Heme

folate vs. B12 deficiency anemia

* B12: macrocytic, neuro sx * folate macrocytic, no nuero sx, glossitis; more associated with ETOH

key points about UTI in children

* MCC is ecoli • The possibility of UTI should be considered in any child younger than 2 years of age with unexplained fever! (Fever without a source) • 3 most useful components of the urinalysis are leukocyte esterase test(waste products the WBCs make), nitrate test(waste product of gram (-) bacteria) and urine microscopy.

Alport syndrome

* progressive, hereditary nephritis, defect in type IV colagen in glomerular basement membrane; sx: hypertension, hematuria, hearing loss, ocular abnormalities

ALL vs. AML vs. hodgkins vs. non-hodkins

1. ALL: MC childhood cancer; fever bone pain, pallor, bruising, hepatosplenomegaly, lymphadenopathy; see leukemic blasts on CBC, confirm lymphoblasts replace bone marrow; tx via induction, consolidation, maintenance; mass spread to testes 2. AML: sx: fever, hepatosplenomegaly, vs. gingival hypertrophy, bleeding; no testicular; dx via aur rods tx. via marrow tranplant 3. hodgkins: a lymphoma, not a leukemia; associated with EBV infection (mono), occurs in older children, cervical and supraclavical lymphadenopathy, rare abdominal findings, painless; Reed Sternberg cells 4. non hodkins: more painful lymphoma, occur in younger kids, abdominal involvement, *anterior medial mass, superior vena cava syndrome, airway obstruction; Mc in children is Burkitt lymphoma, enlargement of tonsils, adenoid, or preyer patach; abdominal mass

AML vs. ALL

1. ALL: malignant proliferation of lymphocytes/lymphoblasts * children <15 *o fever, infxn, pallor, purpura and bone pain, hepatosplenomegaly; * immature blasts on bone marrow bx; anemia, thombocytopenia; leuk blasts; marrow replaced by lymphoblasts * relapse in testes and CNS 2. AML * Malignant proliferation of immature myeloid cells (granulocytes, monocytes, RBC's, megakaryocytes) o fevor, pallor, purpura and pain (acute or insidious onset)→ gingival hypertrophy, no testes involvement o soft tumors→ greenish hue→chlorotomas o Bone marrow bx→ **AUER RODS**

* have cold and then start bruising * associated with hematuria and melana 2. what should vs. not be used for tx

1. ITP 2. • Treatment only warranted when platelets drop below 20,000K • Steroids USE ONLY if diagnosis confirmed ITP and not early leukemia. Bone marrow will show increase megakaryocytes • Platelet transfusions NOT helpful • Transfusions with fresh frozen plasma or cryoprecipitate NOT used • Intravenous gamma globulin (sandoimmune)

1. MCC of airway & lung infxn in infants + kids • Bronchioltitis > viral PNA→ children < 1yo • 98% of children < 2yo have had 2. sx 3. PPx, who get it

1. RSV, Broncholitis 2. • follows URI→tachypnea + wheezing +cough • copious clear rhinorrhea • labored breathing • cough (croupy) followed by wheeing and rapid breathing 3. • Hand hygiene, Breastfeeding, Avoid tobacco exposure • Palivizumab/Synagis (x high risk pt's) very $$$

1. normal MCV, low hmg/hct, elevated ferritin, decreased serum iron, low TIBC 2. microcytic, low ferritin, serum iron and increased TIBC 3. micocytic and normal iron studies

1. anemia of chronic dz; adequate iron stores and inability to properly use them 2. iron deficiency anmia 3. thalassemia

o Hallmark: paroxysmal cough > 2wks + high pitched whoop 1. cause 2. stages 4. tx

1. cause: pertussis; • MCC = Bortadella pertussis (gm - coccobacillus)→highly contagious 2. stages: o Prodrome catarrhal stage→malaise, cough, coryza, anorexia, low grade fever, injection o Paroxysmal stage→bursts of deep high-pitched inspiratory "whooping" cough • Hacking night cough • Cough can last >10wks→"the cough of 100 days" • +/- apnea, flushing, cyanosis o Convalescent stage→sx subside > 4wks of onset 4. tx: o Erythro, Azithro, Clarithro o Bactrim

1. hemophilia A vs. B 2. labs/tx 3. PT vs. PTT

1. hemophilia A vs. B • Hemophilia A *abnormal factor 8 • Hemophilia B: Christmas dz - abnormal factor 9 2. labs/tx: PTT is above normal; PT is normal • Mild cases of hemophilia A can be treated w/ desmopressin acetate (DDAVP) 3. PTT measures the common pathway and the intrinsic pathway (XII,XI, IX, VIII)

non hodgkins vs. hodgkins lymphoma

1. hodkins: * older children o Hodgkin's→PAINLESS ENLARGED MASS/LAD→ MC in neck • Pain in lymph node following EtOH ingestion • Fever, night sweats, wt loss +/- pruritis • +/- SOB, cough * REED-STERNBERG CELLS (Hogkin's) 2. non hodgkins * younger children , Burkitt's MC non-Hodgkin's lymphoma in childhood (assc w/ EBV) o Non-Hodgkin's→ PAINFUL abd. mass + rapidly extending neck mass • May present in the tonsil, appendix, mediastinum, bone, or kidney • Burkitt lymphoma→ abd mass + abd fullness

1. Clinical Manifestations: low grade fever, malaise, headache, anorexia, and abdominal pain Parotitis Orchitis Meningoencephalitis 2, tx

1. mumps 2. tx is supportive

1. o Abd pain + mass (hard, smooth, non-tender), periorbital ecchymoses o Racoon eyes 2. Wilms tumor key words

