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Heme/Onc 3 (genetic)

Step-2

QuestionAnswer
What test is used to r/o urethral injury? retrograde cystourethrogram
A 19 y/o man presents with a palpable flank mass and hematuria, and renal US shows b/l enlarged kidneys with cysts. What brain anomaly is associated with this condition? ADPKD, associated with berry aneurysms
Which lipid-lowering agent(s) matches the following description?: SE: facial flushing niacin
Which lipid-lowering agent(s) matches the following description?: SE: elevated LFTs, myositis statins, fibrates
Which lipid-lowering agent(s) matches the following description?: SE: GI discomfort, bad taste bile acid sequestrants
Which lipid-lowering agent(s) matches the following description?: best effect on HDL niacin
Which lipid-lowering agent(s) matches the following description?: best effect on TGs fibrates
Which lipid-lowering agent(s) matches the following description?: best effect on LDL/cholesterol statins
Which lipid-lowering agent(s) matches the following description?: binds c.diff toxin cholestyramine
Which type of thalassemia is most commonly associated with patients of mediterranean descent? B-thalassemia
Which type of thalssemia is most commonly associated with patients of African or Asian descent? alpha thalassemia
What will you see on the blood smear in the following type of anemia?: alpha-thalassemia target cells, anisocytosis, microcytic
What will you see on the blood smear in the following type of anemia?: beta-thalassemia target cells, anisocytosis, microcytic
What will you see on the blood smear in the following type of anemia?: iron deficiency hypochromic, microcytic
What will you see on the blood smear in the following type of anemia?: lead poisoning basophilic stippling
What will you see on the blood smear in the following type of anemia?: B12/folate deficiency megaloblastic/macrocytic RBCs, hypersegmented neutrophils
What will you see on the blood smear in the following type of anemia?: sideroblastic ringed sideroblasts/erythroblast/RBC precursors, anisocytosis
List 3 acquired causes of sideroblastic anemia. alcoholism, INH, lead poisoning
What complication occurs in 10% of pts with sideroblastic anemia? aucte leukemia
An AA teenager presents to the ER with right hip pain and Hct of 25%. WHat is the most likely dx? sickle cell and osteonecrosis
Besides staph aureus, which organism may be responsible for osteomyelitis is a sickle cell patient? salmonella
What would be the concern in a sickle cell patient that contracts erythema infectiosum (5th disease)? aplastic crisis due to parvovirus B19
Which vaccines are particularly important in children with sickle cell disease? HiB, pneumococcal, menigococcal, influenza, and Hep B vaccines
What medication is used in the long-term management of sickle cell anemia? hyroxyurea
What is a common complication associated with thalassemia? chronic iron overload from repeat transfusions leading to damage to heart and liver.
Why don't newborns with sickle cell dz have symptoms until after 6 months of age? the presence of fetal Hgb in newborns delays the symptoms until after 6 months when the fetal Hgb levels have decreased.
Patients with sickle cell dz are at increased risk of which bone infection? What is the causative organism? salmonella osteomyelitis (although staph aureus is still the most common etiology).
What is the treatment for hereditary cases of sideroblastic anemia? Vit B (peridoxime) may normalize Hgb concentrations and therapeutic phlebotomy or chelation with deferoxamine
What is the tx for aquired cases of sideroblastic anemia? supplemental EPO and therapeutic phlebotomy or chelation with deferoxamine
Created by: shelybel
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