPM. Levels much higher than this (15-25 PPM) have been implicated in a contamination event that resulted in patient deaths. Long-term fluoride exposure can cause or worsen osteomalacia. Fluoride removal occurs during either RO or DI. In this case, the most likely cause of the high fluoride levels is poor function of the RO membranes. Descaling of the RO membranes should result in resolution of this problem. In the interim, the DI system can be used to polish the RO product water and ensure that the final product water meets AAMI standards. Taking either the water softener or the RO off-line will increase the risk of fluoride contamination, as the system would then rely solely on the DI. The low levels of potential fluoride contamination in this case do not warrant closure of the dialysis unit.

Nephrocalcinosis, increased creatinine, calcium kidney stone, and predominantly non-albumin proteinuria is ___ disease. Progressive CKD and low molecular weight proteinuria. X-linked disorder also known as X-linked recessive nephrolithiasis, and X-linked recessive hypercalciuric hypophosphatemic rickets.

Dent disease The majority of patients have Dent disease 1, caused by an inactivating mutation in the lysosomal chloride transporter gene located on the X chromosome, CLCN5. A minority of patients have Dent disease 2, which is caused by a mutation in the OCRL1 gene, also on the X chromosome. elevated or high-normal calcitriol, hypophosphatemia, and hyperphosphaturia that are commonly observed in Dent disease, PTH low

Treatment for uremic bleeding?

Desmopressin is an analogue of vasopressin. This agent is believed to increase the release of von Willebrand factor from the vascular endothelium Administration of desmopressin can improve the bleeding time within 1 hour, an effect that may last for 4-8 hours. Both HD and PD can improve the platelet dysfunction observed in severe uremia, but multiple treatments are required to obtain maximal improvement. Correction of anemia to a hemoglobin >10 g/dL, whether by RBC transfusion or erythropoiesis-stimulating agents, has been shown to decrease bleeding time Conjugated estrogens have also been used to treat bleeding associated with uremic platelet dysfunction, although the mechanism of benefit is unclear. This strategy has been associated with thrombotic events, so it would not be preferred in this patient with a history of deep venous thrombosis. In addition, although the effect of conjugated estrogens begins within the first day, the peak effect is typically seen at days 5-7. Infusion of cryoprecipitate is another option to address uremic platelet dysfunction.

Which clinical characteristic are correlated with AVF maturation failure?

Diabetes Female sex PAD **A preoperative vein diameter of 2.5 mm or greater has been established as an important predictor of adequate maturation. ** The nondominant arm is preferred for initial access

A dialysis patient with LUE AVF is complaining of numbness in the left fingers and fingers are cool to the touch. Left radial pulse is not palpable. Dx and Tx.

Dialysis access steal syndrome (DASS) causing digital ischemia required surgical evaluation Surgical options: - Revascularization of the radial or ulnar arteries - Banding of the fistula to increase in-fistula resistance - Complete ligation of the fistula Although about 10% of patients describe pain or paresthesia due to some degree of ischemia from DASS, only about 4% require surgical intervention. Longstanding ischemia from DASS can lead to gangrene and ulceration of the digits. Risk factors: female sex, tobacco use, DM, HTN, CAD, and PVD. If surgical intervention is not indicated, running the blood flow rate at a slower rate and reducing the amount of steal or retrograde flow into the fistula can help alleviate symptoms, but the first step in management for this patient would be surgical evaluation

The most appropriate regimen to prevent AV graft failure in a patient with a history of graft thrombosis is ___ and ___.

Dipyridamole and Aspirin

First line treatment for hyperkalemia and mild hyperchloremic metabolic acidosis associated with type 4 RTA?

Diuretic Both thiazide and loop diuretics can be effective, but loop diuretics may be more potent for patients with CKD. In addition to inducing natriuresis and kaliuresis, diuretics improve volume overload and correct the metabolic acidosis. In a volume-neutral or volume-depleted patient, oral sodium bicarbonate can be added to address the metabolic acidosis and provide a source of sodium so that diuretic therapy does not result in symptomatic volume contraction.

___ is an inotrope that also causes peripheral vasodilatation. As such, it is most useful in patients with cardiogenic shock.

Dobutamine

For a prospective kidney donor with a family history of ADPKD the best approach is to perform direct mutation analysis for PKD1 and PKD2 mutation in the ____.

Donor Depending on results, the prospective donor could then undergo direct mutation testing to see if she carries the same (identified) mutation. Linkage analysis for PKD mutations requires at least 3 affected family members across at least 2 generations, and would not be an option in this case. A family history of ESRD before age 58 in at least one family member is highly predictive of a PKD1 genotype, whereas a family history of ESRD after the age of 68 is highly predictive of a PKD2 genotype. Linkage analysis for PKD mutations requires at least 3 affected family members across at least 2 generations, and would not be an option in this case. The presence of more than 10 cysts in both kidneys establishes the ADPKD diagnosis and would exclude donation, whereas the absence of any cysts appears to exclude the diagnosis.

Which donor characteristics are important in determining long-term graft outcome?

Donor-recipient size match, donor age, HLA match, and biological first-degree relationship of donor and recipient kidney transplant from a younger donor is associated with a significantly longer expected duration of function, which is of particular importance to younger recipients. The novel allocation policy in the United States has attempted to maximize kidney life-years by matching younger donors (estimated post-transplant survival (EPTS) score <20%) with better quality kidneys (kidney donor profile index (KDPI) score <20%).

___ is used as second-line therapy for patients with septic shock who are at low risk of tachyarrhythmias.

Dopamine Low-dose dopamine, usually described as <5 µg/kg per minute, primarily activates the dopamine-1 receptors in the kidney, heart, and brain, leading to selective vasodilation. At higher doses, dopamine has a primarily α-adrenergic effects, leading to increased cardiac output and vasoconstriction. At these doses, there is an increased risk of arrhythmias.

Treatment for restless leg syndrome (RLS) in ESRD/CKD patients.

Dopamine agonists (ropinirole) - tolerated well CKD and ESRD - start lowest dose and titrate slowly RLS is a neuromuscular disorder characterized by an urgent need to move the limbs (usually legs) coupled with an unpleasant sensation variously described as itching, crawling, burning, or simply discomfort. It is aggravated by periods of inactivity; thus, it is worse at night when attempting to sleep RLS is very common in patients with ESRD (prevalence reportedly as high as 50%) and may have a unique pathophysiology in this population. RLS is also common in the CKD population, with a reported prevalence ranging from 10 to 30%.

How can DKA have a normal anion gap?

Early in the course of DKA, ketoacids are excreted with sodium or potassium in the urine and the serum anion gap can be normal or only minimally elevated. Retention of the ketoacids and expansion of the serum anion gap does not occur until volume contraction (due to urinary sodium loss related to osmotic diuresis and ketonuria) is severe enough to impair glomerular filtration. In select settings, when the patient presents very early in the course or extracellular fluid volume has been maintained by adequate intake, the kidneys can maintain their ability to excrete ketoacids in the urine and the anion gap can be normal. In this situation, the patient would present with classical signs and symptoms of DKA, including hyperglycemia, glycosuria, ketonuria and metabolic acidosis, but without an elevated anion gap. In this case, the patient is euvolemic and has preserved kidney function. The urine anion gap, or measured urinary net charge gap, can be used to identify excretion of an unmeasured anion. Unmeasured anions can be bicarbonate or non-bicarbonate anions such as lactate or ketoacid anions. Urine anion gap = (urine [Na+] + urine [K+]) - urine [Cl-])

Hypokalemia, NAGMA, and negative anion gap suggest? In a patient with abdominal pain, constipation, vomiting, and poor dentition.

Eating disorder-laxative abuse Diarrhea due to laxative abuse causes hyperchloremic acidosis and hypokalemia secondary to fecal bicarbonate and potassium losses.

A patient presents with microangiopathic hemolytic anemia, thrombocytopenia, and AKI that is not responding to plasma exchange. ADAMTS13 activity is >10%. What treatment should be given?

Eculizumab to treat atypical hemolytic uremic syndrome (aHUS) aHUS is a rare disorder caused by defective regulation of the alternative complement pathway C3 may be low, but this finding is neither sensitive nor specific. Mutations can be found in complement factors H, I, and B and many other components of the alternative complement pathway. Complement dysregulation leads to uncontrolled complement activation, endothelial injury, and the clinical findings of aHUS. Eculizumab, a monoclonal antibody that inhibits C5 activation, can be used to down-regulate complement activation and reduce formation of the membrane attack complex, thereby ameliorating endothelial injury.

The most effective treatment for a patient with postpartum hemolytic uremic syndrome is ?

Eculizumab, a monoclonal antibody that inhibits C5 activation, can be used to treat patients with conditions that result from dysregulated alternative complement activation. Atypical HUS: microangiopathic hemolytic anemia, thrombocytopenia, and AKI due to TMA Mutations complement factor H, I, and B

What is used to confirm primary hyperaldosteronism?

Either intravenous saline suppression or 24-hour urine aldosterone after oral sodium loading can be used to confirm primary hyperaldosteronism. Once primary hyperaldosteronism is confirmed, adrenal vein sampling could be pursued to confirm that the adrenal adenoma identified on imaging is the source of the excess aldosterone.

Initial biochemical testing for pheochromocytoma?

Either measurement of plasma free metanephrines or urinary unfractionated metanephrines Imaging to confirm the diagnosis of pheochromocytoma should only be done after biochemical studies have confirmed the diagnosis. Either CT scan or magnetic resonance imaging can be utilized to localize a pheochromocytoma.

___ is a feared and often fatal long-term complication of PD. It most commonly occurs in patients who have utilized PD for a prolonged duration, typically 8 years or more. Some cases may relate to preceding episodes of peritonitis. High mortality and is associated with progressive clinical deterioration with abdominal pain, vomiting, and weight loss. Intermittent and recurrent bouts of bowel obstruction are common.

Encapsulating peritoneal sclerosis (EPS) Patients often have hypoalbuminemia, elevated inflammatory markers, and resistant anemia. Ultrafiltration failure develops, and patients will often be transitioned to hemodialysis. Unfortunately, symptoms from EPS can continue after transitioning to hemodialysis or even after transplantation. Surgery to dissect the fibrous peel ("intestinal cocoon") that adheres to the viscera is complicated and seldom performed, although it maybe necessary in patients with recurrent obstructive symptoms. Tamoxifen, which has some antifibrotic activity, has been used as a treatment with some success. Long-term parenteral nutritional support is sometimes life-sustaining in this condition. A computed tomographic (CT) scan of the abdomen reveals extensive peritoneal calcification with thick-walled loops of small bowel tethered to the mesentery with surrounding fluid.

Treatment for symptomatic Fabry disease ?

Enzyme replacement therapy - Enzyme replacement therapy (ERT) with α-galactosidase A is indicated to prevent disease progression. Guidelines suggest initiation of treatment for all patients with Fabry who have CKD, significant proteinuria, or an abnormal kidney biopsy. The disease is suspected on the basis of family history and clinical manifestations that may include acroparesthesias, angiokeratomas (especially in thigh and buttock regions), ocular abnormalities, and anhidrosis. Angiokeratomas are small, dark, blue, red or gray macules that most commonly appear during teenage years in a bathing trunk distribution. Stroke, restrictive cardiomyopathy, and progressive CKD are more serious sequelae of the disease. A deficiency of α-galactosidase A, an enzyme that metabolizes glycosphingolipids, is the primary abnormality.

___ acts primarily on β-1 adrenergic receptors leading to an increase in cardiac output. It is proarrhythmogenic, causes dose-dependent splanchnic vasoconstriction, and is a second-line therapy added to norepinephrine for patients with sepsis.

Epinephrine

Post-transplant lymphoproliferative disorder is usually caused by ___ infection.

Epstein-Barr virus (EBV) infection EBV-induced PTLD develops in response to immunosuppression leading to decreased T-cell immune surveillance and control of EBV-infected B cells, which can then undergo transformation and progress to lymphoma.

A FF of ___ is a associated with lower tendency for extracorporeal system thrombosis.

FF of 20-25% FF is calculated as the ratio of the total rate of effluent flow (Qeff, which equals the replacement fluid rate [Qr] plus any other prefilter infusions [in this case Qcit], plus the ultrafiltration goal [Quf]) to the total rate of inflow (Qinf, which is equal to the replacement fluid rate [Qr] plus the citrate infusion rates [Qcit] plus the plasma flow rate [Qp]). The plasma flow rate is equal to Qb multiplied by (1 - hematocrit). FF = Qeff/Qinf = (Qr + Qcit + Quf) / (Qr + Qcit + Qp) After the Qb is adjusted to achieve a FF in the 20-25% range, the citrate infusion rate should be adjusted to provide a postfilter ionized calcium concentration of less than 0.35 mmol/L to provide adequate extracorporeal anticoagulation. The infusion of calcium post-circuit is necessary to prevent symptomatic hypocalcemia but does not affect anticoagulation within the CVVH circuit.

___ is the most common cause of adult onset nephrotic syndrome, accounting for about 35% of cases in biopsy series in adults and greater than 50% in African Americans. The increased susceptibility to ____ appears to be related to variants in the apolipoprotein L1 (APOL1) gene, which confer resistance to Trypanosoma brucei rhodesiense, prevalent in eastern Africa. Membranous nephropathy remains an important cause of nephrotic syndrome and is still the most common cause of primary nephrotic syndrome in adults of white ethnicity.

FSGS

Calculate free water deficit

FWD = 0.6 × weight (kg) × ([serum Na / 140] - 1)

Patient has hypercalcemia, hyperparathyroidism and urinary calcium is 70 and urine Cr 1.4. Dx.

Familial hypocalciuric hypercalcemia

___ is characterized by progressive CKD and early-onset gout. This disorder is a type of autosomal dominant tubulointerstitial kidney disease characterized by a mutation in the uromodulin gene. Several mutations have been described and newer classification schema recommend describing this as uromodulin-associated kidney disease (UKD). Uromodulin, also known as Tamm-Horsfall glycoprotein, is produced in the cells of the thick ascending limb of the loop of Henle. This protein may play a role in limiting calcium crystallization and preventing urinary tract infections. The pathology seen in patients with UKD is typically a diffuse tubulointerstitial fibrosis.

Familial juvenile hyperuricemic nephropathy (ADTKD)

___ (drug) is utilized for the treatment of dyslipidemia, as it can lower triglycerides and low-density lipoprotein and raise HDL levels. Can lead to rise in creatinine after 6 months of therapy. The majority of patients recover to baseline creatinine levels after discontinuation of the drug, but approximately 30% will have persistent CKD. Urine sediment is bland and no significant proteinuria.

Fenofibrate

___ derivatives are used for patients with dyslipidemia, particularly when there is a need to control high triglyceride levels. A 20% increase in serum creatinine has been described in patients with CKD after initiation of __ therapy. The mean increase in serum creatinine is 0.37 mg/dL and is typically reversible with cessation of ___. The mechanism of this effect is uncertain.

Fibric acid Fibrate

___ glomerular deposition disease characterized by Congo red-negative (non-amyloid) fibers, which deposit in the mesangium and glomerular capillary walls. These fibrils are randomly arranged and typically 15-24 nm in diameter.

Fibrillary Although most cases are idiopathic, malignancy, plasma cell dyscrasias, and autoimmune diseases have been associated with some cases.

Patient with nephrotic syndrome with randomly arranged 15-30 nm fibrils and DNAJB9 on kidney biopsy has ____.

Fibrillary glomerulonephritis (GN) The clinical presentation of fibrillary GN is variable, ranging from purely nephrotic to nephritic or in between. Because the light microscopic findings can vary, the diagnosis is traditionally made by demonstrating characteristic fibrils on electron microscopy of a biopsy specimen. Congo red staining is usually negative, but Congo red-positivity is occasionally described. DNAJB9, a heat-shock protein, is a highly sensitive and specific marker for fibrillary GN that can be confirmed by immunoelectron microscopy or mass spectrometry of kidney biopsy material. Immunohistochemical staining and measurement of serum DNAJB9 are also emerging as potentially useful markers. Patients confirmed to have fibrillary GN should be investigated for an underlying malignancy, plasma cell dyscrasia, or autoimmune disease.

___ is abnormal in autosomal recessive polycystic kidney disease, which is characterized by massive kidney enlargement and cystic dilation of the intrahepatic biliary system (Caroli disease).

Fibrocystin/polyductin

___ (condition) is a systemic, non-inflammatory vasculopathy affecting small- and medium-sized vessels that can manifest as dissection, aneurysm formation, or stenosis of any vascular bed. Although renal artery involvement is most common, carotid artery involvement occurs in up to 21% of patients and results in a stroke or other cerebrovascular event. Intracranial involvement can also occur. For these reasons, imaging of the cervicocephalic arteries as well as cerebral vasculature has been recommended in patients with ___ because of the possibility of therapeutic intervention. Which imaging modality?

Fibromuscular dysplasia (FMD) MRA of the cervical and cranial arteries.

Management of prolonged bleeding of the AVF?

Fistulagram One clue to the diagnosis is prolonged bleeding from the AVF, which suggests an outflow stenosis, thrombosis, or both. This lesion could be located anywhere from the venous outflow of the fistula to the superior vena cava. Central venous stenosis is common in patients who have received HD for many years and who may have previously received dialysis using a tunneled, cuffed catheter Stenosis in the venous outflow increases BP within the access and can result in prolonged bleeding (as in this patient), edema, and pain, as well as access recirculation, which decreases the efficiency of dialysis. In this patient, decreased efficiency of HD manifests as increased potassium, BUN, and creatinine and decreased bicarbonate. A fistulagram with angioplasty would be diagnostic and therapeutic, respectively, in managing access recirculation associated with outflow stenosis.

In about 25% of the cases, decreased HD adequacy is due to access recirculation, in which dialyzed (venous) blood re-enters the extracorporeal (arterial) circuit, decreasing dialysis efficiency. Recirculation is often attributable to a dysfunctional dialysis access, frequently due to venous stenosis or thrombosis. Access recirculation can be measured by urea-based or non-urea-based methods. Management?

Fistulogram Recirculation of more than 10-15% indicates significant access recirculation.

___ prophylaxis has been recommended to prevent fungal peritonitis after antibiotic treatment of bacterial peritonitis in patients receiving PD.

Fluconazole

Which of the following tests can be used to confirm a proximal tubulopathy caused by tenofovir disoproxil fumarate?

Fractional excretion of uric acid (FEua) TDF (tenofovir disoproxil fumarate) has also been shown to cause a proximal tubulopathy with Fanconi syndrome or AKI, and TDF has also been associated with CKD. These agents are excreted by glomerular filtration and proximal tubular secretion via organic anion transporters Nephrotoxicity has been attributed to mitochondrial dysfunction from disruption of mitochondrial DNA. TDF has also been associated with osteomalacia and osteoporosis, which are likely attributable to chronic phosphaturia Hypouricemia that is associated with renal losses occurs in the setting of proximal tubulopathy or with volume expansion (as with IV fluids or the syndrome of inappropriate antidiuretic hormone). The FEUA should be <10% in hypouricemic individuals with normal proximal tubular function.

Free water deficit = ?

Free water deficit = Total body water x [(SNa/140) - 1]

Which of the anti-neoplastic drugs that can cause TMA?

Gemcitabine Mitomycin C and VEGFi Patient with gemcitabine can get gemcitabine-associated TMA usually after median exposure time of 7 months or cumulative dose of 20,000 mg/m2. Patients will most commonly present with hypertension, edema, and AKI. Non-nephrotic proteinuria and hematuria are frequently present as well. Hematologic findings of TMA include anemia, thrombocytopenia, high LDH, and low haptoglobin. Diagnosis can be made by kidney biopsy or by peripheral smear if there are findings consistent with a TMA such as fragmented red blood cells and schistocytes. Recognition of the clinical entity and discontinuation of the drug is most important. If kidney dysfunction does not improve with discontinuation of the gemcitabine, then options include plasmapheresis, steroids, and eculizumab, which have been used with varying degrees of success.

A pregnant patient with polydipsia, polyuria, and dilute urine that is refractory to exogenous vasopressin but responsive to demopressin has ____.

