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14 Cards in this Set
- Front
- Back
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What is the cause of hereditary spherocytosis?
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mostly autosomal dominant where spectrin, one or more structural proteins of the RBC skeleton is partially deficient and prevents the RBC membrane to adhere properly to the skeleton
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What organ do patients with hereditary spherocytosis usually get taken out and why?
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The spleen. The deformed RBC's remained trapped in the spleen and are attacked by splenic macrophages and cannot get into circulation
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What are signs and symptoms of hereditary spherocytosis?
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Jaundice, pallor, fatigue, malaise, abdominal pain, splenomegaly, cholelithiasis
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What are lab findings for patients with heriditary spherocytosis?
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normocytic, hyperchromic anemia; blood smear can show microspherocytes and polychromasia, high retic count, osmotic fragility is increased and increased unconjugated bilirubin
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What is the treatment for hereditary spherocytosis?
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RBC transfusions, Folic acid, splenectomy after 5 years because of higher rate of sepsis
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What vaccinations do patients need before a splenectomy need and why?
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Hib, Pneumococcal and Meningococcal
Reduced post-splenectomy levels of opsonins, splenic tuftsin, and immunoglobulin (IgM) (which promote phagocytosis of particulate matter and bacteria), hamper the body’s ability to clear encapsulated organisms |
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What do patients who have hereditary spherocytosis usually need to take as prophylaxis lifelong?
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Penicillin
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What is the most common hereditary RBC membrane defect seen that can cause acute hemolysus and hyperbilirubinemia in the newborn?
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herditary spherocytosis
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What is the most common cause of death in SCD and the second most common cause of hospitalization in SCD patients?
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Acute chest syndrome
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What is the pathology behind acute chest syndrome?
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1.) deoxygenation of hemoglobin S is triggered and Hgb S polymerization and sickling of RBC cause vasooclusion, ischemia, endothelial injury
2.) infxn/fat embolus from nectroic bone marrow leading to alveolar hypoxia and subsequent local hypoxia w/obs of smaller airways, collapse of distal air spaces, air trapping and altered ventilation-perfusion relationships 3.) regional alveolar hypoxia from atelectasis, pulmonary edema, bronchospasm, pain from vasooclussive crisis 4.) Endothelial dysfunction ofthe pulmonary microvasc w/increased expression of vacular adhesion molecules, increased platelet and plama coag, activation, and disordered nitric oxide metab leading to thromboembolism |
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What is the most common cause of acute chest syndrome in kids less than 9 years of age?
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infection...mostly viral, but also mycoplasma, chlamydia, bacteria
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How is the diagnosis of acute chest syndrome made?
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clinically and needs:
1.) New pulmonary infiltrate detected by chest radiograph involvingat least one complete lung segment not consistent with atelectasis AND one or more 1.) CP 2.) T >38.5 3.) Tachypnea, wheezing, cough, or increased WOB 4.) Hypoxemia relative to baseline |
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When do you get a CXR for SCD in crises?
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SCD w/fever, CP, resp symptoms; repeat CXR after 24 to 48 hours if pt have restrictive resp efforts or hypoxemia at rest
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What is the best way to treat a patient with SCD who has ACS?
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Pain control w/Ketorolac, etc; O2, bronchodilators for asthma pt; cefotaxime/rocephin plus zithromax
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