PT V Exam 1

Hemophilia has a ____________________________mode of inheritance meaning female carriers do not express disease but can pass it on to the next male generation.

"Skipped generation"

How can referring syndrome be prevented in PN?

"Start low and go slow" Initially providing < 50% of caloric requirements Gradually increase to goal over 3-5 days Correct electrolyte abnormalities before PN initiated Minimize fluid and Na+ during the first few days of PN - Fluid ≤ 1000 mL/day - Sodium ≤ 20 mEq/day Supplement thiamine and folic acid

How can reseeding syndrome be prevented when giving enteral nutrition?

"Start low and go slow" Initially providing < 50% of caloric requirements Gradually increase to goal over 3-5 days Correct electrolyte abnormalities before initiation Minimize fluid and Na+ during the first few days - Fluid ≤ 1000 mL/day - Sodium ≤ 20 mEq/day Supplement thiamine and folic acid

What drug interactions can occur with Phentermine and Topiramate (Qsymia)?

- Alcohol or CNS depressants (ex: benzodiazepines, sleep medications): CNS depression - Non-potassium sparing diuretics: hypokalemia - Carbonic anhydrase inhibitor (ex: zonisamide, acetazolamide, dichlorphenamide): Increases metabolic acidosis and kidney stones (avoid concomitant use) - Phenytoin, carbamazepine: Decreases topiramate - Valproic acid: hyperammonemia with and without encephalopathy, hypothermia (with and without hyperammonemia) - Decreases pioglitazone

Initial evaluation of anemia includes which tests? - *Results of initial evaluation determine need if other tests are needed.

- CBC (including RBC indices) - Reticulocyte index - Examination of a stool sample for occult blood

What is CI with Orlistat (Xenical)?

- Chronic malabsorption syndrome - Cholestasis (reduced bile formation or flow) - Pregnancy

Which prescription medications are not recommended for routine use for decreasing appetite? •Short-term approved medications but NOT recommended(AACE guidelines)

- Dextroamphetamine (Dexedrine®) - Phentermine (see next slides) - Diethylpropion (Tenuate®) - Phendimetrazine (Bontril®) - Methamphetamine (Desoxyn®)

What are the three major mechanisms of acquired aplastic anemia?

- Direct toxicity - Metabolite-driven toxicity - Immune-mediated mechanisms

Which hormones decrease eating?

- Leptin - Insulin - GLP-1 - Norepinephrine alpha 1 and beta 2 - Serotonin 5-HT1B and 5-HT2C - Histamine H1 and H3 - Alpha melanocyte-stimulating hormone

Which hormones stimulate eating?

- Neuropeptide Y (most potent) - Ghrelin - Orexin - Melanocyte-concentrating hormone - Dopamine - Serotonin 5-HT1A - Norepinephrine alpha 2

What patient should avoid taking Orlistat (Alli)?

- Organ transplant - Taking cyclosporine - Problems with absorbing food - Overweight

What is CI with Bupropion and Naltrexone (Contrave)?

- Uncontrolled HTN - Seizure disorders; anorexia nervosa or bulimia; or undergoing abrupt D/C of alcohol, benzodiazepines, barbiturates, antiepileptics. - Using other bupropion-containing products - Chronic opioid use (naltrexone: opioid antagonist) - opioid withdrawal effects. - During or within 14 days of taking MAOI. - Pregnancy.

What are all of the drugs associated with causing Drug-Induced Megaloblastic Anemia?

-Azathioprine -Cotrimoxazole -Hydroxyurea -Phenytoin -Primidone -Phenobarbital -Methotrexate

What drugs are most frequently associated with drug-induced thrombocytopenia?

-Carbamazepine -Eptifibatide -Heparin (Heparin-Induced Thrombocytopenia - HIT) -Ibuprofen -Quinine -Quinidine -Oxaliplatin -Rifampin -Vancomycin -Sulfamethoxazole-trimethoprim

Solid organ transplantation is lifesaving treatment for different disease states including end-stage...

-Cardiac -Kidney -Liver -Lung -Intestinal disease

What are cytolytic agents(e.g. ATG) for acute rejection reserved for?

-Corticosteroid-resistant rejection -Signs of hemodynamic compromise (heart) -More severe rejections

Process of premature RBC destruction referred as hemolysis and can occur because either...

-Defective RBCs -Abnormal changes in intravascular environment -Drugs can promote hemolysis by both processes

What are the 2 proposed mechanisms for drug induced Agranulocytosis?

-Direct toxicity -Immune-mediated toxicity

What are the symptoms associated with Drug-Induced Hemolytic Anemia?

-Fatigue -Malaise -Pallor -SOB

Immunosuppression must be balanced to optimize:

-Graft survival -Patient survival

What are the goals of treating acquired aplastic anemia?

-Improve peripheral blood counts -Limit requirement for transfusions -Minimize risk for infections

What are the goals of acute rejection therapy?

-Minimize intensity of the immune response -Prevent irreversible injury to the allograft

What innovative forms of therapy for persistent or intractable rejection are being investigated? - Acute rejection

-Mycophenolate mofetil -Tacrolimus -Low-dose methotrexate -Sirolimus -Cyclophosphamide -Total lymphoid irradiation -Plasmapheresis and intravenous immunoglobulin

If high doses of immunosuppressants are used to prevent rejection fo a long term, it may lead to what serious complications? - Need rapid dosage reductions to reduce side effects(!)

-Nephrotoxicity -Infection -Thrombocytopenia -Drug-induced diabetes

Which medications are associated with causing agranulocytosis?

-Propylthiouracil -Methimazole -Ticlopidine -Clozapine -Penicillin derivatives

Describe the typical presentation of Agranulocytosis?

-Symptoms may appear immediately or insidiously. -Rare reaction. -Typically presents with fever. -Symptoms arise from increased infection risk associated with due to lack of WBCs.

What patient-specific factors are included in individualizing maintenance therapy?

-Transplant organ and type (cadaveric vs living-donor). -Degree of HLA mismatch. -Time after transplantation. -Posttransplantation complications: number of acute rejections, previous immunosuppressive adverse reactions, compliance, financial considerations.

What prophylactic agents are added to minimize side effects associated with intensive immunosuppression regimens for acute rejection?

-Valganciclovir -Nystatin -Trimethoprim-sulfamethoxazole -H2-receptor antagonists -Proton-pump inhibitors -Antacids

What is the normal protein requirement per day?

0.8-1 (gm/kg/day)

What is the recommended amounts of lipids for PN?

1 gm/kg per day not to exceed 30% of total daily calories. May use < 2.5 gm/kg per day or 60% of total daily calories. **Assess triglycerides first.

What dose of folic acid is used for treating folic acid deficiency anemia?

1 mg daily usually sufficient to replace stores If deficiency due to malabsorption might need doses of 1 to 5 mg daily Parenteral folic acid is available - Rarely needed

For each unit of factor IX infused per kilogram of actual body weight results in about a __% rise in the plasma level of factor IX

1%

The gene for vWF is located on chromosome___. Transcription and translation produce a large primary product that subsequently undergoes complex modifications, resulting in vWFmultimers of various.

12

When giving a hemophilia A patient factor VIII infusion generally it is recommended to administer 50% of the initial dose about every ___ hours to miantain desired factor VIII level.

12

Bupropion and Naltrexone (Contrave) should be D/C if patient does NOT obtain 5% baseline body weight loss after_______________ of maintenance dose

12 weeks

How long do RBCs normally survive for? - Removed by phagocytotic cells of spleen and liver.

120 days

What are in the 2-in-1 and the 3-in-1 admixtures for parenteral nutrition?

2-in-1: - Dextrose - Protein ***Lipids separate 3-in-1: (Total nutrient admixture (TNA)) - Dextrose - Protein - Lipids

Explain the continuous administration method for enteral nutrition.

24 hours a day Stomach - Usually better tolerated than intermittent bolus feeding - Once tolerance established, transition to intermittent bolus method Small intestine - Preferred method - Started at 10-40 mL/hour and advanced to goal rate by 10-20 mL/hour every 8-12 hours as tolerated

What is normal plasma osmolality?

275-290 mOsm/kg

Replacement therapy with plasma-derived vWF-containing products treatment of choice for patients with which types for Von Willebrand Disease?

2B 2M 3 - 1 or 2A vWD UNresponsive to desmopressin!

Each gram of dextrose in PN provides _____ kcal

3.4 kcal

Incidence of new factor VIII inhibitors in patients with severe hemophilia A factor VIII deficiency is about ____%

30% Inhibitors less common in patients with mild or moderate hemophilia A: occur in about 5% to 10% of patients. Risk of developing inhibitors in patients with hemophilia B is much lower: occur in about 3% of patients.

What are the daily fluid requirements for adults?

30-35 mL/kg 1500 mL/m2

Malnutrition occurs in ________% of hospitalized patients.

30-55%

How many mOsm/kg or mmol/kg is considered iso-osmolar?

300 mOsm/kg or mmol/kg

In PN, each gram of protein provides __ kcal

4 kcal

Phentermine (Adipex-P, Lomaira) should be D/C if weight loss not occurred in ___________ or when develop tolerance

4 weeks

What is the formula for BMI?

703 * weights(lbs)/height(in)^2

What therapeutic options can be included in acute rejection therapy?

A. Increasing doses of current immunosuppressive drugs B. Starting "pulse" corticosteroids with subsequent dosage taper C. Addition of another immunosuppressant indefinitely D. Short-term treatment with polyclonal or monoclonal antibody

What are the Antithymocyte Immune Globulins (Antibodies) that are used to prevent rejection? - Depletion of T-lymphocytes

ATG - Equine anti-thymocyte globulin RATG (Thymoglobulin) - Rabbit anti-thymocyte globulin - Less immunogenic(!)

Which electrolytes should be adjusted as needed for acid-base balance in PN?

Acetate and Chloride

What are the goals of Chronic Transfusion Therapy?

Achieve and maintain an HbS concentration of <30% of total Hgb hemoglobin in the primary and secondary prevention of neurologic complications

How is acute pain associated with hemophilia managed? - Acute and chronic pain common in patients with hemophilia. - Cause of acute pain is bleeding

Acute Pain: - Treatment should include factor replacement to stop the bleeding. - PRICE (Protect, Rest, Ice, Compression, and Elevation) - Acetaminophen used for mild pain, - Narcotic analgesia may be required for more severe pain.

Other than chronic and hyperacute rejection, what other types of rejection can occur?

Acute cellular rejection (ACR) Antibody-mediated rejection (AMR)

What complications are associated with Acute Sickle Cell Disease?

Acute chest syndrome Acute splenic sequestration Aplastic crisis Overt stroke Vasoocclusive pain episodes

What are the indications for acute blood transfusions? - Episodic Transfusions for Acute Complications

Acute exacerbation of baseline anemia (e.g. hepatic or splenic sequestration, or severe hemolysis). ACS, stroke, intrahepatic cholestasis, or acute multisystem organ failure. Preparation for procedures which requires use of general anesthesia.

What is ATG indicated for? - Equine anti-thymocyte globulin

Adjunct to delay onset of first rejection - Management of allograft rejection in renal transplant patients

How is hemophilia A treated?

Administration of factor VIII products - Plasma Derived Factor VIII - Recombinant Factor VIII - Extended Half-Life (EHL) Factor VIII Most patients treated with high-purity products: - Recommended as first-line agents - Have the lowest risk of transmitting infectious disease Recombinant products, if available, preferred rather than plasma-derived products.

What factors guide advancement of PN to macronutrient goal?

Advance to macronutrient goal based upon patient response, laboratory monitoring, and clinical course.

What are the advantages and disadvantages of the 2-in-1 solution mixture for parenteral nutrition?

Advantages - Longer stability (1-2 months) - Easier visual inspection Disadvantages - Increased nursing time - Requires two sets of tubing - Increased bacterial growth in lipids

What are the advantages and disadvantages(!) of a Gastrostomy for enteral feeding?

Advantages •Allows for all methods of administration •Low-profile buttons available •Large-bore tubes less likely to clog •Multiple tubes and sizes Disadvantages •Potential increased risk of aspiration •Risk of stoma site complications

What are the advantages and disadvantages of a Jejunostomy for enteral feeding?

Advantages •Allows for early post-injury or post-operative feeding •Potential reduced risk of aspiration •Low-profile buttons •Multiple tubes and sizes Disadvantages(!) •Bolus or intermittent feeding not tolerated •Risk of stoma site complications

What are the advantages and disadvantages of Nasogastric or Orogastric access?

Advantages •Ease of placement •Allows for all methods of administration •Inexpensive •Multiple tubes and sizes Disadvantages •Potential - Tube displacement - Increased risk of aspiration

What are the advantages and disadvantages(!) of Nasoduodenal or Nasojejunal access?

Advantages •Potential reduced aspiration risk •Allows for early post-injury or post-operative feeding •Multiple tubes and sizes Disadvantages(!!!) •Potential tube displacement or clogging •Bolus or intermittent feeding not tolerated

What are the advantages and disadvantages to using umbilical cord blood for an Allogeneic Hematopoietic Stem Cell Transplantation?

Advantages over marrow donors include: - Lower incidence of severe GVHD - Larger donor pool from which to select donors Disadvantages include: - Longer time to engraftment - Higher rate of graft rejection

What are the advantages and disadvantages of the 3-in-1 solution mixture for parenteral nutrition?

Advantages: - Time-efficient for nursing - Single bag with single tubing - Decreased vein irritation - Inhibited bacterial growth - One pump required - Ease of home administration Disadvantages: - Shorter stability (1-2 days) - Complex compounding - Visual inspection is difficult - Emulsion instability - Limited compatibility with medications - Catheter occlusion more common - Larger filter required

What ADRs can occur with Phentermine and Topiramate (Qsymia)?

Adverse reactions: paresthesia, dizziness, dysgeusia, insomnia, constipation, dry mouth, decreased sweating, metabolic acidosis (hyperventilation, fatigue, anorexia, cardiac arrhythmias, stupor), kidney stones, hypokalemia - check potassium before/during treatment, others mentioned on next slide. Increase fluid intake: increase urine output to reduce kidney stones

What are the possible causes of an increased thrombin time (TT)? Clinical manifestations: - Lifelong hemorrhagic disease - Variable clinical symptoms from asymptomatic to either a bleeding diathesis or prothrombotic.

Afibrinogenemia Dysfibrinogenemia Heparin therapy

When treating acute rejection it is almost always starts with "pulse" corticosteroid therapy for several days (PO or IV)(!). However what race may not respond as well to corticosteroids after a kidney transplant and what should they use instead?

African Americans Anti-thymocyte globulin (ATG) preferred (!!)

What people have increased risk of B12 deficiency?

Age Use of gastric acid-suppressing agents (may inhibit cobalamin release from food)

What are all of the factors that alter the requirements for micronutrients?

Age Gender Route of administration Diarrhea Vomiting Fistulas Wound healing Hyper-metabolism/catabolism Burns Severity of malnutrition Organ dysfunction

What are all of the factors that nutrition requirements depend on?

Age Gender Size Disease state Nutrition status Clinical condition Organ dysfunction Physical activity

What condition is defined as decrease in number of mature myeloid cell in blood (granulocytes and immature granulocytes - bands) to a total count of 500 cells/mm3 or less? - Older patients at greater risk due to increased medication us - More common in women than in men!

Agranulocytosis

What drugs can decrease iron absorption?

Al-, Mg-, and Ca2+-containing antacids Tetracycline and doxycycline Histamine-2 antagonists Proton-pump inhibitors Cholestyramine

What protein in the blood maintains colloid oncotic pressure? Binds and transports hormones, anions, drugs, and fatty acids Negative acute phase reactant Insensitive index of malnutrition - Large amount in the body - Highly distributed in extravascular compartment - Long half-life of 18-20 days - Caloric deficiency - Liver, kidney, GI disease

Albumin

What agent is approved for use in B-cell chronic lymphocytic leukemia? -Targets CD52 surface antigen found on T and B lymphocytes, macrophages, monocytes, eosinophils, natural killer cells. -Effects on depleting both T and B lymphocytes make it useful in solid organ transplants. -Not FDA approved for solid organ transplantation. -Increasingly recognized as therapeutic option for induction or treatment of acute rejection.

Alemtuzumab

How is Heparin-Induced Thrombocytopenia treated? Goal: reduce risk of thrombosis or thrombosis-associated complications

All forms of heparin must be discontinued (e.g. heparin flushes) Start alternative anticoagulants: Direct thrombin inhibitors are alternative anticoagulants most commonly used.

What complications are associated with plasma-derived factor replacement?

Allergic reactions Fever Chills Urticaria Nausea PCCs and aPCCs have potential to cause thromboembolic complications. Antifibrinolytic agents should not be given to patients who are receiving PCCs or aPCCs in order to avoid thrombotic complications. Porcine factor VIII which is used for treatment of patients with inhibitors to factor VIII not known to transmit human viruses. Can cause allergic-type reactions (e.g. fever, chills, skin rashes, nausea, and headaches) - Treat with steroids and/or diphenhydramine. Can cause thrombocytopenia.

What is currently the only therapy that can cure patients with SCD?

Allogeneic hematopoietic stem cell transplantation (HSCT) - Optimal candidates for matched sibling donor transplant include SCD patients with severe disease (HbSS and HbSB°) prior to onset of SCD symptoms, stroke, elevated TCD velocity, recurrent ACS, recurrent pain, red cell alloimmunization on chronic transfusion protocol, pulmonary hypertension, sickle nephropathy.