Gestational DI The differential diagnosis of polyuria in pregnancy includes DI, diabetes mellitus, and primary polydipsia. It is distinguished from urinary frequency, which is a common feature of pregnancy, by a urine output that exceeds 3 L/d. Gestational DI is a rare form of DI that occurs when ADH is synthesized and released normally but is degraded by placental vasopressinase, preventing it from reaching its site of action at the collecting tubule. Vasopressinase metabolism is hepatic, so liver impairment due to pregnancy-related disorders such as preeclampsia and the hemolysis, elevated liver function tests, and low platelets (HELLP) syndrome or preexisting liver disease predispose to gestational DI, as does a multiple-gestation pregnancy. Presentation is during the third trimester and gradual resolution over several weeks postpartum is the usual course. Treatment with desmopressin, which is resistant to degradation by vasopressinase, is effective at controlling symptoms while awaiting resolution.

Hypokalemic metabolic alkalosis and inappropriate renal wasting of sodium, chloride, potassium, and magnesium characterize the syndrome. It is caused by an inactivating mutation in the thiazide sensitive sodium chloride cotransporter gene (NCCT). Affected individuals have low to normal blood pressure, often accompanied by salt craving, fatigue, and cramps. Dx.

Gitelman syndrome A calcium-to-creatinine ratio of <0.2 is consistent with Gitelman syndrome

The addition of ___ along with leucovorin is the most appropriate treatment to reduce systemic toxicity of methotrexate in the setting of elevated levels.

Glucarpidase AKI from MTX is due primarily to precipitation of MTX and its metabolites within tubular lumens. This occurs in acid urine and is reduced with alkalinization of the urine by infusing IV sodium bicarbonate. MTX may also lead to direct tubular toxicity, and this is thought to occur from the formation of oxygen radicals associated with decreased adenosine deaminase activity. Risk factors for nephrotoxicity include intravascular volume depletion with sluggish urinary flow, acidic urine, and underlying kidney disease (GFR <60 mL/min/1.73 m2). Based on these factors, prevention is focused on volume repletion before and during drug infusion, appropriate drug dosing, and alkalinization of the urine (pH >7.1). Treatment also includes leucovorin rescue at 24-36 hours of MTX therapy to reduce nonmalignant cell injury. High-flux hemodialysis clears the plasma of MTX fairly well (76%) but is associated with immediate postdialysis plasma rebound. It may have a role when severe AKI is present along with systemic toxicity, but it may become unnecessary with the availability of glucarpidase

A kidney biopsy demonstrates crescentic glomerulonephritis with 40% global glomerulosclerosis. Acute tubular necrosis and moderate interstitial fibrosis with tubular atrophy are also present. Immunofluorescence microscopy shows ribbon-like IgG staining along the glomerular basement membrane. Dx.

Goodpasture disease (anti-GBM) antibody disease

___ also known as pseudohypoaldosteronism type 2 or chloride shunt syndrome, is a rare disorder that arises from genetic mutations encoding WNK1 or WNK4. These are serine-threonine kinases that do not contain lysine (with no lysine (WNK)). Mutations result in upregulation the sodium/chloride (NC) and sodium/potassium/chloride (NKCC) transporters and downregulation the renal outer medullary potassium channel (ROMK), causing hyperkalemia, hyperchloremic metabolic acidosis, volume overload, and resistant hypertension.

Gordan syndrome

The clinical presentation of ___ includes a pulmonary-renal syndrome of lung hemorrhage with rapidly progressive GN, frequently with upper respiratory tract symptoms (70-100% of cases), flu-like symptoms, and subcutaneous nodules. Kidney biopsy typically shows cellular or fibrous crescents, sometimes with fibrinoid necrosis, occasionally with granulomatous inflammation. IF staining is typically negative, but minor positivity can be present, hence the pauci-immune designation.

Granulomatosis with polyangiitis (GPA) Anti-PR3 (cANCA) usually confers a diagnosis of granulomatosis with polyangiitis (GPA) if systemic features are present.

Patient with severe salicylate poisoning should undergo ___ ASAP?

HD Present with n/v/tinnitus/AMS, noncardiogenic pulmonary edema, seizures, coma, coagulopathy, and hyperpyrexia. Extracorporeal removal of salicylates is efficient because of the low molecular weight of the drug and its low volume of distribution Markedly elevated serum concentrations of salicylate (>100 mg/dL), central nervous system dysfunction, renal failure limiting the ability to alkalinize the urine, severe metabolic acidosis, and pulmonary edema/hypoxia should prompt consideration of urgent hemodialysis. Treatment duration of 6-8 hours is often used to maximize salicylate clearance. Alkalization of the serum is first-line therapy for salicylate toxicity not requiring dialysis. - keep the arterial pH between 7.5 and 7.6. - Increasing the urine pH from 5 to 8 can increase salicylate clearance by 10- to 20-fold.

Treatment of lithium poisoning with HD.

HD for a minimum of 6 hours with a blood flow of 350 mL/min and treatments should be repeated until the serum lithium level is <1 mEq/L. The half-life of lithium varies widely from as short as 12 hours to nearly 60 hours depending on age, kidney function, and duration of therapy. Lithium has both acute and chronic toxicity. Acute toxicity includes AKI as well as neurologic and cardiac manifestations. Chronic lithium toxicity may lead to nephrogenic diabetes insipidus or CKD from chronic tubulointerstitial nephropathy. It can also lead to hyperparathyroidism, hypercalcemia, and hypocalciuria.

Patient from South Africa that is African-American presents with nephrotic syndrome and renal biopsy findings of: Biopsy findings in ___ include glomerular capillary collapse, podocyte hypertrophy and proliferation, interstitial fibrosis and edema, and tubular microcystic dilation. Electron microscopy frequently reveals endothelial tubuloreticular inclusions (TRIs). All of these changes are depicted in the biopsy images. The kidneys typically appear large and markedly echogenic by ultrasound, possibly due to pronounced interstitial edema. Dx?

HIVAN - HIV-associated nephropathy The incidence of HIVAN has decreased precipitously with the advent of effective antiretroviral therapy (ART) and has been accompanied by a decrease in the incidence of ESRD in patients who are HIV-positive. However, patients from resource-limited environments without easy access to HIV diagnosis and treatment may present with HIVAN, as may patients unaware of their HIV infection. Patients with African ancestry are at much greater risk of both HIVAN and ESRD, as they are more likely to carry 2 copies of the G1 or G2 alleles of the APOL1 gene. These variants confer protection against resistant Trypanosoma brucei but also predispose patients with 2 copies to disorders such as HIVAN. In a study of South African HIV-positive populations with 2 APOL1 high risk alleles, the odds ratio for developing HIVAN was 89 (95% confidence interval, 18-911).

___ is characterized by a doubling of serum creatinine to a level greater than 2.5 mg/dL during a period of <2 weeks, and it is usually precipitated by a bacterial infection or gastrointestinal bleeding. A protracted decline in GFR manifesting as refractory ascites resistant to diuretics is termed ____.

HRS type 1 - doubling serum Cr > 2.5 in a period of < 2 weeks HRS type 2 - protracted decline GFR, refractory ascites resistant to diuretics, develop over more than 2 weeks Both types are diagnoses of exclusion requiring an absence of evidence for nephrotoxins, glomerulonephritis, obstruction, or acute tubular necrosis. Initial management consists of withdrawal of diuretics coupled with volume expansion with albumin. If this is unsuccessful, vasopressor therapy consisting of midodrine, norepinephrine, or terlipressin is provided with a target of increasing the mean arterial pressure (MAP) by >5 mm Hg (some patients respond better to relatively greater increases in MAP, so this target should be individualized). Vasopressor therapy is combined with ongoing albumin infusions and often with octreotide. Response rates of up to 50% are reported with terlipressin and norepinephrine, though seem to be lower with midodrine. Thus, resorting to midodrine after a lack of response to norepinephrine would not be appropriate.

The optimal duration of maintenance immunosuppression for patients with AAV ___.

Has not been established, but many experts recommend ongoing maintenance for at least 2-3 years after successful induction of remission Patients with high risk for relapse or little reserve may receive maintenance immunosuppression indefinitely.

MOA heparin causing hyperkalemia?

Heparin and low molecular weight heparins can lead to hyperkalemia due to direct toxicity to zona glomerulosa cells, leading to reduced aldosterone production.

ESRD patient with compensated cirrhosis should have ___ measured to determine whether a patient with ESRD and cirrhosis should be listed for dual organ transplant.

Hepatic venous pressure gradient (HVPG) However, prior to listing for a kidney transplant, evaluation for the presence of portal hypertension by direct measurement of HVPG is prudent. Patients with HVPG <10 mm Hg are at only 10% risk of developing clinical decompensation in the next 4 years and can safely proceed with kidney transplantation alone A patient who has elevated HVPG measurements should be considered for a simultaneous liver-kidney transplant, even in the absence of overt clinical signs and symptoms of decompensation.

Herbal tea and hypokalemia.

Herbal tea -> substances with mineralocorticoid properties that can lead -> hypokalemia Many herbal products may contain licorice root for flavor, medicinal purposes, or both. These products may contain significant amounts of glycyrrhetinic acid. This compound produces mineralocorticoid excess effects by blocking 11-β-hydroxysteroid dehydrogenase type 2, leading to increased intracellular cortisol levels, which can activate the mineralocorticoid receptor. Glycyrrhetinic acid can also activate the mineralocorticoid receptor itself and block metabolism of aldosterone, producing further mineralocorticoid activity. Patients taking diuretics may be particularly susceptible to the hypokalemic effects and may also have a blunted hypertensive response.

__ is effective in preventing attacks in individuals with hyperkalemia periodic paralysis.

Hydrochlorothiazide

Muscle weakness, hypotonia, flushing, and hypotension which electrolyte abnormality?

Hypermagnesemia Magnesium-containing antacids, laxatives, and enemas can increase serum magnesium levels in patients with impaired renal function. Patients with chronic constipation, are at risk to overuse magnesium-based over-the-counter remedies. Unless specifically asked, patients might not disclose their use of over-the-counter medications, herbal products, or home remedies. Magnesium is an effective CCB, resulting in vasodilation, flushing, and hypotension when the concentration is elevated. Intracellular magnesium also blocks several cardiac potassium channels, which causes cardiac conduction defects. Bradycardia is seen when magnesium levels exceed 4-5 mEq/L. Additional EKG changes are apparent at concentrations of 5-10 mEq/L. These changes include prolongation of PR and QT intervals and increased QRS duration. Hypermagnesemia can also decrease the release of acetylcholine, blocking transmission at the neuromuscular junction and causing hypotonia and hyporeflexia.

___ is a known complication of denosumab because of the severely reduced osteoclastic activity. Patients with CKD and metastatic bone disease are at higher risk for ___. The majority of patients with denosumab-related ___ are asymptomatic; however, there have been cases of severe symptomatic ___ leading to significant morbidity. Hypophosphatemia has also been reported in 10% of patients given denosumab therapy. To avoid symptomatic ___, calcium and vitamin D supplementation should be initiated prior to denosumab treatment.

Hypocalcemia PTH elevated Denosumab is a monoclonal antibody that mimics osteoprotegerin ("protects the osteo") by binding to receptor activator of nuclear factor κ-β ligand (RANKL). This leads to a decrease in osteoclast activity and thereby a decrease in bone reabsorption and an increase in bone density.

Patient is post-parathyroidectomy presents with tingling of hands and feet, severe muscle cramps, blurry vision, and transient episode of choking and inability to breath. dx?

Hypocalcemia - hungry bone syndrome after parathyroidectomy - tetany, paresthesias, laryngospasm, and seizures - t-wave alternates, prolonged QT, reversible CHF - depression, confusion, psychosis - papilledema and optic neuritis Hypocalcemia due to hungry bone syndrome should be anticipated and prevented or managed by supplementation of calcium and calcitriol. Trousseau sign, carpal spasm induced by mild hypoxia from an inflated blood pressure cuff, is characterized by thumb adduction, wrist and metacarpophalangeal flexion, and interphalangeal extension. Chvostek sign is facial muscle spasm elicited by tapping the parotid gland over the ipsilateral facial nerve.

Hypothermia can cause which electrolyte abnormality?

Hypokalemia Mechanism intracellular K shift attributed to increased activity of Na-K-ATPase. Release of endogenous epi stimulates B-2 receptors, further intracellular movement of K. Polyuria related to cold-induced diuresis occurs leading to urinary K loss. Hypothermia induces peripheral vasoconstriction which increases renal blood flow.

LE weakness following a carbohydrate load and a patient with hypokalemia suggest a diagnosis of ____ and results from a shift of potassium into the intracellular space. Can be acquired form ___ in patient with thyrotoxicosis more common in men, highest in Asians.

Hypokalemic periodic paralysis (HypoPP)

Long-term use of proton pump inhibitors can lead to ___ by decreasing enterocyte absorption. ___ can in turn lead to hypocalcemia by inducing resistance to or deficiency of PTH.

Hypomagnesemia

Cisplatin-based chemotherapy can cause which kidney toxicities?

Hypomagnesemia Hypokalemia Hypophosphatemia AKI Cisplatin is filtered and secreted by organic cation transporters in the proximal tubule. Accumulation in proximal tubule cells can lead to cell injury through multiple mechanisms. This may lead to AKI or necrosis, but tubular dysfunction without AKI can also occur. This may manifest as a proximal tubulopathy, impaired magnesium reabsorption in the ascending limb of the Loop of Henle, or impaired water reabsorption in the collecting duct.

What is the cause of hyponatremia in a euvolemic patient with symptoms of alopecia and hair loss on the outer third of the eyebrow, and confusion, UNa elevated and Uosm elevated?

Hypothyroidism Hypothyroidism has been associated with hyponatremia when moderate or severe but not when mild or subclinical

Refractory hypotension is most likely related to ___, and other pro-inflammatory cytokines, induced by ATG administration. Cytokine release syndrome (CRS) is a form of systemic inflammatory response that can occur after ATG infusion.

IL-6 CRS is thought to be due to immune cell activation and release of inflammatory cytokines, including IL-6 The presentation of CRS can vary from a mild flu-like illness to distributive shock with vascular leakage, disseminated intravascular coagulation, and multiorgan failure. Treatment of CRS-related hypotension consists of volume expansion if tolerated, vasopressors, corticosteroids, and sometimes anticytokine therapy with tocilizumab, an IL-6 receptor targeted monoclonal antibody. A recent case report found that treatment with methylene blue improved hemodynamic status in a patient with vasopressin-refractory hypotension related to ATG.

Dialysis patient with MSSA bacteremia treat with which antibiotic?

IV cefazolin (preferred) after each dialysis or nafcillin (frequent dosing Q6H; less preferred) - 15-20 mg/kg IV after each dialysis for cefazolin

Patient with salicylate poisoning and obtundation should received?

IV dextrose to treat for possible neuroglycopenia, a complication of salicylate poisoning. Salicylate toxicity results in a decreased level of consciousness from multiple mechanisms, including direct central nervous system toxicity, cerebral edema, and neuroglycopenia. Neuroglycopenia can occur despite the presence of a normal serum glucose level and should be empirically treated. Both experimental salicylate poisoning in animals and case reports in humans demonstrate remarkable improvement in neurologic status with IV dextrose administration in this setting. Other treatments for salicylate poisoning include urinary alkalinization to enhance excretion, which should be accomplished with sodium bicarbonate even in the presence of respiratory alkalosis. HD is indicated for severe poisonings with AKI, high salicylate levels, or pulmonary or cerebral edema.

Patient with hypercalcemia of 13, low PTH, weight loss, and fatigue. Radiologic findings of large anterior mediastinal mass, associated lymphadenopathy, and splenomegaly with two splenic masses suggest lymphoma. Tx for the hypercalcemia?

IVF + glucocorticoids extrarenal production of 1,25-dihydroxyvitamin D is the most common cause of hypercalcemia in the setting of lymphoma By reducing extrarenal vitamin D 1-α-hydroxylase activity, glucocorticoids offer the most specific treatment of the hypercalcemia caused by lymphoma

A patient with ANCA-associated vasculitis (AAV), recurrent infections, and hypogammaglobulinemia should be treated with ___.

IVIG Hypogammaglobulinemia is frequently recognized in immunosuppressed patients and is particularly associated with B cell-depleting therapy such as rituximab. Patients receiving maintenance rituximab infusions should have serum immunoglobulin levels measured before and after treatment. Immunoglobulin replacement should be considered in patients with hypogammaglobulinemia who have recurrent infections despite prophylactic antibiotics. IVIG has anti-inflammatory properties, may be considered as an adjunct therapy for AAV, and usually doesn't result in disease flare-up. Even with discontinuation of rituximab, however, the patient may continue to experience recurrent infections, because the effect of rituximab on B cells may last for 6 months or longer.

Treatment for AMR?

IVIG and plasmapheresis AMR is a form of rejection that is mediated by circulating alloantibodies against the transplanted kidney, which trigger complement activation and Fc-mediated cytotoxicity. AMR can be acute or chronic in nature and may relate to either preexisting or de-novo antibodies. Diagnosis of AMR requires evidence of inflammation involving the capillaries on kidney biopsy and the presence of circulating donor-specific antibodies. The majority of cases, but not all, will also have evidence of complement activation through C4d positivity on immunofluorescence.

How does icodextrin cause disparity between levels of serum amylase and serum lipase in PD?

Icodextrin is a glucose polymer developed from cornstarch that is utilized to improve ultrafiltration during long-dwell PD exchanges. Icodextrin is absorbed from the peritoneal cavity by lymphatics and then degraded by amylase into maltose. Patients who use icodextrin may have a falsely low serum amylase because icodextrin competes with amylase as the substrate of the laboratory assay. Lipase measurement is not affected by icodextrin.

A patient with nodular glomerulosclerosis and hyaline arteriosclerosis consistent with diabetic nephropathy however history does not support it what can be another cause of the pathology finding?

Idiopathic nodular glomerulopathy - hypertensive individuals with history of tobacco use

A kidney biopsy reveals light and immunofluorescence microscopy findings of thickened basement membranes and positive, linear staining for IgG and albumin, with negative staining for IgM, IgA, κ, λ, and C3. Acellular periodic acid-Schiff-positive nodules are present in the mesangium as well as areas of nonspecific sclerosis. Blood vessels show changes consistent with chronic hypertension. Electron microscopy demonstrates amorphous material in the mesangium without substructure, thickened basement membranes, and foot process effacement. dx.?

Idiopathic nodular glomerulosclerosis - Nodular glomerulosclerosis is associated with longstanding cigarette smoking and hypertension. The differential diagnosis of nodular glomerulosclerosis includes diabetic nephrosclerosis, idiopathic nodular sclerosis, amyloidosis, and monoclonal immunoglobulin deposition disease.

___ (chemo agent) is metabolized to chloroacetaldehyde, which causes direct tubule toxicity that manifests as AKI and proximal tubule dysfunction (Fanconi syndrome).

Ifosfamide Proximal RTA Hypokalemia Hypophosphatemia Hypouricemia Glucosuria Tubular proteinuria "non-albumin"

Which drug can cause both a pRTA and dRTA?

Ifosfamide - activation produces chloroacetaldehyde, which has been demonstrated to injure renal tubules -> proximal tubulopathy may be severe and result in a proximal RTA-> defect in bicarbonate reabsorption, including glycosuria, phosphaturia, amino aciduria, and loss of low-molecular-weight proteins (Fanconi syndrome) Distal tubular dysfunction has also been described in association with a NAGMA and diminished concentrating ability.

The most common kidney biopsy findings with IBD is ___.

IgA nephropathy

___ is the most common cause of glomerular disease worldwide and is particularly common in individuals of Asian ancestry. This disorder most commonly manifests as intermittent visible hematuria or persistent microscopic hematuria with minimal to nephrotic range proteinuria but usually without the nephrotic syndrome.

IgA nephropathy

__ has been associated with liver cirrhosis and portal hypertension in a patient with microscopic hematuria.

IgA nephropathy - may be found in as many as 35-90% of patients with cirrhosis

The presence of an elevated serum calcium with suppressed PTH and elevated urine N-telopeptide-to-creatinine ratio is consistent with hypercalcemia due to bone resorption. The absence of mechanical stimulation for bone formation, which occurs during ___, reduces osteoblastic activity, leading to relatively unopposed osteoclast-mediated bone resorption. As serum calcium levels rise, PTH is appropriately suppressed and urinary calcium excretion is increased.