1. neuroblastoma 2. Wilm tumor * - Aniridia(absence of iris), *hemihypertrophy(one side of the body or part of one side is larger than another), *Beckwith Wiedeman(growth d/o w/ large body size & large organs) * flank mass, firm tender, smooth; CVAT

1. JAK2 mutation, high platelet count, high hematocrit 2. tx

1. polycythemia vera 2. phebotomy and ASA

1. Painful swelling of the hand, feet, fingers and toes - Microcapillaries are clogged leading to infarction & necrosis - AKA: Dactylitis • Acute chest syndrome (infiltrate, severe hypoxemia) • Splenic sequestration (massive splenomegaly) 2. management/tx

1. sickle cell 2. • Penicillin prophylaxis is very important b/c fxnal asplenia occurs as early as 5-6 months (This begins in infancy and continues into adolescence) • Keep up to date on immunizations • Pneumococcal vaccine (Prevnar) available for all children under 2 years of age to prevent invasive pneumococcal infxn.

1. sx of iron deficiency anemia in infants 2. characteristic of alpha thalassemia minor and beta thalassemia major 3. how is sideroblastic anemia dx

1. sx of iron deficiency anemia in infants: * low birth weight * hemoglobin about 8.0mg/dL *developmental delay 2. characteristic of alpha thalassemia minor and beta thalassemia major: * mild anemia * marked micocytic 3. how is sideroblastic anemia dx: is far less common than iron deficiency anemia and typically diagnosed based on the finding of ringed sideroblasts visible on peripheral smear.

1. what is acute chest syndrome; what disorder causes it 2. what is tx, what are the parameters for tx 3. what is prophylactic tx for above

1. what is acute chest syndrome; what disorder causes it: oPatients with sickle cell disease are prone to acute chest syndrome resulting from sickling of cells within the lung that typically presents with chest pain, tachypnea, cough, fever, and oxygen desaturation. 2. what is tx, what are the parameters for tx: Transfusion is indicated when the hematocrit drops below 30% or oxygen saturation drops below 90%. 3. what is prophylactic tx for above: Hydroxyurea (D) is used prophylactically to reduce the frequency of sickle cell crises and/or acute chest syndrome.

1. when does physiological anemia begin 2. labs for iron def anemia: MCV, MCH, retic, TIBC, ferratin 2. tx

1. when does physiological anemia begin: • Physiologic anemia occurs at 8-12 weeks in full-term infants and 6-8 weeks in the premature infant. 2. labs for iron def anemia: • MCV, MCH are low, low retic count, high TIBC (don't high iron to bind), low ferritin level 2. tx • Prescribe elemental iron 3-5 mg/kg/d • Take with vitamin C source. Milk will inhibit the absorption of iron. • Repeat measurement CBC and retic count in 1 month • Indices improve continue therapy for 2 more months to replace iron stores

A three-year-old girl is brought to the ED because of stridor that developed over 24 hours and is associated with fever (102.9ºF) and dysphagia. PE reveals a child who is sitting up, drooling and exhibiting inspiratory stridor. Her immunization status unknown. Of the following, the MOST likely finding to support the correct diagnosis is

Anteroposterior neck radiography showing subglottic narrowing

A 15 year-old male is seen in the office for a sports physical. A random UA shows 1+ protein on dipstick. The urine specific gravity is 1.010. The most appropriate next step is: Obtain serum creatinine Obtain 24-hr collection of urine Check first morning void for protein Obtain renal ultrasound Tell patient to refrain from contact sports

Check first morning void for protein

Croup 1. cause 2. sx 3. dx/imaging

Croup 1. cause • MCC = parainfluenza, influenza type A, RSV • MC = Winter & spring 2. sx • "barky" or "seal-like" or "brassy" cough @ night • Inspiratory stridor • Hoarseness • Severe resp. distress→hypoxia 3. dx/imaging: o AP Neck XR→ "Steeple" sign (subglottic narrowing)→ r/o epiglottis

Cryptorchidism 1. why tx 2. when to tx

Cryptorchidism 1. why tx: Testicular neoplasm is 20-45 times greater in undescended testes with an overall incidence of 4-11%. Seminoma is the most common malignancy 2. when to tx: by age 2

medical tx for enuresis

DDVAP (DOC)→ x special occasions only, can cause anuria • Imipramine (need EKG)→ can cause cardiac SE's

A 4 year-old boy is brought to the pediatrician's office because he woke up with swelling around his eyes. On PE the pediatrician notes scrotal edema, erythematous throat and BP of 80/50. The next diagnostic test should be:

Dipstick the urine for protein because the most likely diagnosis is minimal change nephrotic syndrome

For all otherwise healthy children, you would MOST likely recommend the use of prophylactic antibiotics for A. Child care contacts of a child who has an invasive HIB infection B. Child care contacts of a child who has pneumococcal meningitis C. Child care contacts of an infant who as pertussis D. Classroom contacts of a child who has scarlet fever E. Classroom contacts of an adolescent who has meningococcal meningitis

E. Classroom contacts of an adolescent who has meningococcal meningitis

A 10 year-old girl has a rash for four days with no other symptoms. She is taking no other medications. PE reveals erythematous cheeks and a lacy, reticulated erythema involving the extremities. Cause, AKA

Erythema infectiosum, caused by parvovirus, AKA fifth disease

• A 32 year-old obese woman presents to the clinic complaining of swelling, warmth, and redness of her left lower extremity. Doppler ultrasound confirms a deep venous thrombosis is the greater saphenous vein. Which of the following best describes the most likely pathophysiology underlying her thrombophilia?

Factor V Leiden is a common genetic mutation that results in resistance to activated protein C.