What risks are associated with transfusions?

Alloimmunization (sensitization to the blood received) Hyperviscosity Transfusion transmitted viral infections Volume overload Iron overload Nonhemolytic transfusion reactions

Rejection mainly mediated by activation of what?

Alloreactive - T cells - Antigen-presenting cells (APCs) (e.g. B lymphocytes, macrophages) **Acute allograft rejection mainly caused by infiltration of T cells into the allograft - leads to inflammatory and cytotoxic effects on graft

What are the 2 currently available Antifibrinolytic therapy options for hemophilia A?

Aminocaproic acid Tranexamic acid

Drug-nutrient Interactions can occur with the following drugs? Antacids Antibiotics Aspirin Loop diuretics Methotrexate Proton pump inhibitors

Antacids - Thiamine deficiency Antibiotics - Vitamin K deficiency Aspirin - Folic acid deficiency + Increased vitamin C excretion Loop diuretics - Thiamine deficiency Methotrexate - Folic acid inhibits effect Proton pump inhibitors - Vitamin B12 deficiency

During a Hyperacute Rejection, tissue damage can be mediated through what processes?

Antibody-dependent cytotoxicity Cell-mediated cytotoxicity Activation of complement cascade

What is the duration of time where only short-term access is required for enteral access? •Easier to place •Less invasive •Lower complication rates •Lower costs

Anticipated enteral feeding duration of less than 4-6 weeks

What anticipated length of time of enteral access is considered long-term. •More invasive •Higher costs

Anticipated enteral feeding duration of more than 4-6 weeks

What medications are associated with weight gain?

Anticonvulsants: carbamazepine, gabapentin, pregabalin, valproic acid Antidepressants: mirtazapine, tricyclic antidepressants Atypical antipsychotics: clozapine, olanzapine, quetiapine, risperidone Conventional antipsychotics: haloperidol Hormones: corticosteroids, insulin, medroxyprogesterone, hormonal contraceptives, sulfonylureas, pioglitazone, rosiglitazone, insulin

What agents for vWD are used for treatment of bleeds associated with tissues rich in plasminogen activators, such as mouth, especially with tooth extractions? - Used in the management of epistaxis, GI bleeding, and menorrhagia. - Should be avoided in urinary tract bleeding due to risk of thrombosis and obstruction.

Antifibrinolytic agents (e.g. aminocaproic acid and tranexamic acid)

What type of therapy is used for hemophilia A treatment that inhibits clot lysis? - Useful adjunctive therapy for treatment of hemophilia.

Antifibrinolytic therapy

Due to pharmacological action on DNA replication, what class of chemotherapeutic agents is most frequently associated with drug-induced megaloblastic anemia?

Antimetabolite class Methotrexate (MTX): Irreversible inhibitor of dihydrofolate reductase. - Dihydrofolate reductase, an enzyme responsible for generating tetrahydrofolate essential factor in making deoxythymidine triphosphate necessary for DNA synthesis.

What alternative anticoagulants are FDA approved for treating heparin induced thrombocytopenia?

Argatroban - Preferred in renal insufficiency - Metabolized in liver Bivalirudin - Preferred if hepatic impairment - Renally metabolized and requires dosage adjustments if renal impairment

Which amino acid modulates immune function, inflammation, and response to sepsis? •Supplementation is controversial

Arginine

How should hyperglycemia from PN be assessed and managed?

Assess chemsticks and sliding scale insulin use - 0.1 unit of insulin per gram of dextrose - Give 1/2 to 2/3 of insulin requirement since new PN initiated Usually adjust in 5-10 unit increments Advance dextrose based on chemsticks and insulin usage.

How often should patients being treated for weight loss receive behavioral modification? Group or individual sessions Determine safe lifestyle change that keeps weight loss sustained Techniques - Monitor weight and exercise - Use of tools (pedometer, food scale) - Determine weight loss barriers Combined with diet and exercise

At least one or two times a month with professional or leader (another source: at least 14 sessions in 6 months with trained professional)

What are the benefits of initiating enteral nutrition early instead of delayed? - First 24-72 hours

Attenuates stress response Reduces disease severity and infectious complications Decreases release of inflammatory cytokines Fewer alterations in gut permeability

Which patients should avoid bolus enteral administration?

Avoid in patients with delayed gastric emptying and those at risk of aspiration

What anti-rejection agent is a prodrug for 6-mercaptopurine (6-MP)? - Metabolized to 3 different compounds - Active = 6-thioguanine nucleotides (6-TGNs) - Xanthine oxidase converts 6-MP and 6-TGNs to inactive compounds. Incorporated into nucleic acids via de novo& salvage pathways - Reduce lymphocyte proliferation

Azathioprine

What drugs can cause folic acid deficiency (directly inhibit DNA synthesis)?

Azathioprine 6-mercaptopurine 5-fluorouracil Hydroxyurea Zidovudine

What should be monitored at baseline when starting PN?

Basic metabolic panel Mg2+, PO4- Liver function tests Lipid panel (triglyceride) CBC with differential PT, PTT Capillary glucose (QID) Weight Intake and output (Daily)

What anti-rejection agent is a non depleting antibody that is a chimeric monoclonal antibody? - Bind IL-2 receptor on the surface of activated T-lymphocytes - IL-2 can't bind à No T-cell proliferation

Basiliximab (Simulect®)

What foods are high in folic acid?

Beef liver Fortified cereals Lentils Green leafy vegetables Orange juice Rice

What immunosuppressive agent is a Costimulatory Signal Inhibitor that os the only drug currently approved in this class of immunosuppressive agents? - May replace calcineurin inhibitors in the majority of immunosuppressive regimens -Derived from abatacept(!!)

Belatacept

Describe how macrolides work to prevent rejection?

Bind to FKBP12 - complex binds mTOR - blocks activation & proliferation of B & T lymphocytes by stopping progression of cell cycle. - Inhibit T-cell response to IL-2.

What is a BIA? - Electric current applied to wrist and ankle or both feet - Resistance to electrical conductivity measured

Bioelectrial Impedance Analysis (BIA)

What herbal supplement is used in combination with caffeine for weight loss? - Has synephrine (similar structure to epinephrine) and octopamine (similar to norepinephrine). - Serious adverse effects (ex: HTN, CV toxicity, MI, stroke, seizure)

Bitter orange

How do the corticosteroids work to prevent rejection?

Block cytokine activation - Inhibit IL-1,2,3,6, γ-interferon, TNF-α - Interfere with cell migration, cell recognition, and cytotoxic effector mechanisms.

What are the causes of normocytic anemia?

Blood loss Chronic disease

Which enteral nutrition administration method is described here? •Delivered by gravity or feeding pump over approximately 15 minutes

Bolus

What ADRs can occur with hydroxyurea?

Bone marrow suppression: Neutropenia Thrombocytopenia Anemia Decreased reticulocyte count

What agent is a proteosomal inhibitor used in multiple myeloma? - Works against B-cells

Bortezomib

Which weight-loss medication is described here? Prescription - Adjunct to reduced-calorie diet and increased exercise for chronic management of weight in adults with BMI > 30 kg/m2 or > 27 kg/m2 with at least 1 weight-related comorbidity (ex: HTN, Type 2 DM, dyslipidemia) MOA unknown - (opioid antagonist) and (weak inhibitor of dopamine and norepinephrine neuronal reuptake) - _____________________ may affect 2 different areas of brain that regulate food intake: hypothalamus (appetite regulatory center) and mesolimbic dopamine circuit (reward system) Average weight loss at 1 year: 4.9 kg (10.8 lbs) May decrease seizure risk by following dose schedule

Bupropion and Naltrexone (Contrave)

What is the greatest stress factor that effects total daily expenditure?

Burns: Stress factor up to 2.

What disease can cause hypermagnesemia? - Mg > 2.4

CKD

What type of delivery system for enteral nutrition is described here? •Ready-to-hang •No modifications can be made •Decreased nursing time •Less risk of contamination

CLOSED delivery system

What two electrolytes can precipitate and cause a Fatal microvascular emboli? Factors associated with precipitation •Increased pH •Low amino acid concentration •Increased temperature •Order of mixing additives What levels prevent precipitation?

Ca2+ and Phos Low risk of precipitation: •Ca2+ ≤ 10 mEq/L •Phos ≤ 30 mmol/L

What agents are the central component in most maintenance regimens? _________________________-free immunosuppression remains future goal due to significant nephrotoxicity associated with these agents.

Calcineurin inhibitors

Which electrolyte regulates endocrine functions, blood coagulation, and bone and tooth metabolism? - Propagate of neuromuscular activity

Calcium

What is the Harris-Benedict equations for?

Calculates basal energy expenditure (BEE)

What is the DRI equation for?

Calculates estimated energy requirement (EER) - M: 662 - (9.53 x age) + (physical activity factor x 15.91 x wt{kg}) + (539.6 x ht{m}) - F: 354 - (6.91 x age) + (physical activity factor x 9.36 x wt{kg}) + (726 x ht{m}) Physical activity factor (PA) - Sedentary = 1 - Low active = 1.12 - Active = 1.27 - Very active = 1.45

How often should Desmopressin be administered?

Can be administered every 12 to 24 hours Response decreases with repeated treatment After 3-4 doses usually no longer effective Need alternative replacement therapy if prolonged treatment is needed(!!!)

What are the signs and symptoms associated with vitamin B12 deficiency?

Can cause neurologic and hematologic complications. Usually start with bilateral paraesthesia in extremities. Deficits in proprioception and vibration. If not treated can progress to ataxia, dementia-like symptoms, psychosis, and vision loss. In children can lead to poor brain development.

Which macronutrient is a major source of calories?

Carbohydrate

How many kcal/gm are in each of the following macronutrients for enteral feeding? - Carbohydrates - Protein - Fat

Carbohydrate - 3.4 kcal/gm Protein - 4 kcal/gm Fat - 9 - 10 kcal/gm

How are nutrient and fluid requirements are altered by chronic kidney disease?

Carbohydrate - Hyperglycemia and insulin resistance common. Fat - Changes in metabolism. Protein - Increased requirements with dialysis Fluid status - Often overhydrated - Depends on type of dialysis

What molecule is a quaternary amine that is required for transport of long-chain fatty acids and energy production?

Carnitine

What BBW is associated with Liraglutide (Saxenda)?

Causes thyroid C-cell tumors in rats and mice; not known if causes thyroid C-cell tumors (including medullary thyroid carcinoma) in humans.

What are the risk groups for Iron-Deficiency Anemia (IDA)?

Children <2 YO Adolescent girls Pregnant/lactating females >65 YO

What complication associated with PN is a liver complication that can progress to cirrhosis and liver failure? - Obstruction of bile secretion

Cholestasis

What tests can be done to diagnose hemophilia prenatally?

Chorionic villus sampling in gestational weeks 9 to 14 or Amniocentesis after 15 to 17 weeks of gestation

How can Sirolimus levels be measured? - These methods are not interchangeable (!!!) - Concentrations depend on the type of assay used(!) **If different assays are used in monitoring a single patient then dose of sirolimus might be adjusted improperly

Chromatographic or immunoassay methods.

What therapy is important in management of SCD? - In acute illness transfusions can be life-saving - _____________________________ can prevent serious complications of SCD Primary indication for __________________________: - Primary and secondary stroke prevention - Amelioration of organ damage

Chronic Transfusion Therapy

What factors can decrease Transferrin (TFN) values

Chronic infection Cirrhosis Burns Enteropathies Nephrotic syndrome Cortisone Testosterone

What complications can develop later in life as a result of sickle cell disease? Acute complications can be: - Unpredictable - Rapidly progressive - Life threatening

Chronic organ damage Cognitive or emotional impairment

What factors can decrease Prealbumin values?

Cirrhosis Hepatitis Stress Surgery Inflammation Hyperthyroidism Cystic fibrosis Burns Kidney dysfunction Zinc deficiency

When diagnosing a coagulation disorder, _____________________ should determine if there is a family history of bleeding or known bleeding disorders.

Clinical history Diagnosis based on: - Detailed clinical history - Physical examination - Laboratory tests

How is Von Willebrand Disease typically presented?

Clinical manifestations are variable some patients are asymptomatic Signs and Symptoms: - Mucocutaneous bleeding: epistaxis, gingival bleeding with minor manipulation, menorrhagia. - Easy bruising - Postoperative bleeding

What proteins in the blood circulate as inactive precursors (zymogens)? - Activation leads to cascading series of proteolytic reactions. - At each step, a _______________________ undergoes limited proteolysis and becomes an active protease. - Designated by a lowercase a

Coagulation Factors

Bleeding disorders can be a result of what main defects?

Coagulation factor defect Quantitative platelet defect Qualitative platelet defect Enhanced fibrinolytic activity

What is the ADR Probability Scale (Algorithm)?

Common tool employed by clinicians to rate the likelihood of causality in ADRs investigations E.g. Naranjo scale - Series of scored questions which can lead investigator to likelihood that an ADR was caused by a specific suspected medication Rated as - Doubtful - Possible - Probable - Definite

What is the physiology of a chronic transplant rejection?

Complex interactions between allograft and cellular cytokines, cell-to-cell interactions, CD4+ and CD8+ T cells, and B cells. Ultimately lead to graft loss if adequate immunosuppression is not maintained. Major cause of graft loss.

What all is involved with the coagulation system?

Complex interactions between: - Vascular endothelium - Platelets - Procoagulant proteins - Anticoagulant proteins - Fibrinolytic proteins

What are the physiologic consequences of liver transplantations?

Complex physiologic consequences with changes in: -Metabolic function -Synthetic function Many abnormalities: -Fluid -Electrolyte -Nutritional Biliary tract dysfunction may alter absorption of: -Fats -Fat-soluble drugs

How is nutrition assessed?

Comprehensive medical, surgical, and dietary history. Nutrition focused history and physical exam (NFPE) to identify factors that predispose to malnutrition.

Describe the mechanism that causes Metabolic Drug-Induced Hemolytic Anemia

Considered to be oxidative(!) Most often occur in presence of a glucose-6-phosphate dehydrogenase (G6PD) deficiency G6PD deficiency: disorder of the hexose monophosphate shunt responsible for producing nicotinamide adenine dinucleotide phosphate hydrogen (NADPH) in RBCs (keeps glutathione in reduced state) Reduced glutathione: substrate for glutathione peroxidase, enzyme that removes peroxide from RBCs and protecting them from oxidative stress. Without reduced glutathione, oxidative drugs can oxidize the sulfhydryl groups of hemoglobin, removing them prematurely from the circulation (e.g. causing hemolysis).

What is CI with Phentermine and Topiramate (Qsymia)?

Contraindications - Pregnancy - Glaucoma - Hyperthyroidism - During or within 14 days of MAOI (hypertensive crisis) Avoid: severe liver disease or end-stage renal disease

What is CI with Belatacept?

Contraindications: Recipients who are EBV seronegative or with unknown EBV serostatus. Consider PTLD in patient reporting new or worsening neurological, cognitive, or behavioral signs or symptoms. - Another risk factor for PTLD is CMV infection - CMV prophylaxis is recommended for at least 3 months after transplantation(!!)

What are the goals with parenteral nutrition?

Correct - Caloric nitrogen imbalances - Fluid and electrolyte abnormalities - Known vitamin or trace element abnormalities - Lessen metabolic response to injury - Minimize oxidant stress - Modulate immune response

Why do we need to have a formula for corrected calcium and what is the formula?

Correct calcium based on albumin. - Normal albumin: 3.6-5 gm/dL Corrected Ca2+= current Ca2 + 0.8(4 - current albumin)

What is the MOA of Belatacept?

Costimulatory Signal Inhibitor. Mammalian soluble fusion protein Selective T-cell co-stimulation blocker - Binds to CD80 and CD86 on antigen-presenting cells - Blocks CD28 mediated co-stimulation of T lymphocytes

How is Drug-Induced Megaloblastic Anemia caused by Cotrimoxazole treated?

Course of folinic acid 5-10mg up to QID

What agent historically was the mainstay of therapy for vWD? - Contains about 80 to 100 units of vWFper unit (5-10X more vWF and factor VIII than fresh-frozen plasma). - Since _______________________ is not virally inactivated so it should not be used as first-line treatment.

Cryoprecipitate

Which enteral nutrition administration method is described here? •Less than 24 hours a day •Typically overnight

Cyclical

How is chronic pain associated with hemophilia treated? - Secondary to hemophilic arthropathy

Cyclooxygenase-2 inhibitors can also be helpful in managing chronic pain. Surgical interventions may help to alleviate chronic pain. More advanced joint disease can benefit from joint replacement.

What agents can be used to treat the occurrence of inhibitors when treating hemophilia?

Cyclophosphamide IV immune globulin Rituximab

What specific ADRs can occur with each calcineurin inhibitor?

Cyclosporine •Acne •Gingival hyperplasia •Hirsutism Tacrolimus •Alopecia •Diarrhea, nausea •Decreased Mg++ •Pruritis

Name the calcineurin inhibitors and where they bind.