Immobilization the presence of elevated NTX with a normal bone-specific alkaline phosphatase indicates active bone resorption with the absence of bone formation, which suggests hypercalcemia due to immobilization. Bone-specific alkaline phosphatase is found in osteoblasts and is a marker of bone formation. Conversely, bone resorption mediated by osteoclasts leads to liberation of the collagen peptides N-telopeptide crosslink (NTX) and C-telopeptide crosslink, whose levels reflect the rate of bone resorption. Bisphosphonates and denosumab, an inhibitor of receptor activator of nuclear factor-κB (RANK), have been used to treat immobilization hypercalcemia by inhibiting osteoclast activity.

How does pseudohyperphosphatemia happen in multiple myeloma?

Immunoglobulin excess due to multiple myeloma, monoclonal gammopathy, or Waldenström macroglobulinemia may cause interference with colorimetric assays for phosphorus, causing pseudohyperphosphatemia. Alternatively, increased phosphate binding capacity of the paraproteins may result in an increase in total serum phosphate without an increase in biologically active phosphate. Reanalysis after removal of the paraprotein results in normalization of the spurious hyperphosphatemia.

Treatment for membranous nephropathy.

Immunosuppressive treatment is not recommended for those patients with advanced CKD as manifested by a high serum creatinine (>2.5 mg/dL) or advanced tubulointerstitial fibrosis on biopsy, or kidney size < 8. Tx secondary MN is treatment of the original disease.

Patient with CKD, proteinuria, and microtubular structures on EM is ___. EM showing organized fibrillar deposits > 30 nm in diameter, arranged in parallel arrays of microtubular structures.

Immunotactoid glomerulonephritis (GN) - associated with autoimmune conditions, plasma cell dycrasias, or malignancy Case reports describe the use of rituximab, corticosteroids, cyclophosphamide, and mycophenolate to treat immunotactoid GN, but no therapy should be initiated prior to investigation for an underlying cause.

What can be done during HD to reduce apnea/hypopnea index in HD patients with OSA?

Increase UF volume Sleep apnea is common in ESRD, with some studies reporting a prevalence as high as 60%. The etiology includes both central and obstructive mechanisms and is not readily explained by comorbidities. The pattern and prevalence are very similar to what is observed in congestive heart failure, another condition marked by chronic fluid overload. Increase in neck circumference due to fluid shifting from the legs to the upper body has been described in both conditions. Limited data have shown that ultrafiltration reduces the AHI.

What is the best strategy to improve weekly Kt/V in a patient treated with low dialysate volume HD?

Increase dialysate volume per treatment

Patient with recurrent calcium oxalate stones with normal citrate in the urine, low urine volume, hypercalciuria, with high sodium excretion in the urine, and K in the urine is normal. What would you recommend to the patient?

Increase fluid intake and sodium restriction Advised to reduce sodium intake to <2 g (88 mmol) per day. Numerous studies have demonstrated that urinary calcium excretion is correlated with urinary sodium excretion and that hypercalciuria and risk for recurrent stones can be reduced by limiting dietary sodium intake. While she may ultimately benefit from thiazide therapy, an initial trial of dietary sodium restriction is warranted.

Microalbumin testing in DM.

Increased albuminuria is one of the earliest markers of diabetic nephropathy. This can occur at the time of diagnosis of type 2 diabetes mellitus, whereas in type 1 diabetes mellitus, the mean onset of increased albuminuria is 15 years after diagnosis. This discrepancy is likely related to the fact that those with type 2 diabetes mellitus may have had hyperglycemia for a significant period of time preceding the diagnosis, which allowed for the development of microvascular complications; in type 1 diabetes mellitus, the approximate date of diabetes onset is usually known. The current recommendation for screening for diabetic nephropathy in patients with type 2 diabetes mellitus is to perform an annual test for albuminuria and assessment of the eGFR. If both are normal, then repeat testing is recommended annually. If there is evidence of an elevated urinary albumin, then repeat testing is recommended in 3-6 months. There is a high false-positive rate for urine albumin because other factors such as exercise, infections, hyperglycemia, and other acute illnesses may transiently increase the urinary albumin such that the increase does not necessarily reflect established diabetic nephropathy. Based on 2017 Hypertension guidelines, the recommended blood pressure targets for those with diabetes and/or CKD are a blood pressure <130/80 mmHg with a preference for the use of angiotensin converting enzyme (ACE) inhibitors or angiotensin receptor blockers because these agents have been shown to reduce proteinuria and decrease the rate of decline of kidney function.

Patient is switching from in-center hemodialysis to home hemodialysis and is becoming uremic. Sessions per week is 3 treatments at home. What changes can be made to the prescription for home hemodialysis to improve uremia symptoms?

Increasing the frequency of dialysis from 3 to 5 treatments per week is the option most likely to relieve uremic symptoms by increasing her weekly clearance. the drastic reduction in dialysate used in home hemodialysis has led to decreased clearance of uremic toxins. Increasing treatment frequency to 5 days weekly will increase clearance and relieve uremic symptoms, allowing her to remain on home hemodialysis. A standard weekly Kt/V of at least 2.0 is recommended to achieve adequate clearance, irrespective of modality. While this can be achieved by thrice-weekly conventional in-center hemodialysis, home hemodialysis using a low-volume dialysate platform or shorter treatments usually requires 5-7 sessions per week.

Indications for biopsy during pregnancy?

Indications for biopsy during pregnancy include new-onset glomerulonephritis (including lupus nephritis), severe nephrotic syndrome that requires therapy, and an acute decline in kidney function. Kidney biopsy can be safely undertaken during the first trimester. Biopsy can also be performed later in pregnancy, but the procedure may need to be performed with the patient lying in the lateral decubitus position rather than prone because of the gravid uterus. In addition, the risk for complications may be increased after mid-trimester.

Patient with staph aureus and low c3 levels. Urine sediment showing RBC with dysmorphic cells. kidney biopsy demonstrates both immunoglobulin A and C3 deposition with subepithelial humps, which are characteristic of ___.

Infection-associated GN This pattern of glomerular injury is often referred to as "post-infectious GN," but in the case of staphylococcal infection, the development of GN occurs during the active infection. Because "post-infectious" implies that the infection has resolved, the terms "infection-related" or "infection-associated" GN are now favored. Staphylococcus-associated GN is most commonly seen in older individuals with underlying risk factors for staphylococcal infections, such as diabetes or alcoholism

MOA NSAIDs and how they cause AKI?

Inhibition of prostaglandin-mediated afferent arteriolar vasodilation Prostaglandins are produced by the enzyme cyclooxygenase and can contribute to both local and systemic pain and inflammation. Nonsteroidal anti-inflammatory drugs (NSAIDs) are cyclooxygenase inhibitors that are widely used to treat these symptoms Under normal circumstances, this is well tolerated because renal vasodilatory prostaglandins are not necessary to maintain renal perfusion and glomerular filtration. However, when conditions such as heart failure or volume depletion are present, renal vasodilatory prostaglandins are needed to offset the vasoconstriction mediated by angiotensin II, norepinephrine, vasopressin, and endothelin. Inhibition of prostaglandin synthesis in this setting can lead to a decline in renal perfusion and AKI that may be functional or parenchymal.

Mainstay of therapy staghorn calculi?

Initial therapy includes treatment with an appropriate antibiotic to treat the urinary tract infection, although antibiotics are typically unable to penetrate the stones themselves. The mainstay of therapy is urologic removal of the stones. Any infected stone fragments left in place can lead to recurrent stone formation. Once most of the stone burden is removed, antibiotics are continued until the urine is sterile, with subsequent tapering. If small fragments remain that cannot be surgically removed, chemolysis with hemacidrin, an acid substance used to dissolve calculi, can be infused via a nephrostomy tube or a catheter in the ureter. Chemolysis is not without side effects and is only considered after significant surgical debulking of the stone has been performed and if the patient is medically stable.

Kidney biopsy findings of AIN.

Interstitial lymphocytic infiltrates

A dialysis patient with symptomatic anemia with functional iron deficiency (defined as transferrin sat < 20% and a ferritin level < 200) should be treated with ___.

Iron sucrose, IV formation of iron supplementation - relative iron deficiency that results in hyporesponsiveness to an ESA - due to GI losses, phlebotomy, impaired gastric absorption of iron, loss of blood in the dialysis circuit (called functional iron deficiency), can also be from chronic inflammation associated with ESRD with increased liver hepcidin

Causes of osmolar gap post-op from a hysteroscopy?

Irrigation solution such as glycine and sorbitol are hypotonic with an osmolality of 200 and 165 respectively

Treatment to limit cisplatin-induced nephrotoxicity?

Isotonic saline prior to chemotherapy the alkylating agent cisplatin is nephrotoxic and can cause injury by several mechanisms including direct proximal tubular toxicity, renal vasoconstriction, and induction of an inflammatory response. * furosemide and mannitol have not been found to be beneficial, although furosemide may be required in patients with symptomatic hypervolemia, n-acetylcysteine no benefit **it is worth noting that hypomagnesemia secondary to urinary magnesium wasting is commonly observed with cisplatin-mediated nephrotoxicity and this deficit may exacerbate cisplatin toxicity.

Which electrolyte abnormality can Zosyn (piperacillin/tazobactam) cause?

It is thought piperacillin causes hypokalemia and metabolic alkalosis by the same mechanism as that of penicillin. Excretion of more than 25-30 mEq of potassium per day indicates inappropriate renal potassium loss spot urine potassium-to-creatinine ratio >15 mEq/g creatinine (or 1.5 mEq/mmol) usually indicates inappropriate renal potassium loss.

Ketamine and AKI

Ketamine, an analgesic and anesthetic agent, has also been used as a recreational drug. AKI has been described in patients who use ketamine chronically. The mechanism of AKI is urinary obstruction due to precipitation of ketamine metabolites in the renal calyces and pelvis. Case reports have documented the presence of hydronephrosis

Induction therapy ATG versus Basiliximab which one to use and what factors are considered high immunologic risk?

Kidney Disease Improving Global Outcomes (KDIGO) guidelines recommend basiliximab as first line induction therapy in kidney transplant recipients of standard risk (1B), whereas a lymphocyte-depleting agent is suggested for patients with high immunological risk (2B). High immunologic risk - Young age (<40) - Race (African-American) - PRA > 20% - Greater HLA mismatch between recipient and donor

Noncontrast CT has become the gold standard to confirm urinary stone disease with a sensitivity and specificity of 97% and 95%, respectively. However, in order to avoid repeated exposure to relatively high dose ionizing radiation, either ___ is preferred for patients who require serial imaging studies for stone surveillance.

Kidney US or plain radiography

Patient with multiorgan involvement including liver failure, AKI, and pulmonary hemorrhage. It is most often recognized as a tropical or subtropical illness related to exposure to water that may be contaminated by the urine of animals (particularly rodents, but also dogs, cattle, and sheep). What does the patient have?

Leptospirosis The organism is suspected to gain entry through minor breaks in the skin or via conjunctival or mucosal exposure.

Patient hypertension, hypokalemia, metabolic alkalosis, and suppressed levels of renin and aldosterone resulting in apparent mineralcorticoid excess can be caused by food ?

Licorice, which contains the compound glycyrrhetinic acid, is the best recognized inhibitor of 11 β-hydroxysteroid dehydrogenase type 2, but azole antifungals including posaconazole have also been associated with this effect. the diagnosis of AME by this mechanism can be established by the ratio of urinary free cortisol to urinary free cortisone in a 24-hour urine collection. If 11 β-hydroxysteroid dehydrogenase type 2 is functioning normally, urinary free cortisone levels exceed urinary cortisol levels, and the ratio of cortisol-to-cortisone is approximately 0.3-0.5.

Presence of hypertension, hypokalemia, and metabolic alkalosis is consistent with normal aldosterone and renin which condition?

Liddle syndrome - tx ENaC blocker such as amiloride Liddle syndrome is a rare, AD cause of HTN. It usually presents as early onset hypertension, but occasionally, this syndrome may escape detection until adulthood. Liddle syndrome results from an activating mutation of ENaC in the distal nephron. Increased ENaC activity results in increased sodium reabsorption. In addition, the increased luminal electronegativity leads to potassium secretion from the principal cells and favors H+ excretion via the H+ATPase at the apical membrane of the α-intercalated cells. Because of the resultant volume expansion, mineralocorticoid activity is suppressed. Thus, treatment with spironolactone is ineffective. Liddle's syndrome is treated with blockade of ENaC by agents such as amiloride or triamterene.

Characteristic electron microscopy findings include randomly distributed small fibrils, 10-12 nm in diameter.

Light microscopy in amyloidosis shows acellular periodic acid-Schiff staining. Deposits can appear amorphous. The gold standard for diagnosis is Congo red positivity. Characteristic electron microscopy findings include randomly distributed small fibrils, 10-12 nm in diameter. Light chain (AL) amyloidosis will demonstrate a light chain predominance by immunofluorescence microscopy.

Which amphotericin preparation is associated with lower risk of AKI?

Liposomal amphotericin B is associated with a lower risk for AKI than other formulations in the setting of neutropenic fever. The nephrotoxicity is thought to be a result of both tubular injury and vasoconstriction. The direct tubular injury results from an alteration in membrane permeability, which occurs with any formulation of amphotericin B. Risk factors include the formulation of amphotericin, high doses, cumulative dose, and the concomitant exposure to other nephrotoxic medications. By formulating amphotericin in a lipid package, nephrotoxicity is reduced and antifungal effectiveness is potentially enhanced. Compared with conventional deoxycholate amphotericin B, both liposomal amphotericin B (L-Amp) and amphotericin B lipid complex (ABLC) have reduced nephrotoxicity. A head-to-head trial of L-Amp compared with ABLC in patients with neutropenic fever found a significantly lower rate of AKI with L-Amp (approximately 14% versus 42%), making it the preferred choice in this setting. The direct tubular toxicity from amphotericin B exposure results in renal magnesium and potassium wasting, with resultant hypomagnesemia and hypokalemia. Although saline infusions prior to administration attenuate the risk of AKI, they do not affect the likelihood of hypokalemia.

The combination of CKD, multiple small kidney cysts, and preserved kidney size in a patient with history of long-term lithium exposure is typical of ___.

Lithium nephrotoxicity. The cysts are very small and measure 1-2 mm; thus, they may occasionally be difficult to assess on ultrasound. Features of lithium nephropathy - including leukocyturia - possible nephrogenic diabetes insipidus - hyperparathyroidism with mild hypercalcemia

A patient with hyperuricemia, gout, and persistent HTN after 6 months of nonpharmacologic intervention should be treated with ___.

Losartan - Losartan reduces serum urate via a modest uricosuric effect that appears to plateau at a dose of 50 mg/d. - This phenomenon appears to be specific to losartan and is not observed with alternative ARBs or with ACE inhibitors such as lisinopril. In contrast, diuretic therapy, such as with hydrochlorothiazide, has been shown to moderately increase both serum urate levels and the risk of gout by increasing net urate reabsorption in the proximal tubule. ß-Blockers have also been associated with increased serum uric acid levels.

LM normal. EM effacement of epithelial cell food processes and normal thickness of the glomerular capillary walls dx.

MCD Approximately 15%-20% of adults with nephrotic syndrome will have biopsy-proven MCD. These patients typically present with classic findings of nephrotic syndrome, including proteinuria >3.5 g/day, hypoalbuminemia, edema, and hyperlipidemia.

Defects in ___ lead to intracellular accumulation of mucin-1 resulting in chronic interstitial nephritis, formerly known as medullary cystic kidney disease type 1. Gout is prominent, but more typically occurs as kidney function declines. Inheritance is autosomal dominant.

MUC1 MUC1 encodes mucin-1, a transmembrane protein expressed throughout the kidney that plays a role in maintaining the epithelial barrier.

Which bowel prep in CKD can lead to hypotension, hyporeflexia, paralysis, bradyarrhythmias, and respiratory depression?

Magnesium citrate The most likely cause of the patient's presentation is magnesium toxicity resulting from magnesium citrate ingestion, decreased magnesium clearance, and hypermagnesemia. Patients with advanced CKD are unable to renally excrete the magnesium load ingested with a magnesium citrate bowel preparation. In cardiac cells, magnesium regulates the sodium-potassium ATPase pump and can affect the resting membrane potential by altering the intracellular to extracellular ratio of potassium. In addition, magnesium blocks the slow inward calcium channels. Therefore, ECG findings seen with hypermagnesemia are similar to those of hyperkalemia. Flushing, nausea, and vomiting are symptoms of moderate magnesium toxicity that develop when the magnesium level reaches 4-6 mg/dl. Safe to use: Miralax, polyethylene glycol, lactulose, sorbitol, senna

Which factors in ESKD are associated with 3 month mortality?

Male sex Age >85 years CHF Severe peripheral vascular disease (PVD) Dysrhythmia Severe behavioral disorder Active malignancy Decreased serum albumin (significant if <3.5 g/dL; greatest effect if <2.5 g/dL) Impaired mobility were independently associated with 3-month mortality.

Weight loss and fatigue in a patient more than 5 years after kidney transplant warrants investigation for?

Malignancy The most prevalent malignancies after kidney transplant include skin cancer, Kaposi sarcoma, renal cell carcinoma, and post-transplant lymphoproliferative disorder (PTLD). PTLD is a heterogenous group of diseases characterized by abnormal lymphoid proliferation following organ transplantation. The most common type is a non-Hodgkin lymphoma with approximately 50% exhibiting extra-nodal involvement including the allograft. PTLD usually has a bimodal presentation, peaking at the first year and then 7-10 years after transplant. Presentation within the first year after transplantation is strongly associated with EBV infection, while later PTLD is EBV-negative in approximately 40% of cases. The main risk factors for PTLD include recipient EBV seronegative status, older age, and higher degree of immunosuppression (including the use of T-cell depletion therapy and higher levels of maintenance immunosuppression, in particular tacrolimus). The use of belatacept, a selective T-cell costimulatory blocker, for maintenance therapy for patients who are EBV-naïve has also been associated with higher risk of PTLD. Dx: needs a tissue biopsy for PTLD, if no localizing signs then need kidney biopsy for cell type, EBV encoding (EBER-1 and EBER-2), clonality, also may need additional imaging PET-CT Treatment options range from immunosuppression reduction to rituximab to combination chemotherapy with rituximab, depending on the malignant nature and extent of the PTLD

Treatment of SIADH in a patient with recent SAH and worsening hyponatremia and neurologic impairment?

Management should include administration of 3% saline, which will maintain intravascular volume to allow for adequate cerebral perfusion and will also correct the hyponatremia. Hyponatremia occurs in up to 30-50% of patients with SAH. The most common cause is SIADH. Cerebral salt wasting (CSW) can also occur but is much less common and more difficult to diagnose. These two etiologies are distinguished on the basis of volume status: patients with CSW have evidence of hypovolemia, whereas patients with SAIDH are clinically euvolemic. Patients with SIADH as a result of SAH present unique management challenges. Hypovolemia should be avoided, because it increases the risk of cerebral ischemia due to vasospasm. Fluid restriction should not be used as a treatment strategy

Patient with hyperkalemia but platelet count > 500,000 c/f pseudohyperkelmia due to thrombocytosis. Next step?

Measure whole blood potassium (K) to confirm diagnosis of pseudohyperkaelmia due to thrombocytosis Pseudohyperkalemia refers to a spurious increase in the measured serum K compared with a normal plasma or whole blood K, typically by at least 0.3 mEq/L Patients with spurious hyperkalemia are asymptomatic and display no manifestations of hyperkalemia on electrocardiogram. In one study, up to 14% of patients with platelet counts >500,000/µ L showed a >1 mEq/L increase in serum compared with plasma or whole blood K. Similar phenomenon can occur with extreme leukocytosis, although pseudohypokalemia is more common. Common causes of pseudohyperkalemia are mechanical trauma, fist clenching, or tourniquet use during phlebotomy that can result in a hemolyzed sample

A patient with signs and symptoms of CHF following creation of an AVF should undergo?

Measurement of flow in the AVF AVF have expected hemodynamic consequences including INC cardiac output, DEC peripheral vascular resistance, and INC sympathetic tone (resulting in INC heart rate, stroke volume, and contractility). These changes are usually tolerated among patients with good ventricular function but can result in high-output CHF among patients with limited cardiac reserve. Rarely, patients with preserved ventricular function can experience high-output CHF if AVF flow is high (>2L/min). A ratio of the access flow (QA) to the cardiac output (CO) > 0.3 has been identified as a potential risk factor for CHF. If high-output CHF is identified, flow reduction by surgical revision of the AVF is often successful in terms of both symptom improvement and access patency.