A three-year-old boy has a 2-day hx of fever, irritability, decreased appetite, and a rash. Findings of PE include several 3-4mm ulcers on the tongue and buccal mucosa, but the gums are spared. There are elliptical vesicles with surrounding erythema on the feet.

Hand, foot and mouth disease

A 3-year-old boy presents with a 2-day hx of pain in both knees & a rash. He has been afebrile. PE reveals abdominal tenderness, decreased range of motion and mild edema of the knees, and a purpuric rash over the lower extremities.

Henoch-Schonlein purpura

Henoch-Schonlein purpura 1. cause 2. sx

Henoch-Schonlein purpura 1. cause • Def = IgA mediated vasculitis→small bv inflamm + leukocytic infiltration of tissue + hemorrhage + ischemia→ Follows URI 2. sx: • palpable purpura/rash (LE + buttock) • crampy abd. pain/bowel angina • arthritis→ankles + knees • nephritis (w/in 3 mo of rash) hemorrhage→intussusceptions • HTN

In addition to vascular thrombosis, the MOST likely complications observed in children who have minimal-change nephrotic syndrome are:

Hypercholesterolemia

1. Recurrent episodes of gross hematuria (coca cola urine) usually 1-2 days after a URI or GI infection. 2. Gross hematuria (tea or coke colored urine) 2 weeks after acute infection with HTN, edema, and impaired renal function 3. Familial form of nephritis with neurosensory hearing loss and slow progression to renal failure

IGa nephropathy or Berger's syndrome 2. post strep glomeronephritis 3. alports syndrome

ITP vs. HSP vs. TTP

ITP: follows viral infection, bleeding, bruising, labs: large, sticky platelets; supportive; if platelets <20K, treat with IVIG or coticosteroid TTP: is a rare disorder of the blood-coagulation system, causing extensive microscopic clots to form in the small blood vessels throughout the body.[2][3] These small blood clots, called thrombi, can damage many organs including the kidneys, heart and brain. In the era before effective treatment with plasma exchange, the fatality rate was about 90%. * sx: thrombocytopenia (purpura), anemia, fever, neuro sx HSP: IgA vasculitis, palpable purpura, renal insufficiency, arthirits, abdominal pain; platelet count is normal

A 6 year-old presents for evaluation of hematuria. Over the last several years, he has had approximately 6 episodes of hematuria, usually associated with URI symptoms. The most likely diagnosis is: Alport's syndrome Idiopathic nephrotic syndrome Wilm's tumor IgA nephropathy Hemophilia

IgA nephropathy

You are concerned because varicella is the MOST commonly identified risk factor in children who have

Invasive group A streptococcal infection

meningitis; CSF finding if bacterial vs. viral

Meningitis 1. bacterial CSF: (WBC is H); proteine is H; glucose is L 2. viral: WBC (<1000); protein (20-100); glucose is normal

A 6-year-old boy presents with a 24-hour hx of fever, malaise and bruising. PE reveals and ill-appearing child whose temperature is 103.1ºF. He has widespread petechiae and areas of palpable purpura, especially on the buttocks and lower extremities. The hemoglobin is 10.5 g/dL, WBC is 18.5K, and platelet count is 25K

Meningococcemia

A four-year-old girl developed a fever to 104ºF and a diffuse, raised purpuric rash this morning. Lab studies reveal: WBC 5.1K, Hgb 11.8, plts 189K, PT 11.8sec, PTT 28 and fibrinogen 250.

Meningococcemia

A six-year-old boy presents with a 24-hour hx of fever, malaise and bruising. PE reveals an ill-appearing child whose temperature is 103.1ºF. He has widespread petechiae and areas of palpable purpura, especially on the buttocks and lower extremities. The Hgb is 10.5, WBC is 18.5K, and platelet count is 25K.

Meningococcemia

Minimal change dz 1. cause/definition 2. sx

Minimal change dz 1. cause/definition: • Def: Fusion of glomerular epithelial foot processes 2. sx: o SUDDEN WEIGHT GAIN (over 2 wks) o orbital EDEMA(MC in AM) + ascites + pleural effusion +/- scrotal/labia edema • mild puffiness around the eyes in AM o Nephrotic sx

Minimal change dz 1. labs 2. tx 3. what does it put you at risk for

Minimal change dz 1. labs o UA/dipstick→ > +1 protein/, hypoalbuminemia (<1.5g/dL), hyperlipidemia o 24 hr urine→ massive proteinuria (> 4g-5g/d) 2. tx o corticosteroids (prednisone) 2mg/kg/day 4-6 weeks, tapering for another 4-6 weeks o 25% albumin (x severe edema) 1g/kg 25% +/- diuretics and fluid o salt restriction 3. what does it put you at risk for ↑ risk for infxn (cellulitis, PNA + peritonitis)

Urinalysis obtained during a health supervision visit of a 10-year-old boy reveals 2+ protein. The remainder of the urinalysis is nl. Results of family and personal medical hx as well as PE of the boy are negative. Of the following, the BEST next step is to: Arrange consultation with a nephrologist Examine the urine of family members Obtain a 24-hour urine sample for protein quantification Obtain renal ultrasonography Perform a urinalysis on the first morning void

Perform a urinalysis on the first morning void

• A 48-year-old man presents to the clinic for a routine employment physical. The patient is asymptomatic. A pre-employment CBC reveals hemoglobin of 13.2g/dl, hematocrit of 39.5%, and MCV of 60.6 fL. Subsequent iron studies, hemoglobin electrophoresis, and sickle cell screening are "normal." After the labs are reviewed, the patient states "they are always normal." What is the most likely diagnosis?