Cyclosporine (Sandimmune®, Gengraf®, Neoral®) Tacrolimus/FK506 (Prograf®) Drug binds to immunophilin in cytoplasm - Cyclosporine = cyclophilin - Tacrolimus = FK-binding protein 12 *Inhibits action of calcineurin *Without calcineurin, no transcription of key cytokines (IL-2) needed for T-cell activity

What drug interactions can occur with Orlistat (Xenical)

Cyclosporine (reduced cyclosporine levels) - take cyclosporine 3 hours after orlistat -measure cyclosporine levels more frequently Warfarin: orlistat may reduce vitamin K absorption - INR (monitor INR) Levothyroxine: hypothyroidism occurs - monitor thyroid & separate levothyroxine & orlistat by at least 4 hours Oral contraceptives: severe diarrhea - need back up birth control if taking oral contraceptive (since decreased absorption) Decreased fat absorption - may affect absorption lipophilic meds (ex: gabapentin, amiodarone, valproic acid, lamotrigine) Decreased antiretroviral medication effectiveness (ex: atazanavir, ritonavir, emtricitabine)

What does cyclosporine depend on for absorption? - Standard/USP formulation: Sandimmune - Modified/microemulsion: Gengraf, Neoral

Cyclosporine depends on enterohepatic recirculation/bile for absorption(!!) - Metabolized by CYP3A4

What are the contraindications to the different calcineurin inhibitor injections?

Cyclosporine injection - Hypersensitivity to Cremophor® EL (polyoxyethylated castor oil) Tacrolimus injection - Hypersensitivity to HCO-60 (polyoxyl 60 hydrogenated castor oil)

What drugs are associated with causing metabolic Hemolytic Anemia

Dapsone, rasburicase, ascorbic acid, metformin, nitrofurantoin

What mTOR can be added to cyclosporine to decrease the dose of it to prevent renal toxicity?

Decrease dose of cyclosporine with Everolimus(!) to decrease renal dysfunction. - Increased risk of kidney arterial and venous thrombosis resulting in graft loss with everolimus.

What are the goals of treatment for sickle cell disease?

Decrease hospitalizations Decrease complications Decrease mortality Improve quality of life

How should PN be tapered when discontinuing?

Decrease the rate by ½ and run for a few hours then discontinue. Decrease: - Macronutrients by a percentage - Rate Check CS ½ to 1 hour after discontinuation

_____________________ (Increased/Decreased) skeletal muscle function is an indicator of malnutrition. Tests - Hand-grip strength - Respiratory muscle strength - Muscle response to electrical stimulation

Decreased

Laboratory testing can distinguish bleeding disorders caused by... 1. 2. 3.

Defects in coagulation pathways Fibrinolytic pathways Alterations in the number or function of platelets

What are the signs and symptoms of a Carnitine deficiency, and what conditions can cause a Carnitine deficiency?

Deficiency predisposing factors - Chronic kidney or liver disease - Chronic use of valproic acid and zidovudine - Vegetarian diet Signs/symptoms of deficiency - Skeletal muscle weakness - Fatty liver - Fasting hypoglycemia

What factors can caused an increased Albumin measurement?

Dehydration Anabolic steroids Insulin Infection

What are the possible consequences of malnutrition?

Delayed wound healing Prolonged hospitalization Increased - Rate of rehospitalization - Healthcare costs

Explain what intermittent enteral administration is.

Delivered via feeding container or bag over 30-45 minutes with or without enteral feeding pump. More consistent physiologically with normal eating patters.

What are the symptoms of fatty acid deficiency?

Dermatitis Alopecia Impaired wound healing Growth failure Thrombocytopenia Anemia

What agent for vWD stimulates endothelial cell release of vWF and factor VIII? Temporarily effective for patients with vWDwho have adequate endogenous stores of functional vWF including: - Patients with type 1 disease and some patients with type 2A disease - Not used for treatment of patients with type 3 disease who do not have stores of vWF

Desmopressin

What is nutrition screen used for?

Detect those who are overweight, obese, malnourished, or at risk for malnutrition Predict outcome of nutritional factors Provide an indication as to whether nutritional treatment will influence outcomes

What is the rate of administration determined by for PN?

Determined based on volume contributed by dextrose, protein, electrolytes, multivitamins and trace elements.

What is the most serious complication of factor replacement therapy? - Associated with considerable morbidity and decreased quality of life - Neutralizing antibodies to factors VIII and IX - Develop in a subset of patients with hemophilia

Development of inhibitors

What macronutrient for PN is a carbohydrate that is the primary energy source in PN? - Minimum of 50 grams in PN for stability

Dextrose

How is minimum rate calculated for PN?

Dextrose: - 70 gm/100 mL = # gm/x1 mL - x1 mL Protein: - 100 gm/1000 mL = # gm/x2 mL - x2 mL 200-300 mL for electrolytes, multivitamins, trace elements, and other agents - x1 mL + x2 mL + 200-300 mL = total volume (mL) Total volume (mL)/24 hr = minimum rate (mL/hr)

What is diagnosis of Drug-Induced Aplastic Anemia based on and how can it be classified?

Diagnosis based on bone marrow aspirate and biopsy to exclude other causes of pancytopenia. Classified based on WBCs, platelets, Hgb, and reticulocyte count as: - Moderate aplastic anemia - Severe aplastic anemia - Very severe aplastic anemia

What all is the diagnosis of drug-induced immune hemolytic anemia based on and what 2 categories can drug induced hemolytic anemia be divided into?

Diagnosis based on: - Direct Coombs test or - Direct antiglobulin test [DAT]) - Identifies foreign immunoglobulins either in serum or on RBCs Can be divided into two categories - Immune - Metabolic (e.g. oxidative)

What are the different caloric restriction options for non-pharmacologic treatment of obesity?

Diet: deficit of ≥ 500 kcalories/day Low calorie diet (LCD): deficit of 500-750 kcal/day - 1200-1500 kcal/day (females) and 1500-1800 kcal/day (males) - Weight loss: 1-2 lbs/week (6-month weight loss average: 8%) Very low caloric diet (VLCD): < 800 calories/day - under PCP supervision - Not recommended - Usually given as liquid - Multivitamin and multimineral needed if consume < 1200 calories/day for long time - Quicker weight loss but no better than LCD in long-term(!) Short-Term Fasting and Time-Restricted Eating - Many different methods - Randomized, controlled trials needed

What can be used to treat Iron-Deficiency Anemia?

Dietary supplementation Oral iron preparations

Which weight-loss med is described here? - Prescription - Short-term (few weeks) weight loss with BMI ≥ 30 kg/m2 or ≥ 27 kg/m2 with risk factor (ex: HTN, DM, dyslipidemia) - Used in conjunction with diet, exercise, behavior modification MOA: increase NE Some adverse effects: - Insomnia, nervousness - Palpitations, tachycardia, BP - Pulmonary HTN - Valvular heart disease - Blurred vision - Mydriasis (dilated pupil) - Psychosis •Baseline cardiac evaluation for preexisting valvular heart disease, pulmonary hypertension; EKG during therapy; monitor BP, weight, weight circumference

Diethylpropion (Tenuate)

What tests are used to diagnose Von Willebrand disease? - Initial and specific tests

Different laboratory tests used in the diagnosis of this hemostatic abnormality. Initial screening tests include: - Determinations of PT - Activated partial thromboplastin time (aPTT) - Platelet Specific laboratory tests for the diagnosis of vWD include measurement of: - vWF antigen (vWF:Ag) level - Factor VIII assay - Determination of vWF ristocetin cofactor (vWF:RCo) activity and vWF multimer analysis.

What types of agents can be used for vWF replacement?

Different virus-inactivated, intermediate- or high-purity plasma-derived factor VIII concentrates have sufficient amounts of functional vWF(!!) - Plasma Derived Factor VIII - Recombinant Factor VIII - Extended Half-Life (EHL) Factor VIII - Plasma Derived Factor VIII + vWF - Recombinant Factor vWF **Ultrahigh-purity (monoclonal antibody-derived) plasma-derived products contain only negligible amounts of vWF and recombinant factor VIII products contain no vWF so they are not used for treatment of vWD.

Where exactly is waist circumference measured?

Distance around the smallest area below the rib cage and the top of the iliac crest.

What DDIs can occur with Bupropion and Naltrexone (Contrave)?

Do NOT co-administer CYP 2B6 inducers (ex: ritonavir, lopinavir, efavirenz, phenytoin, phenobarbital, carbamazepine): Decrease bupropion effects. NOT more than one tablet BID when taking CYP2B6 inhibitor (ex: ticlopidine or clopidogrel): bupropion Bupropion inhibits CYP2D6 and can increase antidepressants (ex: SSRIs, many tricyclics), antipsychotics (ex: haloperidol, risperidone, thioridazine), beta-blockers (ex: metoprolol), Type 1C antiarrhythmics (ex: propafenone, flecainide): consider decreased dose if used with Contrave Use with dopaminergic medications (levodopa, amantadine): CNS toxicity Decreased digoxin Not get complete benefit from opioid medicines (ex: cough/cold, antidiarrheal, opioid analgesics) ® if need intermittent opiate - D/C CONTRAVE and do NOT increase opiate above standard dose

Describe the administration information for cyclosporine modified?

Do not remove capsules from original foil container until right before use. Can mix solution with milk or juice Should NOT be mixed in plastic or styrofoam container(!)

What formula can be used to estimate total dose of parenteral iron needed to correct anemia?

Dose of iron (mg) = whole blood hemoglobin deficit (g/dL) × body weight (lb) or Dose of iron (mg) = whole blood hemoglobin deficit (g/L) × body weight (kg)×0.22 Additional quantity of iron to replenish stores should be added(!!) - About 600 mg for women and 1,000 mg for men

What is a rare but serious form of anemia that has an unclear etiology? Suggests relationship between environment and risk Bimodal risk distribution with peak incidences: -10 - 25 YO and -> 60 YO Characterized by: Pancytopenia - Anemia - Neutropenia - Thrombocytopenia Hypocellular bone marrow. No gross evidence of increased peripheral blood cell destruction. Men and women equally affected

Drug-Induced Aplastic Anemia

What drug induced hematological disorder is described here? - Abnormal development of RBC precursors called megaloblasts in bone marrow - Deficiency in either vitamin B12 or folate responsible for impaired proliferation and maturation of hematopoietic cells - Result in cell arrest and subsequent sequestration

Drug-Induced Megaloblastic Anemia

What other assessment tools can be used to assess malnutrition?

Dual-energy X-ray absorptiometry (DXA) Magnetic resonance imaging (MRI) and computed tomography (CT) Neutron activation Isotope dilution Underwater weighing

What is CI with Phentermine (Adipex-P, Lomaira)

During or within 14 days following use of MAOI (ex: phenelzine/Nardil) (!!!!) - hypertensive crisis Glaucoma (due to mydriasis) Agitated Hyperthyroidism History of drug abuse Cardiovascular disease (ex: arrhythmias, heart failure, CAD, stroke, uncontrolled hypertension) Pregnancy Breast-feeding

What is CI with Diethylpropion(Tenuate) and when should it be D/C?

During or within 14 days following use of MAOI (ex: phenelzine/Nardil) - hypertensive crisis) (!!) Also: - Advanced arteriosclerosis - Severe hypertension - Pulmonary hypertension - Hyperthyroidism - Glaucoma (due to mydriasis) - Agitated - History of drug abuse - Concurrent use with other anorectic medications •D/C if weight loss not occurred in 4 weeks or if develop tolerance

What are the signs and symptoms associated with hemophilia?

Ecchymoses (palpable/raised) Hemarthroses (especially knee, ankle, and elbow) Joint pain/Joint swelling and erythema Decreased range of motion Muscle hemorrhage Swelling at the site of muscle bleeding Pain with motion of affected muscle Signs of nerve compression Excessive bleeding with surgery

What factors can cause an overestimation of body fat with BMI?

Edema, a lot of muscle, wasting of muscle, status of hydration, shortened stature, tumor that is large.

What laboratory tests are able to quantify vWF:Ag?

Electroimmunoassay Immunoradiometric assay Enzyme-linked immunosorbent assay (ELISA)

How are electrolyte levels and vitamins altered by Short Bowel Syndrome?

Electrolytes - Hypomagnesemia - Hypokalemia - Hypocalcemia - Hypochloremia - Metabolic acidosis Trace elements - Zinc, copper, selenium, and manganese deficiency Vitamins - Vitamin B12 deficiency - Deficiencies in vitamins A, D, E, and K

How are electrolyte levels altered by a chronic kidney disease?

Electrolytes - Hyponatremia - Hyper/hypokalemia - Hyper/hypophosphatemia - Metabolic acidosis Trace elements - Zinc redistribution - Increased chromium levels - Selenium losses during dialysis

What micronutrients are important an a normal diet?

Electrolytes and minerals Trace elements Vitamins

What BBWs are associated with Bupropion and Naltrexone (Contrave)?

Elevated risk of suicidal thinking and behavior in children, young adults, adolescents taking antidepressants for major depressive disorders and other psychiatric disorders. Monitor for worsening and starting of suicidal thoughts and behaviors. Not studied in pediatric patients.

What precautions and warnings are associated with Phentermine and Topiramate (Qsymia)?

Embryo-Fetal toxicity: harmful to fetus - obtain negative pregnancy test before treatment and monthly, use effective contraception. Increase heart rate: monitor heart rate Suicidal behavior and ideation: monitor depression or suicidal thoughts - D/C if have symptoms Acute myopia and secondary angle closure glaucoma: d/c medication Mood and sleep disorders: reduce dose or withdrawal for clinically significant/ persistent symptoms Cognitive impairment: attention or memory problems - caution operating automobiles or hazardous machinery Metabolic acidosis: measure electrolytes before and during therapy (including bicarbonate) Elevated Scr: measure Scr before and during therapy Diabetes medications: loss of weight loss may result in hypoglycemia - measure glucose before and during therapy

What nutrient and fluid requirements are altered by an Acute Kidney Injury (AKI)?

Energy - Requirements influenced by comorbid illness and type of renal replacement therapy. Carbohydrate - Hyperglycemia and insulin resistance common Fat - Changes in metabolism Protein - Breakdown and urea accumulation - Increased requirements with dialysis Fluid status - Depends on residual urine output and type of dialysis

How are nutrient and fluid requirements altered by hepatic failure?

Energy - Variability in expenditure Carbohydrate - Cirrhosis - hyperglycemia is common - Fulminant hepatitis - prone to hypoglycemia Fat - Increased triglyceride and free fatty acid concentrations - Risk of essential fatty acid deficiency in severe failure Protein - No change in nitrogen requirements Fluid status - Overhydrated

How are nutrient and fluid requirements effected by pulmonary failure?

Energy - Variable Carbohydrate - Abundance may led to increased respiratory demand Fat - Lowest respiratory quotient - May decrease oxygenation in acute respiratory distress syndrome (ARDS) Protein - Influences ventilatory demand Fluid status - Avoid

How are nutrient and fluid requirements effected by Short Bowel Syndrome?

Energy - Variable Carbohydrate - Malabsorption Fat - Malabsorption Protein - Well tolerated Fluid status - Imbalances

What type of nutrition is defined as the delivery of nutrients by tube or mouth into a functioning gastointestinal (GI) tract? - Medical food Includes - Oral supplements - Tube feeds Goal •Provide calories, macronutrients, and micronutrients to those who are unable to achieve nutritional requirements from an oral diet

Enteral Nutrition (EN)

What herbal weight loss supplement is a stimulant banned by FDA (nervousness, headache, increases BP and HR, MI, stroke, death)

Ephedra

Describe how the dose of factor IX concentrate replacement is calculated? - Factor IX: small protein - Not limited to intravascular space (!) - Passes into extravascular compartment (!) - Vd about twice that of factor VIII

Equation can be used to calculate the initial dose: - Plasma-derived Unknown node type: a (units) = (Desired level - Baseline level) × (Weight [in kilograms]) - Baseline level can be omitted from the formula if it is negligible compared to the desired level

__________________________________________ content determines quality of protein composition. - Molecular form determines amount of digestion

Essential amino acid

What are all of the Dietary Reference Intakes (DRIs) that are determined for Nutrition Requirements?

Estimated Average Requirements (EARs) Recommended Dietary Allowances (RDAs) Adequate Intakes (AI) Tolerable Upper Intake Levels (ULs)

What is the MOA of hydroxyurea?

Exact mechanism of HbF production is unknown Proposed that hydroxyurea and its myelosuppressive effect: - Stimulates stress erythropoiesis - Triggers rapid erythroid regeneration - Shifts erythrocyte hemoglobin production to HbF

What coagulation factors are assessed with a prothrombin time?

Factors I, II, V, VII, X

What coagulation factors are assessed with an Activated partial thromboplastin time (aPTT)?

Factors I, II, V, VIII, IX, X

T or F: Phentermine and Topiramate (Qsymia) does NOT have a REMS program yet.

False, Controlled substance (schedule IV), REMS program (training programs). - Only available in Qsymia-certified retail pharmacies or Qsymia Home Delivery Network.

T or F: Drug-induced thrombocytopenia only results from immune-mediated mechanisms.

False, Drug-induced thrombocytopenia can result from: -Immune-mediated mechanisms -Nonimmune-mediated mechanisms

T or F: Re-challenging patients with suspected agent to confirm diagnosis a method for determining drug-induced blood disorders.

False, Re-challenging patients with suspected agent to confirm diagnosis NOT recommended! - In vitro studies with offending agents and cells or plasma from patient's blood performed to identify cause(!)

T or F: The incidence of hemophilia is affected by race.