The most common causes of acquired prolonged QT?

Medications (methadone, amiodarone, procainamide, levofloxacin/ciprofloxacin, erythromycin, ketoconazole, amitriptyline, sertraline/fluoxetine, haloperidol, seroquel, ziprasidone, sumatriptan) Hypokalemia - dialysis patients prone with low potassium bath especially at the end of each HD session when K lowest Hypomagnesemia prolonged QT can lead to tornadoes de pointes - polymorphic v.tach -> sudden death

____ congenital disorder characterized by cystic dilation of the terminal collecting ducts. It is commonly associated with nephrocalcinosis and recurrent calcium stones.

Medullary sponge kidney The diagnosis has historically been made by IV pyelography, which demonstrates pooling of contrast material within the cystic dilations, creating a characteristic appearance that may be compared to a "paintbrush" or a "bouquet of flowers." CT scanning without radiocontrast can detect medullary nephrocalcinosis, which suggests medullary sponge kidney, but tubular ectasia is not well visualized. Kidney ultrasound may show cysts with calcified sediment, which forms a fluid level in the dependent portion of the cyst; this may be described as a cyst with "milk of calcium." The kidney stones are primarily composed of calcium oxalate, calcium phosphate, or both.

Patient with nephrotic syndrome and history of HSCT. Which GN? EM showing subepithelial deposits and diffuse effacement of foot processes

Membranous nephropathy is the most common pathogenesis is unknown, likely that GVHD plays a role When nephrotic syndrome occurs in the setting of GVHD, the treatment is directed at GVHD, although use of additional agents such as rituximab has been reported

___ is a form of non-proteinuric CKD of unknown cause. It is seen predominantly in young agricultural workers in or from the Pacific coast lowland regions of Central America, although similar syndromes are increasingly recognized in other parts of the world. The etiology of this condition has not been elucidated, but it is speculated to be due to recurrent episodes of volume depletion, dehydration, and AKI, possibly in conjunction with sucrose exposure. Excessive exposure to NSAID drugs and agricultural chemicals are also possible contributing factors. This condition typically develops chronically over time.

Mesoamerican nephropathy

_____ (condition) primarily affects young men who are exposed to heat stress from laboring outdoors in extreme temperatures. Repeated episodes of AKI from volume depletion or cyclical hypertonicity may play a role in the pathogenesis of this disease. In addition, it has been postulated that some workers may be reluctant to drink the water because of concern for contamination. An increase in uric acid out of proportion to the severity CKD is often seen and may also contribute to the pathogenesis. Risk factors appear to include low body mass index, male sex, and consumption of sugar-containing rehydration beverages or sugar cane itself. Other potential factors include heavy metal exposure, insecticides, and non-steroidal anti-inflammatory drugs.

Mesoamerican nephropathy (MaN) - The majority of cases to date have been reported in Nicaragua and El Salvador, but there are also reports from Guatemala and Costa Rica. patients present with non-proteinuric CKD that is most consistent with chronic tubular interstitial nephritis. They are usually asymptomatic until late in the course. Kidney biopsy reveals interstitial fibrosis but also evidence of glomerular ischemia with glomerular basement wrinkling.

The classic presentation of aHUS is? Mutations?

Microangiopathic hemolytic anemia (MAHA) accompanied by severely advanced renal failure and moderate to severe hypertension Mutations in regulatory proteins of the alternative complement pathway (e.g., factor H (CFH mutation), factor I, membrane cofactor protein) are detected in approximately 50% of aHUS cases; loss-of-function mutations of factor H, a key inhibitor of the alternative pathway, have the highest frequency.

Patient with resistant hypertension receiving ___ (medication) is likely to present with hypotension, dilated neck veins, muffled heart sounds seen in the setting of cardiac tamponade. Other potential physical findings include a paradoxical increase in JVP during inspiration, known as Kussmaul sign, and dullness to percussion and egophony at the left lung base, known as Ewart or Pins sign. Low cardiac voltage and electrical alternans may be present on EKG.

Minodixil Pericardial effusion attributable to minoxidil is observed in about 3% of patients receiving this drug and is occasionally complicated by tamponade. Patients with CKD appear to be more likely to experience this complication. Minoxidil causes arteriolar dilation without venous dilation, resulting in reflex tachycardia and volume retention with edema. Therefore, the drug is sometimes prescribed in conjunction with a loop diuretic and a β-blocker. In some cases, pericardial and pleural fluid may accumulate. Management includes discontinuation of minoxidil and pericardial drainage when the effusion is large and symptomatic.

When should prednisone be tapered in ANCA associated vasculitis.

Most experts recommend that clinicians begin to taper prednisone once the patient has achieved remission, with a goal of discontinuing the steroids after 4-8 months of therapy.

The application of __ to the catheter exit site reduces the risk for catheter infection and bacteremia in patients receiving dialysis.

Mupirocin The application of topical mupirocin ointment to the catheter exit site until healed was found to reduce the rate of bloodstream infection by approximately 85%. Povidone-iodine ointment, polysporin triple antibiotic ointment, and medical grade honey have also been shown to be effective in reducing catheter-related bloodstream infections (CRBI).

Patients with ileal conduits or neobladders are at risk for ____ acidosis.

NAGMA This typically occurs when urine remains in contact with the bowel mucosa for prolonged periods due to stasis or obstruction. Urea in the urine traveling through an intestinal conduit is metabolized to ammonium (NH4+) and bicarbonate (HCO3-) by intestinal bacteria. NH4+ has a similar ionic radius to sodium (Na+) and competes with Na+ for interaction with the Na+-H+ exchanger 3 (NHE3) on the luminal membrane of enterocytes that line the neobladder. Consequently, the bowel segment absorbs NH4+ from the urine in exchange for H+. Chloride (Cl-) is also absorbed from the urine in exchange for HCO3-. Thus, the intestinal conduit absorbs NH4Cl and secretes NaHCO3. Absorbed NH4+ is metabolized back to urea and H+ by the liver resulting in a gain of acid. Excreted HCO3- in the urine represents a loss of bicarbonate. Older age, diabetes, and CKD are risk factors for metabolic acidosis in patients with ileal neobladders.

How do neobladders cause NAGMA?

Neobladders and urinary conduits are often formed by using a portion of the ileum, which is conveniently mobilized on its vascular supply and less prone to metabolic complications than neobladders formed from colon. Nevertheless, ileal neobladders and conduits can predispose patients to developing NAGMA, particularly if there is urinary stasis. The mechanism underlying this predisposition is the presence of chloride-bicarbonate exchangers in the ileal epithelium. Chloride in urine that remains in contact with the neobladder is exchanged for bicarbonate, which is subsequently excreted in the urine, resulting in NAGMA. Diabetes, chronic renal impairment, and a large-capacity neobladder have been identified as risk factors. The severity of the acidosis in this case implies stasis. Investigation for urinary retention, urinary tract infection, and urologic consultation should be considered.

Treatment of propofol-related uric acid crystalluria.

No treatment is necessary because these effects on urine appearance are self-limited and resolve shortly after stopping propofol. Appearance of cloudy or pink urine due to the presence of uric acid crystals. Propofol is structurally similar to probenecid, and it is thought that this similarity results in a uricosuric effect leading to increased uric acid excretion. Risk factors for precipitation include a low urine pH and inadequate fluid replacement.

Patient with skin biopsy findings of alpha5 chain of type 4 collagen along the epithelial BM should she donate her kidney?

No. - counsel patient against donation - potential donor's skin biopsy is consistent with mosaic expression of the α5 chain of type 4 collagen, indicating a heterozygous carrier state for the mutation. Although the clinical course is less severe than for affected males, females with X-linked Alport syndrome have a risk of ESRD approaching that of a patient with diabetes, reaching 30% by age 60 and 40% by age 80. For this reason, female carriers under age 45 are discouraged from kidney donation.

Which PD modality helps with OSA.

Nocturnal dialysis has been shown to improve the apnea-hypopnea index (the number of apnea or hypopnea episodes per hour of sleep). Even a modest degree of fluid shift while recumbent can increase neck circumference and airway resistance. Nocturnal PD offers improvements in the apnea-hypopnea index compared with chronic ambulatory PD, possibly by mitigating any fluid shift. Nocturnal HD has also been shown to reduce the apnea-hypopnea index compared with conventional HD, but there are no data comparing PD with HD.

___ acts on both α-1 and β-1 adrenergic receptors, leading to an increase in cardiac output and peripheral vasoconstriction. It is first-line therapy for the treatment of hypotension in adequately volume-resuscitated patients with septic shock.

Norepinephrine

__ is an independent risk factor for CKD. Earliest manifestation of ___ related glomerulopathy is hyperfiltration, which has been attributed to an increase in renal plasma flow and afferent arteriolar vasodilation. This results in an increased GFR, glomerular hypertension, and subsequently proteinuria. Can also see FSGS, mesangial expansion, and podocyte hypertrophy. Increase in Na reabsorption also leads to hypertension and glomerular hyperfiltration.

Obesity Sustained weight loss is most likely to decrease proteinuria by >25%. One study found a 33% reduction in proteinuria after 6 months among patients with morbid obesity who lost weight compared with no reduction in patients who did not lose or who gained weight. GFR is likely to decline after weight loss because intraglomerular pressure and hyperfiltration decrease.

Treatment for AKI caused by NSAIDs.

Observation and IVF NSAID-mediated inhibition of cyclooxygenase leads to decreased production of prostaglandins, which results in decreased glomerular blood flow

Insulin deficiency results in hyperkalemia by reducing the activity of Na-K-ATPase, resulting in reduced cellular uptake. ___ is a potent inhibitor of insulin secretion, and patients can experience up to a 1 mEq/L increase in serum potassium after its administration. This effect can be more pronounced in patients with ESRD. Patients with relative insulin deficiency, as can occur in inflammatory bowel disease and undernourishment, are also at greater risk. If discontinuation of ___ is not feasible, patients may require exogenous insulin. Hyperkalemia in this case responded readily to exogenous insulin challenge.

Octreotide Lisinopril and heparin both cause hyperkalemia by decreasing the production of aldosterone. Heparin inhibits aldosterone synthase in the zona glomerulosa. Lisinopril inhibits ACE, decreasing the angiotensin II-mediated stimulus for aldosterone production. Hypoaldosteronism results in a reduction in renal potassium excretion. The urinary potassium concentration is of limited utility in evaluation of hyperkalemia, but low concentration (urinary potassium concentration of <40 mEq/L or a urine potassium-to-creatinine ratio of <20 mEq/g) is consistent with impaired renal potassium excretion. In this case, the urine potassium concentration of 100 mEq/L suggests that renal potassium excretion is intact, which indicates that neither the lisinopril nor the heparin is the primary cause of hyperkalemia.

Patient has known CKD and want to limit the risk of postoperative AKI. The patient is scheduled for a CABG. What is a strategy to limit the risk of post-op AKI?

Off-pump CABG surgery rather than conventional CABG with cardiopulmonary bypass is the best strategy to limit risk of postoperative AKI. The CORONARY trial randomized more than 4500 patients to on-pump versus off-pump cardiac surgery. The primary end point of dialysis-requiring AKI was indistinguishable between study groups. However, in patients with CKD, the overall incidence of AKI was lower in the off-pump arm, and the relative risk of dialysis-requiring AKI was lower at 30 days.

Prognosis of Goodpasture disease and long-term dialysis requirement based on serum creatine.

One of the largest cohort studies of patients with anti-GBM disease demonstrated that patients who required immediate dialysis and had 100% crescents on their kidney biopsy remained dialysis-dependent. Patients who presented with serum creatinine values <5.7 mg/dL were likely to have renal recovery. Patients with serum creatinine of >5.7 mg/dL but who did not require dialysis immediately had an 82% chance of renal survival at 1 year. Those patients who required dialysis on presentation, had a poor renal prognosis with only a small minority achieving dialysis independence.

KDIGO guidelines recommend ICA screening in ADPKD patients when?

Only for patients without limited life expectancy who have a family history of ICA or SAH, are employed in high-risk occupations (e.g., airline pilots or bus drivers), those who request screening, those preparing for major surgery. Patients with family history as their only risk factor are advised to have MRI/MRA every 5-10 years. Patients who have known aneurysms <7 mm in diameter, engage in high-risk occupations, or are planning conception, transplantation, or other major surgery are advised to have MRI/MRA every 1-5 years. ICA >10 mm in diameter have a relatively high risk of rupture and, should be repaired by surgical clipping or endovascular coil

___ is the classic pathology associated with renal failure. This disorder is associated with increased bone turnover and elevated PTH levels and alkaline phosphatase.

Osteitis fibrosa

Treatment for fibrin obstruction in PD.

Outflow obstruction can also occur from occlusion of the peritoneal dialysis catheter by fibrin. Fibrin obstruction usually impairs both inflow and outflow of dialysate. In addition, fibrin is often observed in the effluent. When fibrin clots are suspected, instillation of intraluminal thrombolytics may be considered if vigorous flushing does not improve flow.

Ingestion of vitamin C at high doses (>1-2 g/d), particularly in those with CKD, is a risk factor for ___ nephropathy.

Oxalate nephropathy Oxalate nephropathy is characterized by calcium oxalate nephrolithiasis, calcium oxalate tubular deposition, or both. It can be a primary condition due to an inborn error of metabolism, the most common form of which presents early in life and leads to ESRD. Secondary forms relate to either excessive consumption of oxalate or precursors or increased intestinal absorption. Ethylene glycol ingestion is associated with an acute form of oxalate nephropathy. Vitamin C (1-2 g/d) is at least partly metabolized to oxalate and has been associated with both kidney stones and oxalate nephropathy. The B vitamins thiamine and pyridoxine are important in the metabolism of oxalate precursors, and their deficiency can result in increased oxalate production.

____ is a rare, medium-vessel vasculitis that can affect almost any organ. Inflammation of medium-sized arteries leads to aneurysm formation as well as areas of luminal narrowing. In the renal circulation, this results in glomerular ischemia and can cause renin-mediated hypertension and impairment in function. Urinalysis usually shows minimal proteinuria and hematuria, but typically there are no red cell casts, as the pathologic lesion is preglomerular in location. ___ can also affect other organs including the skin where it can present with purpura, erythematous nodules, livedo reticularis, and ulcers. Abdominal pain can be seen with mesenteric arteritis. ____antigenemia is the most recognized serologic marker, but only 5-35% of patients with ___ are positive for hepatitis ___ surface antigen.

PAN Hepatitis B

Kidney biopsy findings in myeloma cast nephropathy

PAS-negative fractured cast material

The United States Center for Disease Control (CDC) recommends that patients with ESRD who have never been vaccinated against pneumococci (or patients for whom the vaccination history is unknown or incomplete) receive one dose of ___ followed by one dose of ___ at least 8 weeks later.

PCV13 23-valent pneumococcal polysaccharide vaccine (PPSV23) at least 8 weeks later A second dose of PPSV23 should be administered 5 years later to complete the sequence If the patient has received PPSV23 first, PCV13 vaccination should be delayed 1 year.

A mutation in __ is responsible for X-linked hypophosphatemic rickets, which presents in early childhood with hypophosphatemia, growth retardation, rickets, and osteomalacia.

PHEX

In the setting of hypomagnesemia, renal conservation should achieve a FeMg of < 2% what are the cause of non renal hypomagnesemia?

PPI - PPIs appear to inhibit magnesium absorption by interfering with a protein, transient receptor potential melastatin 6 (TRPM6), which is involved in the active transcellular transport of magnesium by intestinal epithelial cells. - A passive, paracellular mechanism remains intact, as evidenced by correction of hypomagnesemia with large doses of oral magnesium. Because magnesium is important in renal conservation of potassium, these patients often have a concomitant hypokalemia - PPI for more than 1 year, although has been shown for use of 3 months Patiromer Large bowel resection Chronic diarrhea Hungry bone Alcohol Poor nutrition Pancreatitis

Patient hypokalemic and hypomagnesemia with Uk > 100 can be cause by ___ use.

PPI use The expected renal response to hypokalemia is potassium conservation manifested by a daily urinary potassium excretion of <20 mEq. The high urinary potassium in this patient signals renal wasting. The expected renal response to hypomagnesemia is magnesium conservation, which is demonstrated in this case by the low magnesium excretion. The hypomagnesemia therefore must be of nonrenal origin, such as gastrointestinal (GI) losses or failure to absorb dietary magnesium. PPIs such as pantoprazole are well known to cause hypomagnesemia, possibly because PPI-mediated alkalinization of the intestine inhibits TRPM6/7-mediated magnesium absorption. The proposed mechanism of urinary potassium wasting in hypomagnesemia is insufficient intracellular magnesium causing persistently open potassium channels (renal outer medullary potassium channel, or ROMK) in the distal tubule and cortical collecting duct. Thus, this patient's hypokalemia may be attributed to hypomagnesemia, which in turn has resulted from pantoprazole toxicity.

What is post-transplant diabetes mellitus?

PTDM, also referred to as new-onset diabetes after transplant, is a common complication of kidney transplantation. 5 to 20% of patients develop post-transplant diabetes within 1 year of transplantation. Age >40 years, obesity, family history, and African-American race have been identified as some of the most important risk factors for PTDM.

Treatment for calcific uremic arteriolopathy?

Pain control, wound management Sodium thiosulfate Cinacalcet reduce PTH level Discontinue warfarin Discontinue calcium based phosphate binders

Patient with sickle cell trait presents with dysuria, gross hematuria, left flank pain, and difficulty urinating. Dx.

Papillary necrosis 50% of patients with SCT will have papillary necrosis, 3rd and 4th decade of life High osmolality and low O2 tension of the renal medulla favorable for sickling in the vasa recta -> hyperviscosity, microthormbus, and infarction Renal papillae may slough off cause urine flow obstruction -> hydronephrosis and flank pain -> isosthenuria (inability to max concentrate urine) volume resuscitation, urology for percutaneous nephrostomy or ureteral stent, red blood cell transfusion to reduce sickling

The most likely diagnosis for a patient with gross hematuria and post-micturition anxiety, headaches, palpitations, and hypertension is a ___ .

Paraganglioma of the bladder - Micturition leads to shrinking of the bladder, which stimulates the tumor and results in secretion of catecholamines. The bladder tumor can also explain the gross hematuria Pheos that arise outside the adrenal glands are referred to as paragangliomas. Presenting symptoms depend on whether the catecholamine secreted is predominantly NE, which is most common, or epinephrine. The secretion of dopamine is least common and may correlate with a higher tendency to be malignant. NE binds to α-receptors, which leads to vasoconstriction and HTN. Epinephrine typically exerts its effects on β-2 receptors, leading to vasodilation and orthostatic hypotension. Paroxysmal HTN is a classic feature of pheos, but sustained hypertension may be equally common. Other characteristic symptoms and signs include angina, anxiety, nausea, hyperglycemia, tremor, and weight loss. - plasma free metanephrines and urinary fractionated metanephrines are laboratory tests with excellent sensitivity and specificity for the detection of pheos. - CT and MRI are both excellent initial imaging tests, although the latter may be better for detecting metastatic disease. 123I-metaiodobenzylguanidine (MIBG) scintigraphy is a functional test that may also be helpful in excluding metastatic disease or identifying an occult pheo not visualized with conventional imaging. Given the high frequency of hereditary tumors, genetic testing for mutations is warranted.

____ has been associated with collapsing glomerulopathy (variant FSGS) in a patient with fever, nausea, and diarrhea and proteinuria. A kidney biopsy demonstrates segmental collapse of the glomerular basement membrane and occlusion of the glomerular capillaries with hypertrophic podocytes and pseudocrescents consistent with collapsing glomerulopathy. Moderately severe interstitial inflammation is also present.

Parvovirus B19 Parvovirus B19 has also been associated with membranoproliferative glomerulonephritis (MPGN) with either IgA or IgG predominance.

___ is an AR genetic condition, sensorineural hearing loss, young age, and a goiter. Patients usually have normal electrolytes, but thiazide administration or vomiting may cause severe metabolic alkalosis and profound hypokalemia, hypomagnesemia, and hyponatremia.

Pendred syndrome, which is caused by a defect in pendrin, the apical chloride/bicarbonate exchanger in type B (or non-A, non-B) intercalated cells in the collecting duct of the kidneys. This transporter, which functions to secrete bicarbonate into the tubular lumen, appears play an important role in the setting of a metabolic alkalosis by blunting the severity of the alkalosis.

