Hemolytic uremic syndrome: Difference between revisions
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==Background==
==Background==
*Similar to but different from [[TTP]] (which is more common in adults)
*Similar to [[TTP]] (which is more common in adults), but associated with less central nervous system and more renal involvement.
*Most cases occur in children <10yr (2/3 of cases in <5yr)
*Most cases occur in children <10yr (⅔ of cases in < 5yr)
*Most cases due to E. coli O157:H7 infection
*Most cases due to E. coli O157:H7 infection
**Shiga toxin may induce autoantibody to CD36 (found on endothelial cells and platelets)
*Shiga toxin may induce autoantibody to CD36 (found on endothelial cells and platelets)
**May also be caused by shigella, yersinia, campylobacter, salmonella
*May also be caused by shigella, yersinia, campylobacter, salmonella
*Types:
===Types===
**Typical
#Typical
***Occurs 2-14d after development of infectious diarrhea (bloody, no fever)
#*Occurs 2-14d after development of infectious diarrhea (bloody, no fever)
**Atypical
#Atypical
***Occurs in older children and adults
#*Occurs in older children and adults
***Extrarenal involvement is common (difficult to distinguish from TTP)
#*Extrarenal involvement is common (difficult to distinguish from TTP)
***May be caused by atypical pathogens (EBV, S. pneumo) or immunosuppression
#*May be caused by atypical pathogens (EBV, S. pneumo) or immunosuppression


==Clinical Features==
==Clinical Features==
#Triad:
===Triad===
##Acute renal failure
#[[Renal Failure|Acute renal failure]]
###Oliguria
#[[Thrombocytopenia]]
##Thrombocytopenia
#[[Microangiopathic Hemolytic Anemia (MAHA)]]
##Microangiopathic hemolytic anemia
 
===Other Associated Conditions===
#Enteritis
#Enteritis
##N/V, diarrhea (usually bloody), +/- fever
#N/V, diarrhea (usually bloody), +/- fever
#Hyperglycemia
#Hyperglycemia
##Pancreatic beta-cell dealth due to microthrombi within pancreas
#Pancreatic beta-cell dealth due to microthrombi within pancreas
 
*Within a week develops pallor, oliguria, lethargy, sz, encephalopathy


==DDx==
==Differential Diagnosis==
#Acute gastroenteritis
#[[Gastroenteritis]]
#Appendicitis
#[[Appendicitis]]
#Colitis
#Colitis
#Intussusception
#[[Intussusception]]
#IBD
#IBD
#Perforation
#Perforation
#DIC
#[[DIC]]
#TTP
#[[TTP]]
#SLE
#[[SLE]]


==Work-Up==
==Work-Up==
*CBC
*CBC
**WBC may be elevated
**Checking for Schistocytes and Thrombocytopenia
**Anemia
***Schistocytes
**Thrombocytopenia
*Stool tests
*Stool tests
**Shiga toxin, E. coli O157:H7 test
**Shiga toxin, E. coli O157:H7 test
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==Treatment==
==Treatment==
*Supportive care
#'''Plasma exchange (plasmapheresis)'''
**Fluid for hypovolemia
#'''Transfusion of RBCs''' (only severe bleeding)
**Transfusion for anemia
#*Generally only indicated if plasma exchange cannot be performed immediately
*Abx are not indicated
#'''Platelet Transfusion is AVOIDED'''
*Antimotility agents are contraindicated
#*Only used for life-threatening bleeding or intracranial hemorrhage under guidance from hematologist
*Platelets are contraindicated
#*Platelet infusion may lead to acutely worsened thrombosis, renal failure, and death
*Emergency dialysis for acute renal failure
#'''Hemodialysis'''
#*Often needed for renal failure and hyperkalemia treatment
#'''AVOID Antibiotics'''
#*May lead to worsening lysis of bacteria and further toxin release
#'''AVOID Antimotility agents'''
#*Leads to prolonged gut exposure to toxins


==Disposition==
==Disposition==
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==Source==
==Source==
Tintinalli
*Corrigan J. Boineau FG. Hemolytic-uremic syndrome. Pediatr Rev. Nov 2001;22(11):365-9
*George J.  Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med 2006; 354:1927


[[Category:Peds]]
[[Category:Peds]]

Revision as of 21:54, 5 June 2014

Background

  • Similar to TTP (which is more common in adults), but associated with less central nervous system and more renal involvement.
  • Most cases occur in children <10yr (⅔ of cases in < 5yr)
  • Most cases due to E. coli O157:H7 infection
  • Shiga toxin may induce autoantibody to CD36 (found on endothelial cells and platelets)
  • May also be caused by shigella, yersinia, campylobacter, salmonella

Types

  1. Typical
    • Occurs 2-14d after development of infectious diarrhea (bloody, no fever)
  2. Atypical
    • Occurs in older children and adults
    • Extrarenal involvement is common (difficult to distinguish from TTP)
    • May be caused by atypical pathogens (EBV, S. pneumo) or immunosuppression

Clinical Features

Triad

  1. Acute renal failure
  2. Thrombocytopenia
  3. Microangiopathic Hemolytic Anemia (MAHA)

Other Associated Conditions

  1. Enteritis
  2. N/V, diarrhea (usually bloody), +/- fever
  3. Hyperglycemia
  4. Pancreatic beta-cell dealth due to microthrombi within pancreas

Differential Diagnosis

  1. Gastroenteritis
  2. Appendicitis
  3. Colitis
  4. Intussusception
  5. IBD
  6. Perforation
  7. DIC
  8. TTP
  9. SLE

Work-Up

  • CBC
    • Checking for Schistocytes and Thrombocytopenia
  • Stool tests
    • Shiga toxin, E. coli O157:H7 test
  • UA
    • Hematuria, casts
  • LFT
    • Increased bilirubin
  • Chemistry
    • Creatinine, hyperkalemia (renal failure)

Treatment

  1. Plasma exchange (plasmapheresis)
  2. Transfusion of RBCs (only severe bleeding)
    • Generally only indicated if plasma exchange cannot be performed immediately
  3. Platelet Transfusion is AVOIDED
    • Only used for life-threatening bleeding or intracranial hemorrhage under guidance from hematologist
    • Platelet infusion may lead to acutely worsened thrombosis, renal failure, and death
  4. Hemodialysis
    • Often needed for renal failure and hyperkalemia treatment
  5. AVOID Antibiotics
    • May lead to worsening lysis of bacteria and further toxin release
  6. AVOID Antimotility agents
    • Leads to prolonged gut exposure to toxins

Disposition

  • Admit

Source

  • Corrigan J. Boineau FG. Hemolytic-uremic syndrome. Pediatr Rev. Nov 2001;22(11):365-9
  • George J. Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med 2006; 354:1927
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