Hemolytic uremic syndrome: Difference between revisions
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==Background== | ==Background== | ||
*Abbreviation: HUS | |||
*Similar to [[TTP]] (which is more common in adults), but associated with less central nervous system and more renal involvement. | *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 occur in children <10yr (⅔ of cases in < 5yr) | ||
* | *80-90% 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== | ||
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===Other Associated Conditions=== | ===Other Associated Conditions=== | ||
*Enteritis | |||
*[[Nausea/vomiting]], [[diarrhea]] (usually bloody), +/- [[fever]] | |||
*[[Hyperglycemia]] | |||
**Pancreatic beta-cell death due to microthrombi within pancreas | |||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
*[[Gastroenteritis]] | |||
*[[Appendicitis]] | |||
*[[Colitis]] | |||
*[[Intussusception]] | |||
*[[IBD]] | |||
*Perforation | |||
*[[SLE]] | |||
== | {{Glomerulonephritis causes}} | ||
{{Hemolytic anemia DDX}} | |||
{{Thrombocytopenia}} | |||
==Evaluation== | |||
*CBC | *CBC | ||
** | **[[Anemia]] | ||
**[[Thrombocytopenia]] | |||
**Peripheral smear checking for schistocytes, burr cells, helmet cells, spherocytes and segmented red blood cells | |||
*LDH (elevated) | |||
*Haptoglobin (decreased) | |||
*Reticulocyte count (appropriate) | |||
*PT/PTT/INR (normal; differentiates from [[DIC]]) | |||
*Stool tests | *Stool tests | ||
**Shiga toxin, E. coli O157:H7 test | **Shiga toxin, E. coli O157:H7 test | ||
* | *[[Urinalysis]] | ||
**Hematuria, casts | **[[Hematuria (peds)|Hematuria]], casts | ||
* | *[[LFTs]] | ||
**Increased bilirubin | **Increased bilirubin | ||
*Chemistry | *Chemistry | ||
**Creatinine, hyperkalemia (renal failure) | **Creatinine elevation, [[hyperkalemia]] ([[renal failure]]) | ||
== | ==Management== | ||
*[[IVF|Fluid resuscitation]] | |||
*[[Insulin]] therapy if hyperglycemic and ketones (pancreatic insufficiency complication) | |||
*[[Antihypertensives]] | |||
**[[Nifedipine]]ER (0.25-0.5 mg/kg/day oral) | |||
**[[Labetalol]] 1-3 mg/kg/day, divided into twice daily dosing (12 mg/kg/day up to 1200 mg/day) | |||
**[[Nitroprusside]] 0.3-0.5 ug/kg/min IV (max 10 ug/kg/min) | |||
*[[Plasma exchange]] (plasmapheresis) | |||
**Usually performed if anuria or neurologic sequela | |||
*[[Eculizumab]] | |||
**Monoclonal anti-C5 antibody that interrupts complement cascade | |||
*Transfusion of RBCs(only severe bleeding) | |||
**Generally only indicated if plasma exchange cannot be performed immediately | |||
*[[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 | |||
*[[Hemodialysis/Hemoperfusion]] | |||
**Often needed for renal failure and hyperkalemia treatment | |||
*AVOID Antibiotics | |||
**May lead to worsening lysis of bacteria and further shiga toxin release | |||
*AVOID Antimotility agents | |||
**Leads to prolonged gut exposure to toxins | |||
**Risk of [[toxic megacolon]] | |||
==Disposition== | ==Disposition== | ||
*Admit | *Admit | ||
== | ==See Also== | ||
*[[TTP]] | |||
==References== | |||
*Corrigan J. Boineau FG. Hemolytic-uremic syndrome. Pediatr Rev. Nov 2001;22(11):365-9 | *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 | *George J. Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med 2006; 354:1927 | ||
[[Category: | [[Category:Pediatrics]][[Category:Heme/Onc]] |
Revision as of 00:44, 15 September 2019
Background
- Abbreviation: HUS
- 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)
- 80-90% 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
- Typical
- Occurs 2-14d after development of infectious diarrhea (bloody, no fever)
- Atypical
Clinical Features
Triad
Other Associated Conditions
- Enteritis