A young patient with uncontrolled HTN despite being on 3 antihypertensive medications, patient with fibromuscular dysplasia (FMD) should undergo?

Percutaneous transluminal angioplasty (PTA) - recommended for: young, recent onset of hypertension, those with uncontrolled or drug-resistant HTN, or those with CKD - Aspirin 75-100 mg daily is recommended post-procedure. FMD is a rare systemic vascular disorder that results in the non-atherosclerotic narrowing of the affected vessel. FMD is classified according to the nature of the stenotic lesions. Any of the arterial layers can be affected. FMD-related stenoses may be discrete and unifocal, multifocal, or web-like. The renal and carotid arteries are the most commonly recognized sites of involvement. Renal artery stenoses related to FMD usually occur in the mid- to distal artery, as opposed to atherosclerotic lesions that tend to occur in the proximal segment, at or near the origin. The condition affects women preferentially and likely has a genetic component. This patient has a carotid bruit, and all patients in whom FMD is identified should be screened for carotid artery disease, with subsequent management dictated by imaging findings. Hypertension may emerge spontaneously or be provoked by pregnancy or oral contraceptive use. Occasional patients may be identified later in life after a presentation more typical of essential hypertension.

Treatment for anemia in a patient with CKD, anemia and history of cancer.

Periodic transfusion of RBC EPO agents -> increased risk of progression or recurrence of neoplasm in patients treated with ESA KDIGO guidelines suggest that ESA be "used with great caution" in patients with active malignancy, particularly if there is potential for cure, and grade the evidence for this recommendation 1B. They make the same recommendation for patients with a history of malignancy but grade the evidence 2C.

___ (condition) rare systemic vasculitis involving medium-sized arteries and results in aneurysms, stenosis, occlusion, and potentially rupture of the involved vessel and distal ischemia. Arteriography is considered the gold standard for imaging, but CT and MRI may also be useful. Complement levels are typically normal and anti-neutrophil cytoplasmic antibody (ANCA) is usually negative (in contrast, ANCA testing is often positive in cases of small vessel vasculitis). May be associated with hep B or C infection or hairy cell leukemia.

Polyarteritis nodosa (PAN) If PAN is associated with hepatitis B or C infection, antiviral therapy is indicated. Most cases in the United States are primary and are seen in middle-aged or older adults with a slight male predominance PAN may affect any organ; skin, kidney, and neurologic involvement are particularly common. Skin manifestations are diverse and may include painful nodules, purpura, bullae, vesicles, or livedo reticularis. Because of the "preglomerular" location of the kidney lesion, kidney involvement is manifested by hypertension and reduced filtration. The urine sediment may be surprisingly bland without nephritic or nephrotic features. Mononeuritis multiplex occurs in up to 70% of patients, affecting both motor and sensory nerves. Patients can also have central nervous system involvement, including stroke, hemorrhage, and seizures.

What is the equation to calculate electrolyte-free water clearance?

Precise measurement of insensible losses is impractical, but can be estimated to be 0.8-1.2 L daily under usual circumstances Example: Renal electrolyte-free water clearance is estimated by the following equation: CeH2O = V (1 - [UNa + UK]/[SNa]) where CeH2O represents electrolyte-free water clearance, V is the total daily volume of urine, UNa is urine [Na], UK is urine [K], and SNa is serum [Na]. In this example, the equation becomes:CeH2O = 2.8 L (1 - [62 mmol/L + 43 mmol/L]/[149 mmol/L]) = 0.82 L daily Intake of electrolyte-free water supplementation = 200 mL x 4 daily or 0.8 L daily. In this case, it is reasonable to disregard metabolic water generation and to estimate insensible losses to be 0.8 L/day. Therefore, his net daily electrolyte-free water balance is 0.8 L intake − (0.8 L urinary loss + 0.8 L insensible loss) = −0.8 L.

Conservative management of ESRD is associated with?

Preservation of quality of life Fewer hospitalizations Shortened life expectancy on the order of 12-24 months

When is emergent repair of an AVF needed, what are the signs of impending rupture?

Progressive enlargement Thin and shiny overlying skin Ulceration Spontaneous bleeding Rupture can cause rapid exsanguination. An aneurysm three times the size of adjacent vein, generally 2-3 cm in diameter, is considered a large aneurysm that mandates frequent evaluation. Immediate surgical consultation and repair are warranted

Anion gap metabolic acidosis, AKI, increased serum creatinine kinase level, Brugada-type electrocardiogram changes, increased serum potassium level, and cardiovascular compromise (bradycardia with hypotension refractory to pressors) in an ICU patient diagnosis?

Propofol-related infusion syndrome Risks: - critically ill patients who require vasopressor support - propofol dose >4-5 mg/kg per hour - infusions > 48 hours The toxicity is felt to be due to disruption of intracellular energy production, which leads to lactic acidosis, rhabdomyolysis, and cardiac instability. Mortality has been reported to be as high as 60%. Tx: d/c infusion and supportive care

___ is a solvent used as a vehicle for both diazepam and lorazepam. High dose infusions of either agent can lead to ___ toxicity. Toxicity of ___ causes increased anion gap metabolic acidosis because of accumulation of both D- and L-isomers of lactic acid and an increase in the osmolal gap.

Propylene glycol

Common drug causes of ANCA-associated vasculitis.

Propylthiouracil Hydralazine Minocycline Allopurinol Penicillamine Procainamide Methimazole Clozapine Phenytoin Rifampin Cefotaxime Isoniazid Indomethacin Drug-induced AAV is typically associated with constitutional symptoms, arthralgias, arthritis, and cutaneous vasculitis. Patients with drug-induced AAV most often display very high titers of antimyeloperoxidase (MPO) antibodies and may also have strongly positive ANA tests Both cytoplasmic (C-ANCA) and perinuclear (P-ANCA) staining patterns have been described in drug-induced AAV.

Hyperkalemia, HTN, and mild metabolic acidosis in a young individual is characteristic of ___ which caused by either a gain of function mutation in WNK1 (with no lysine kinase-1) or a loss of function mutation in WNK4.

Pseudohypoaldosteronism type II (Gordon syndrome) - Both abnormalities result in increased function of the NaCl cotransporter and the epithelial Na channel, as well as profound inhibition of the renal outer medullary potassium channel (ROMK) channels in the cortical collecting duct. The net effect is decreased Na and K excretion. - Renin and aldosterone are typically suppressed due to volume expansion from the enhanced sodium reabsorption at the distal nephron - Administration of fludrocortisone alone will not substantially increase the rate of potassium excretion given that there is still a marked inhibition of ROMK. However, when combined with sodium bicarbonate administration sufficient to cause bicarbonaturia, there will be an increase in K trapping in the lumen from more proximal sites, thereby resulting in kaliuresis.

Causes pseudohyponatremia?

Pseudohyponatremia can be caused by extreme elevation in plasma proteins (as with plasma cell dyscrasias), triglycerides (as with lipemic serum), or total cholesterol and lipoprotein X (as with obstructive jaundice). Note that an abnormality related to lipoprotein X does not result in lipemic serum. Multiple myeloma can result in hyperproteinemia and cause pseudohyponatremia.

Treatment for diffuse alveolar hemorrhage and evidence of acute GN?

Pulse methylprednisolone and plasma exchange Kidney biopsy and serologic evaluation should be pursued, but treatment for the possibility of an autoimmune pulmonary-renal syndrome should be initiated even before a diagnosis is established. Guidelines from the American Society for Apheresis (ASA) lists AAV with severe renal involvement as a class I indication for TPE, as it does for pulmonary hemorrhage related to either anti-GBM or ANCA antibodies. In this patient, for whom the clinical suspicion of an autoimmune process is high, and in whom anti-GBM disease has not been excluded, empirical TPE and corticosteroids would be indicated. Either Rituxan or cyclophosphamide (or both) is often added once the diagnosis is established.

Treatment for primary hyperoxaluria.

Pyridoxine primary hyperoxaluria based on his presentation with calcium oxalate monohydrate stone disease, nephrocalcinosis, CKD, and severe hyperoxaluria. Primary hyperoxaluria is a rare autosomal recessive disorder, which results in overproduction of oxalate. Type 1 is the most common of the three types of primary hyperoxaluria Because the liver is the source of the underlying enzymatic defect, liver transplantation is considered a curative procedure. Transplantation is usually delayed and medical therapy maintained until CKD has reached stage 3b or worse. Many patients undergo SLKT because there is already significant kidney damage, and kidney function may worsen with the transplant procedure or with calcineurin inhibitor therapy after the transplant. ***a new therapy for primary hyperoxaluria has been approved. Lumasiran is an RNA-interference drug (RNAi) that reduces hepatic oxalate synthesis and increases excretion of an oxalate precursor, glycolate. A recent study found significant reduction in oxalate excretion and normalization of oxalate levels without serious adverse effects. Although experience is preliminary, nephrologists should be aware that lumasiran is a promising, emerging option for patients with primary hyperoxaluria.

What is reflex anuria (RA)?

RA is described as cessation of urine output from both kidneys in response to irritation of or trauma to one kidney or its ureter or severely painful stimuli to other organs. The pathogenesis remains incompletely understood. Proposed mechanisms of RA involve reflex bilateral intrarenal arteriolar spasm or ureteral spasm. RA has been reported in cases of unilateral ureteral obstruction, in which the contralateral kidney appears nonfunctional despite an absence of anatomic obstruction or any direct manipulation of the unobstructed kidney or ureter. RA is a diagnosis of exclusion. Other causes of anuria, such as bilateral ureteral obstruction, renovascular occlusion, acute tubular necrosis (ATN), cortical necrosis, intrinsic kidney disease, severe infection, and hemodynamic instability, must all be excluded. Importantly, RA is a functional condition, not a parenchymal disease.

Mutations in the ___ gene for renin, previously termed FJHN type 2, are associated with hyperuricemia, hyperkalemia, childhood anemia, and mild hypotension.

REN

Patient with positive HBsAg but negative result for core antibody what is the cause?

Recently vaccinated with recombinant HBsAg There are several hepatitis B vaccines approved for use, including a recent 2-dose formulation. All use recombinant HBsAg to provoke protective immunity, manifested in the recipient by the brief appearance of HBsAg followed by the longer persistence of HBsAb in the serum. The hepatitis B vaccine is typically administered as 2-3 doses over 1-6 months, depending on the generation of vaccine administered. Measurement of HBsAg within 2 weeks of administration can potentially result in a positive test, erroneously suggesting acute or chronic infection. The HBsAg typically becomes undetectable (negative) by 14-20 days after vaccination.

After a kidney transplant failure should someone continue immunosuppressants?

Recommend maintenance of immunosuppressive therapy after transplant failure for those patients seeking another transplantation who are likely to undergo subsequent transplantation quickly through living donation or early relisting. Additional reasons to maintain immunosuppressant medications include minimization of systemic inflammation from the failed renal allograft and, possibly, preservation of residual allograft function, particularly in peritoneal dialysis patients. Weaning immunosuppression is an independent predictor of the development of de novo donor-specific antibodies, and when these agents are weaned without allograft removal, this can trigger late rejection in the failed kidney and mandate allograft nephrectomy.

Patient with history of MN s/p renal transplant 14 months ago from sister now develops peripheral edema. DSA negative, PRA 0%. Cr stable. Proteinuria with UPC 3.1. What is the likely cause of the patient's proteinuria?

Recurrence of membranous nephropathy - may recur in 10-45% of patients and typically presents with proteinuria 13-15 months after transplantation, although, rarely, it may be observed earlier. Surveillance for proteinuria, followed by biopsy of the allograft, is necessary for early diagnosis. Spontaneous remission of MN is rare after transplantation; instead, early initiation of rituximab is recommended.

Most appropriate modification for a patient with mixed calcium and uric acid stone disease?

Reduce animal protein intake - 24 hour urine will have low pH ~ associated with increased dietary intake of protein - associated with uric acid stones, both calcium and uric acid stones form acidic urine - elevation of urine sulfate, urea nitrogen, and ammonium, high catabolic rate = high protein consumption Dietary protein intake increases acid load -> increased urinary calcium excretion -> decrease citrate excretion urine and low urine pH -> calcium and uric acid stones form

___ rate to minimize the frequency and severity of hypotensive episodes during dialysis is a reasonable strategy to try to decrease the progression of cognitive impairment in a patient who receives HD.

Reduction in ultrafiltration (UF) Repeated bouts of hypotension leading to reduced cerebral perfusion is emerging as a potentially modifiable risk factor for complications, including cognitive impairment, in patients receiving dialysis. The reported prevalence of intradialytic hypotensive episodes is 7.5-69% depending on the definition. A high UF rate is increasingly implicated as a risk factor for these events.

What is refeeding syndrome?

Refeeding syndrome is a potentially fatal medical complication that occurs as a result of shifts in fluids and electrolytes in malnourished patients during the initial stage of oral, enteral, or parenteral nutritional replenishment. Hypophosphatemia is the most common complication and is also considered a surrogate marker for refeeding syndrome. When patients are fed carbohydrates after a period of nutritional deficiency, the glucose load causes the release of insulin, triggering cellular uptake of glucose, phosphate, potassium, and magnesium, with corresponding reduction in their respective serum concentrations. Insulin also stimulates protein and glycogen synthesis, which require phosphate for adenosine triphosphate and 2,3-bisphosphoglycerate, further depleting total body phosphate stores. The ensuing acute hypophosphatemia can lead to acute respiratory failure, cardiac arrhythmias, hemolytic anemia, rhabdomyolysis, colonic ischemia, altered mental status, and seizures.

CT findings of a "rim sign" and the persistent anuric AKI precipitated by severe acute pancreatitis are diagnostic of ___.

Renal cortical necrosis Bilateral renal cortical necrosis, a rare form of severe AKI usually associated with severe shock and characterized by oligo-anuria and limited or absent recovery. Pregnancy-related events such as septic abortion, placental abruption, and post-partum thrombotic microangiopathy account for the largest number of cases. HUS, severe forms of glomerulonephritis, pancreatitis, and other forms of shock are among the various causes as well. The abrupt onset of severe oligo-anuria (<100 mL per day of urine) limits the differential diagnosis to bilateral renal artery occlusion, complete urinary tract obstruction, severe RPGN, and bilateral acute cortical necrosis. Two patterns of renal cortical necrosis have been described: 1. Diffuse cortical necrosis, which involves global cortical destruction except for a thin rim of preserved subcapsular and juxtamedullary tissue 2. Patchy cortical necrosis of up to half of the cortical tissue

___ is a nuclear medicine technique that uses radioactive isotopes, commonly technetium 99m (Tc-99m), to evaluate kidney function. Tc-99m is conjugated to ligands eliminated by glomerular filtration or tubular secretion to assess GFR or renal plasma flow. It is an effective tool to identify obstruction in situations in which hydronephrosis is absent.

Renal scintigraphy Three components comprise a normal renogram, as shown in the figure below. The first phase reflects the bolus of the radioactivity that arrives via the renal artery to the kidney. The second phase indicates the accumulation of the tracer in the kidney up to the peak. The third and final phase reflects the excretion of the radiotracer from the kidney. In obstructive AKI, phase 3 is absent, and the curve generated from the affected kidney shows a slow continuous accumulation of tracer with no downslope. Phase 3 may also be absent or blunted in the presence of significant CKD or AKI. A dose of a loop diuretic can be administered to induce tracer elimination to distinguish between intrinsic AKI (elimination occurs and phase 3 is observed) and obstruction (no elimination).

Patient with granulomatosis with polyangiitis develops symptomatic hyponatremia 2/2 SIADH while on cyclophosphamide therapy. What change should be made?

Replace cyclophosphamide with rituximab Cyclophosphamide is the likely cause of this patient's SIADH and should be replaced with an alternative agent to treat the underlying granulomatosis with polyangiitis (GPA). The risk of SIADH appears greatest in those receiving high dose IV cyclophosphamide, but there are case reports of severe hyponatremia in patients treated with low dose IV or even oral preparations. Because patients receive IV fluids with the cyclophosphamide infusion and are advised to have a high oral fluid intake when taking cyclophosphamide to prevent bladder toxicity, the risk for hyponatremia may be inadvertently increased. Hyponatremia related to cyclophosphamide infusion is typically apparent by 12-48 hours after exposure and usually resolves over several days.

A kidney transplant patient has severe cutaneous squamous cell carcinomas and he is currently on tacrolimus, mycophenolate, and prednisone. Which changes in the patient's IST can be made to decrease the risk of subsequent squamous cell carcinomas?

Replacement of tacrolimus with sirolimus is the most appropriate change in this patient's immunosuppressive regimen because it is likely to decrease the incidence of further squamous cell carcinomas (SCCs). Conversion from a CNI-based regimen to a sirolimus-based regimen reduces the risk of cancer but is associated with a higher risk for proteinuria, edema, and pneumonitis.

Of the commonly prescribed anti-TB agents which of the drugs most likely to cause AKI?

Rifampin The pathogenesis of AKI from rifampin is likely from anti-rifampin antibodies, which may explain the observation that the syndrome develops rapidly after a second exposure. Patients tend to present with n/v, abdominal pain, fever, and flu-like symptoms. When kidney biopsy has been performed, the histopathologic findings reported include acute interstitial nephritis, acute tubular necrosis, or both. Although the AKI can be severe and dialysis may be needed, recovery is the rule, occurring in >90% of patients when the drug is discontinued. Abnormalities of aminotransferases, anemia, thrombocytopenia, and hemolysis are often observed.

Which latent TB drug is a potent inducer of cytochrome P450 3A4 which is responsible for tacrolimus metabolism results in rapid metabolism of tacrolimus?

Rifampin tacrolimus levels can become subtherapeutic or even undetectable if the tacrolimus dose is not increased in anticipation of this drug interaction. In this case, failure to increase the tacrolimus dose when rifampin was prescribed predisposed the patient to rejection. Use of an alternative agent, such as isoniazid, would also have been reasonable. Regardless of the known propensity of an agent to interact with immunosuppressant medications, it is good practice to monitor drug levels and assess the patient within a week or two whenever medications are added or withdrawn.

Hydronephrosis occurs in up to 80% of pregnant women and has been attributed to mechanical compressive forces on the ureters by the gravid uterus. Hormones, such as progesterone and relaxin, may also play a role. ___-sided hydronephrosis is more common and may be more pronounced. This is attributed to the anatomy of the ___ ureter, which crosses the iliac and ovarian vessels.

Right

A patient with refractory hypertension and evidence of unilateral overproduction of aldosterone by the right adrenal gland should undergo ___.

Right adrenalectomy patient presents with early-onset hypertension that is refractory to control with 4 drugs, including a diuretic. This presentation suggests a secondary cause. His elevated aldosterone/renin ratio (ARR) strongly suggests primary aldosteronism, primary aldosteronism is confirmed by the failure of IV saline loading to suppress the aldosterone level Patients who are surgical candidates for adrenalectomy should then undergo adrenal CT or MRI scanning. Because incidental adrenal nodules are identified in up to 4% of patients undergoing abdominal CT for other reasons, and many of these "incidentalomas" are nonfunctional, the presence of an adrenal nodule is insufficient to justify adrenalectomy. Instead, it is crucial to prove that the overproduction of aldosterone lateralizes to the side of the nodule by adrenal vein sampling (AVS). In unilateral aldosterone excess, the cortisol-corrected aldosterone level is >4:1 (when the high side is compared with the low side), and the ratio from the suppressed adrenal gland is typically clearly lower than that from the inferior vena cava.

The most appropriate treatment for a patient who developed recurrent diffuse proliferative lupus nephritis despite cytoxan induction and maintenance therapy with MMF is ___.

Rituximab - rituximab is not recommended as a first-line therapy for the treatment of lupus nephritis (based on LUNAR study) - However, efficacy of rituximab in the treatment of refractory lupus nephritis has been demonstrated in observational studies in which rituximab therapy led to complete or partial remissions in >50% of patients who did not respond to first-line therapy. ** combo tacrolimus, MMF, cyclophosphamide (multi target therapy) has been used as an induction for lupus nephritis

___ is characterized by the abrupt onset of hypertension and AKI. Pulmonary edema and hypertensive encephalopathy may also occur. Raynaud syndrome with swelling and stiffness of the hands. ACEi are the mainstay of therapy.