False, incidence of hemophilia NOT affected by race. About one-third of patients have negative family history - Presumably representing spontaneous mutation

T or F: Sickle-cell anemia occurs most often in Asian Americans.

False, it is most common in people with African heritage! - Affects millions of people worldwide - Estimated 300 million people worldwide are carriers RBCs carrying abnormal sickle Hgb prevent normal growth and development of Plasmodium falciparum within RBCs so individuals with SCT are more likely to survive acute malarial illness

T or F: Pharmacologic treatment options for obesity can be used alone for treatment.

False, only used in conjunction with lifestyle modifications.

What are the risk factors for obesity?

Family history of obesity Certain medical diagnosis - Polycystic ovary syndrome, Prader-Willi syndrome, Cushing's syndrome. Poor dietary habits Inadequate exercise Drug therapies

What deficiency may occur with 1-3 weeks of fat-free PN formulation Signs and symptoms •Excessive thirst •Frequent urination •Rough, dry or scaly skin •Dull or "lifeless" hair •Dandruff •Soft, brittle nails

Fatty Acid Deficiency Prevention •Supply 2-4% of total calories as lipid

What mechanical complications can occur with enteral feeding?

Feeding tube Occlusion - Accumulation of formula sediment - Improper medication administration - Kinking of tube - Flushing technique Malposition

What is the recommended adult feeding weight for a critically ill obese (BMI > 30) person.

Feeding weight - 11-14 kcal/kg ABW - 22-25 kcal/kg IBW

Name all of the oral iron products available and how much elemental iron is in each.

Ferrous sulfate (20% elemental iron) Ferrous sulfate (exsiccated) (30% elemental iron) Ferrous gluconate (12% elemental iron) Ferrous fumarate (33% elemental iron)

What type of hemoglobin is present predominantly in fetal RBCs? - Tetramer of two α-globin chains and two γ-globin chains (α2γ2) Around 32-week gestation: - Switch from production of γ chains to β chains - Increase in HbA production

Fetal hemoglobin (HbF)

What factors can increase fluid requirements?

Fever Radiant warmers Diuretics Vomiting Nasogastric suction Ostomy/fistula output Diarrhea Glycosuria Phototherapy Diabetes Insipidus Increased temperature Hyperventilation Prematurity Excessive sweating Increased metabolism

How should an infection and fever be treated if it occurs due to an Allogeneic Hematopoietic Stem Cell Transplantation?

Fever should be considered medical emergency Need rapid administration of IV antibiotics due to risk of overwhelming sepsis Ceftriaxone should be used for outpatient management If cephalosporin allergy can use clindamycin For fever control use acetaminophen or ibuprofen

What nutrient included in enteral feeding is described here? •In the form of soy polysaccharides •Trophic effects on colon •Energy source •Regulates bowel function •Promotes Na+ and H20 absorption •Soluble versus insoluble

Fiber

What nutrient is necessary for the maintenance of normal bowel habits? - Lower blood pressure and cholesterol - Prevent colon cancer - Promote weight loss

Fiber

What coagulation factors are assessed with a thrombin time (TT)?

Fibrinogen Inhibitors of fibrin aggregation

What occurs during the first 24-72 hours of starvation during reseeding syndrome, and then what happens > 72 hours after.

First 24-72 hours •Liver uses glycogen stores for energy and skeletal muscle to provide amino acids as a source for new glucose > 72 hours •Metabolic pathways shift to derive energy from ketones as result of free fatty acid oxidation •Protein mobilization from skeletal muscle is spared •Decrease in liver gluconeogenesis •Decline in metabolic rate •Decrease of insulin secretion •Increased use of free fatty acids

What factors can decrease fluid requirements?

Fluid overload Cardiac failure Decreased urinary output Heat shields High humidity Humidified air via endotracheal tube Kidney failure Hypoalbuminemia Syndrome of inappropriate secretion of antidiuretic hormone (SIADH)

When should the enteral feeding tube be flushed when giving medications?

Flush feeding tube before and after administration Multiple medications - Administer separately with flush in-between

What type of anemia is one of the most common vitamin deficiencies in the U.S.? Association with: - Excessive alcohol intake - Pregnancy Major causes: - Inadequate intake - Decreased absorption - Increased folate requirements

Folic Acid Deficiency Anemia

How is Drug-Induced Megaloblastic Anemia caused by Phenytoin or phenobarbital treated?

Folic acid 1 mg QD - Some clinicians suggest that folic acid supplementation can decrease effectiveness of antiepileptic medications.

What foods are high in iron?

Foods that are high in iron: animal liver, fortified cereals/oatmeal, beef, eggs, spinach, lentils, tofu, and beans Iron best absorbed from meat, fish, poultry Milk and tea decrease absorption - Consume in moderation

How should Acute Sickle Cell Pain be treated?

For vasoocclusive (painful) episodes mainstays of treatment: - Hydration - Analgesia

What foods are high in B12?

Fortified cereals, Fish Animal liver Milk Clams Yogurt

What are the physiologic consequences of kidney transplantations?

GFR may be near normal almost immediately after transplantation. Concentration of standard biochemical indicators of kidney function (e.g. SCr, BUN) may remain elevated for several days.

What ADRs can occur with Mycophenolic Acid?

GI- abdominal pain, N/V/D Infections Malignancy/lymphoma Neutropenia, anemia & thrombocytopenia - Monitor CBC weekly during 1st month, twice monthly 2nd & 3rd months, then monthly through 1st year Pure Red Cell Aplasia

What are the instances when either gastric or small bowel access is preferred for enteral nutrition?

Gastric - Intact gag and cough reflexes - Functional stomach - No delay of gastric emptying, obstruction, or fistula Small bowel - Appropriate for gastric outlet obstruction, gastroparesis, pancreatitis, and known reflux and aspiration of gastric contents. - Early postoperative feeding after major abdominal procedure planned.

What route of enteral access is used for a long term duration in patients with the following characteristics? Patient characteristics •Normal gastric emptying

Gastrostomy

What possible treatment that is being developed offers potential of a long-term and possibly life- long correction of vWF deficiency? - Candidates for _________________ include patients with most severe bleeding phenotypes of vWD (type 3 and some severe cases of types 1 and 2). - Preclinical trials are being conducted to test feasibility of gene transfer in the management of vWD

Gene therapy

What are the physiologic consequences of lung transplantations?

Generally, more complications than other solid organ transplant recipients Primary graft dysfunction: noncardiogenic pulmonary edema Airway complications include: -Ischemia and associated anastomotic dehiscence -Bronchial stenosis -Bronchiolitis obliterans

What is an essential AA that is a precursor for nicotinamide adenine dinucleotide (NAD+) synthesis that is used for sickle cell? Data shows increased uptake of __________________ by sickle RBCs, especially to produce NAD+. Children with SCD have lower ___________________ levels Increase of NAD+ can potentially restore redox balance in oxidative stressed cells

Glutamine

Which amino acid is described here? •Fuel source for rapidly dividing cells •Skeletal muscle is the primary site of production •Potential for depletion during periods of prolonged critical illness •Favorable outcomes demonstrated for critically ill patients' enteral feeds when supplemented

Glutamine

What are the 2 CONDITIONALLY essential amino acids? - Need increases beyond body's synthetic ability during periods of high physiologic stress.

Glutamine Arginine

What is the goal of systemic treatment for Von Willebrand Disease?

Goal of systemic therapy is to correct platelet adhesion and coagulation defects by stimulating the release of endogenous vWF or by administering products that contain vWF and factor VIII or vWF alone. - Used for bleeding that cannot be controlled by local measures and - For the prevention of bleeding with surgery

What are the goals of treating a vitamin B12 deficiency and what does treatment typically include?

Goals of treatment: - Reversal of hematologic manifestations - Replacement of body stores of vitamin B12 - Prevention or resolution of neurologic manifestations Treatment incudes: - Replacement therapy - Correction of any underlying etiology that is treatable

Normal adult RBCs contain predominantly which type of hemoglobin?

HbA

How does HbF help treat sickle cell disease?

HbF reduces polymer formation of HbS due to its high-oxygen affinity. Higher HbF levels associated with decreased RBC: - Sickling - RBCs adhesion

How should the patient be positioned when they are receiving enteral nutrition?

Head-of-bed (HOB) elevation of 30-45º to prevent aspiration and pneumonia, unless otherwise specified or contraindicated.

When do heart failure patients typically receive a heart transplant?

Heart transplant candidates typically patients with New York Heart Association class III or IV signs and symptoms despite maximal medical management. and Have an expected 1-year mortality risk of 50% or greater without transplant.

How are plasma-derived factor concentrates virally inactivated?

Heat treatment Chemicals (solvent detergent) Affinity chromatography with monoclonal antibodies

When RBCs are destroyed (in the spleen), which portion of the hemoglobin molecule gets converted into bilirubin?

Heme

What does The Who classify as anemia? - Anemia: group of diseases characterized by decrease in either Hb or circulating red blood cells (RBCs) àLeads to reduced oxygen-carrying capacity of the blood.

Hemoglobin (Hb) <13 g/dL in men Hemoglobin (Hb) <12 g/dL in women

What is a bleeding disorder which results from a congenital deficiency in a plasma coagulation protein? What are the different types of this disorder as well? - Affects about 400,000 males worldwide

Hemophilia Hemophilia A (classic hemophilia) Hemophilia B (Christmas disease)

What are the different factors affected in each type of hemophilia?

Hemophilia A (classic hemophilia): caused by deficiency of factor VIII Hemophilia B (Christmas disease): caused by a deficiency of factor IX - Hemophilia A is about 1 in 5,000 male births\ - Hemophilia B occurs in 1 in 30,000 male births

Describe the genetic inheritance of hemophilia?

Hemophilia A and hemophilia B: - Recessive X-linked diseases - Defective gene is located on the X chromosome - Affects only males - Females are carriers

Could hemophilia be treated with gene therapy in the future? - Why or why not?

Hemophilia is a candidate for gene therapy because tight control of gene expression is not required. Low levels of factor expression can reduce bleeding episodes in patients with severe hemophilia - Similar to rationale for prophylactic factor replacement. Gene therapy for treatment of hemophilia is in early clinical stages.

What term is used to describe the stopping of a bleed at the site of vascular injury? - There is formation of impermeable platelet and fibrin plug.

Hemostasis

What type of thrombocytopenia is described here? Thrombosis can occur in up to 50% of patients with ______. High risk of thrombosis continues for days to weeks after heparin discontinuation and platelet recovery. Need continued anticoagulation with alternative agent. Recovery starts within 1 to 2 days of discontinuation of offending agent and completes at one week. Antibodies may persist for years and need to advise patients to avoid drug indefinitely.

Heparin-Induced Thrombocytopenia

Describe sickle cell disease (SCD).

Heterozygous inheritance of HbS with another qualitative or quantitative β-globin mutation results in: - Sickle cell hemoglobin C (HbSC) - Sickle cell β-thalassemia (HbSβ+-thaland HbSβ0-thal) - Some other rare phenotypes Chronic illness

What is sickle cell trait defined as and how is it typically presented?

Heterozygous inheritance of one normal β-globin gene producing hemoglobin A (HbA) and one sickle gene producing HbS (HbAS). - Asymptomatic

What are all of the laboratory tests can be used to determine anemia?

Hgb, Hct, and RBC: May stay normal early in disease and later decrease as the anemia progresses Serum iron: Low in IDA and AI Ferritin levels: Low in IDA and normal or elevated in AI Total iron-binding capacity: High in IDA and is low or normal in AI Mean cell volume: Elevated in vitamin B12 deficiency and folate deficiency Vitamin B12 and folate levels: Low in their respective types of anemia Homocysteine: Elevated in vitamin B12 deficiency and folate deficiency Methylmalonic acid: Elevated in vitamin B12 deficiency

Describe induction therapy for preventing rejection?

High level of immunosuppression at time of transplantation With or without immediate start of cyclosporine or tacrolimus Two perioperative immunosuppressive strategies used: 1. Highly intense immunosuppression usually based on patient-specific risk factors. 2. Use of antibody therapy to provide enough immunosuppression to delay initiation of therapy with the potentially nephrotoxic calcineurin inhibitors

How are the corticosteroids dosed for preventing rejection?

High-dose IV methylprednisolone (250 - 1,000 mg) given peri-operatively or during acute rejection. - Rapidly tapered and discontinued. Oral prednisone started - Given once daily in the AM with food. - Eventually tapered to lowest possible dose, some centers discontinue completely.

What all should be assessed during a nutritional assessment?

Histories - Medical, nutrition, medication Physical examination Anthropometric measurements - Height, weight (current, usual, ideal), body mass index (BMI) Laboratory data - Albumin, pre-albumin

Patients with what triglyceride levels should not receive lipids in PN?

Hold for triglycerides > 400 mg/dL

What agent for sickle cell anemia is a chemotherapeutic agent that stimulates HbF production? - Increases number of HbF-containing reticulocytes and intracellular HbF. - Inhibits DNA synthesis by blocking the conversion of ribonucleoside to deoxyribonucleotides FDA approved >2 YO with recurrent moderate-to-severe painful crises to decrease frequency of painful crises and need for blood transfusions

Hydroxyurea

What drugs can cause megaloblastic anemia?

Hydroxyurea Zidovudine Cytarabine Methotrexate Azathioprine 6-mercaptopurine Cladribine

How are electrolyte levels altered by an acute kidney injury?

Hyper/hyponatremia Hyper/hypokalemia Hyper/hypophosphatemia Hypermagnesemia Metabolic acidosis

What type of rejection is described here? -May be evident within minutes of transplantation procedure when donor-specific antibodies are present in recipient at time of transplant. -Can be induced by IgG antibodies that bind to antigens on the vascular endothelium (e.g. class I MHC, ABO, and vascular endothelial cell antigens.

Hyperacute Rejection

What are all of the metabolic complications that can occur with enteral nutrition?

Hyperglycemia Dehydration Electrolyte imbalance - Hypernatremia - Require ~ 1 mL of water for each calorie Refeeding syndrome

What side effects are associated with vitamin B12 supplementation?

Hyperuricemia Hypokalemia

What initiation strategy should be considered for starting PN in ICU critical care patients?

Hypocaloric dosing of ≤ 20 kcal/kg/day or 80% of estimated energy needs may be considered in appropriate patients over the first week. - High risk - Severely malnourished

How are electrolyte levels altered by hepatic failure?

Hyponatremia Hypokalemia Hypophosphatemia Hypomagnesemia

What are all of the clinical manifestations of refeeding syndrome?

Hypophosphatemia - Neurologic, cardiac, respiratory, hematologic abnormalities, death Hypokalemia - Impaired muscle contractions, respiratory compromise, rhabdomyolysis, cardiac abnormalities, death Hypomagnesemia - Weakness, muscle twitching, altered mental status, nausea, vomiting, diarrhea, cardiac abnormalities, death Na+ retention/Fluid shifts - Fluid overload, pulmonary edema, cardiac decompensation Vitamin deficiencies - Encephalopathy, lactic acidosis, death

How are electrolyte levels altered by pulmonary failure?

Hypophosphatemia Respiratory acidosis

Based on functional classification of anemia there are three major categories of anemia. What are they?

Hypoproliferative (Ex. Iron deficiency) Maturation disorders (Ex. Cytoplasmic defects) Hemorrhage/hemolysis

What patients should consider holding lipids for 7 days? And what are lipids in PN contraindicated with? (!!)

ICU patients - consider holding for at least 7 days(!) Contraindications - Severe egg allergy - Triglycerides > 400 mg/dL - Hold until level is normalized Pancreatitis is not a contraindication; however, use with caution - Triglycerides < 400 mg/dL but > 300 mg/dL and history of pancreatitis may warrant 50% of goal x 3-4 days.

Actual body weight can be compared to what to evaluate weight?

Ideal body weight (IBW)

What are the goals of nutrition assessment?

Identify those who are malnourished or at risk for malnutrition Determine risk for malnutrition-associated complications Estimate nutritional needs Establish baseline nutrition status Monitor nutrition therapy

Describe the mechanism that causes Immune Drug-Induced Hemolytic Anemia?

IgG, IgM or both bind to antigens on surface of RBCs and initiate their destruction through complement and mononuclear phagocytic system. - Can be either drug dependent or independent.

How is Immune hemolytic anemia treated?

Immediate removal of the offending agent and supportive care Glucocorticoids may be helpful for severe cases

What immunizations are recommended for sickle cell patients? - Routine immunizations very important in preventive care in managing SCD.

Impaired splenic function increases susceptibility to infection by encapsulated organisms especially S. pneumoniae. Risk of meningococcal disease higher in SCD - Vaccination recommended for individuals with functional or acquired asplenia. For prophylaxis against invasive pneumococcal infections penicillin prophylaxis until at least 5 YO recommended in children with SCD HbSS or HbSβ0-thal. Penicillin recommended even if received PCV13 or PPSV23 immunization - If allergic to penicillin can give erythromycin

What are the three major causes of vitamin B12 deficiency?

Inadequate intake Malabsorption syndromes Inadequate utilization

What immunizations should patients with hemophilia receive?

Including immunization against hepatitis B. Hepatitis A vaccine also recommended for patients with hemophilia due to risk of transmitting the causative agent through factor concentrates.

What laboratory finding indicates Drug-Induced Megaloblastic Anemia?