The patient has a mild anemia with pronounced microcytosis consistent with alpha thalassemia minor. Alpha thalassemia minor hemoglobin electrophoresis reveals normal results.

labs for 1. alpha thalassemia minor 2. iron deficiency 3. beta thalassemia major 4. sickle cells

The patient has a mild anemia with pronounced microcytosis consistent with alpha thalassemia minor. Alpha thalassemia minor hemoglobin electrophoresis reveals normal results. Patients with anemia of chronic disease (A) and iron deficiency anemia (D) have abnormal iron studies; while patients with beta thalassemia major (C) and sickle cell disease (E) have abnormal hemoglobin electrophoresis results

A 12-year-old girl complains of a sore throat, headache, and abdominal pain. On PE, her temperature is 100.6ºF, her pharynx appears erythematous with exudate on both tonsils, and she has bilateral tender anterior cervical adenopathy. A number of students in her class have been absent with sore throats. Of the following, the MOST appropriate diagnostic specimen to obtain at this time is

Throat swab for rapid streptococcal antigen testing

A 15-year-old girl has had malaise, myalgias, fever, diarrhea, rash and vaginal discharge for two days. PE reveals a temperature of 102.2ºF and BP of 80/56. She is alert but disoriented and has diffuse cutaneous erythema that is accentuated in the flexures. Lab eval demonstrates WBC count of 16.8K, Hgb of 12.6, and Plt count of 92K.

Toxic shock syndrome

o Initially→ low grade fever, malaise, vomiting o Followed by→ worse malaise, weakness, vomiting, abd pain o Subquently→watery diarrhea, hypoTN, oliguria o Convolescence→desquamation of skin→palms, soles, fingers, toes +/- hair & nail loss o Diffuse erythematous macular rash →sunburn-like or scarlatiniform

Toxic shock syndrome

A 5-year-old otherwise healthy AA girl, who was fully toilet trained at age 3, begins to wet her bed. She complains of some dysuria, but has normal findings on PE. Of the following, the MOST likely etiology for this child's enuresis is: Constipation with rectal distension Diabetes mellitus Nocturnal enuresis Sickle cell trait Urinary tract infection

UTI

Results of a routine urinalysis on a 10-year-old boy include: S.G. 1.015, pH 6.0, 2+ bld, neg protein, 10-25 RBC's, & 0-2 WBC's. His hx is negative for gross hematuria, abd or flank pain, edema, dysuria, trauma or other evidence of bleeding. There is no family hx of renal dz, & he has not taken any meds, including antibx, since 3yo. VS & the results of his PE are nl. Of the following, the MOST important next step to determine the cause of the microscopic hematuria is to:

Wait 2 weeks and obtain another urinalysis

What tests are used to confirm the following: 1. pernicious anemia 2. hemolytic anemia 3. serum folate levels 4. what is schilling test associated with

What tests are used to confirm the following: 1. pernicious anemia: anti-intrinsic antibodies factor 2. hemolytic anemia: Coomb test 3. serum folate levels: macrocytic anemia, but not dx PA 4. what is schilling test associated with: was once used to dx PA, but no used b/c no reliable radiolabled human intrinsic factor

o erythematous, hot, tender, ill-defined, edematous plaques o lateral margins indistinct o constitutional sx→F/C, maliaise o regional LAD o Pitting edema o staph→localized infxn + purulent center o strep→infxn spreads rapidly + lymphangitis 1. complication of above

cellulitis 1. periorbital infection: proptotic air sign

common types of glommerulonephritis in children: post-streptococcal glomerulonephritis vs. Iga (Berger's disease) vs. henoch-Schonlien purpura (HSP)

common types of glommerulonephritis in children: post-streptococcal glomerulonephritis vs. Iga (Berger's disease) vs. henoch-Schonlien purpura (HSP) 1. post-streptococcal glomerulonephritis: develops 8-14 days post Group A hemolytic strep; hematuria, HTN (fluid overload), low C3; dx with ASO; supportive management 2. IgA/Burger's disease: occurs in Asian, Native american; recurrent hematuria (chronic), respiratory infection; renal failure; elevated IgA; manage me with ACE, steroid, 3. Henoch-Schonlein purpura: HSP, igA mediated vasculitis, palpable purpura, abdominal pain, arthritis, and hematura; self limited

• sudden onset of HIGH fever • sore throat o Lateral XR of neck→ "thumb sign"• difficulty swallowing • drooling • "hot potato voice" CAUSE

epiglottis Haemophilus influenzae type B, although some cases are attributable to Streptococcus pneumoniae, Streptococcus agalactiae, Staphylococcus aureus, Streptococcus pyogenes, Haemophilus influenzae, and Moraxella catarrhalis.

etiology/pathophys of each 1. schizocytes 2. Howell-Jolly bodies 3. basophilic stippling 4. Burr cells

etiology/pathophys of each 1. schizocytes * look like small cell fragments; intravascular hemoysis, hemolytic anemia 2. Howell-Jolly bodies * nucleus seen inside cell; lack of removal of nuclear material b/c spleen not work well or absent; seen in sickle cell, hemolytic anemia, megalobastic anemia, 3. basophilic stippling * numerous ribosomes seen in cells; seen in sideroblastic anemia, beta and alpha thalassemia, TTP 4. Burr cells RBC that not have smooth round circumference; look like waves; due to change in pH