Scleroderma renal crisis (SRC) - ANA antibodies, speckled and centromere pattern - Anti-RNA polymerase III antibodies are associated with increased risk of SRC as well as more rapid progression of cutaneous involvement - Topoisomerase antibodies (Scl-70) are present in 30% of patients with diffuse SS and absent in limited SS - Anti-centromere antibodies are present in about half of patients with limited SS.

___ is indicated for this patient with immune complex-mediated membranoproliferative glomerulonephritis (MPGN) with predominant kappa deposition.

Serum protein electrophoresis The immunofluorescence (IF) pattern of the biopsy provides clues to the pathogenesis and helps define the subtypes. 1. Immunoglobulin-positive, complement-positive (these can be monoclonal immunoglobulins as with a plasma cell dyscrasia, or polyclonal as with the case of hepatitis C) 2. Immunoglobulin-negative, complement-positive (disorders of complement, as with dense deposit disease or C3 glomerulonephritis) 3. Immunoglobulin-negative, complement-negative (as with a thrombotic microangiopathy) Etiologies of immune complex MPGN include autoimmune disease such as lupus nephritis. Lupus nephritis, however, usually demonstrates IgA and C1q in addition to IgG, IgM, C3, kappa, and lambda by IF, often referred to as a "full house pattern." MPGN related to hepatitis C (with or without cryoglobulins) is associated with the deposition of polyclonal immune complexes, and shows granular deposition of IgM, C3, and relatively equal kappa and lambda; IgG deposition is variable. MPGN related to chronic bacterial infection as with bacterial endocarditis would also produce polyclonal immune complex deposition, rather than a monoclonal pattern by IF. Deposition of a monoclonal immunoglobulin can also result in an immunoglobulin-positive, complement-positive MPGN-pattern of inflammation, as in this case.

1-2 weeks after thymoglobulin treatment patient presents with fever, rash, and polyarthralgias. Causes?

Serum sickness - the heterologous protein, thymoglobulin, can cause a type III hypersensitivity reaction typical of serum sickness. This immune complex-mediated reaction activates the complement cascade and results in systemic symptoms that most commonly include fever, rash, and polyarthralgias occurring 1-2 weeks after first exposure to the responsible agent. - Lab: neutropenia or leukocytosis, mild thrombocytopenia, elevated ESR and C-reactive protein, low serum complement levels, and elevated anti-heterologous antibodies against rabbit immunoglobulin IgG and IgM. Occasionally, serum creatinine is elevated, but renal dysfunction tends to resolve within a few days. - Tx is discontinuation of the offending agent/avoidance of its use in the future. Corticosteroids are recommended for patients with severe systemic symptoms such as high fever/severe arthralgias and rash. Plasmapheresis has also been used for severe or refractory cases.

Treatment of erectile dysfunction in ESKD patient?

Sildenafil - phosphodiesterase-5 inhibitor Erectile dysfunction (ED) is common in patients with ESRD, affecting 80% and rated as severe in 50%, often adversely affecting quality of life.

The mainstay of treatment for calcific uremic ateriolopathy (CUA) is ___.

Sodium thiosulfate administered IV thrice weekly during the last 30-60 minutes of HD - observational studies have shown complete remission of calciphylaxis lesions in 26-52% of patients - Adverse effects of sodium thiosulfate include nausea and vomiting, volume overload, and an anion gap metabolic acidosis (accumulation of thiosulfate This medication is thought to inhibit calcification in adipocytes and adipocyte-induced calcification in vascular smooth muscle cells, possibly by exchanging with phosphate as a counterion with calcium.

The ___ class of medications decreases glomerular hyperfiltration and the risk of progression of diabetic nephropathy.

Sodium-glucose transporter (SGLT2) inhibitor The Empagliflozin, Cardiovascular Outcome Event Trial in Type 2 Diabetes Mellitus Patients enrolled 7020 patients with type 2 diabetes (hemoglobin A1c 7-10%) and high cardiovascular risk. Patients randomly assigned to empagliflozin, compared with those receiving placebo, had lower risks of all-cause mortality (32%), cardiovascular mortality (38%), and hospitalization for heart failure (35%). A prespecified subgroup of patients with CKD (estimated GFR <60 mL/min/1.73 m2) experienced these benefits, as well as a lower risk of renal endpoints (doubling of creatinine, worsening nephropathy, ESRD, or renal death).

Hypophosphatemia with high fractional excretion of phosphorus. Normal PTH. Normal vitamin D levels. Dx?

Spurious hypophosphatemia, rare condition associated with paraproteins - Paraproteins can occasionally interfere with the serum phosphorus assay, and if suspected, the plasma sample can be deproteinated prior to measurement, after which the assay will be accurate. - Paraproteins have also been associated with spurious hyperphosphatemia related to binding of phosphate to the paraproteins.

How do patients with chronic alcohol abuse develop hypophosphatemia?

Such patients have decreased intestinal phosphate absorption due to poor intake of both phosphate and vitamin D. Decreased absorption may be further exacerbated in the presence of chronic diarrhea. Alcohol abuse may lead to generalized proximal tubular dysfunction, increasing urinary phosphate excretion as well as decreasing phosphate absorption caused by hyperparathyroidism associated with vitamin D deficiency. Chronically low total body stores of phosphorus place patients with chronic alcoholism at an elevated risk of developing severe hypophosphatemia during refeeding syndrome.

___ medications can also cause crystal-induced AKI. The crystals are commonly fan-shaped, often described as a "shock of wheat." ___ , in the high doses needed to treat infections such as toxoplasmosis or Pneumocystis jiroveci pneumonia, can cause crystal precipitation and intratubular obstruction or even nephrolithiasis.

Sulfa-containing

What is the best confirmatory diagnosis for the following diagnosis? Long duration of dialysis dependence Distribution of symptoms (involving the neck, both shoulders, and hands), patient with pain/stiffness Soft tissue prominence over the shoulders ("shoulder pad sign") Bone cysts on radiography

Synovial biopsy to establish the diagnosis of β-2 microglobulin amyloidosis β-2 microglobulin is normally filtered at the glomerulus with reabsorption and catabolism in tubules. Reduced clearance in patients with ESRD results in deposition of β-2 microglobulin fibrils in bones and periarticular and other soft tissues, which causes dialysis-related amyloidosis. Osteoarticular deposition causes an erosive and destructive osteoarthropathy, spondyloarthropathy, and carpal tunnel syndrome Conventional radiographs of the joint affected by dialysis-associated amyloidosis can show radiolucencies of variable size within the medullary or cortical bone due to cortical destruction. These bone cysts are identified most commonly around hips, shoulders, and carpal bones. Fine sclerotic margins are usually present. Definitive diagnosis of dialysis-related amyloidosis is made by staining biopsy tissue from the affected joint or bone cyst with Congo red and the demonstration of telltale apple-green birefringence under polarized light. Once the diagnosis of β-2 microglobulin amyloidosis is established, efforts can be directed to enhance clearance. Considerations might include high-flux dialysis using more biocompatible dialyzer membranes, hemofiltration, immunoadsorption, and kidney transplantation.

Patient with n/v/abdominal and back pain, and AKI. Kidney biopsy consistent with ATN. Utox is negative. CK normal. Which drug?

Synthetic cannabinoid "spice" or "K2" - supportive tx and recovery within days

TRUE/FALSE: Medication non adherence is a risk factor for tx failure and is a contraindication for PD.

TRUE

TRUE/FALSE: Most patients with ESRD will require maintenance therapy with a combination of ESA and iron to maintain target levels of hemoglobin >9 g/dl with adequate iron stores (additional iron is suggested when transferrin saturation [TSAT] ≤30% and ferritin ≤500 ng/ml).T

TRUE

TRUE/FALSE: Normally, the serum level of kappa free light chains is 3.3-19.4 mg/L and that of lambda free light chains is 5.7-26.3 mg/L, so the normal kappa/lambda ratio is typically 0.26-1.65

TRUE Although this effect is modest, some studies suggest that the kappa/lambda ratio expected range in patients with AKI or CKD should be adjusted to 0.37-3.1.

TRUE/FALSE: KDIGO defines anemia in adults with CKD as a hemoglobin concentration <13 g/dL in men and <12 g/dL in women.

TRUE In patients with CKD, iron deficiency is defined as a relative deficiency resulting in impaired response to an erythropoiesis-stimulating agent (ESA). Iron therapy should be started in CKD patients with anemia, ferritin <500 ng/mL, and TSAT <30%. A variety of IV iron preparations are available, and each may be used in a variety of regimens. Iron sucrose is often infused in 100-mg or 200-mg increments for a total dose of 1 g over 2 weeks. Larger doses of 300-500 mg requiring only 2-3 infusions may be more convenient for patients not yet receiving dialysis.

TRUE/FALSE: Blood group B transplant recipients with low anti-A titers can receive a kidney from blood group A2 donors without the need for desensitization or any additional immunosuppression than what is used in ABO-compatible transplantation

TRUE blood type A2, have low antigen expression and functionally act similarly to blood group O donors

TRUE/FALSE AKI requiring dialysis is a common condition affecting up to 7% of critically ill patients in the ICU and associated with mortality in 40-60%. This number is consistent across multiple studies and does not appear to be modified by the dose of renal replacement therapy.

TRUE Acute kidney injury requiring dialysis (AKI-D) Approximately 10-30% of ICU survivors will remain dialysis-dependent at 90 days, although this prevalence decreases to 8.5% at 180 days. 2009 study of all patients requiring renal replacement therapy for AKI in northern California stratified by baseline eGFR found that the proportion of patients who developed ESRD within 30 days of discharge was 27%, 45%, and 77% among those with CKD stage 3b, 4, and 5, respectively.

TRUE/FASLE: A modest rise in serum creatinine by up to 30% that does not progress is commonly observed and is not associated with any adverse outcome when starting a patient on ACEi/ARB.

TRUE, recheck serum chemistries in 1 week to confirm that kidney function is stable Angiotensin-converting enzyme inhibitors (ACE inhibitors) and angiotensin receptor blockers (ARBs) are associated with a physiologic reduction in intraglomerular pressure, which may lead to a reduction in the glomerular filtration rate (GFR). These agents both decrease arterial pressure by blocking angiotensin's effect on the vasculature and also decrease vasoconstriction of the efferent arteriole If the GFR declines >30% during the first 6-8 weeks of ACE inhibitors or ARBs, the drug should be discontinued and look for RAS and duplex renal US.

The Kidney Disease Improving Global Outcomes (KDIGO) Clinical Practice Guideline for Anemia recommends maintaining transferrin saturation (TSAT) > ___ % and ferritin > ___ ng/mL for hemodialysis or >100 ng/mL for peritoneal dialysis patients. IV iron therapy is suggested if TSAT ≤30% and ferritin ≤500 ng/mL.

TSAT > 20% Ferritin > 200 ng/mL A recent meta-analysis suggested that hemoglobin typically improves by 0.3-0.5 g/dL after an IV iron load.

__ , an α1-adrenergic receptor antagonist, is often used for "medical expulsive therapy" for patients with renal colic, in order to promote relaxation of the ureter and hasten stone passage.

Tamsulosin

Dosing of Zosyn during CRRT?

Target levels of antibiotics including piperacillin-tazobactam (piptazo) are associated with more effective bacterial killing and are more likely to be achieved with extended infusions (3 hours) during continuous renal replacement therapy (CRRT). Because of the potentially high clearance of antibiotics by CRRT, conventionally dosed antibiotics may fail to achieve this goal. Extension of infusion time increases the time at which the concentration of pip-tazo exceeds the MIC. This strategy has shown benefit for some patients with severe infections and sepsis.

Calculate weekly Kt/V urea provided by residual renal function.

The daily residual renal Kt/V is calculated as (UUN × UV)/(BUN × TBW), where TBW is total body water, estimated as 60% of body weight. UUN = urine urea UV = urine volume Multiplied by 7 days, the weekly residual renal Kt/V Daily peritoneal urea clearance may be calculated similarly, but total dialysate effluent (TDE) substitutes for urine volume. In this case, TDE is the sum of dialysate infused (four 2-L exchanges = 8 L) plus the UF volume (2.4 L), for a value of 10.4 L:

Sodium correction for hyperglycemia?

The most commonly cited correction factor for the serum sodium concentration is 1.6 mmol/L for every 100 mg/dL increase in glucose concentration above normal

First line treatment PRES

The primary goal of therapy is to lower the BP, but it should not be lowered by more than 25% in the initial period. Labetalol and nicardipine are commonly used as first-line therapies. Nitroprusside can theoretically increase intracranial pressure because of its vasodilatory effects and as such is second-line therapy.

How can alkaline urine cause a false-positive urinary dipstick?

The protein reagent on the urinary dipstick is a colorimetric dye, which displays a color change depending on the concentration of urinary albumin. This reagent changes from pale green to dark blue based on pH changes promoted by urinary albumin. If the urine is alkaline, a false-positive urinary dipstick for albumin can occur. When the urine is very concentrated, the urine dipstick may overestimate the amount of urinary protein, but this will not result in a high false positive (i.e., 3+)

When to use saline suppression test?

The saline suppression test is most commonly used for confirmation of primary aldosteronism when plasma aldosterone and renin levels are equivocal. The plasma aldosterone level is measured before and after infusion of 2 L of normal saline over 4 hours. Patients with primary hyperaldosteronism have higher basal levels, but more importantly, they fail to suppress below 5 ng/dL after saline administration.

Treatment for renal infarction?

The treatment of a renal infarction is usually conservative when symptoms have been present for >24 hours. Thrombectomy or thrombolysis can be considered if the event is more recent and there may be an opportunity to salvage tissue. Angioplasty, with or without stenting, can be performed if there is an underlying anatomic abnormality, such as fibromuscular dysplasia or dissection. In this case, the presence of atrial fibrillation, which likely accounts for the renal infarction, as well as the delay in presentation suggest that anticoagulation is preferable to angiography. Longer-term therapeutic anticoagulation should be considered for patients with risk for additional events, such as this patient with atrial fibrillation or patients with thrombophilia. Other clinical manifestations include livedo reticularis, purpura, digital ischemia, bowel ischemia, and pancreatitis. Eosinophilia and C3 hypocomplementemia may be seen in laboratory investigation.

The most appropriate tx for a patient presenting with features of alcoholic ketoacidosis (AKA) is ____.

Thiamine and dextrose Measure osmolar gap Measured osmolality - calculated osmolality = OG Measured osmolality - (Na × 2 + BUN/2.8 + glucose/18) Profound ketoacidosis can develop in patients with chronic alcohol abuse. Contributing factors include a poor nutritional status, which results in a reduction in the insulin-to-glucagon ratio, which leads to accelerated fatty acid utilization as a source of energy. Lower insulin levels decrease carnitine acyltransferase activity, impairing the entry of fatty acids into mitochondria, where they would normally enter the citric acid cycle, and instead shunting them to ketone production. Chronic metabolism of ethanol in the liver leads to an increased NADH/NAD+ ratio, causing a mass balance shift of acetoacetate to β-hydroxybutyrate to a greater extent than typically seen in diabetic ketoacidosis (DKA). Patients most often present after a period of abstinence when the ethanol level is low, because withdrawal increases circulating catecholamines, provoking more peripheral mobilization of fatty acids.

Which deficiency can cause high-output HF, nystagmus ophthalmoplegia, ataxia, confusion, confabulation, and impaired short-term memory. B lactic acidosis.

Thiamine deficiency - acute Wernicke encephalopathy or chronic Korsakoff syndrome Patients on either PD or HD can get thiamine deficiency, decreased plasma thiamine levels by 6% after HD Nutritional supplementation (thiamine 100 mg oral daily) is recommend in ESRD

A patient with recurrent kidney stones with adequate UOP has hypercalciuria and hypocituria. Tx?

Thiazide-like diuretic + potassium citrate supplementation Potassium citrate is preferred to sodium citrate because an increase in sodium excretion will tend to increase calcium excretion, potentially off-setting some of the benefit of the chlorthalidone. In addition, the use of a thiazide is likely to cause hypokalemia and potassium citrate can offset these losses.

Define relapsing peritonitis, repeat peritonitis, and recurrent.

This patient has relapsing peritonitis, which is defined as a second episode with the same organism within 4 weeks of completion of antibiotics for the initial episode. Repeat peritonitis is another episode of peritonitis with the same organism >4 weeks after completion of antibiotics for the initial episode. Relapsing and repeat peritonitis may relate to failure to eradicate the infecting organism due to either biofilm adherent to the catheter or a persistent nidus of infection associated with the exit site or tunnel. Recurrent peritonitis, defined as another episode of peritonitis with a different organism within 4 weeks of stopping antibiotics, is usually due to episodes of touch contamination with poor patient technique.

What is thyrotoxic hypokalemic periodic paralysis?

Thyrotoxic hypokalemic PP is an acquired form of the disorder that is common in Asian populations and primarily affects males. It has been associated with defects in the gene encoding Kir2.2, an inwardly-rectifying potassium channel on skeletal muscle that is transcriptionally regulated by thyroid hormone. Propranolol has been shown to be effective in thyrotoxic PP but has no role in familial hypokalemic PP.

The most likely intervention to improve the abdominal pain associated with PD is for the patient to switch to a ___ of peritoneal dialysis. ___ peritoneal dialysis involves incomplete drainage of the peritoneum prior to the addition of the next dwell.

Tidal mode Automated peritoneal dialysis uses hydraulic suction to facilitate drainage. It has been proposed that the suction transmits negative pressure to the sensitive parietal peritoneum when the peritoneum is nearly empty, resulting in pain. Residual fluid in the cavity acts as a liquid barrier and may cushion, or otherwise protect, the parietal peritoneum from direct exposure to suction, thereby preventing drain pain. If tidal peritoneal dialysis is unsuccessful at alleviating pain, a trial of continuous ambulatory peritoneal dialysis can be attempted, because gravity drainage may be less prone to provoke drain pain.

Patient with combined anion and NAGMA with normal osmolal gap and profound hypokalemia. Which toxicity?

Toluene Toluene toxicity typically results from glue or solvent sniffing. Toluene is metabolized to form hippuric acid, which titrates extracellular bicarbonate and leads to an anion gap metabolic acidosis. However, hippurate is freely filtered in the urine. This both reduces the anion gap and leads to potassium wasting as the hippurate is excreted with potassium as a "non-reabsorbable" anion and thus mimics a distal renal tubular acidosis A patient presenting soon after toluene exposure may display an anion gap acidosis whereas a later presentation typically displays a non-anion gap acidosis.

___ , the only V2 receptor antagonist approved for use in the United States, is approved for the short-term treatment of hypervolemic or euvolemic hyponatremia refractory to fluid restriction. There is concern regarding the use of ___ in patients with liver disease based on the observation of elevated liver enzyme levels in patients receiving ___ compared with placebo in the ___ Efficacy and Safety in Management of Autosomal Dominant Polycystic Kidney Disease and Its Outcomes (TEMPO) trial

Tolvaptan

____ was approved to slow kidney function decline in adults with rapidly progressive ADPKD based on the TEMPO 3:4 and REPRISE studies.

Tolvaptan Before beginning treatment, patients must be counseled about the adverse effects of tolvaptan, including liver toxicity, and both patient and provider must participate in a risk evaluation and mitigation strategy for the duration of treatment.

How does bactrim cause hyperkalemia?

Trimethoprim is structurally similar to amiloride, and competitively inhibits the luminal epithelial sodium channel in the collecting duct. This leads to impairment of distal potassium excretion and can provoke hyperkalemia. Note that trimethoprim also inhibits tubular secretion of creatinine so it is common for the serum creatinine to rise without actual kidney injury or a change in GFR.

___ is the most likely diagnosis in a patient with ESRD and recurrent tertiary hyperparathyroidism, severe hyperphosphatemia, and evidence of soft tissue calcification on x-ray. Uncommon complication in patients with ESRD, in which extensive calcification of soft tissues leads to the formation of nodules that can be painful.

Tumoral calcinosis This diagnosis is usually associated with severe perturbations in mineral metabolism parameters, including calcium, phosphorus, and PTH. Although more common in large joints, tumoral calcinosis has been described in the fingertip.

Treatment for primary hyperaldosteronism in a patient that is a poor surgical candidate.