Increased Mean Corpuscular Hemoglobin (MCV) concentration on blood tests. - Megaloblastic changes caused by direct or indirect effects of the drug on DNA synthesis. Some patients can have normal-appearing cell line - Diagnosis must be made by measurement of vitamin B12 and folate concentration. Abnormality can be seen in any portion of replication process. - DNA assembly - Base precursor metabolism - RNA synthesis

What warnings are associated with Belatacept?

Increased risk for post-transplant lymphoproliferative disorder (PTLD) Use in liver transplant patients not recommended due to increased risk of graft loss and death Increased susceptibility to infection and malignancy - Limit exposure to UV light because of malignancy risk! - JC virus-associated progressive multifocal leukoencephalopathy (PML) - BK virus nephropathy Only experienced physicians and adequate facilities

What are the goals of treating folic acid deficiency?

Induce hematologic remission Replace body stores Resolve signs and symptoms - Treatment includes administration of exogenous folic acid

What are the 3 main phases of treatment for preventing transplant rejection?

Induction therapy Acute rejection Maintenance therapy

What other complications can occur with enteral feeding?

Infection Around exit site - Leaking and/or bleeding - Formation of excessive granulation tissue

Anemia can be a manifestation of patients' systemic disorders such as...

Infection Chronic renal disease Malignancy

Parenteral iron is used in patients with due to poor oral absorption caused by what conditions?

Inflammatory bowel disease (IBD) Gastric bypass/gastric resection Chronic kidney disease

What pharmacokinetic values of drugs can be altered by enteral feeding?

Influence •Bioavailability •Pharmacological effect •Stability

What ADRs are associated with ATG and RATG?

Infusion-associated reactions (ATG and RATG) - Fever, chills, dyspnea, N/V/D, hypotension or hypertension, malaise, rash, headache - Premedicate with acetaminophen, diphenhydramine, corticosteroids(!!!!) - Reduce rate of infusion Cytokine release syndrome (CRS) (RATG) - Cardiorespiratory dysfunction, hypotension, ARDS, MI, tachycardia, pulmonary edema, death. Anaphylaxis Anemia, leukopenia, thrombocytopenia Infections Malignancy Serum sickness (more with ATG) - Fever, rash, arthralgia, myalgia - 5 to 15 days after onset of treatment - Manage with corticosteroids

What are the 2 major categories of aplastic anemia, and what does each one result in?

Inherited and Acquired Inherited aplastic anemias (e.g. Fanconi's) result in: -Bone marrow failure -Fatty infiltration of marrow -Loss of circulating blood cells Acquired aplastic anemia: - Results from exposure to drugs, radiation, viruses, or chemicals - Accounts for most cases of aplastic anemia - An idiosyncratic reaction - Unpredictable severity and time to recovery

What are the possible causes of an increase aPTT caused by an altered number of Factors I, II, V, VIII, IX, X?

Inherited factor deficiencies* Lupus anticoagulant Heparin therapy Liver disease Afibrinogenemia

What effect on insulin can occur upon discontinuing TPN?

Insulin production increases in response to dextrose Rebound hypoglycemia can theoretically result from abrupt cessation of dextrose. - If abrupt discontinuation, D10 at same rate as PN

How is RQ interpreted?

Interpretation - > 1 - lipogenesis/hyperventilation. - < 0.7 - ketogenic diet, fat gluconeogenesis, or ethanol oxidation.

When is parenteral iron indicated for iron-deficiency anemia?

Intolerance to oral iron Malabsorption Nonadherence

What is the mainstay treatment for hemophilia?

Intravenous factor replacement therapy for the treatment or prevention of bleeding

What are all of the different ways that malnutrition can be classified as? Any disorder of nutritional status - Deficiency of nutrient intake, impaired nutrient metabolism, over-nutrition

Involuntary loss or gain of ≥ 10% of usual body weight in six months. Involuntary loss or gain of ≥ 5% of usual body weight in one month. Body weight of 20% over or under ideal body weight (IBW). Inadequate nutrition intake.

What are the causes of microcytic anemia?

Iron deficiency

What factors can increase Transferrin (TFN) values?

Iron deficiency Pregnancy Hypoxia Chronic blood loss Estrogens

What are the five different parenteral iron preparations currently available in the U.S.?

Iron dextran Sodium ferric gluconate Iron sucrose Ferumoxytol Ferric carboxymaltose

What is the most common nutritional deficiency in developing and developed countries? Results from prolonged negative iron balance which can occur due to: - Increased iron demand or hematopoiesis - Increased loss - Decreased intake and/or absorption

Iron-Deficiency Anemia (IDA)

What route of enteral access is used for a long term duration in patients with the following characteristics? Patient characteristics •Impaired gastric motility or emptying.

Jejunostomy

What factors increase Prealbumin values?

Kidney dysfunction

When is delaying calcineurin inhibitor necessary depending on transplant type? (!!)

Kidney transplantation: newly transplanted kidney is very susceptible to nephrotoxic injury. Liver and heart transplantation: need to protect patients with preexisting kidney impairment from further insults during perioperative period. Need calcineurin inhibitor dosage adjustment to maintain target concentration. Sometimes difficult to maintain target concentration in perioperative period due to fluctuation in GI absorption and enteral intake.

What condition associated with undernutrition occurs rapidly and is caused by an inadequate protein and antioxidant micronutrient intake? Develops in areas of famine, limited food supply, and low levels of education. May appear well nourished due to adipose tissue preservation, but with depletion of visceral protein. "Pot-belly" appearance.

Kwashiorkor

What glutamine product was FDA-approved in 2017? - First product approved for pediatric patients Most common gastrointestinal side effects: - Constipation - Abdominal pain - Nausea

L-glutamine (Endari®)

How is the dose of Factor VIII calculated when giving it to a patient with hemophilia A?

Large molecule which remains in intravascular space Plasma volume (about 50 mL/kg) used to estimate Vd Each unit of factor VIII concentrate infused per kilogram of actual body weight results in 2% rise in plasma factor VIII levels Equation can be used to calculate an initial dose of factor VIII: Factor VIII (units) = (Desired level - Baseline level) × 0.5 × (Weight [in kilograms]) - Baseline level usually omitted from equation if it is is negligible compared to desired level

What is LBM and how is it assessed biochemically?

Lean body mass - Skeletal muscle - Somatic protein - Functional proteins Assessed by measuring visceral proteins - Albumin - Transferrin - Prealbumin

What is a Bioelectrial Impedance Analysis (BIA) used to assess?

Lean body mass (LBM) Total body weight (TBW) Water distribution

What drugs can be effected by taking iron supplementation?

Levodopa ↓ (chelates with iron) Methyldopa ↓ (decreases efficacy of methyldopa) Levothyroxine ↓ (decreased efficacy of levothyroxine) Penicillamine ↓ (chelates with iron) Fluoroquinolones ↓ (forms ferric ion quinolone complex) Tetracycline and doxycycline ↓ (when administered within 2 hours of iron salt) Mycophenolate ↓ (decreases absorption)

Which weight loss medication is described here? GLP 1 agonist, prescription Adjunct to reduced-calorie diet and increased exercise for chronic management of weight in adults with BMI > 30 kg/m2 or > 27 kg/m2 with at least 1 weight-related comorbidity (ex: HTN, Type 2 DM, dyslipidemia) Indicated > 12 YO with body weight > 60 kg and BMI > 30 kg/m2 for adults Not indicated for treatment of type 2 DM or used with insulin or not used in combination with any other GLP-1 receptor agonist Dose: 3 mg daily subcutaneous injection (any time of day; without regard to timing of meals; abdomen, thigh upper arm) - Start at 0.6 mg per day for one week - Every week, increase dose by 0.6 mg until obtain 3 mg dose (5-week dosing escalation) - D/C if not lost at least 4% of baseline body weight by week 16

Liraglutide (Saxenda)

When is central access recommended for TPN? - Also what is the maximum osmolarity allowed with central access

Long-term nutrition - > 10-14 days Up to 1-3 years Can provide full nutritional support Ability to concentrate PN for fluid restriction May be > 900 mOsm/L (!)

What should be the initial weight loss goal in people who are obese to improve obesity complications?

Lose 5-10% of BW - Initial goal: lose 5-10% over 6 months. - 1st 10% weight loss: most health benefits and easiest to achieve. - Weight normally plateaus after 6 months since decreased metabolic rate at lower weight. - Lose 1-2 pounds/week. (!)

When is parenteral nutrition indicated in the ICU critical care setting?

Low nutrition risk (NRS-2002 ≤ 3 or NUTRIC score ≤ 5), PN be withheld over the first 7 days if patient cannot maintain intake and early EN not feasible PN-dependent diagnosis unless bacteremia High nutrition risk (NRS-2002 ≥ 5 or NUTRIC score ≥ 6) or severely malnourished when EN not feasible Low or high nutrition risk if unable to meet > 60% of energy and protein requirements by EN after 7 to 10 days

What laboratory findings are seen with Iron-Deficiency Anemia?

Low serum iron Low ferritin levels High total-iron binding capacity (TIBC) - Microcytic anemia

What laboratory findings are seen with a vitamin B12 deficiency?

MCV is elevated (>100 fL), some patients deficient normal MCV. Mild leukopenia and thrombocytopenia often present due to abnormal DNA synthesis. - Can affect all blood cell lines Peripheral blood shows macrocytosis accompanied by hypersegmentedpolymorphonuclear leukocytes.

How should Maintenance IV Fluids (MIVFs) be adjusted in patients on PN?

MIVFs containing dextrose and/or potassium should be converted to appropriate MIVFs without dextrose or potassium upon PN initiation. Examples: - D51/2NS → 1/2NS - D5NS + 20 mEq KCl → NS - LR ok as is. Total rate of MIVF + PN = previous ordered rate of MIVF - Some instances, may discontinue MIVF

What measurement looks at total energy expended during a time period? Reflects alteration in energy requirements as a result of disease or clinical status. Accounts for nutritional repletion energy with metabolic/activity factor

MREE [(3.94 VO2) + (1.11 VCO2) + (2.17 uN2)] x 1.44 - uN2 - urinary nitrogen component - Multiple by metabolic/activity factor

What are the 3 classifications of anemia based on RBC size?

Macrocytic Normocytic Microcytic

What is seen in megaloblastic anemias that is caused by abnormal DNA metabolism resulting from vitamin B12 or folate deficiency?

Macrocytosis

What are all of the components that can be included in parenteral nutrition?

Macronutrients: - Dextrose - Protein - Lipids REGULAR insulin Electrolytes Micronutrients: - Multi-vitamins - Trace elements Other additives

Which electrolyte is mostly is located in the skeleton, while the remainder is located in the intracellular fluid? Maintains neuromuscular and enzymatic functions - Cofactor for phosphorylation of adenosine triphosphates (ATP) from adenosine diphosphates (ADP)

Magnesium

What is the formula for ideal body weight?

Males = 50 + (2.3 x inches > 60 inches) Females = 45.5 + (2.3 x inches > 60 inches)

What is defined as an acute, subacute or chronic state of nutrition, in which varying degrees of OVERnutrition or UNDERnutrition with or without inflammatory activity have led to a change in body composition and diminished function?

Malnutrition

What condition associated with undernutrition is a chronic condition that results from prolonged inadequate intake or utilization of protein and calories? Inadequate food supply, anorexia nervosa, malabsorption syndromes. Deficit of subcutaneous adipose and skeletal muscle Visceral protein preserved - Albumin, transferrin Weight loss exceeds 10% of usual body weight(!) Skin and bones appearance

Marasmus

What patients require their TPNs to be at the maximum concentration. - Least amount of fluid.

Max concentrated for fluid restricted patients •Renal failure •Hepatic failure •Heart failure

What are the general signs and symptoms of anemia?

May be asymptomatic or have non-specific complaints. If vitamin B12 deficiency may develop neurologic consequences. In anemia of inflammation, signs and symptoms of underlying disorder can overshadow sign and symptoms of anemia. Signs: - Decreased exercise tolerance - Fatigue - Dizziness - Irritability - Weakness - Palpitations - Vertigo - SOB Symptoms: - Tachycardia - Pale appearance - Decreased mental acuity

What BMI must patients be above in order to consider pharmacologic treatment options for weight-loss?

May consider if not obtain adequate or maintain weight loss after lifestyle changes alone or history of many attempts to lose or maintain weight with lifestyle interventions alone AND - BMI ≥ 30 with no obesity related risk factors or diseases. (!!!!!) - BMI ≥ 27 with obesity related risk factors or diseases. (!!!) - Nonprescription orlistat: indicated with BMI > 25 - Need to consider patient specific factors and safety and efficacy of medications

When should the dose of Orlistat (Xenical) be omitted? - Dose: 120 mg TID with each main meal containing fat (during or up to 1 hour after each main meal)

Meal is occasionally missed or does not have fat - Dose can be omitted.

What all is measured in an evaluation of stature when assessing someones body mass? - Determined by genetics and nutrition

Measures Adults: - Standing height - Demispan (Distance from throat to ring finger) - Knee height

What all is assessed in the Subjective Global Assessment which is the Rating and Classification System for malnutrition? For several years, the Subjective Global Assessment, a simple, reproducible, cost-effective, bedside approach to nutrition assess has been used by many practitioners in a variety of patient populations.

Medical and dietary history - Weight changes in the previous six months - Dietary intake changes - Gastrointestinal (GI) symptoms - Functional capacity of GI tract - Presence of disease states known to affect nutrition Physical exam - Loss of subcutaneous fat - Muscle wasting - Edema - Ascites

What are the 2 types of macrocytic anemia?

Megaloblastic Nonmegaloblastic

What waist-to-hip ratio indicates a much higher risk for many diseases in men and women? - Waist circumference/hip circumference - Independent risk factor for morbidity

Men > 0.9 Women > 0.85

What waist circumference is an independent risk factor for obesity-related complications in men and women?

Men > 40 in Women > 35 in

What acid-base imbalances can be cause by PN? - Most acid-base disorders related to underlying conditions

Metabolic alkalosis: •Excessive acetate salts •Administer Cl- salts Metabolic acidosis: •Excessive Cl- salts •Administer acetate salts

What drug and food interactions can occur with the mTOR inhibitors?

Metabolized by 3A4 & P-glycoprotein - Consistent with calcineurin inhibitors Cyclosporine inhibits 3A4 & P-gp - Sirolimus: take 4 hours after cyclosporine - Everolimus: take same time as cyclosporine - Caution with dose adjustments Tacrolimus and sirolimus in heart transplants not recommended Administer consistently in regards to meals Grapefruit juice may increase levels

What corticosteroids are used for acute rejection and can be used with USP calcineurin inhibitors?

Methylprednisolone, prednisone

What are the different fat substitutes?

Microparticulated protein: desserts that are frozen, salad dressing Soluble fiber: from oats, fat replacement in meats, cheese, baked goods, desserts that are frozen Sucrose (Olestra): fast-soluble vitamin malabsorption, GI adverse effects, can cook and fry

What measurement estimates skeletal muscle mass? (!) - Compared to tricep skin fold thickness to calculate.

Midarm muscle circumference

What laboratory sign indicates Cholestasis? - Also when does this become elevated?

Mild to moderate elevation of liver enzymes commonly seen within 2 weeks of starting PN. - Return to normal after PN discontinued Rise in serum conjugated bilirubin of ≥ 2 mg/dL - May be accompanied by increases of alkaline phosphatase, ALT and AST

What type of products come as a powder or liquid form of nutrients? - Used to supplement an enteral formulation - Mixing process increases potential for bacterial contamination and incorrect preparation

Modular Products

How often do overweight and obese patients need to have their weight monitored?

Monitor every month for 1st 3 months and then every 3 months.

What is the most common cause of megaloblastic anemia in pregnancy? - Megaloblastic anemia during pregnancy is rare

Most common cause is folate deficiency Manifests as underweight premature infant and suboptimal health of mother Folic acid supplementation of 400 mcg daily is recommended for all women Prenatal vitamins usually have higher amount of folic acid as compared with general multivitamins to make sure adequate supplementation is provided

What laboratory findings are seen with folic acid deficiency anemia?

Most important to rule out vitamin B12deficiency Vitamin B12 and MMA levels normal Serum folate levels decrease to <3 ng/mL within few days of reduced dietary folate intake

How are most other rare bleeding disorders treated? - Rare bleeding disorders include congenital deficiencies in fibrinogen, in factors II, V, VII, X, XI, and XIII, and in combinations of factor deficiencies.

Most treated with fresh-frozen plasma

What is the general approach for treatment for preventing transplant rejection?

Multidrug approach Many agents have: - Overlapping mechanisms of action - Potentially synergistic mechanisms of action Multidrug immunosuppression regimen may allow use of lower doses of individual agents - Can reduce severity of dose-related side effects

What anti-rejection drug blocks inosine monophosphate dehydrogenase - Enzyme responsible for guanosine synthesis via the de novo pathway? - T and B cells only use de novo pathway - Decrease lymphocyte proliferation

Mycophenolic Acid: - Mycophenolate mofetil - Mycophenolate sodium - delayed release

Name all of the important electrolytes.

Na+ K+ Ca2+ Mg2+ Phos Cl- CO2-

What is the estimated daily requirements for each of the electrolytes?