Small yellow ulcers develop on the tongue, soft palate, uvula and anterior tonsillar pillars Cutaneous lesions begin as red macules on the palmer aspect of the hands and fingers, the plantar surfaces of the feet If no cutaneous lesions, then disease is called HERPANGINA

hand, foot, mouth disease; caused by coxsackie A16

Present with high fever, irritability, anorexia and sore mouth Infants and toddlers drool copiously Gingivae are red, edematous and friable Yellow ulcerations with red halos are seen mainly on buccal mucosa, tongue and gingivae

herpes simplex 1

Which one of the following is not a common manifestation of classic pertussis? Paroxysmal cough High fever No systemic manifestations Lymphocytosis Post-tussive vomiting

high fever

meningicoccemia a. cause b. presentation c. treatment

meningicoccemia a. cause: nisseria meningitidis b. presentation: include fever, nausea, myalgia, headache, arthralgia, chills, diarrhea, stiff neck, and malaise. Later symptoms include septic shock, purpura, hypotension, cyanosis, petechiae, seizures, anxiety, and multiple organ dysfunction syndrome. c. treatment: 3rd generation celasporin, cefotaxime or centriaxone

• Tachy, HypoTN • Petichae, purpura, erythematous macular rash (usually develops rapidly) • ⊕ Kernig sign ( intrathecal pressure) • ⊕ Brudzinski sign ( intrathecal pressure) • Bulging fontanel or diastasis (widening) of sutures * nuchal rigidity

meningitis

A 3-month-old girl presents with a 2 day hx of a temp of 103ºF, irritability, decreased oral intake, & vomiting. She has not received any of her IZ. On PE, she appears lethargic & has a bulging fontanelle. You suspect meningitis & perform a LP. Results of the LP are: WBC 800 (88% neutrophils, 12% monocytes), RBC 2, glucose 15, and protein 150.

meningitis due to pneumococcal

A 48-year-old female presents with a gradual progression of fatigue, pallor, and dyspnea on exertion over the last few months. Initial CBC results show a hemoglobin of 10.2 mg/dL, hematocrit of 31%, an MCV of 74 fL, and a reticulocyte count of 0.1%. Which of the following is the most likely pathophysiologic mechanism responsible for her anemia?

o : The patient most likely has an underlying iron deficiency anemia (IDA), which requires confirmation and then evaluation for sources of chronic blood loss. IDA results from defective hemoglobin production. o Acute blood loss (A) would present more acutely and with normal hemoglobin, hematocrit, and MCV until hemodilution occurs and lowers the hemoglobin concentration and hematocrit. Defective bone marrow/stem cell function (B) tends to produce normocytic red blood cells. Defective DNA production (C) results in failure of RBC maturation and macrocytosis (elevated MCV). Hemolysis (E) is less likely than IDA, but can be confirmed or ruled out through the assessment of iron studies, the reticulocyte count and further hematologic labs as necessary.

• A 63 year-old woman complains of fatigue, loss of appetite, a sore-red tongue, paresthesias of her feet and hands, and unsteadiness of her gait. Which of the following tests is be used to confirm the patient's suspected diagnosis?

o A diagnosis of pernicious anemia (PA) can be supported through the presence of anti-intrinsic factor antibodies. o Antiparietal (B) cell antibodies aren't associated with the development of PA. The Coomb's test (C) is used in the evaluation of hemolytic anemias. Serum folate (D) levels are beneficial in evaluating macrocytic anemias, but will not establish a diagnosis of PA. The Schilling test (D) was once commonly used to diagnosis PA, but is no longer available due to lack of available radiolabeled human intrinsic factor.

• A 73 year-old woman with a past medical history of diabetes and hypertension presents to the clinic complaining of progressive fatigue. An initial CBC reveals a hemoglobin 10.1 mg/dL and MCV of 91 fL. Which of the following best describes the cause of the abnormality pictured in the patient's peripheral smear? looks like flowers

o Burr cells are present on the peripheral smear and are associated with uremia; look like flowers, o Howell-Jolly bodies result from the lack of removal of nuclear material due to an absent or non-functioning spleen (A). Macrocytosis results from failure of nuclear maturation (B) commonly secondary to vitamin B12 or folate deficiency. Intravascular hemolysis (C) creates schizocytes, and lead intoxication (D) or thalassemia cause basophilic stippling.

A 52-year-old female with a past medical history of dyslipidemia, GERD, hypothyroidism, and osteoarthritis presents with a gradual progression of fatigue and pallor over the last few months. Initial CBC results show a hemoglobin of 10.7 mg/dL, hematocrit of 33%, an MCV of 117 fL, and a reticulocyte count of 0.0%. Which of the following medications should be considered as a potential cause of her anemia?

o Cholestyramine is associated with folate malabsorption and may rarely be a medication cause for macrocytic anemia. o Celecoxib (A), levothyroxine (C), omeprazole (D), and ranitidine (E) are not strongly associated with vitamin B12 or folate deficiency leading to macrocytic anemia

• A 22-year-old non-obese female presents to the clinic with sign and symptoms of deep vein thrombosis (DVT). She denies recent trauma, prolonged immobilization, smoking, use of oral contraceptives, or changes in her health. Her last menstrual period was one week ago. Which of the following is the most likely diagnosis that contributed to the development of her DVT?

o Factor V Leiden mutation is one of the most common genetically inherited prothrombotic states.

MCC of inherited prothrombotic states that leads to DVT in young females; what is underlying pathophys

o Factor V Leiden mutation is one of the most common genetically inherited prothrombotic states. o Factor V Leiden is a common genetic mutation that results in resistance to activated protein C.

• A 41-year-old alcoholic male, who lives primarily on the streets, appears pale, cachectic, and mildly icteric. He is complaining of several weeks of increasing fatigue. Laboratory findings note an elevated MCV of 128. What other physical finding would most support the diagnosis for megaloblastic anemia?

o Features of folate deficiency are similar to vitamin B12 deficiency. However, there are none of the neurologic abnormalities associated with vitamin B12. Glossitis is the only non-neurologic finding in the PE that would support folate deficiency. Alcoholism and poor dietary intake also support the diagnosis of folate deficiency.

