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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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hemolytic uremic syndrome1
Hemolytic Uremic Syndrome
  • Classic Triad
    • Microangiopathic hemolytic Anemia
    • Thrombocytopenia
    • Acute Renal Failure
  • Children < 4y/o
    • Most frequent cause of ARF
clinical presentation
Clinical Presentation
  • 1 Diarrheal Prodrome
      • Watery initially
      • 5-7 days
  • 2 Hemolysis and Thrombocytopenia
  • 3 Oliguria/Anuria
etiology
Etiology
  • Toxin producing E. coli
    • O157:H7
  • EnterohemorrhagicE.coli
  • Shigelladysenteriae
  • Sources
    • Undercooked beef
    • Unpasteurized milk
    • Contaminated juice
    • Person-to-person
atypical sporadic hus
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
hus pathogenesis
HUS: Pathogenesis
  • Toxin destroys colonic mucosal cells
    • Bloody diarrhea
  • Toxins enter circulation
    • Attach/damage other endothelial cells
      • Kidneys
      • Brain
      • Pancreas
    • Platelet/fibrin thrombi form
clinical picture
Clinical Picture
  • Previously healthy child
  • Acute Onset
    • Pallor
    • Irritability
    • Lethargy
    • Oliguria
    • Following 5-7 days diarrheal illness
other possible findings
Other possible findings
  • CNS findings (20-30%)
    • Seizures
    • Lethargy/ coma
    • Personality change
    • Infarcts
      • Focal deficits
  • Abdominal findings
    • Gangrenous bowel
    • Intussusception
    • Hepatosplenomegaly
    • Pancreatic insufficiency
renal aspects
Renal Aspects
  • Wide spectrum
  • Mild
    • Microscopic hematuria
    • Mild proteinuria
    • Normal or increased UOP
  • Severe
    • Renal cortical necrosis
    • Anuric renal failure
    • May be permanent
renal aspects1
Renal Aspects
  • 50% anuric
    • average 3 days
  • 50% Hypertension
  • All have hematuria and protienuria
  • RBC casts
  • Fluid overload
  • Electrolyte abnormalities
management
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
prognosis
Prognosis
  • 5% mortality
  • 5% lifelong dialysis
  • Late development
    • ESRD 10%
    • HTN
  • Atypical may have worse prognosis