Na+ - 1-2 mEq/kg K+ - 1-2 mEq/kg Ca2+ - 10-15 mEq Mg2+ - 8-20 mEq Phos - 20-40 mmol Cl- - As needed to maintain acid-base balance CO2- - As needed to maintain acid-base balance

What is the normal value of each electrolyte in healthy individuals?

Na+ - 135-145 mmol/L K+ - 3.5-5.2 mmol/L Ca2+ - 8.5-10.4 mg/dL Mg2+ - 1.6-2.4 mEq/L Phos - 2.5-4.5 mg/dL Cl- - 97-109 mmol/L CO2- - 20-29 mmol/L

What level is hyponatremia defined as? Caused by: •Gastrointestinal losses •Skin losses •Renal failure •Cortisol deficiency •Heart failure (HF) •Syndrome of inappropriate antidiuretic hormone hypersecretion (SIADH) •Diuretics

Na+ < 135 mmol/L:

What level is hypernatremia defined as? Caused by: •Loss of water: •Fever, burns, infection, renal loss, GI loss •Retention of Na+ because of hypertonic saline administration

Na+ > 145 mmol/L

What route of enteral access is used for a short term duration in patients with the following characteristics? Patient characteristics •Impaired gastric motility or emptying. •High risk of gastroesophageal reflux.

Nasoduodenal or Nasojejunal

What route of enteral access is used for a short term duration in patients with the following characteristics? Patient characteristics •Intact gag reflex •Normal gastric emptying

Nasogastric or Orogastric

How long do overweight / obese patients need to continue treatment measures?

Need to continue measures lifelong!!!

What are all of the indications for enteral nutrition?

Neoplastic disease - Chemotherapy - Radiation therapy - Upper GI tumors - Cancer cachexia GI disease - Inflammatory bowel disease - Short bowel syndrome - Esophageal motility disorder - Pancreatitis - Fistulas - Severe gastroesophageal reflux disease (GERD) - Esophageal or intestinal atresia Organ dysfunction - Liver disease/failure - Kidney insufficiency/failure - Cardiac cachexia - Acute Respiratory Distress Syndrome (ARDS)/ - Acute Lung Injury (ALI) - Bronchopulmonary dysplasia - Congenital heart disease - Organ transplantation Neurologic impairment - Comatose state - Cerebrovascular accident - Demyelinating disease - Severe depression - Cerebral palsy Hypermetabolic stress - Closed head injury - Burns - Postoperative major surgery - Sepsis Other indications - AIDS - Anorexia nervosa - Complications during pregnancy - Failure-to-thrive - Geriatric patients with multiple chronic diseases - Extreme prematurity - Inborn errors of metabolism - Cystic fibrosis

What are the possible causes of an increase prothrombin time caused by an altered number of Factors I, II, V, VII, X?

Newborn Vitamin K deficiency Inherited factor deficiencies* Warfarin therapy Liver disease Lupus anticoagulant (rare) Afibrinogenemia

What value reflects protein use or protein catabolic rate? Determined by measuring urinary nitrogen excretion and comparing with nitrogen intake. Stress causes increased protein use and nitrogen excretion.

Nitrogen balance

How is Drug-Induced Megaloblastic Anemia caused by chemotherapy agents treated?

No real therapeutic options available - Anemia becomes accepted side effect of therapy, resolve after removing the causative agent.

Can diuretics or laxatives be used for weight loss? Why or why not?

No! Diuretics - May see initial weight loss BUT within few days effect not seen - Electrolyte imbalance, sudden death Laxatives - Many botanicals are stimulants - No weight loss effect: act in colon and not small intestine where food mainly absorbed - Prolonged use: laxative dependence for regular bowel movements, electrolyte problems.

Can NSAIDs be used to treat acute pain associated with hemophilia?

No, Nonsteroidal anti-inflammatory drugs (NSAIDs) impair platelet function and may complicate bleeding so not recommended! Cyclooxygenase-2 inhibitors have less antiplatelet activity, an option for acute and chronic pain management.

What is the normal plasma levels range of either factors VIII or IX?

Normal plasma levels range from 0.5 to 1.5 units/mL (50%-150%) Factor VIII and factor IX activity levels are measured in units per milliliter, with 1 unit/mL representing 100% of the factor found in 1 mL of normal plasma.

What is the function of Willebrand factor (vWF)? - Large multimeric glycoprotein

Normal platelet adhesion Normal bleeding time Stabilization of factor VIII

What are the limitations ti using indirect calorimetry?

Not widely utilized. Calibration errors. Overestimates REE - Hyperventilation - Metabolic acidosis - Overfeeding - System air leaks Underestimates REE - Hypoventilation - Metabolic alkalosis - Underfeeding - Gluconeogenesis Requirements of patient

Visceral proteins should NOT be solely used to assess nutrition status because serum concentrations are affected by many variables, including what?

Nutrition Age Kidney function Gastrointestinal (GI) tract Skin Hydration status Liver function Metabolic stress

What type of delivery system for enteral nutrition is described here? •Product is transferred to a feeding bag •Can modify feedings •Increased nursing time •Risk of contamination

OPEN delivery system

What are the 2 different approaches to treating hemophilia?

One approach for treatment is on-demand therapy: treating of hemophilia patients to administer the necessary factor only for acute bleeding episodes Second approach to treatment known as prophylactic factor replacement therapy

What type nutritional substances are described here? •Enhance oral diet •Sweetened to improve taste •Hypertonic

Oral Supplements

Which weight loss agent is described here? OTC (!!) 60mg (lower dose than RX) 3 times/day before each meal containing fat Personalized tools (meal planner and tracker, recipes, weight tracker, food report) Not indicated for weight maintenance (just weight loss) Indications(!!!!) - ≥ 18 YO (different than RX) - Used with reduced-calorie, low-fat diet, and exercise program - Use in overweight adults (BMI ≥ 25) May lose about 5-10 lbs over 6 months

Orlistat (Alli)

What weight loss medication is described here? Prescription Indication: weight loss and maintenance in combination with reduced-calorie diet in persons ≥ 12 YO with BMI ≥ 30 kg/m2 or ≥ 27 kg/m2 with other risk factors (ex: HTN, DM, dyslipidemia) Inhibits gastric and pancreatic lipases - inhibit absorption of dietary TG (fat) - Decreases fat absorption (Rx: approximately 30% dietary fat; OTC: approximately 25%) Long-term treatment (safety and effectiveness after 4 years not determined) Several weeks to see "scale" effect but see stool changes in few days Used with low-fat/low-cal diet, exercise modifications - Diet with 30% fat calories and fat, carbohydrate, protein daily intake should be distributed over 3 meals Average weight loss at one year: 2.9-3.4 kg (6.5-7.5 lbs)

Orlistat (Xenical)

What is the function of size and quantity of ionic and molecular particles? Unit of measure •mOsm/kg •mmol/kg

Osmolality

What factor can cause decreased albumin?

Overhydration Edema Kidney dysfunction Nephrotic syndrome Poor dietary intake Impaired digestion Burns Congestive heart failure (CHF) Cirrhosis Thyroid/adrenal/pituitary hormones Trauma Sepsis

What are the goals of weight loss treatment?

Overweight and obesity-related medical conditions improvement (ex: DM, CVD) Psychological health and quality of life/well-being improvement Lose weight Prevent weight gain Have weight loss goal that is realistic Have exercise goal that is realistic Maintain weight that is healthy

What dosage forms and doses of B12 are used to treat vitamin B12 deficiency?

PO vitamin B12 used effectively to treat - Daily oral doses (1,000-2,000 mcg) of vitamin B12 is as effective as IM administration in achieving hematologic and neurologic responses Nonprescription 1,000 mcg cobalamin tablets available (among other strengths) Initial parenteral vitamin B12 regimen includes: - Daily injections of 1,000 mcg of cyanocobalamin for 1 week to saturate vitamin B12 stores in the body and resolve clinical manifestations of the deficiency - Then it can be given weekly for 1 month - Then monthly for maintenance

What laboratory findings indicate Von Willebrand disease?

PT is normal aPTT may be normal or prolonged in relation to the reduction in plasma factor VIII levels Normal aPTT does not rule out vWD Specific laboratory assessment of the vWF is needed

What are the clinical features seen with Sickle Cell Anemia (SCA- HbSS)?

Pain episodes Microvascular disruption of organs (spleen, liver, bone marrow, kidney, brain, lung) Gallstones Priapism Leg ulcers Anemia (Hb 6-9 g/dL)

What clinical features are seen with Sickle Cell Hemoglobin C (HbSC)?

Painless hematuria Rare septic necrosis of bone Pain episodes are less common and occur later in life Ocular disease Pregnancy-related problems Mild anemia (Hb 9-14g/dL)

What are the characteristic bleeding manifestations of hemophilia?

Palpable ecchymosis Bleeding into joint spaces (hemarthroses) Muscle hemorrhages Excessive bleeding after surgery or trauma

What are the different adult protein requirements?

Patient Status - Protein (gm/kg/day) Normal, healthy - 0.8-1 Metabolic stress - 1.2-2 Critical care - 1.2-2 Renal failure (no dialysis) - 1.2-2 Dialysis/CRRT - 1.5-2.5 Hepatic disease - 1.2-2 Surgery - 1.2-2 Wound healing - 1.2-2

What are the different adult fluid requirements depending on age?

Patient age Fluid requirement(mL/kg) 16-24 - 40 ml/kg 25-55 - 35 ml/kg 56-65 - 30 ml/kg > 65 - 25 ml/kg or 1500 mL + (20 mL x number of kg > 20 kg)

What are all of the different adult parenteral nutrition indications? (!!!!!!)

Patient who is well nourished prior to admission, but after 7 days(!!) of hospitalization enteral nutrition (EN) has not been feasible or target goal calories have not been met consistently by EN alone. On admission, the patient is malnourished (recent weight loss of > 10-15% or ABW < 90% of IBW) and EN is not feasible. Patient expected to undergo major upper gastrointestinal (GI) surgery (esophagectomy, gastrectomy, pancreatectomy, or other major re-operative abdominal procedures) and EN is not feasible: - Malnourished patient, initiate 5-7 days pre-operatively and continued post-operatively - Delayed for 5-7 days post-operatively - Duration of therapy is anticipated to be ≥ 7 days A major surgical procedure is planned for a critically ill ICU malnourished patient and EN is not feasible through the peri-operative period Patient with non-functional GI tract or cannot be accessed: - Paralytic ileus - Mesenteric ischemia - Small bowel obstruction (SBO) - GI fistula (except when enteral access may be placed distal to the fistula or volume of output (< 200 mL/day)) - Diffuse peritonitis - Intestinal obstruction - Intractable vomiting or diarrhea - GI ischemia Patient with severe acute pancreatitis and EN is not anticipated to be feasible after the first 7 days of hospitalization.

What are the different types of IV access used for TPN?

Peripheral Midline - Treat as PPN (peripheral) Central - Peripherally inserted central catheter (PICC) - Arrow multi-lumen - MedPort - Hickman - Groshong

Which weight loss medication is described here? - Prescription - Short-term (few weeks) weight loss (tolerance develops) - Used in conjunction with diet, exercise, behavior modification Similar structure to amphetamine, less CNS stimulation & abuse MOA: increase NE and dopamine (less)(!!!) Some adverse effects: - Insomnia (do not give in evening or HS), nervousness - Palpitations, tachycardia, BP - Primary pulmonary HTN - Valvular heart disease - Mydriasis (dilated pupil) - Psychosis Baseline cardiac evaluation for preexisting valvular heart disease, pulmonary hypertension; EKG during therapy; monitor BP, weight, weight circumference

Phentermine (Adipex-P, Lomaira)

Which weight loss medication is described here? Prescription Indication: combination with reduced-calorie diet and exercise for chronic management of weight in adults with BMI > 30 kg/m2 or > 27 kg/m2 with at least 1 weight-related comorbidity (ex: HTN, Type 2 DM, dyslipidemia) One of the components is an antiepileptic (MOA in weight loss not known) Approved for long-term(!!) CV morbidity and mortality effect not known Once daily in morning (avoid evening due to insomnia)(!) Average weight loss one year: 6.6-8.6 kg (14.5-18.9 lbs)

Phentermine and Topiramate(Qsymia)

What drugs are associated with causing Immune Hemolytic Anemia?

Phenytoin, acetaminophen, ribavirin, ciprofloxacin, amlodipine, bevacizumab

What electrolyte is a major intracellular anion? (!!!!) Important for metabolism of proteins, lipids, and carbohydrates Component of phospholipid membranes, RNAs, nicotinamide diphosphate, cyclic adenine and guanine nucleotides, and phosphoproteins Forms high energy bonds for the production of ATP Component of 2,3-diphosphoglycerate (2,3-DPG)

Phosphorus

What are anthropometric measurements?

Physical measurements of size, weight, and proportions of human body: - Weight - Stature - Growth velocity - Head and waist circumference - Limb size Bioelectrical impedance analysis (BIA) - Used to compare to normative standards and monitor response.

What are all of the Factor IX agents that are used for hemophilia B?

Plasma Derived Factor IX Recombinant Factor Factor IX Extended Half-Life (EHL) Factor IX - There also has been development of monoclonal antibody-purified plasma-derived products.

What lab procedure is the gold standard to assess platelet function? Possible cause of abnormal value: - Qualitative platelet defects, antiplatelet medications

Platelet aggregation

What lab measurement could indicate thrombocytopenia? Possible Cause of Abnormal Value: - Quantitative platelet disorder, type 2B von Willebrand disease, immune thrombocytopenia, other cause of thrombocytopenia. Clinical Manifestations - Mucocutaneous bleeding

Platelet count

What is thrombocytopenia defined as? - Annual incidence of drug-induced thrombocytopenia about 10 cases per 1,000,000 (excluding cases associated with heparin)

Platelet count <100,000 cells/mm2 or >50% reduction from baseline value.

Describe the normal process of the maturation and development of RBCs?

Pluripotent stem cell produces erythroid burst-forming units Erythropoietin (EPO) and cytokines stimulate this cell to form an erythroid colony-forming unit in the marrow. Nucleus becomes smaller with each division and disappears normal erythrocyte. Hb and iron get incorporated into gradually maturing RBC - RBCs eventually released from marrow into the circulating blood as a reticulocyte.

What is an inhibitor? - Most serious complication of factor replacement therapy?

Polyclonal high-affinity immunoglobulin G (IgG) directed against the factor VIII or IX protein. - Interfere with infused factor concentrate and reduce/make ineffective therapy - Measured with the Bethesda assay. (Titers reported in Bethesda units (BUs))

What is the difference between elemental and polymeric/intact carbohydrate formulations?

Polymeric or intact - starches and glucose polymers - Require digestion - Not as sweet Elemental - glucose and galactose - Contribute to osmolality

What do the numbers calculated from a nitrogen balance indicate? Balance = (Protein intake (gm)/(6.25)) - (24 hr urinary urea (gm) + 4 gm).

Positive # - Building muscle Negative # - Protein breakdown

What electrolyte is the most abundant intracellular cation? - Maintains electroneutrality with Na+ - Regulates muscle and nerve excitability

Potassium

What is the transport protein for thyroxine and carrier for retinol-binding protein? Most useful for monitoring acute effects of nutrition - Low body content - Responds quickly to changes in nutrition - Short half-life of 2-3 days

Prealbumin

How should vaccines be administered differently to patients with hemophilia?

Preferred subcutaneously in patients with severe disease (!) If intramuscular administration is used need to: - Use of a small-gauge needle. - With cold compresses and pressure to the site can prevent excessive bleeding.

What side effects can occur with therapeutic doses of iron?

Primarily GI in nature: - Dark discoloration of feces - Constipation or diarrhea - Nausea - Vomiting

What patients usually receive liver transplants?

Primary cause of end-stage liver disease (> 70% of liver transplant recipients) have noncholestaticcirrhosis (hepatitis C, alcoholic cirrhosis, hepatitis B, nonalcoholic steatohepatitis, autoimmune hepatitis)

How is Drug-Induced Thrombocytopenia treated?

Primary treatment - Removal of offending drug Need to treat symptomatic patients Use of corticosteroids is controversial - Some experts recommend in severe cases

How can drug induced agranulocytosis be treated?

Primary treatment - Removal of the offending agent - Blood cell counts usually returns to normal range within 2 to 4 weeks Sargramostim (GM-CSF) and filgrastim (G-CSF) shorten duration of neutropenia - Shorten duration of neutropenia, length of antibiotic therapy and hospital length of stay - Recommended for patients with neutrophil nadir <100 cells/mm3 regardless presence of infection

How is Metabolic hemolytic anemia treated?

Primary treatment is removal of offending drug No other therapy usually necessary If known G6PD enzyme deficiencies need to avoid medications capable of inducing the hemolysis

What labratory testing would be expected of someone with hemophilia?

Prolonged activated partial thromboplastin time (aPTT) Decreased factor VIII or factor IX level Normal prothrombin time (PT) Normal platelet count Normal von Willebrand factor antigen and activity Normal bleeding time

Local measures to control superficial bleeding caused by vWd include...

Prolonged pressure Ice Topical thrombin

What is Basiliximab (Simulect®) indicated for?

Prophylaxis of acute organ rejection in patients receiving renal transplantation when used as part of an immunosuppressive regimen that includes cyclosporine and corticosteroids. Only used by experienced physicians and in adequate facilities ADRS: Hypersensitivity reactions

What are the general indications for Belatacept?