• A 12 year-old African-American male with a history of sickle cell disease presents to the clinic for routine follow up. A peripheral smear is performed. Which of the following best describes the cause of the abnormality pictured in the patient's peripheral smear?

o Howell-Jolly bodies are noted in the peripheral smear and result from the lack of removal of nuclear material due to an absent or non-functioning spleen. o Failure of nuclear maturation (B) produces macrocytosis, intravascular hemolysis (C) leads to the creation of schizocytes, lead intoxication (D) and thalassemia cause basophilic stippling, and uremia (E) is associated with Burr cells.

• An 11-month-old African-American male presents to the pediatric office with lethargy, jaundice and splenomegaly. A CBC reveals hemoglobin of 8.0 mg/dl and a hematocrit of 25%. Peripheral smear appearance is available below. Which of the following treatments will reduce hemolysis and increase hemoglobin levels for this patient?

o Hydroxyurea directly reduces hemolysis and increases levels of fetal hemoglobin and reduces complications and transfusion frequency. o Sickle cell disease can be cured in 80% of individuals who receive a suitable transplant (A), but this procedure carries great risk and donors are difficult to identify.

An 8-month-old male presents to the clinic due to irritability, fatigue and parental concerns about developmental delays. He is fifth percentile for weight and 33rd percentile for height. An office based hemoglobin level is 8.4 mg/dL. Which of the following is the most likely diagnosis?

o Infants with poor nutrition are at greatest risk of iron deficiency anemia between the ages of 6 and 24 months. o Alpha thalassemia minor (A) and beta thalassemia minor (B) present with a markedly microcytic, but mild anemia. Folate deficiency (C) due to inadequate nutrition in the absence of co-morbid disease is rare. Sideroblastic anemia (E) is far less common than iron deficiency anemia and typically diagnosed based on the finding of ringed sideroblasts visible on peripheral smear.

A 68-year-old female with a past medical history of diabetes, dyslipidemia, hypertension, and gout presents with a gradual progression of fatigue and pallor over the last few months. Initial CBC results show a hemoglobin of 10.4 mg/dL, hematocrit of 32%, an MCV of 112 fL, and a reticulocyte count of 0.1%. Which of the following medications should be considered as a potential cause of her anemia?

o Metformin is associated with vitamin B12 deficiency and may rarely be a medication cause for macrocytic anemia. o Nicotinic acid (E) administration may help reduce the risk of vitamin B12 deficiency secondary to nicotinic acid deficiency. Allopurinol (A), atorvastatin (B), and lisinopril (C) are not strongly associated with vitamin B12 or folate deficiency.

•A 63 year-old woman developed severe thrombocytopenia after undergoing her first treatment of a cisplatin-based chemotherapy regimen. Which of the following agents may be administered to prevent thrombocytopenia if the patient continues her cisplatin-based regimen?

o Oprelvekin, or recombinant interleukin-11, is a megakaryocyte growth factor that promotes platelet production. o Erythropoietin (B) and darbepoetin alfa (A) are both stimulators of red blood cell production, while filgrastim (C) and sargramostim (E) stimulate granulocyte production.

What is used to stimulate 1. platelet production 2. RBC 3. granulocytes

o Oprelvekin, or recombinant interleukin-11, is a megakaryocyte growth factor that promotes platelet production. o Erythropoietin (B) and darbepoetin alfa (A) are both stimulators of red blood cell production, while filgrastim (C) and sargramostim (E) stimulate granulocyte production. •

• A 48-year-old man presents to the clinic for a routine employment physical. The patient is asymptomatic. A pre-employment CBC reveals hemoglobin of 13.2g/dl, hematocrit of 39.5%, and MCV of 60.6 fL. Subsequent iron studies, hemoglobin electrophoresis, and sickle cell screening are "normal." After the labs are reviewed, the patient states "they are always normal." Which of the following is the most appropriate management for this patient?

o The patient has a mild anemia with pronounced microcytosis consistent with alpha thalassemia minor and requires reassurance. o Alpha thalassemia minor hemoglobin electrophoresis reveals normal results. Patients with alpha thalassemia are often inappropriately advised to take iron supplements (B) and don't require iron, folic acid (C) or vitamin B12 supplements unless a coexisting deficiency is confirmed. Referral to an oncologist (E) and a bone marrow aspirate (A) aren't necessary for this patient.

A 18 year-old man presents to the clinic with a lifelong history of transfusion dependent anemia and iron chelation therapy. Which of the following hemoglobin electrophoresis findings is most likely present?

o The patient history is most consistent with beta thalassemia major that presents with increased HbA2 and/or HbF levels.

• A 78-year-old obese male smoker presents with a gradual progression of fatigue and pallor over the last few months. Initial CBC results show a hemoglobin of 10.4 mg/dL, hematocrit of 32%, an MCV of 74 fL, and a reticulocyte count of 0.1%. Iron studies reveal an elevated ferritin, decreased serum iron and TIBC. Which of the following is the most likely cause of this patient's anemia?

o The patient most likely has an underlying chronic disease resulting in anemia, as evidenced by his adequate iron stores and inability to properly use that iron for RBC production. o Iron studies in patients with thalassemia (A and B) are typically normal. Iron deficiency anemia (E) is confirmed with low ferritin, serum iron, and increased TIBC.

