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November 22, 2010

November 22, 2010. Hemolytic Uremic Syndrome. Hemolytic Uremic Syndrome. Classic Triad Microangiopathic hemolytic Anemia Thrombocytopenia Acute Renal Failure Children < 4y/o Most frequent cause of ARF. Clinical Presentation. 1 Diarrheal Prodrome Watery initially 5-7 days

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November 22, 2010

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  1. November 22, 2010

  2. Hemolytic Uremic Syndrome

  3. Hemolytic Uremic Syndrome • Classic Triad • Microangiopathic hemolytic Anemia • Thrombocytopenia • Acute Renal Failure • Children < 4y/o • Most frequent cause of ARF

  4. Clinical Presentation • 1 Diarrheal Prodrome • Watery initially • 5-7 days • 2 Hemolysis and Thrombocytopenia • 3 Oliguria/Anuria

  5. Etiology • Toxin producing E. coli • O157:H7 • EnterohemorrhagicE.coli • Shigelladysenteriae • Sources • Undercooked beef • Unpasteurized milk • Contaminated juice • Person-to-person

  6. Atypical (sporadic) HUS • Rare • Nonprodromal • No preceding diarrhea • Worse prognosis • Relapse more likely • Associations • Family History • Chemo meds, oral contraceptives, bone marrow transplant, vasculitic diseases

  7. HUS: Pathogenesis • Toxin destroys colonic mucosal cells • Bloody diarrhea • Toxins enter circulation • Attach/damage other endothelial cells • Kidneys • Brain • Pancreas • Platelet/fibrin thrombi form

  8. Microangiopathic hemolytic anemia

  9. Clinical Picture • Previously healthy child • Acute Onset • Pallor • Irritability • Lethargy • Oliguria • Following 5-7 days diarrheal illness

  10. Other possible findings • CNS findings (20-30%) • Seizures • Lethargy/ coma • Personality change • Infarcts • Focal deficits • Abdominal findings • Gangrenous bowel • Intussusception • Hepatosplenomegaly • Pancreatic insufficiency

  11. Renal Aspects • Wide spectrum • Mild • Microscopic hematuria • Mild proteinuria • Normal or increased UOP • Severe • Renal cortical necrosis • Anuric renal failure • May be permanent

  12. Renal Aspects • 50% anuric • average 3 days • 50% Hypertension • All have hematuria and protienuria • RBC casts • Fluid overload • Electrolyte abnormalities

  13. Management • Supportive • Renal failure • Fluids, lytes, dialysis may be needed • Hypertension • RBC’s for symptomatic anemia • Platelets not usually given • Surgical procedures • Acute bleed (rare) • Avoidance of antibiotics if E.Coli • Antibiotics may be used for Shigella

  14. Prognosis • 5% mortality • 5% lifelong dialysis • Late development • ESRD 10% • HTN • Atypical may have worse prognosis

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