Prophylaxis of organ rejection in adult patients receiving a kidney transplant with: - Basiliximab induction - Mycophenolate mofetil - Corticosteroids Use only in patients who are EBV seropositive patients(!!)

What is •Mycophenolic acid delayed-release indicated for an what should it be administered with?

Prophylaxis of organ rejection in patients receiving renal transplants - Use with cyclosporine and corticosteroids

What is mycophenolate mofetil (Regular release) indicated for and what should it be administered with?

Prophylaxis of organ rejection in patients receiving renal, cardiac or hepatic transplants. - Use with cyclosporine and corticosteroids - IV admin for up to 14 days; switch to oral ASAP

Which medications count towards lipid total in PN patients?

Propofol (Diprovan®) - 10% lipid emulsion Clevidipine (Cleviprex®) - 20% lipid emulsion

Which macronutrient in PN is provided as crystalline amino acids (CAAs)? - Categorized as standard or modified solutions Standard: - "Normal" organ function Modified: - Disease specific

Protein

What all is required for proper nutrition?

Protein Fat Micronutrients Fiber Fluids

What are the DRIs for the 3 main macronutrients in adults?

Protein - 10-25% Fat - 15-30% Carbohydrate - 45-60%

In adults what is the percent of daily calories for each of the macronutrients?

Protein - 10-25% of daily calories. Fat - 15-30% Carbohydrate - 45-60%

REVIEW PPT 12

REVIEW PPT 12

What measurement reflects substrate oxidation and characterizes substrate use? - Calculated as VCO2/VO2 ___________ nutrient values - Fat - 0.7 - Carbohydrate - 1 - Protein - 0.8 - Mixed substrate - 0.85

RQ

What clinical features are seen with Sickle Cell β+-Thalassemia (HbSβ+-Thal)?

Rare pain Milder severity than HbSS due to production of some HbA Hb 9-12 g/dL Microcytosis

What are the clinical feature of Sickle Cell Trait (SCT) presentation?

Rare painless hematuria. Heavy exercise under extreme conditions can provoke gross hematuria. Complications (normal Hb).

Which vWF replacement was approved by the FDA in 2015 for on-demand treatment and control of bleeding episodes and for perioperative management of bleeding in patients with vWD?

Recombinant vWF alone

What are the dosing directions for Phentermine and Topiramate (Qsymia)?

Recommended dose: Phentermine 3.75mg/topiramate ER 23mg daily for 14 days; then increase to 7.5 mg/46 mg daily Discontinue or escalate dose (as described) if 3% weight loss not obtained after 12 weeks of taking 7.5 mg/46 mg dose Discontinue if 5% weight loss not obtained after 12 weeks on maximum daily dose (15 mg/92 mg) Discontinue 15 mg/92 mg dose gradually to prevent seizure (refer to PI) Do not exceed 7.5 mg/46mg dose: moderate or severe renal impairment moderate hepatic impairment.

Describe prophylactic factor replacement therapy.

Recurrent joint bleeding can damage joint and lead to the development of severe physical disability Recommended to prevent bleeding episodes and avoid the resultant damage Goal: to maintain a patient's minimum factor level at or above 0.01 units/mL (1%) with regular infusions of factor products Converts severe hemophilia into a milder form of the disease

How does Ristocetin cofactor activity (RCo) differ between each type of vWd? - Functional assay of vWF activity based on platelet aggregation with ristocetin.

Reduced by the same degree as vWF:Ag in types 1 and 3 Reduced to a greater extent in type 2 disease (except 2B)

What ADRs are associated with Orlistat (Xenical)?

Reduction in fat soluble vitamin absorption - Take MVI (at least 2 hours before or after orlistat, such as HS) Soft, oily spotting, loose and frequent, fatty/oily stools Flatulence Fecal urgency and incontinence Bloating Abdominal pain Severe liver injury Nephrolithiasis Cholelithiasis Pancreatitis

What metabolic complication may occur in severely malnourished patients who receive rapid nutritional repletion? Re-introduction of carbohydrate causes sudden shift back to glucose as a fuel source with inhibition of fat metabolism. Demand for phosphorylated intermediates for glycolysis - Adenosine triphosphate (ATP) - 2,3-diphosphoglycerate (DPG)

Refeeding Syndrome

What severe complication is a metabolic complication that may occur in severely malnourished patients who receive rapid nutritional repletion? Re-introduction of carbohydrate causes sudden shift back to glucose as a fuel source with inhibition of fat metabolism. •Demand for phosphorylated intermediates for glycolysis •Adenosine triphosphate (ATP) •2,3-diphosphoglycerate (DPG)

Refeeding Syndrome

What type of insulin is the only type compatible with PN? - Typically order sliding scale coverage when PN initiated - Usually, adjust at increments of 5-10 units - Monitor blood glucose every 4-6 hours - Recommended to add 0.1 unit per gram of dextrose - Goal maintenance blood glucose readings: 140-180 mg/dL

Regular insulin

What enteral nutrition products are useful to maintain hydration or treat dehydration? - Administered orally or via feeding tube - May decrease fecal water loss and generate positive electrolyte balance

Rehydration Products

How is acquired aplastic anemia treated? - List all options

Remove/stop suspected offending agent Provide adequate supportive care -Antimicrobial therapy - (Prophylactic antimicrobial and antifungal therapy in patients with neutrophil count <500 cells/mm3) -Transfusion support with erythrocytes and platelets -Use of G-CSF may be considered only if life threatening infections. -Allogeneic HSCT -Immunosuppressive therapy - Antithymocyte globulin (ATG) - Cyclosporine

What is determined by protein, Na+, K+, Cl- content? - Greater _______________ increases obligatory H20 loss - Affects tolerance

Renal Solute Load

How should Chronic Sickle Cell Pain be treated?

Requires an interprofessional team approach Pharmacological approach: - Different agents Nonpharmacological approach: acupuncture, massage, cognitive behavioral therapy, relaxation therapy

What are the goals of treating anemias?

Reversal of hematologic parameters to normal Return of normal function and quality of life Prevention or reversal of long-term complications

What are the goals of treating Iron-Deficiency Anemia?

Reversal of hematologic parameters to normal. Return of normal function and quality of life. Prevention or reversal of long-term complications.

Even though hunger and satiety mainly regulated by hypothalamus people eat due to...

Rewards, pleasure, learning, memory

What is the antibiotic that is used to measure vWF activity that causes platelet aggregation in the presence of functional vWF? - Assay is performed by mixing platelet-free patient plasma, normal formalin-fixed platelets, and _______________ and then quantitating extent of platelet agglutination.

Ristocetin

What agent sometimes used for rejection works at the CD20+ antigen?

Rituximab

What are the different sugar substitutes?

Saccharin: tastes bitter, Replaced by newer sweeteners Aspartame - Warning on label - contains phenylalanine - do NOT take if have phenylketonuria since cannot metabolize it! - Do not cook or bake with it - bitter taste Fructose, sorbitol, xylitol - Have calories - NOT "sugar-free"!!! - Xylitol and sorbitol do not cause tooth decay - Sorbitol: diarrhea Stevia: food additive Sucralose (Splenda) and Neotame: used in cooking

What are the different coagulation factor levels associated with each severity of hemophilia? - Mild, moderate, severe

Severe hemophilia: <0.01 units/mL (1% of patients) of either factor Moderate hemophilia: 0.01 - 0.05 units/mL (1%-5% of patients) Mild hemophilia: 0.05 - 0.4 units/mL (5%-40% of patients)

When is peripheral access recommended for TPN? - Also what is the maximum osmolarity allowed with peripheral access?

Short-term nutrition - < 14 days Cannot provide full nutritional support Large volumes may be required to meet nutritional goals Max of 900 mOsm/L (!!!) May require line changes every 48-72 hours

What warnings are associated with Mycophenolic Acid?

Should be only used by experienced health care providers and in adequate facilities Serious infections Malignancies Embryo-fetal toxicity - Use during pregnancy associated with ↑risk of pregnancy loss and congenital malformations: Risk Evaluation and Mitigation Strategy (REMS), females and males of childbearing potential must use contraception

What is a group of hereditary conditions characterized by presence of sickle cell hemoglobin (HbS) in RBCs? - What 2 syndromes is it divided into?

Sickle cell syndromes Divided into: - Sickle cell trait (SCT) - Sickle cell disease (SCD)

What are the target drug levels for each of the mTORs?

Sirolimus - Whole blood with CIs target trough: 10-15 ng/mL - Whole blood without CIs target trough: 15-25 ng/mL Everolimus - Target trough with CIs (HPLC): 3-8 ng/mL

What anti-rejection agents are proliferation aignal inhibitors of Mammalian Target of Rapamycin (mTOR)?

Sirolimus/Rapamycin Everolimus Macrolides

Which mTOR inhibitors can be used for prophylaxis of organ rejection in patients receiving renal transplants?

Sirolimus: 13 and older Everolimus: adults at low-moderate immunologic risk

What measurement estimates subcutaneous fat(!)? - Triceps skin is most commonly utilized

Skin fold thickness

What electrolyte is the most abundant extracellular (!) cation? - Major regulating factor of water balance - Extracellular and intracellular content affected by fluid status

Sodium

Describe the administration directions for Sirolimus?

Solution given in glass or plastic 2 oz of water or orange juice Mix and drink immediately Refill with 4 oz water or orange juice and drink again

How can solutions and suspensions be effected by enteral feeding?

Solutions - Increased potential for drug instability Suspensions - Increased viscosity and potential for settling of dispersed particles. - Shake well immediately prior to drug administration

What ADRs can occur with Liraglutide (Saxenda)?

Some adverse effects: nausea, hypoglycemia (consider lowering dose of anti-diabetic drugs to reduce hypoglycemia risk), diarrhea, constipation, vomiting, headache, decreased appetite, dyspepsia, fatigue, dizziness, abdominal pain, increased lipase, increased heart rate (monitor), pancreatitis, depression, suicidal thoughts or behavior (!!!!!!!!), acute gallbladder disease, fever, gastroenteritis, acute renal failure, worsen chronic renal failure. Avoid if history suicidal attempts or currently have suicidal ideation (!)

What lipid product for PN is available as 10%, 20%, and 30% concentrations

Soybean-oil-based products

Which calcineurin inhibitors can only be administered with corticosteroids? - All should be only used by experienced health care providers and in adequate facilities

Standard/USP - Only administered with corticosteroids! Modified: - May be administered with other suppressive agents in kidney, liver, and heart transplant patients

Describe how the pharmacokinetics of cyclosporine differs between the standard and modified formulations? - Standard/USP formulation: Sandimmune - Modified/microemulsion: Gengraf, Neoral

Standard/USP: - Decreased AUC and Cmax - F = 30% - t1/2 = 19 hours Modified: - Increased AUC and Cmax (decreased by food) - F>Standard formulation - t1/2 = 8.4 hours

What are the different type of solution formulas for calculating TPNs?

Standardized - Fixed amounts of macronutrients, electrolytes, MVI, and trace elements Specialized - "Patient-specific"

What microbiologic species most commonly cause catheter-related infections/thrombosis in PN patients? •May require anticoagulation •May require removal of IV access

Staphylococcus aureus and Candida albicans

How are calcineurin inhibitors dosed and what should be monitored for safety and efficacy?

Start with weight-based dose q12h & then adjust based on levels - IV dose is 1/3 the PO dose Measure trough concentrations - Cyclosporine (HPLC) whole blood 100-300 ng/mL, plasma 75-100 - Tacrolimus whole blood HPLC 8-13 ng/mL, RIA 5-20

What are the main risk factors for hyperglycemia with PN?

Stress (sepsis, surgery, organ failure) Cirrhosis, uremia, obesity Corticosteroids (!!!!) Medications (!!!!) Catecholamine vasopressors (!!!!!) Diabetes, pancreatitis, old age Parenteral dextrose infusion

Which formulations of drug agents should not to be crushed and administered via feed tube? - Institute for Safe Medication Practices (ISMP)

Sublingual, sustained-release, and enteric-coated formulations. Pellets - Generally not crush **Solid dosage forms - Crush to fine powder and mix with solvent **Capsules - Many may be opened

What biochemical defect causes sickle cell? Normal RBCs: - Biconcave shape - Able to deform to squeeze through capillaries - SCD RBCs not able to easily pass through capillaries

Substitution of Valine for Glutamic acid as 6th aa in β-polypeptide chain. - V -> E Another abnormal hemoglobin, hemoglobin C (HbC): produced by substitution of lysine for glutamic acid as 6th aa in β-chain. Structurally, α chains of HbS, HbA, and HbC are identical Chemical differences in β-chain which account for sickling and its related sequelae

What drug and food interactions can occur with the calcineurin inhibitors?

Substrates of 3A4 and P-gp - 3A4 inhibitors - decrease dose of calcineurin inhibitors Calcineurin Inhibitors are inhibitors of 3A4 - Caution HMG-CoA reductase inhibitors Administer consistently in regard to meals(!!!) Tacrolimus must be separated from antacids/positive cations by 2 hrs(!) Other drugs causing ↑K+ Other nephrotoxic drugs

Where is Von-Willebrand factor synthesized?

Synthesized in endothelial cells and stored in Weibel-Palade bodies or secreted constitutively. Also synthesized in megakaryocytes and stored in α-granules from which it is released after platelet activation.

which calcineurin inhibitor is described here? •Can be opened and administered by sublingual route •Avoids first-pass metabolism •Start with ½ of the PO dose •Leave under tongue for at least 15 min •Increased risk of oral cancers

Tacrolimus

What drug and foods can interact with Mycophenolic Acid?

Take on empty stomach(!!) - But with food if severe GI upset Cholestyramine - Decreased AUC because of decreased enterohepatic recirculation à Avoid use Antacids - Avoid use Oral contraceptives - Decreased blood levels of hormones Acyclovir/Ganciclovir - Increased concentration of antiviral & mycophenolic acid. - Caution in renal impairment Proton-pump inhibitors - Mycophenolic acid regular release needs acidic pH

How long should folic acid therapy be taken for when treating Folic Acid Deficiency Anemia? - Synthetic folic acid almost completely absorbed by GI tract

Therapy should continue for about 4 months if underlying cause of deficiency can be identified and corrected - Need to allow for clearance of all folate-deficient RBCs from circulation.

Name the important water-soluble vitamins.

Thiamine (B1) Riboflavin (B2) Pantothenic acid Niacin Pyridoxine (B6) Folic acid Cyanocobalamin (B12) Biotin Ascorbic acid (C)

What are the requirements to initiate enteral nutrition?

Those who cannot or will not eat a sufficient amount to meet nutritional requirements. Sufficient functioning GI tract to allow nutrient absorption. Method of enteral access safely obtained.

What is the goal of treatment for Von Willebrand Disease?

To prevent bleeding episodes and their short-term and long-term consequences.

When calculating basal energy expenditure (BEE), what can effect the total calories used per day? - Daily adult requirements.

Total Calories* (kcal/kg/day) Healthy/Normal nutrition/Minimal illness severity: 10-40 (kcal/kg/day) Illness/Metabolic stress: BMI < 30 kg/m2 - 25-30 (kcal/kg/day) BMI ≥ 30 kg/m2 - 11-14 (kcal/kg/day) Major burn injury: ≥ 30 (kcal/kg/day)

What immune function tests can be used to asses malnutrition?

Total lymphocyte count - Number of circulating T and B lymphocytes. - Tissues that generate T cells are very sensitive to malnutrition. - T cell count < 1200 cells/mm3 (1.5 x 109 cells/L) is a nonspecific marker for nutrition depletion. Delayed cutaneous hypersensitivity (DCH) reactions - Uses antigens to which individual was previously sensitized. - Anergy associated with severe malnutrition.

What protein binds iron to the liver and reticuloendothelial system? Serum concentrations may be determined by - Direct measure - Indirectly from measurement of total iron-binding capacity - _____________________ (mg/dL) = (total iron binding capacity X 0.8) - 43 Relatively small body pool Half-life of 8-9 days

Transferrin (TFN)

What risks are associated with Allogeneic hematopoietic stem cell transplantation (HSCT)?

Transplant-related mortality rate (5% - 10%) Graft rejection (about 10%) Secondary malignancies Neurologic events (e.g. intracranial hemorrhage, seizures during transplant). - More frequently in patients with history of stroke.

What are the physiologic consequences of heart transplantations?

Transplanted heart denervated so no longer normal response to: -Physiologic stimuli -Pharmacologic agents If there is increased HR (e.g. due to exercise or hypotension) -Denervated heart is unable to increase HR -Instead increases the stroke volume

What are the different protein products used for PN?

Travasol® (Mainly use this one!) Aminosyn® Hepatamine® (for pts with liver failure)(More BCAA's)

What is RATG (Thymoglobulin) indicated for? - Rabbit anti-thymocyte globulin

Treatment of renal transplant acute rejection in conjunction with immunosuppression.

T or F: All currently available plasma-derived factor concentrates: - Come from screened donors - Undergo viral inactivation procedures

True

T or F: Homozygous HbS (HbSS) previously called sickle cell anemia (SCA) now includes HbSβ0-thal due to similarities in clinical severity.

True

T or F: In enteral feeding partially hydrolyzed or elemental substrates and caloric density can INCREASE osmolality.

True

T or F: Increased HbF production can occur under severe erythroid stress (e.g. anemia)?

True

T or F: More than one etiology of anemia can occur concurrently

True

T or F: intravenous factor replacement therapy can be administered at home.