Tx with spironolactone. Patients with an elevated aldosterone-to-renin ratio (ARR) have about a 30%-50% chance of having primary aldosteronism. In addition, there is a unilateral adrenal mass. In a patient with a good functional status, further evaluation of hyperaldosteronism would be indicated in anticipation of surgical intervention. In a patient with advanced age, who has poor functional status, it is reasonable to treat conservatively with medical therapy. Spironolactone is the first-line agent mineralocorticoid antagonist (MRA). If this is poorly tolerated (most commonly because of gastrointestinal side effects), eplerenone can be prescribed. Because of the excellent response of primary aldosteronism to MRA treatment and the costs/risks associated with preoperative evaluation and surgery, some have argued for wider adoption of empiric MRA therapy in patients with an increased ARR (with suppressed renin and a plasma aldosterone level >15 ng/dl). Adrenal vein sampling is used to localize the source of excess aldosterone secretion in patients who are deemed candidates for surgical therapy.

What is a type A versus type B dialyzer reaction?

Type A: - soon after starting HD, within 5-15 minutes - symptoms range from mild allergic-type symptoms to life-threatening bronchospasm, hypotension, and cardiac arrest, cyanosis, sense of doom, anaphylaxis - IgE-mediated reaction to substances such as ethylene oxide that leach from the dialyzer or circuit - Patients with allergies or eosinophilia may be predisposed. - dialysis should be discontinued without returning the patient's blood from the circuit. - allergy to ethylene oxide sterilant Type B: - occur 20-30 minutes after treatment is initiated - chest and back pain, nausea, and hypotension, all of which gradually subside even if dialysis is continued - complement activation via the alternative pathway by the dialyzer membrane is thought to be the primary cause - supportive management, oxygen supplementation, can continue tx as sxs abate within 1 hour

Patient with post-obstructive diuresis (POD) with dilute UOP of 2.5 L last 4 hours. What replacement fluid would you choose?

UO should be replaced with 0.45% saline at a rate of approximately 0.75 mL/mL of urine output over the first 24 hours in a patient with post-obstructive diuresis (POD) In most cases, a physiologic POD will begin to abate by 24 hours, whereas a pathologic POD may persist for >48 hours. A physiologic POD can be perpetuated by excessive IV fluid. This can be avoided by replacing the urine output at a rate less than the urinary output. Because the urine is relatively hypotonic, a hypotonic replacement fluid should be chosen. In all cases, the patient's electrolytes and volume status should be monitored closely, and fluid management should be tailored to the clinical circumstances. If the patient is able to eat and drink and the polyuria is not severe, IV replacement can be rapidly discontinued in favor of oral intake.

Patient with advanced CKD, volume overload, and moderate pericardial effusion most likely has ____.

Uremic pericarditis - common in patients initiating dialysis - often asymptomatic associated with volume overload - tx is HD

A patient with retroperitoneal fibrosis and AKI should be treated with ____ to relieve obstruction.

Ureteral stenting RPF are idiopathic (>75%). Dx more in men and fifth/sixth decade of life. Fibrosis in the retroperitoneum usually envelopes the aorta or iliac arteries and surrounding structures, including the ureters. Histopathology reveals inflammatory cells and collagen bundles. Sxs: fatigue, weight loss, low-grade fever, flank/ abdominal pain, testicular complaints, and constipation. AKI due to obstruction, w/o hydronephrosis (30-50%), and kidney atrophy. Extrinsic compression of the renal arteries resulting in renal artery stenosis (RAS). ESR and CRP elevated. Because ureteral obstruction is common when there is peri-iliac involvement as in this case, relief of obstruction with ureteral stents would be the first step. If kidney function failed to improve, or if clinical signs of RAS ensued, it would be reasonable to consider renal artery intervention. RF for idiopathic RPF are toxin exposure (tobacco and asbestos) and inflammatory diseases (particularly autoimmune thyroiditis, but also immunoglobulin G4-related [IgG4] disease, RA, vasculitis, SLE, and psoriasis). Medications (ergot alkaloids, bromocriptine, methyldopa, hydralazine, etanercept, and infliximab), malignancy (carcinoid, lymphoma, sarcomas, and numerous carcinomas), infections (tuberculosis, histoplasmosis), radiation exposure, and abdominal surgery. Diagnosis of RPF is made by contrast CT showing fibrous encasement of the aorta or iliac vessels, with or without ureteral involvement. This can also be seen on MRI. Ultrasound has low sensitivity. Biopsy is also diagnostic. After relief of ureteral obstruction, medical therapy with glucocorticoids and other immunosuppression (mycophenolate mofetil, azathioprine, methotrexate, or cyclophosphamide) may be added; rituximab or tocilizumab is suggested for highly resistant cases.

How do patients with nephrotic syndrome develop vitamin D deficiency?

Urinary losses of vitamin D bound to vitamin D-binding protein Prolonged, severe vitamin D deficiency with serum values <10 ng/mL results in secondary hyperparathyroidism and osteomalacia (bone pain/tenderness, weakness)

Urine anion gap equation?

Urine anion gap = [Na+] + [K+] - [Cl-]

___ manifests as increased wound drainage, abdominal pain, ipsilateral leg edema, and delayed or worsening allograft function, sometimes with fever. Ultrasound reveals an anechoic fluid collection with sharp borders and no septations. Chemical analysis of the fluid reveals high creatinine and potassium compared with serum levels.

Urinoma Urinomas may become infected, and management requires drainage and correction of the leak. Surgical exploration and intervention may be necessary, but a percutaneous nephrostomy tube or ureteral stent is often the initial approach. Urinomas tend to form within the first 2 weeks after kidney transplantation. These urine-containing fluid collections usually arise from extravasation of urine due to necrosis of the ureter or failure of the surgical ureteroneocystostomy. Many transplant surgeons place a ureteral stent at the time of transplantation. This strategy is associated with fewer major early urologic complications, including urine leak, but increases the risk for urinary tract infection and possibly other complications.

___ is used as second-line therapy in patients with septic shock who have not responded to norepinephrine. Its use is limited by potential adverse effects, including hyponatremia and rebound hypotension, as well as a smaller literature base.

Vasopressin

Patient with hx renal transplant presents with fever, hypotension, confusion, hemorrhagic cellulitis of the legs, mixed acid-base disorder (anion gap metabolic acidosis and respiratory alkalosis) likely has ___ infection. Patient ate at a seafood buffet.

Vibio vulnificus (Vv) - gram negative bacterium - thrives coastal environments - infection can be acquired through ingestion of contaminated seafood, usually raw oysters. Can also occur through exposure to seawater via inoculation through abrasion by shells or coral, exposure of an open wound, or injury by marine organisms. Transplant patients, ESKD and liver disease patients increased risk: advise patients to avoid raw seafood **At a minimum, recipients of a solid organ transplant should not consume raw or undercooked shellfish (especially oysters), poultry, meat, or fish to reduce their risk of foodborne illness. Sxs: gastroenteritis, primary sepsis with bacteremia, necrotizing soft tissue infections - Hemorrhagic bullae may be present, and culture of this fluid can yield Vv Tx: Third-generation cephalosporin with a tetracycline (e.g., ceftazidime plus doxycycline), but fluoroquinolones, aminoglycosides, and trimethoprim-sulfamethoxazole have also been used.

Which supplements should be started in a patient that is starting denosumab?

Vitamin D and calcium to limit the hypocalcemia associated with denosumab Denosumab is a monoclonal antibody that targets the receptor activator of nuclear factor-κB ligand (RANKL), inhibiting osteoclast formation and activation. Denosumab is indicated for the management of osteoporosis and in prevention of fractures in patients with bone metastases from solid tumors. Hypocalcemia is a prominent side effect of denosumab and occurs in 1.7% of patients treated for osteoporosis (utilizing the low-dose formulation, marketed as Prolia) and 12.4% of patients treated for solid tumor bone metastases (using the high-dose formulation, marketed as Xgeva). This risk is increased in patients with CKD and occurs in up to 30% of patients with creatinine clearance <30 mL/min.

Metabolic alkalosis, hypokalemia, low urine chloride is ___.

Vomiting Ddx of metabolic alkalosis includes GI hydrogen ion loss, renal hydrogen ion loss, and the addition of exogenous alkali. In this case, the divergence of urine chloride (very low) and urine sodium (not low) suggests vomiting as the primary driver of the derangements. Vomiting results in loss of hydrochloric acid in the emesis, which is accompanied by the addition of bicarbonate ion to blood. The resulting high level of filtered bicarbonate then exceeds the resorptive threshold in the proximal tubule and functions as a nonreabsorbable anion, carrying sodium and potassium into the urine. Urine Cl is low because of concomitant volume contraction and increased reclamation of chloride along the nephron, as well as a reduced filtered load due to the loss of chloride in the emesis. Hypomagnesemia is commonly identified in patients with disordered eating. Inadequate intake and urinary losses may contribute. Refeeding can exacerbate this problem. **Diuretic abuse, unless remote, is characterized by an inappropriately high urine chloride. Bartter + Gitelman, inherited disorders of Na reabsorption in the loop of Henle or distal tubule, respectively, mimic chronic exposure to loop or thiazide diuretics and manifest with metabolic alkalosis and hypokalemia and often hypomagnesemia. The urine Cl is inappropriately HIGH.

Patient present with hypokalemia, hypochloremia, metabolic alkalosis, high urine K > 25 and low urine chrloride < 25. Dx.

Vomiting In the case of nonrenal chloride loss, such as vomiting, urine chloride should be appropriately low (<25 mEq/L). Hypokalemia is due to both hypovolemia-stimulated aldosterone secretion and increased delivery of sodium to the distal tubule related to the increased filtered load of bicarbonate ion. Gitelman syndrome, Bartter syndrome, and diuretic use would all cause inappropriately high urine chloride excretion.

__ can lead to hypovolemia, hypochloremia, and metabolic alkalosis. Low urine chloride. Patient with history of bulimia.

Vomiting - chlorine responsive metabolic alkalosis

Patient doing home-hemodialysis lives on a farm. Develops HA, dizziness, cyanosis, and methemoglobinemia what is the contaminant in the water supply?

Well water can be contaminated by agricultural runoff, potentially exposing dialysis patients to chemical and heavy metal toxicity. Fertilizer contamination results in high levels of nitrates in dialysate produced from the contaminated water. Nitrates are potent oxidizers that result in the formation of ferric (3+) hemoglobin from ferrous (2+) hemoglobin. Ferric hemoglobin is unable to bind oxygen efficiently and also impairs oxygen release from the remaining ferrous hemoglobin. The net effect is marked impairment in oxygen delivery. The diagnosis is established by multi-wavelength pulse oximetry or blood gas analysis. Levels higher than 3% can cause skin color changes, cyanosis, headaches, and dizziness. Levels above 15% can cause cardiac and neurologic symptoms associated with hypoxia. Higher levels of methemoglobin can cause seizures and death. Symptomatic patients should be hospitalized and treated with IV methylene blue.

Patient is having a hernia repair and is a PD patient when can PD be resumed?

Withholding PD for 2 days and then resuming with low dialysate volumes with the patient supine during exchanges PD is then gradually advanced over the next month back to the preoperative regimen. The regimen used is as follows: PD thrice weekly for 10-hour sessions using low volumes for 2 weeks, then PD daily using 1.5 L per exchange for 2 weeks, then resume preoperative regimen.

___ deficiency in patients with a history of gastric bypass surgery is a risk factor for acquired hyperammonemia.

Zinc Ammonia is primarily produced in the intestine through metabolism of intraluminal amino acids as well as bacterial degradation of urea. Ammonia is eliminated by conversion to urea in the liver and to glutamine in the brain and skeletal muscle. Hyperammonemia results from both decreased intrinsic hepatic function as well as defects in the urea cycle. Although most individuals with urea cycle enzyme deficiencies present in infancy, mild defects, particularly in ornithine carbamoyltransferase, can present in adulthood with hyperammonemia precipitated by stress.

ADPKD is associated with numerous extrarenal manifestations, including intracranial aneurysms, valvular heart disease (e.g., mitral valve prolapse), hepatic and pancreatic cysts, colonic and duodenal diverticular disease, and _____.

abdominal and inguinal hernias The prevalence of hernia (inguinal, paraumbilical, and incisional) among patients with ADPKD and ESRD was 45%, compared with 16% for patients with other causes of ESRD and 4% for surgical controls. Among patients undergoing PD, hernia is 2-15 times more common among patients with ADPKD than those with other causes of ESRD. Hernia pathogenesis is attributed to abnormal extracellular matrix and increased intra-abdominal pressure caused by nephromegaly.

Osteomalacia refers to a defect with both low turnover and abnormal mineralization of bone. This disorder can be seen by vitamin D deficiency, but in the past, it was a common complication of ___ toxicity.

aluminum

Infliximab is a chimeric IgG monoclonal antibody directed against tumor necrosis factor-α. This agent is used to induce remission in patients with Crohn's disease and then may be used for maintenance therapy. This agent has been associated with drug-induced SLE, although renal involvement has only rarely been reported in this setting. This phenomenon may be distinguished from primary SLE by the presence of ___.

anti-histone antibodies

This patient with asymptomatic fibromuscular dysplasia (FMD) of the carotid and renal arteries should receive antiplatelet therapy with __ because of the increased risk for transient ischemic attack (TIA) and other thrombotic events in this syndrome.

aspirin This strategy has not been proven, but experts agree on this recommendation for all patients with carotid FMD whether symptomatic or asymptomatic, according to the American Heart Association Scientific Statement on FMD.

Postpartum appearance of AKI, hemolysis, and thombocytopenia is most consistent with a diagnosis of ___.

atypical HUS (aHUS) Pregnancy-induced aHUS (P-aHUS) can be seen in the postpartum period and often presents with severe AKI, thrombocytopenia, and hemolysis. It is caused by the dysregulation of the alternative complement pathway. Spontaneous activation of the alternative pathway of complement is prevented by plasma and membrane-bound regulators, including factors H and I, membrane cofactor protein, and decay accelerating factor. Acquired (antibodies) or congenital (activating or inactivating mutations) dysregulation of various factors involved in the regulation of the alternative pathway can lead to complement-induced injury to endothelial cells. Pregnancy has been identified as a trigger for episodes of aHUS in patients with these mutations.

___ has demonstrated to result in substantial and sustained weight loss among obese dialysis patients thus increasing the opportunity for transplation in this population

bariatric surgery

Tumor-induced osteomalacia results from fibroblast growth factor-23 release from (typically benign) mesenchymal tumors, leading to severe urinary phosphate wasting. In this setting, ___ levels are usually low due to a defect in ___ synthesis.

calcitriol

Patiromer is a potassium binding polymer that utilizes calcium sorbitol as a counterion to exchange for potassium. It is associated with a modest increase in ___ absorption and also therefore urinary excretion.

calcium Patiromer is an oral non-absorbable potassium binding agent approved for the treatment of chronic hyperkalemia and is expected to decrease the serum potassium by 0.5-1 mEq/L over the course of 3 days to 2 weeks. This agent has been studied in patients at risk for hyperkalemia associated with renin-angiotensin-aldosterone blockade and allows for the potential continuation of this blockade in settings where hyperkalemia may otherwise limit or prohibit this strategy. One of the most commonly reported adverse effects of patiromer is a reduction in serum magnesium as stool losses of magnesium are increased.

What is the pathophysiologic explanation for autosomal dominant hypercalciuric hypocalcemia (ADHH) is a condition of overactivity of the ___.

calcium-sensing receptor (CaSR) ADHH is a genetic disorder characterized by an activating mutation of the CASR gene. The result of this mutation is that the relationship between calcium and PTH is altered such that calcium concentrations that normally trigger PTH release no longer do so. PTH levels are often low or low-normal. Patients with ADHH have low calcium levels due to insufficient PTH release. In the kidney, insufficient PTH leads to a decrease in active calcium reabsorption and hypercalciuria. Activation of the CaSR in the kidneys also exerts a loop diuretic-like effect in the thick ascending loop of Henle (TAL), reducing sodium chloride reabsorption and decreasing lumen positivity, leading to a decrease in renal calcium reabsorption. Medullary nephrocalcinosis can be precipitated by attempts to normalize the serum calcium via administration of calcium and vitamin D.

Patient with accelerated hypertension associated with retinopathy and AKI ("malignant hypertension"), and a kidney biopsy demonstrating marked arteriolosclerosis, onion-skinning, and luminal thrombosis, has scleroderma renal crisis (SRC) and should be treated with ___ .

captopril - most often used ACEi in acute setting, short half-life SRC is characterized by 3 major features in a patient with systemic sclerosis: (1) abrupt onset of moderate-to-severe hypertension (2) AKI (3) urinalysis that is normal or reveals only mild proteinuria with few cells or casts. The key therapy in SRC is prompt BP control. Compared with other antihypertensive agents, a number of observational and uncontrolled studies have shown that angiotensin-converting enzyme (ACE) inhibitors are associated with better preservation of kidney function and improved survival in patients with SRC. Even patients who require dialysis should be treated with ACE inhibitors because some will recover kidney function after prolonged dialysis dependence.

A patient has acute onset of hemolytic anemia during hemodialysis, and the presence of bite cells and confirmation of Heinz bodies is most consistent with chloramine breakthrough related to dysfunction of the ___.

carbon filtration system - Hemolysis during hemodialysis may be due to breakthrough of chloramine, bleach, copper, nitrates, formaldehyde, improper mixing of the dialysate, increased dialysate temperature, or from mechanical trauma from kinking or narrowing of the dialysis blood tubing. When there is any suspicion of hemolysis, such as when there is an acute drop in hemoglobin, acute onset of back or chest pain, or pink discoloration of dialysis filtrate, the tubing should be clamped with immediate cessation of dialysis. Blood should not be returned to the patient because there is a high risk of hyperkalemia in this setting. The tubing should be saved for further analysis because defective tubing with narrowing or kinks may cause extravascular hemolysis. The composition of the dialysate should also be analyzed. When there is suspicion of a contaminated water product based on the findings in a single patient at a unit with shared water products, strong consideration should be given to discontinuing dialysis for the entire unit until a safe water supply can be confirmed with certainty.

The presence of arm edema, visible collateral veins, and prolonged bleeding after dialysis should prompt evaluation for ____, with angioplasty if indicated.

central venous stenosis patients have a history of ipsilateral central vein catheters, with a reported prevalence of central stenosis as high as 27% the gold standard for diagnosis is central venography. In addition, therapeutic angioplasty can be performed in the same setting as the diagnostic procedure, restoring blood flow and normalizing high venous pressures, allowing for resolution of arm edema, healing of cannulation sites, and improvement in dialysis efficiency.

___ can reduce polyuria due to nephrogenic DI.

chlorthalidone Amiloride as monotherapy to treat the polyuria could be considered if the patient is intolerant of a thiazide

Bisphosphonates, such as zoledronic acid, are used for prevention and treatment of osteoporosis. These agents is not recommended for patients with CKD. Zoledronic acid has been linked to acute tubular necrosis and pamidronate has been associated with___.

collapsing glomerulopathy

A urine osmolality < 100 generally indicates?

complete suppression of ADH, maximally dilute urine and water diuresis 1.) Primary polydipsia 2.) Low solute intake (<300 mOsm/d) 3.) Beer potomania 4.) Advanced renal failure Primary polydipsia when water intake exceeds excretory capacity, estimated 10% of GFR, normal kidney function can excrete total 15 L or more without hyponatremia, decreased GFR may have hyponatremia with lower water intake levels

Dx post-transplant diabetes mellitus (PTDM)

confers an increased risk for cardiovascular events, mortality, and graft loss The diagnosis is established using the same criteria as ADA: elevation of fasting plasma glucose ≥126 mg/dL, elevation of random plasma glucose ≥200 mg/dL with symptoms of diabetes, or a 2-hour plasma glucose concentration ≥200 mg/dL during an oral glucose tolerance test. ***Hemoglobin A1c (HbA1c) reflects glycemic control over the preceding 2-3 months. Blood glucose level may fluctuate substantially in the early post-transplant interval due to perioperative stressors and induction immunosuppression (particularly with corticosteroids). - it is recommended that HbA1c not be used to determine the presence of PTDM until at least 12 weeks after transplant.

Patient on PD finds out they are pregnant what is the next step regarding HD/PD?

conversion to an intensive HD regimen providing ≥36 hours treatment per week and maintaining BUN <50 mg/dL is the preferred strategy - for better maternal and fetal outcomes

IgA vasculitis (HSP) is a systemic vasculitis that involve the skin, GI tract, and kidneys. Treatment?

corticosteroids Generally, patients with renal involvement related to HSP have an excellent long-term renal prognosis. Patients with mild proteinuria and preserved renal function are managed conservatively. However, patients who present with nephrotic syndrome (or nephrotic range proteinuria) or crescentic glomerulonephritis and rapidly deteriorating renal function can develop long-term renal insufficiency and ESRD. These patients are candidates for immunosuppression. A variety of immunosuppressive agents have been used, but corticosteroids are usually considered "first line." Rituximab has been used successfully in cases of refractory or steroid-dependent HSP and rarely as primary therapy, but corticosteroids are considered first line for severe HSP renal involvement.