A 48-year-old female presents with a gradual progression of fatigue, pallor, and dyspnea on exertion over the last few months. Initial CBC results show a hemoglobin of 10.2 mg/dL, hematocrit of 31%, an MCV of 74 fL, and a reticulocyte count of 0.1%. After treatment for her anemia begins, which of the following diagnostic studies can be ordered first (earliest) to demonstrate a response to therapy?

o The patient most likely has an underlying iron deficiency anemia (IDA) which requires confirmation and then evaluation for sources of chronic blood loss. IDA is treated with iron replacement (e.g., ferrous sulfate) and response to therapy can be assessed in 7-10 days via a reticulocyte count.

A 28-year-old female presents with a gradual progression of fatigue and pallor over the last few months. Initial CBC results show a hemoglobin of 10.4 mg/dL, hematocrit of 32%, an MCV of 112 fL, and a reticulocyte count of 0.1%. Which of the following is the most likely pathophysiologic mechanism responsible for her anemia?

o The patient most likely has an underlying vitamin B12 or folate deficiency resulting in macrocytosis (MCV of 112 fL). o Defective DNA production (C) results in failure of RBC maturation and macrocytosis (elevated MCV). Acute blood loss (A) would present more acutely and with normal hemoglobin, hematocrit, and MCV until hemodilution occurs and lowers the hemoglobin concentration and hematocrit. Defective bone marrow/stem cell function (B) tends to produce normocytic red blood cells. Microcytic, hypochromic anemia results from defective hemoglobin production (D). Hemolysis (E) is less likely than vitamin B12 and folate deficiency, but can be confirmed or ruled out through the assessment of the reticulocyte count (increased reticulocyte will increase the MCV and be inconsistent with a nutritional deficiency) and further hematologic labs as necessary.

• A 73-year-old, male with a 30 pack-year smoking history presents to the clinic with complaints of headache and dizziness. Labs reveal a hemoglobin of 21.3 g/dl, hematocrit of 63%, and platelet count of 498,000. He tests positive for the JAK2 mutation. Which of the following should be recommended to this patient to prevent secondary complications from his diagnosis?

o The patient most likely has polycythemia vera and is at risk for thrombotic events that can be reduced through the use of daily aspirin. In addition, he should be counseled on smoking cessation if he is still currently smoking. o The patient should not take iron supplements (B) as they compete with phlebotomy therapy that seeks to reduce iron stores.

1. s/sx of B12 deficiency 2. s/sx of anemia d/2 chronic kidney dz 3. iron deficiency 4. folate deficiency

o The patient most likely has vitamin B12 deficiency, as evidenced by the macrocytic anemia and neurological signs and symptoms. The patient should be treated with vitamin B12. o Anemia secondary to chronic kidney disease (A) typically presents as a normocytic, normochromic anemia. Iron deficiency anemia (B) presents as a microcytic, hypochromic anemia. Folate deficiency (C) typically presents as a macrocytic anemia, but without the classic neurological symptoms consistent with vitamin B12 deficiency.

• A 65-year-old man presents to the clinic complaining of fatigue, weakness, paresthesias, and an unsteady gait. Physical exam reveals decreased vibratory sensation and lack of proprioceptive feedback, noted on Romberg testing. CBC reveals hemoglobin of 10.7 g/dl, hematocrit of 32.1% and MCV 117.6 fL. Which of the following is the most appropriate treatment?

o The patient most likely has vitamin B12 deficiency, as evidenced by the macrocytic anemia and neurological signs and symptoms. The patient should be treated with vitamin B12. o Anemia secondary to chronic kidney disease (A) typically presents as a normocytic, normochromic anemia. Iron deficiency anemia (B) presents as a microcytic, hypochromic anemia. Folate deficiency (C) typically presents as a macrocytic anemia, but without the classic neurological symptoms consistent with vitamin B12 deficiency.

A 33 year-old man with long-standing, recurrent, abdominal pain is diagnosed with anemia secondary to vitamin B12 deficiency. Which of the following gastrointestinal disorders most likely explains his diagnoses?

o Vitamin B12 complexed with intrinsic factor produced by gastric parietal cells is absorbed in the ileum, which is commonly affected by Crohn's disease (B). Achalasia (A) can lead to malnutrition and anemia secondary to vitamin B12, folate, and iron, but presents as difficulty swallowing, chest pain, and heartburn. Duodenal ulcer (C) and ulcerative colitis (E) may result in chronic blood loss and the development of iron deficiency anemia. Pancreatitis (D) causes malabsorption of fat soluble vitamins and may also lead to vitamin B12 deficiency in rarer instances.

A 5 year-old girl presents to the clinic with her mother who is concerned about her daughter's energy level since starting full day kindergarten. CBC reveals a hemoglobin of 12.3 g/dl, hematocrit of 36%, MCV of 62 fL, and an MCHC of 34 g/dL. Serum ferritin levels are normal. What is the most likely hemoglobin electrophoresis results for this patient?

oAlpha thalassemia presents with a normal hemoglobin electrophoresis, since all adult hemoglobin are alpha-containing and affected equally. oBeta thalassemia major may present with increased HbA2 and/or HbF levels (C and D). Beta thalassemia minor typically presents with increased HbA2 (C).

electrophoresis for: 1. Alpha thalassemia 2. beta thalassemia major 3. beta thalassemia minor

oAlpha thalassemia presents with a normal hemoglobin electrophoresis, since all adult hemoglobin are alpha-containing and affected equally. oBeta thalassemia major may present with increased HbA2 and/or HbF levels (C and D). Beta thalassemia minor typically presents with increased HbA2 (C).