True More convenient for families Allows for earlier treatment of acute bleeding episodes

T or F: Females who are carries of hemophilia will also have lover factor VIII counts.

True, Female carriers have lower factor VIII levels than females who are not carriers! - Do not have bleeding tendency tho!!

T or F: After a transplant the risk of acute rejection decreases over time.

True, the risk of acute rejection decreases over time so clinicians: - Gradually reduce doses of immunosuppressants - Sometimes total discontinuation of immunosuppressants over period of 6 to 12 months

T or F: When administering a TPN a filter is required.

True, use a 0.22 µm filter.

T or F: Diagnosis of hemophilia should be considered in any male with unusual bleeding.

True. Family history of bleeding: - Helpful in the diagnosis - Absent in up to 50% of patients

Normal hemoglobin (HbA) composed of:

Two α chains and Two β chains

What type of Von Willebrand Disease is described here? Most common type Acounts for 70% to 80% Characterized by a mild-to-moderate quantitative reduction in the level of vWF. Usually inherited in autosomal dominant fashion with variable penetrance and expression. Bleeding symptoms often very mild to moderate.

Type 1 vWD

What type of Von Willebrand Disease is described here? Diagnosed in 20% to 30% of affected patients Characterized by a qualitative abnormality of vWF Bleeding manifestations more severe than with type 1 disease Inheritance: - Most often autosomal dominant - May be recessive

Type 2 vWD

What type of Von Willebrand Disease is described here? Severe quantitative variant of the disease in which vWF is almost undetectable. Factor VIII levels are very low (<20 IU/dL [0.2 IU/mL]). Often inherited in an autosomal recessive fashion. Accounts for <5% of all cases. Clinical phenotype is severe, reflecting major deficits in primary hemostasis and coagulation.

Type 3 vWD

What are the 3 major subtypes of Von Willebrand Disease Classification?

Types 1 and 3 are associated with quantitative defects in vWF. Type 2 mutations associated with functional abnormalities in vWF.

How is Drug-Induced Thrombocytopenia typically presented?

Typically presents 1 to 2 weeks after starting a new drug. May present immediately after dose if an agent has been used intermittently in the past. Development of thrombocytopenia may be associated with the systemic drug concentration (e.g. with linezolid)

How is the infusion rate for cyclical infusions calculated?

Typically, first and last hour 75-125 mL/hr Rate calculation - Total volume/(# of hours - 1) = rate for in-between first and last hours. - Round to nearest 10 mL/hr Answer x (# of hours - 2 hours) = volume between first and last hours. Total volume - answer = volume for first and last hours. Volume/2 = rate for first and last hours.

What type of malnutrition results from - Starvation - Impaired absorption - Altered metabolism

Undernutrition

How long are TPNs usually administered for?

Usually administered for 24 hours Cyclical infusions - Administered for < 24 hours - Typically anywhere between 10-18 hours - If patient is not diabetic, schedule chemsticks (CS) during infusion and 1 hour after administration completed.

How often should chronic transfusion therapy be given to sickle cell patients?

Usually given every 3 to 4 weeks Frequency of transfusion adjusted to maintain desired HbS levels

How does the levels of von Willebrand factor antigen (vWF:Ag) differ between each type of vWd? - Antigenic determinant(s) on vWFmeasured by immunoassays

Usually low in types 1 and 2 Virtually absent in type 3

What are the potential limitations of a Bioelectrial Impedance Analysis (BIA)?

Variability with electrolyte imbalances Interference by obesity Lack of reference standards

What are the key mechanisms involved with hemostasis?

Vascular constriction Primary platelet plug formation (primary hemostasis) Clot propagation through fibrin formation (secondary hemostasis)

What are the causes of macrocytic anemia?

Vitamin B12 deficiency Folic acid deficiency

Coagulation factors are divided into 3 groups, what are they?

Vitamin K dependent (II, VII, IX and X) Contact activation factors (XI, XII, prekallikrein, high-molecular weight kininogen) Thrombin sensitive (V, VIII, XIII and fibrinogen)

What coagulation disorder is the most common congenital bleeding disorder? - Prevalence of 0.1% - 1% Family of disorders caused by: - Quantitative and/or - Qualitative defect of von Willebrand factor (vWF) Has autosomal inheritance pattern and usually results in equal frequency of disease in males and females (!!!)

Von Willebrand Disease

What risk factors are associated with metabolic syndrome? - ≥ 3 risk factors indicate metabolic syndrome.

Waist circumference > 40 inches (men) or 35 inches (women) - Can vary depending on ethnic group TG > 150 mg/dL HDL < 40 mg/dL (men) or < 50 mg/dL (women) BP > 130/85 mm Hg Fasting serum glucose > 100 mg/dL

What is the formula for BMI?

Weight (kg)/height (m2) OR [Weight (lb)/height (in2)] x 703

Can Bupropion and Naltrexone (Contrave) be administered with food?

Yes but do NOT administer with high-fat meal. Neuropsychiatric events or decreased tolerance of alcohol if have alcohol while taking bupropion → minimize or avoid alcohol.

Can marasmus and kwashiorkor occur together?

Yes they can occur together in someone with a severe protein-calorie malnutrition? Develops in chronically ill, starved individuals during periods of hypermetabolic stress. Reduced visceral protein synthesis along with somatic protein and adipose wasting.

Can patients ever receive enteral tube feeding and PN at the same time?

Yes! When discontinuing PN patients tolerating PO or tube feeds (TF) should transition to enteral feeding! - TF + PN near goal nutrition - Consider when consuming 50-75% of goal calories

Name the important trace elements.

Zinc Copper Manganese Selenium Chromium

Treatment with ______________________________ often adequate for minor bleeding episodes in patients with mild hemophilia A. Synthetic analog of antidiuretic hormone vasopressin - Causes release of vWF and factor VIII from endogenous endothelial storage sites Most effective in patients with higher baseline factor VIII levels (0.1-0.15 units/mL) - Can be administered intranasally via a concentrated nasal spray

desmopressin acetate

Most recent guidelines by the American College of Chest Physicians recommend that ___________________________ is most appropriate option if history of HIT and have present clot with normal renal function.

fondaparinux

What is the most common complication of excessive dextrose infusion? Stress and/or critical illness may also contribute Uncontrolled ________________ can lead to: •Fluid and electrolyte imbalances •Increased susceptibility to infection •Hyperosmolar non-ketotic syndrome •Hypertriglyceridemia •Hepatic steatosis

hyperglycemia

The body synthesizes all fatty acids except for which ones? - Essential fatty acids. - Fatty acid deficiency may be prevented if 5% of total calories ingested as these fatty acids.

linoleic acid and α-linolenic acid

What agent sometimes used for rejection is a Janus kinase inhibitor?

tofacitinib

What is von Willebrand factor (vWF)?

von Willebrand factor (vWF): glycoprotein which plays a role in both platelet aggregation and coagulation Binds factor VIII and protects it from degradation by plasma proteases and prolongs its half-life. Important for primary and secondary hemostases

What signs/symptoms of intolerance should be monitored for when giving enteral nutrition?

•Abdominal distention •Cramping •High gastric residual volumes •Aspiration •Diarrhea

What ADRs can occur with corticosteroids?

•Adrenal suppression/ insufficiency •Cataracts/glaucoma •Fat redistribution •Hyperglycemia •Hypertension •Immunosuppression •Impaired wound healing •Infection •Muscular atrophy •Na+/H2O retention •Osteoporosis •Peptic ulcers •Psych effects •Stunted growth •Thinning of the skin •Weight gain

What ADRs are associated with the mTOR inhibitors?

•Anemia/ thrombocytopenia •Angioedema •Arthralgia •Delayed wound healing/ dehiscence •Fluid accumulation •Hypercholesterolemia/ triglyceridemia •Hypertension •Infections •Interstitial lung disease •Malignancy •Nephrotoxicity Everolimus only •Kidney thrombosis •Male infertility

What patients are at risk for Refeeding Syndrome? (!!!!)

•Anorexia nervosa •Residents admitted from skilled nursing facilities •Unfed x 7-10 days with evidence of stress/depletion •Chronic diseases causing undernutrition •History of excessive alcohol intake •Morbid obesity with massive weight loss

What patients are at risk of Refeeding Syndrome?

•Anorexia nervosa •Residents admitted from skilled nursing facilities •Unfed x 7-10 days with evidence of stress/depletion •Chronic diseases causing undernutrition •History of excessive alcohol intake •Morbid obesity with massive weight loss

Name all of the water soluble vitamins

•C - ascorbic acid •B - folate •Niacin •B1 - thiamine •B2 - riboflavin •B6 - pyridoxine •B12 - cobalamin •Pantothenic acid •Biotin

What are the possible causes of hyperphosphatemia?

•CKD •Hypoparathyroidism

What nonpharmacologic dietary changes can be used for treating obesity?

•Caloric restriction (portion size) •Change fat, protein, carbohydrate, protein proportions •Use of macronutrient substitutes (ex: sugar & fat) •Change timing or meal frequency •Drinking water instead of beverages with sugar •Use smaller plates •Healthier snacks •Make changes that can last a long-time

What complications can occur with parenteral feeding?

•Catheter-related infections •Catheter-related thrombosis •Pneumothorax •Fluid imbalance •Acid-base imbalance •Gut atrophy •Hyperglycemia •Essential fatty acid deficiency •Refeeding syndrome •Cholestasis

Which trace elements are included in Multitrace?

•Chromium 4 mcg •Copper 0.4 mg •Manganese 0.1 mg •Zinc 1 mg

What are the primary indications for a lung transplant?

•Chronic obstructive lung disease (COPD)/emphysema •Idiopathic pulmonary arterial hypertension •Cystic fibrosis •Idiopathic pulmonary fibrosis -Becoming increasing viable life-saving option for patients with end-stage pulmonary failure who are not amenable to other treatment

What are the different pediatric enteral feeding formulation classifications for infants?

•Cow's milk-based •Soy protein-based •Prematurity •Transition •Semi-elemental/elemental •Special diets

What drugs are associated with drug associated aplastic anemia?

•Cytotoxic chemotherapy •Chloramphenicol •Phenytoin •Carbamazepine

What are the advantages of enteral nutrition vs parenteral nutrition?

•Decreased infectious complications •Fewer metabolic complications •Fewer technical complications •Less costly

What are all of the potential causes of hyperkalemia? - K+ > 5.2 mol/L

•Decreased pH •Insulin deficiency •β2-agonist blockade •Digoxin overdose •Re-warming after hypothermia Reduced urinary excretion: •Kidney dysfunction •Intravascular volume depletion •Hypoaldosteronism •K+-sparing diuretics

Which medications are commonly administered via PN?

•Famotidine (Pepcid®) •Ranitidine (Zantac®) •Heparin

What is defined as the volume of remaining contents in the stomach? - Used to monitor safety, efficacy, and tolerance No definitive definition of high residual volume - Adults - greater than 200-500 mL - Check every 4-5 hours when initiating feeds until plateau of less than 50 mL achieved

•Gastric residuals

What symptoms are associated with high osmolality?

•Gastric retention •Diarrhea •Abdominal distention •Nausea •Vomiting

What ADRs can occur with both calcineurin inhibitors?

•Hepatotoxicity •Increased K+ •Hyperglycemia •Hyperlipidemia •Hypertension •Infections - new & reactivation •Malignancy •Nephrotoxicity •Neurotoxicity •Headache

What are the possible negative consequences of transplantation?

•Hypertension •Hyperlipidemia •New-onset of diabetes •Infection •Malignancy

What are the possible causes of hypocalcemia? - Hypocalcemia Ca2+ < 8.5 mg/dL:

•Hypoparathyroidism •Vitamin D deficiency •Alcoholism •Hyperphosphatemia •Receipt of large amounts of blood •Continuous renal replacement therapy (CRRT)

What are all of the different disease-specific formulation classifications of enteral feeding in adults

•Immune-modulating •Kidney •Liver •Lung •Diabetes Mellitus

What are the potential causes fo hypomagnesemia? - Mg2+ < 1.6 mEq/L

•Impaired intestinal absorption •Inadequate intake •Hypokalemia •Increased renal excretion

What are all of the potential causes of hypokalemia? - K+ < 3.5 mmol/L

•Increased pH •Insulin •β2-agonists •Hypothermia •GI losses •Hypomagnesemia •Diuretics

What are the possible causes of hypophosphatemia? - Phos < 2.5 mg/dL:

•Increased renal elimination •Refeeding syndrome •Respiratory alkalosis •Treatment of diabetic ketoacidosis (DKA)

What factors regarding gastric vs intestinal enteral tubes should be considered when administering medications?

•Length of functional bowel •Internal diameter and length of tube •Composition of tube •Routine flushing regimen •Location of distal end of feeding tube relative to site of drug absorption •Need to keep drug separate from tube feeding formula •Size of oral syringe

How can cholestasis be prevented?

•Maintain PN at < 25 kcal/kg/day •Substitute lipids for 30% of energy from dextrose •Cyclical schedule •Dextrose < 5-7 gm/kg/day •Lipids < 1-2 gm/kg/day •Protein < 2 gm/kg/day •Metronidazole (Flagyl®)

What are the possible causes of hypercalcemia? - •Hypercalcemia Ca2+ > 10.4 mg/dL

•Malignancy •Hyperparathyroidism

What is the role of the clinical pharmacist regarding solid organ transplant patients?

•Monitoring drug levels •Monitoring for adverse drug reactions •Preventing drug-drug interactions that could cause adverse effects or rejection •Educating patients and other healthcare practitioners about the medications •Encouraging adherence

What are all of the GI complications that can occur with enteral nutrition?

•Nausea •Vomiting •Abdominal distention •Cramping •Aspiration •Diarrhea •Constipation

What are the 3 main types of fatty acids included in the fat and fatty acid component of enteral feeding?

•Polyunsaturated fatty acid •Medium chain triglyceride (MCT) •Long chain triglyceride

What are the risk factors for cholestasis?

•Sepsis •Presence of jejunostomy •GI surgery •Duration of PN and enteral fasting

What all does an assessment of a Na+ imbalance involve? Changes in Na+ usually reflect changes in water balance(!) - Not simply fixed by adding/removing Na+ from PN

•Serum osmolality •Volume status •Renal function •Comorbidities

What are the exclusions to self-treatment for weight loss?

•Severe obesity (BMI > 40) •Pregnancy •Breastfeeding •< 18 YO (need enough food to grow) •> 65 YO •CVD •Dyslipidemia •DM •HTN •Eating Disorders

What are all of the nonpharmacologic for obesity?

•Small, gradual eating & exercise changes •Maintain realistic weight loss and exercise goals •Eat low-calorie, balanced diet •Eat meals at table (do no other activity while eating-no television, no phone) •Do not skip meals (have regular eating schedule) •Eat slowly •Place fork or spoon down between bites of food •Leave food on plate •Wait 5 minutes before placing more food on the plate •Get rid of serving dishes from table after served 1st portion of food •Leave table after eating •Use smaller plates so servings do not seem too small •Start meal with low-salt, broth-based soup to feel full •Eat 5 or more servings/day of fruits and vegetables •Have healthy snacks available •Drink at least 8 glasses of noncaloric beverage (water)/day to feel full •Craving occurs - do something else (walk) - pass within minutes •Do not eat after dinner •Grocery shop after meal and use grocery list •Slowly increase exercise (goal: 60 minutes moderate-intensity most days of week) •Increase lifestyle activity (walk, stand more, park further away, climb stairs) •Limit time watching television, computer/internet, playing video games •Keep diary of weight, physical activity, caloric intake - track progress and success

What symptoms are associated with agranulocytosis?

•Sore throat •Fever •Malaise •Weakness •Chills

What are the criteria for medications to be administered via PN?

•Stable for at least 24 hours. •Drug dosage is constant during a 24 hour period. •Pharmacokinetic properties appropriate for continuous infusion. •Chemical and physical compatibility.

What are the different pediatric enteral feeding formulation classifications for ages 1-10 years old?

•Standard •Semi-elemental/elemental

What are all of the different formulation classification categories of enteral feeding in adults?

•Standard polymeric •High protein •High caloric density •Elemental •Peptide-based

What all should be monitored in patients receiving enteral nutrition?

•Vitals •Clinical assessment •GI tolerance •Tube placement •Labs

Name all of the vitamins included in a multi-vitamin (MVI)? (Per 10 mL)

•Vitamin A 3300 IU •Vitamin B1 3 mg •Vitamin B2 3.6 mg •Vitamin B6 4 mg •Vitamin B12 12.5 mcg •Vitamin C 100 mg •Vitamin D 200 IU •Vitamin E 10 IU •Vitamin K 150 mcg •Biotin 60 mcg •Folic acid 400 mcg •Niacinamide 40 mg •Dexpanthenol 15 mg

Name all of the trace elements included in PN?

•Zinc •Selenium •Copper •Chromium •Manganese

Which trace elements need to be adjusted in the following scenarios? Decrease ________________________ with elevated dbili > 2 mg/dL Decrease _________________________ in renal failure/no dialysis.

↓ Copper and manganese with elevated dbili > 2 mg/dL ↓ Selenium and chromium in renal failure/no dialysis

What BMI is obesity?

≥ 30 kg/m2

What percentile for child BMI is classified as obese?

≥ 85th percentile Underweight = < 5th percentile