Curved microtubules around 30 nm in diameter, often described to resemble a fingerprint pattern, are typical of GN related to ___ .

cryoglobulinemia

Protein goals for PD and ESRD with wounds.

dietary protein intake of 1.2-1.3 g/kg per day for clinically stable patients receiving PD less so the higher target of up to 1.5 g/kg per day for ESRD patients with wounds Nutritional supplementation is indicated if malnutrition persists after an attempt at optimizing diet and intake under the supervision of a dietician

Which urinary biomarker confers a higher likelihood of progression of AKI in patients with acute decompensated heart failure?

elevated urine albumin/creatinine ratio (UAC)

What can be causing a metabolic acidosis in a patient taking SGLT2?

euglycemic diabetic ketoacidosis (euDKA) SGLT2 inhibitors work by increasing renal excretion of glucose by blocking glucose reabsorption via the SGLT2 transporters in the proximal tubule. The mechanism of euDKA is thought to relate to a decreased insulin-to-glucagon ratio. Patients often need to administer less insulin while taking SGLT2 inhibitors because of the reduction in serum glucose from glycosuria. At the same time, there is an increase in glucagon release due to both decreased insulin levels and inhibition of SGLT2-mediated transport of glucose into pancreatic a-cells. This low insulin-to-glucagon ratio leads to increased lipolysis and release of free fatty acids, which are converted to ketone bodies by ß-oxidation.

Risk factors for 5-oxoprolinemia?

female sex, advanced age, malnutrition, diabetes, and liver disease acquired 5-oxoprolinemia most commonly results from chronic acetaminophen ingestion, which depletes glutathione stores can lead to metabolic acidosis due to accumulation of pyroglutamic acid, or 5-oxoproline

Rasburicase, a recombinant form of urate oxidase, rapidly degrades uric acid into allantoin, which is more water soluble and less toxic than its precursor. This agent is highly effective in controlling uric acid levels compared with allopurinol, with a low rate of adverse events. Patients of Mediterranean or black ethnicity should be screened for ___deficiency.

glucose-6-phosphate dehydrogenase (G6PD) G6PD deficiency is a contraindication to rasburicase therapy as it results in hemolysis in individuals with this metabolic defect.

Defects in the UMOD gene for uromodulin are also associated with hyperuricemia and early-onset __.

gout Previous terminology for this disorder includes medullary cystic kidney disease type 2, familial juvenile hyperuricemic nephropathy (FJHN) type 1, and uromodulin-associated kidney disease.

Membranous nephropathy related to ___, while rare in United State natives, should be considered in immigrants or travelers from ___-endemic regions presenting with proteinuria.

hepatitis B Approximately 60-80% of patients with membranous nephropathy associated with hepatitis B will have circulating serum HBeAg; the remainder will have antibodies to HBeAg. Adult onset hepatitis B-associated membranous nephropathy carries a higher risk of progressive kidney disease.

Patients with ANCA-associated glomerulonephritis related to ___ use frequently have detectable ANA, antibodies to double-stranded DNA, and antihistone antibodies, along with anti-myeloperoxidase ANCA. Cessation of the drug and immunosuppressive treatment are usually necessary for resolution.

hydralazine

A point mutation in the gene encoding the α subunit of the skeletal muscle cell sodium channel leads to ___ (condition).

hyperkalemic periodic paralysis The abnormal sodium channel closes slowly and the muscle can become desensitized; this results in paralysis of the muscle and release of potassium. Precipitants may include cold exposure, rest after exercise, fasting, ingestion of even small amounts of potassium, and anesthesia. Potassium levels may be normal or only modestly elevated during attacks. mechanism involved in hyperkalemia is one of transcellular shift

Chronic hypokalemia may cause ___ ___ , characterized by interstitial fibrosis, tubular vacuolization, and multiple renal cysts. The urine sediment is bland. Mild low-molecular-weight proteinuria, with only modest albuminuria, is observed. Chronic hypokalemia may also cause a urinary concentrating defect with a fixed low urine specific gravity that can present as a form of nephrogenic diabetes insipidus.

hypokalemic nephropathy

Parallel arrays of microtubules with a diameter usually >30 nm are seen in ___ GN.

immunotactoid

Patient with severe hyperammonemia and acute cerebral edema should undergo ___.

intermittent HD

Patient with suspected air embolism should be placed on __side in Trendelenburg position to attempt to trap the air in the apex of the right ventricle and prevent airlock of the right ventricular outflow or the migration of air up the venous circulation to the brain.

left Air embolism during dialysis is a serious but rare complication resulting in chest pain, dyspnea, and possible death. Although small amounts of air can diffuse across the arteriolar wall into the alveolar spaces, larger amounts (≥50 mL) can cause pulmonary outflow tract obstruction, also termed "airlock." Rate of air entry is also important; it is estimated that 300-500 mL of gas introduced at a rate of 100 mL/s can be acutely fatal. This rate can be attained through a 14-gauge catheter with a pressure gradient of only 5 cm H2O. Foam in the venous line suggests the diagnosis of air embolism. A "millwheel" continuous cardiac murmur may be present. Treating air embolism includes clamping the venous line and stopping the blood pump immediately. In case of a crack in the hub or in the catheter, it is imperative to clamp proximally to the site of the air leak.

Left shoulder pain in PD patient. Cause?

left shoulder pain in this patient is referred pain from subdiaphragmatic irritation by air instilled inadvertently into the peritoneal cavity during peritoneal dialysis exchange Air can enter the peritoneum if the dialysate bag and inflow tubing are not properly flushed prior to instillation of fluid. Intra-abdominal free air is demonstrated on the chest x-ray as a crescent-shaped air-density lucency seen just underneath the left hemidiaphragm. In contrast, a normal gastric air bubble would typically appear as a round or ovoid air density rather than crescent shaped. Air trapped in this position can cause referred pain to the shoulder via irritation of the phrenic nerve. During PD training, patients are instructed to flush the dialysate inflow line to clear residual air prior to connecting the line to the peritoneal dialysis catheter. If this step is not completed, air enters the peritoneal cavity and often tracks superiorly to interpose between the liver and diaphragm, although in this case, it accumulated under the left hemidiaphragm. The pain usually gradually resolves over a week's time. The condition is benign, and no further evaluation or management is required, although it may be important to review instillation technique with the patient to prevent future episodes.

Cocaine is a potent vasoconstrictor. Patients abusing cocaine can present with severe hypertension and AKI related to rhabdomyolysis and ATN. ANCA-associated vasculitis with AKI has also been identified in patients using cocaine contaminated with ___. In these cases, active urine sediment and systemic features of vasculitis have been present.

levamisole

This disease is characterized by early onset gout and progressive tubulointerstitial nephritis, leading to ESRD in 80% of affected individuals by the fifth or sixth decades. Dx?

mutation in UMOD, leading to uromodulin kidney disease, a subtype of autosomal dominant tubulointerstitial disease (ADTKD) (previously known as familial juvenile hyperuricemic nephropathy and medullary cystic kidney disease, type 2) UMOD encodes uromodulin, also known as Tamm-Horsfall protein. In affected individuals, uromodulin accumulates in the tubular epithelium leading to cell atrophy and loss. There are no specific therapies for this disease. Instead, therapy is directed at limiting the hyperuricemia and recurrent episodes of gout. This disease is one of several disorders recently reclassified as autosomal dominant tubulointerstitial kidney disease.

Nephrogenic DI (nDI) is a common renal manifestation of ___ use.

nDI is a common renal manifestation of lithium use and can occur after only weeks of treatment. Nephrogenic DI may resolve if lithium is stopped early in the course, but the defect can be permanent. Chronic lithium exposure can also cause a chronic tubulointerstitial disease. Discontinuation of lithium may prevent further damage to the kidneys, but severe kidney disease may progress despite cessation. Amiloride, which impairs lithium uptake by the epithelial sodium channel (ENaC) of the principal cell in the cortical collecting duct, reduces lithium absorption in the kidney and theoretically may protect against lithium-induced kidney damage, but this agent has no effect after lithium is discontinued.

Criteria to diagnose peritonitis in peritoneal HD?

need 2 or more of the following 1. Consistent clinical features (abdominal pain or cloudy effluent) 2. Peritoneal WBC count > 100 cc/mm3 (or 0.1x10^9/L after dwell time of 2 hours) and percentage of neutrophils > 50% 3. Positive effluent culture

Patients undergoing PD who receive any antibiotic should receive concurrent antifungal prophylaxis to prevent fungal peritonitis. Candida is the most common species responsible for fungal peritonitis, which typically necessitates urgent removal of the PD catheter for successful eradication. Prophylaxis can be provided with ___ or ___ every 48 hours during antibiotic treatment and a few days afterward.

nystatin 500,000 units three times daily fluconazole 200 mg Although nystatin requires more frequent dosing, some fungal species have developed fluconazole resistance, so nystatin is preferred by some experts.

Patient with hyperphosphatemia associated with refractory secondary hyperparathyroidism (SHPT) should undergo ___ , in accordance with KDIGO 2017 guidelines.

parathyroidectomy (PTX) PTX should be considered for patients with persistent hypercalcemia or hyperphosphatemia despite optimized medical therapy, as well as those with symptomatic complications such as renal osteodystrophy, calciphylaxis, erythropoiesis-stimulating agent resistance, or severe vascular calcification

Define resistant hypertension.

persistent hypertension despite treatment with 3 different classes of antihypertensive medications including an optimally dosed diuretic agent * diagnosis of resistant HTN requires confirmation of medication adherence and proper BP measurement technique as well as either 24-hour ambulatory or home BP monitoring to exclude white coat HTN

Initial treatment for tachycardia, palpitations, hypertensive urgency, headache, tachypnea and diaphoresis causes by pheochromocytoma.

phenoxybenzamine, a nonselective a-adrenergic receptor blocker

Hypokalemic PP is a rare (prevalence, 1 in 100,000) autosomal dominant defect of calcium or, less commonly, sodium channels in skeletal muscle. Men are more often affected. Hypokalemic PP tends to present before age 20 years and is precipitated by stress, exercise, and carbohydrate loading. Treatment includes avoiding these triggers and taking ___.

potassium-sparing diuretics, carbonic anhydrase inhibitors, or both Hypokalemic periodic paralysis (PP) is a rare inherited neuromuscular disorder, related to a defect in muscle ion channels, and manifest by episodes of painless muscle weakness that may be triggered by rest after vigorous exercise, stress, or a high-carbohydrate meal.

Despite reasons to be cautious about choosing PD, the patient's __ is the most important factor in planning for his initial dialysis modality as he approaches ESRD.

preference

In kidney transplant patients, the median gestational age at delivery is 35 weeks with up to half of all pregnancies resulting in ___.

premature birth

One of the most commonly reported observations is the appearance of green urine after ___ administration; this is due to the presence of phenolic green metabolites in the urine.

propofol

A patient with a small renal mass who is a poor surgical candidate should undergo ___.

radiofrequency ablation (RFA) with biopsy SRMs are suspicious solid or cystic lesions seen on either high-quality CT or MRI performed with and without contrast that measure <4 cm and are limited to the kidney (stage T1a). RFA is most effective for SRMs <3 cm It is associated with an increased risk for local recurrence, so post-intervention imaging is necessary, with repeat RFA for persistent or recurrent disease.A biopsy of the mass can be obtained at the time of the procedure and may be helpful, because the histology can guide prognosis and inform the need for additional therapy or surveillance.

Severe hypokalemia (serum K <2.5 mEq/L) is associated with significant muscle weakness and ___. Hypokalemia can cause a wide range of arrhythmias.

rhabdomyolysis

Renal biopsy finding: pigmented casts and associated tubular injury would be expected in pigment nephropathy consequent to ___ , ___, and ____.

rhabdomyolysis, hemolysis, and bilirubin

The first step in managing an enlarging AVF aneurysm is to ensure that needle access is rotated along the fistula using the ___ technique.

rope ladder technique repeated venipuncture of the same site can cause weakness of the vessel wall and lead to or worsen aneurysmal dilation signs of imminent aneurysmal rupture include rapidly increasing size, thinning of overlying ski, shiny and unable to be pinched by the examiner

The majority of patients with ___ from cisplatin present with polyuria, orthostatic hypotension, and inappropriately elevated urine sodium concentration and fractional excretion of sodium.

salt wasting Salt wasting after cisplatin exposure has been reported in up to 10% of patients treated with this agent.

Canagliflozin is a ___that reduces blood glucose levels by lowering the renal threshold for glucose reabsorption and promotes urinary glucose excretion. Early transient reductions in eGFR have been noted with canagliflozin; these changes generally stabilize or attenuate over time and reverse after discontinuation, suggesting the cause is hemodynamic rather than structural glomerular or tubular damage. However, in 2016, the US FDA strengthened the existing warning regarding the risk of AKI for ___ after receiving notification of >100 confirmed cases of AKI associated with canagliflozin or dapagliflozin use, including some that required hospitalization and dialysis. Treatment for AKI due to canagliflozin?

sodium-glucose cotransporter-2 (SGLT-2) inhibitor In most of these cases, discontinuation of canagliflozin resulted in improvement or recovery of kidney function. Many of the patients who developed AKI were also being treated with blockers of the renin-angiotensin system, diuretics, or non-steroidal anti-inflammatory agents. changes in kidney function associated with canagliflozin manifested within 4-12 weeks

The best treatment for a patient with hyponatremia with low solute intake and high fluid intake is to increase her ___.

solute intake with protein supplements This syndrome occurs when solute intake is so low that water excretion cannot meet intake, even with maximal dilution of the urine. In this syndrome, carbohydrate intake is usually sufficient to suppress catabolism of endogenous protein so that there is insufficient endogenous urea. When this syndrome occurs in individuals who consume large quantities of beer but almost no other food, it referred to as beer drinker's potomania; when related to dietary restriction, it has been referred to as "tea and toast" hyponatremia

Causes of AVF maturation failure?

stenosis of the inflow artery, stenosis at the arteriovenous anastomosis, stenosis of the outflow vein, or the presence of large accessory veins

Experts recommend ___ prior to transplantation for patients with moderate or severe symptoms or PTH >800 pg/mL despite adequate medical therapy

subtotal parathyroidectomy After transplantation, parathyroidectomy is still the preferred treatment. Although higher doses are used in parathyroid carcinoma or when severe hypercalcemia complicates primary hyperparathyroidism, cinacalcet has not yet been approved by the US Food and Drug Administration for use in patients with renal transplant, and doses >180 mg daily are currently not recommended for secondary hyperparathyroidism.

Treatment HUS

supportive care should receive supportive care for AKI secondary to thrombotic microangiopathy, most likely representing hemolytic uremic syndrome (HUS) secondary to Shiga toxin-producing Escherichia coli (STEC) infection of the colon. The prodrome of bloody diarrhea followed by such symptoms as fatigue, nausea, bruising, and decreased urine output are typical of STEC HUS, as are the urinary findings.

Patient with peritonitis fluid growing gram positive cocci chains, gram negative rods, and gram positive rods with acute abdomen (guarding, rebound tenderness) what's the best next step?

surgical evaluation evidence of polymicrobial peritonitis, which should raise suspicion for secondary peritonitis from a perforated abdominal viscus -> requires urgent surgical evaluation. Peritonitis in patients receiving peritoneal dialysis is typically acquired by contamination of the catheter or its exit site and usually consists of monomicrobial skin flora such as Staphylococcus or Streptococcus species. Patients may be febrile but rarely demonstrate signs of septic shock. Both polymicrobial infection and septic shock should increase suspicion for intra-abdominal pathology, such as diverticulitis with perforation.

African American patients without CKD initial therapy should be ___ for HTN?

thiazide diuretic or CCB This recommendation is based on results from a subgroup analysis of African American individuals in the ALLHAT study that demonstrated fewer cerebrovascular events, less heart failure, and a reduction in combined cardiovascular outcomes with a thiazide-type diuretic versus an ACE inhibitor. A calcium channel blocker was equally effective in this trial for all end points except heart failure.

A ____ is the most appropriate initial treatment for stone prevention in this patient with calcium stones and hypercalciuria.

thiazide diuretic, such as hydrochlorothiazide The hypocalciuric effect of thiazide diuretics has been demonstrated to lower calcium stone recurrence in patients with hypercalciuria.

Acute calcineurin inhibitor (CNI) ___ can develop in response to drug interactions from CYP3A4 inhibitors, including antifungal azoles, non-dihydropyridine calcium channel blockers, and macrolide antibiotics, which result increased CNI concentrations.

toxicity

Measurement of the ___ level in the dialysate effluent is the most effective method to establish the diagnosis of chyloperitoneum.

triglyceride Opaque, milky discoloration suggests chyloperitoneum, which occurs when lymphatic drainage from the gut is obstructed or interrupted Management is aimed at the underlying condition, but a low-fat diet can decrease lymphatic flow, providing a useful adjunct to therapy, particularly when trauma is implicated Computed tomography is an important step to diagnose contributory anatomic abnormalities once the diagnosis of chyloperitoneum is confirmed, but triglyceride determination of the effluent would be the initial step.

A patient with lower urinary tract dysfunction due to spinal cord injury should undergo ___ . Lower urinary tract dysfunction is a heterogeneous entity whose presentation depends on the level of spinal cord injury. Injuries occurring between the infrapontine and suprasacral regions of the spinal cord result in both detrusor overactivity as well as loss of the coordinated activation of the detrusor and inhibition of the urethral sphincter. Detrusor-sphincter dyssynergia may lead to elevated bladder pressures and vesicoureteral reflux due to bladder contractions against a closed sphincter, which poses a substantially higher risk of progressive CKD. Despite therapy with clean intermittent catheterization and an anti-muscarinic agent, this patient has developed CKD; therefore, ___ are indicated for guidance regarding additional treatment.

urodynamic testing

Checking ___ would be helpful in suspected cases of pseudohyperkalemia, including severe leukocytosis or thrombocytosis, or simply mechanical disruption of RBCs during phlebotomy.

whole blood or plasma potassium In patients with severe leukocytosis or thrombocytosis, cellular elements may degrade after phlebotomy but before laboratory analysis, causing a spurious elevation in potassium determination. In such cases, measuring whole blood or plasma potassium in a heparinized tube will yield a normal value. Fist clenching and tourniquet use during phlebotomy, vigorous aspiration of blood via a small-gauge needle, and pneumatic tube transport of blood samples can all result in mechanical disruption of blood cells in vitro and lead to pseudohyperkalemia ("hemolyzed sample").

__ deficiency is more common in HD patients than in healthy controls. Signs and symptoms include acrodermatitis enteropathica, decreased taste and smell, and increased susceptibility to infections.

zinc

___ amyloidosis is usually seen in patients who have been on dialysis at least 5 years.

β2-Microglobulin-associated This disorder involves osteoarticular sites, and patients may present with carpal tunnel syndrome or shoulder pain. Bone cysts may be visible on x-ray.

Median neuropathy due to carpal tunnel syndrome may be related to ___ amyloidosis in dialysis patients.

β2-microglobulin amyloidosis - Production is stimulated by inflammation, and clearance is through glomerular filtration and catabolism by the proximal tubule β2-microglobulin may accumulate in dialysis patients because of limited clearance, especially with older, cellulose-based dialysis membranes, and possibly because of increased production. β2-microglobulin is capable of forming amyloid that has a predilection for deposition in bone and articular locations. The syndrome of dialysis-associated β2-microglobulin amyloidosis commonly includes CTS due to infiltration of the carpal synovium. Anterior shoulder involvement and infiltration of the flexor tendons of the hands is also common. Additional features can include bone pain, pathologic fractures, and spinal cord compression due to vertebral involvement. Visceral organ involvement does not occur. Long-term dialysis patients are at greatest risk. The incidence of β2-microglobulin amyloidosis appears to be declining, perhaps because current biocompatible, high-flux dialyzers have better clearance of β2-microglobulin and less propensity to stimulate its production compared with older, cellulose-based membranes. Optimal treatment consists of surgical release and debridement of the synovium. Recurrence is common.