•A 22-year-old patient with sickle cell disease presents to the emergency department complaining of chest pain, fever, and non-productive cough. On physical exam his temperature is 100.6°F, BP is 144/88, pulse is 110, respiratory rate is 24, and pulse oximetry is 84%. CBC shows a WBC of 11,500, hemoglobin of 8.3%, and hematocrit of 28%. What is the most critical/emergent treatment for this patient?

oPatients with sickle cell disease are prone to acute chest syndrome resulting from sickling of cells within the lung that typically presents with chest pain, tachypnea, cough, fever, and oxygen desaturation. Transfusion is indicated when the hematocrit drops below 30% or oxygen saturation drops below 90%. oBone marrow transplant is only an option in children (B) and not indicated for emergent therapy. Furosemide (C) would promote dehydration and further exacerbation of cell sickling. Hydroxyurea (D) is used prophylactically to reduce the frequency of sickle cell crises and/or acute chest syndrome. Acetaminophen (A) may be used for symptomatic treatment, but is less critical than transfusion and may not provide adequate pain management.

•A 6-week-old male with sickle cell disease presents to the pediatric office for his well-child visit. When should this child begin taking daily prophylactic penicillin?

oPatients with sickle cell disease develop functional asplenia as early as 3 months of age and should begin treatment with prophylactic penicillin at 2 months of age to prevent infection by encapsulated organisms (i.e., pneumococcus).

A 23-year-old male with a recent diagnosis of Non-Hodgkin's lymphoma presents complaining of swelling of the neck and face, cough, and dyspnea on exertion. On exam you note dilated neck veins. • Which of the following is the most appropriate initial treatment for this patient?

oThe classic presentation signs and symptoms of superior vena cava syndrome (SVCS) are present in this patient. Patients with lymphoma often respond with a decrease in tumor mass and improvement in SVCS with glucocorticoid therapy. oBeta-blockers (A) and vena cava stenting (E) aren't routinely used in the management of SVCS. Other initial symptomatic therapies include low salt diet, diuretics, head elevation, and diuretics. IV fluids (C) and Trendelenburg position (D) are contraindicated.

• SEVERE ST→pt c/o "worst ST I've ever had" • HOT POTATO VOICE • Swollen/fluctuant tonsil + uvula deviation • odynophagia • Fever

peritonsillar abscess

• Fever • Dysphagia/Odynophagia • hot potato/muffled voice +/- drooling • stridor • decreased PO's • torticolitis • Neck hyperextension • Bulging of posterior pharyngeal wall • Medial displacement of tonsils

retropharangeal abscess; extend from base of neck to the mediastinum

Clinical presentation begins abruptly with high fever (39ºC to 104ºF) Anorexia, irritability, rarely febrile seizures After 3 days, fever defervesces suddenly and a discrete rose-pink macules or maulopapules begin on trunk and spread

roseola

o Sudden, unabating scrotal pain without relief + w/ "high lie" appearance o Swollen/edematous, retracted testicle + hyperemia +/- infarction o (-) Prehn's Sign→ NO relief w/ scrotal elevation o CREMESTERIC REFLEX is absent

testicular torsion

what conditions cause the following? 1. hypersegmented neutrophils 2. aur rods 3. left shift and basophilia 4. hodgkin's lymphoma 5. multiple myeloma

what conditions cause the following? 1. hypersegmented neutrophils: B12 or folate deficiency 2. aur rods: AML 3. left shift and basophilia: PCV 4. hodgkin's lymphoma: no neutrophils 5. multiple myeloma no specific neutrophil on peripheral smear

what is H/H, MCV, and retic of: 1. anemia of chronic blood loss 2. acute blood loss 3. defective bone marrow/stem cell function 4. defective DNA production 5. hemolysis

what is H/H, MCV, and retic of: 1. anemia of chronic blood loss: * low H/H, low MCV, retic of 0.1% 2. acute blood loss: * normal H/H 3. defective bone marrow/stem cell function: * normocytic RBC 4. defective DNA production: * failure of RBC maturation; macrocytosis 5. hemolysis: * need to look at iron stores and retic count

what increases the absorption of iron in the ileum?

• An acidic achieved through presence of additional acids (C) (e.g., ascorbic acid) increases absorption of iron. Food (E), other heavy metals (A), and basic stomach environments (B, D) negatively affect the absorption of iron.

describe the centor criteria for tonsillitis

• Centor criteria→FEVER >38°C, tender cervical LAD, NO cough, pharyngotonsillar exudate = GABHS

fever of unknown origin; tx

• FUO = FEVER (>38 °C/100.4 °F) x > 8-14d w/o apparent dx o Fever w/o localizing signs (FWLS) = fever w/o source (FWS)→ any fever w/ NO cause found on PE • Dx of exclusion • MC = infxn & rheumatic dz (CT or autoimmune) o ZERO empiric tx w/ abx or anti-inflammatories→ may delay dx of infxns (endocarditis, meningitis) o Antipyretics (x fever)→ APAP

Henoch-Schonlein purpura 1. labs 2. tx

• Labs o Hematuria +/- proteinuria o CBC, stool guiac, CT o Renal bx o ANA, ANCA, RF→ negative o Dx (2/4)⇒ palpable purpura, bowel angina, bx (granulocytes), peds (<20yo) • Tx o Supportive o Po/IV Steroids (controversial)→ if severe intussusception or abd. pain o AZA, Cyclophosphamide, plasmapheresis

tx of UTI in children

• Oral Abx prophylaxis is indicated in children w/ significant VUR (3-6 months) • Children < 2 months - Cephalexin 10mg/k/d or amoxicillin 20 mg/k/d • Children > 2 months - Trimethoprim-sulfamethoxazole 10-20mg/k/d - Nitrofurantoin 1-2 mg/k/d