- Nausea/vomiting, diarrhea (usually bloody), +/- fever
- Hyperglycemia
- Pancreatic beta-cell death due to microthrombi within pancreas
Differential Diagnosis
- Gastroenteritis
- Appendicitis
- Colitis
- Intussusception
- IBD
- Perforation
- SLE
Causes of Glomerulonephritis
- Poststreptococcal glomerulonephritis
- Hemolytic-uremic syndrome
- Henoch-Schonlein purpura
- IgA nephropathy
- Lupus nephritis
- Alport syndrome
- Goodpasture syndrome
- Paraneoplastic
Microangiopathic Hemolytic Anemia (MAHA)
- Disseminated Intravascular Coagulation (DIC)
- Thrombotic Thrombocytopenic Purpura (TTP)
- Hemolytic Uremic Syndrome (HUS)
- HELLP syndrome
- Heparin-Induced Thrombocytopenia (HIT)
- Hereditary spherocytosis
- Paroxysmal nocturnal hemoglobinuria (PNH)
- Malignant hypertension
- Scleroderma
- Antiphospholipid Syndrome (APS)
- Other medical causes: malignancy, renal allograft rejection, vasculitides like polyarteritis nodosa and Wegener's granulomatosis
- Drugs: chemotherapy; Clopidogrel (Plavix) associated with TTP
- Nonvascular causes: prosthetic valve (more common with mechanical, more common at aortic valve), LVAD, TIPS, coil embolization, patched AV shunt, AVM
Thrombocytopenia
Decreased production
- Marrow infiltration (tumor or infection)
- Viral infections (rubella, HIV)
- Marrow suppression (commonly chemotherapy or radiation)
- Congenital thrombocytopenia
- Fanconi anemia
- Alport syndrome
- Bernand Soulier
- Vitamin B12 and/or folate deficiency
Increased platelet destruction or use
- Idiopathic thrombocytopenic purpura
- Thrombotic Thrombocytopenic Purpura (TTP)
- Hemolytic Uremic Syndrome (HUS)
- Disseminated Intravascular Coagulation (DIC)
- Viral infections (HIV, mumps, varicella, EBV)
- Drugs (heparin, protamine)
- Postransfusion or Posttransplantation
- Autoimmune destruction (SLE or Sarcoidosis)
- Mechanical destruction
- Artificial valves
- ECMO
- HELLP syndrome
- Excessive hemorrhage
- Hemodialysis, extracorporeal circulation
- Splenic Sequestration
- Occurs in Sickle cell disease and Cirrhosis
Drug Induced
- sulfa antibiotics, ETOH, ASA, thiazide diuretics/furosemide
Comparison by Etiology
ITP | TTP | HUS | HIT | DIC | |
---|---|---|---|---|---|
↓ PLT | Yes | Yes | Yes | Yes | Yes |
↑PT/INR | No | No | No | +/- | Yes |
MAHA | No | Yes | Yes | No | Yes |
↓ Fibrinogen | No | No | No | No | Yes |
Ok to give PLT | Yes | No | No | No | Yes |
Evaluation
- CBC
- Anemia
- Thrombocytopenia
- Peripheral smear checking for schistocytes, burr cells, helmet cells, spherocytes and segmented red blood cells
- LDH (elevated)
- Haptoglobin (decreased)
- Reticulocyte count (appropriate)
- PT/PTT/INR (normal; differentiates from DIC)
- Stool tests
- Shiga toxin, E. coli O157:H7 test
- Urinalysis
- Hematuria, casts
- LFTs
- Increased bilirubin
- Chemistry
- Creatinine elevation, hyperkalemia (renal failure)
Management
- Fluid resuscitation
- Insulin therapy if hyperglycemic and ketones (pancreatic insufficiency complication)
- Antihypertensives
- NifedipineER (0.25-0.5 mg/kg/day oral)
- Labetalol 1-3 mg/kg/day, divided into twice daily dosing (12 mg/kg/day up to 1200 mg/day)
- Nitroprusside 0.3-0.5 ug/kg/min IV (max 10 ug/kg/min)
- Plasma exchange (plasmapheresis)
- Usually performed if anuria or neurologic sequela
- Eculizumab
- Monoclonal anti-C5 antibody that interrupts complement cascade
- Transfusion of RBCs(only severe bleeding)
- Generally only indicated if plasma exchange cannot be performed immediately
- 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
- Hemodialysis/Hemoperfusion
- Often needed for renal failure and hyperkalemia treatment
- AVOID Antibiotics
- May lead to worsening lysis of bacteria and further shiga toxin release
- AVOID Antimotility agents
- Leads to prolonged gut exposure to toxins
- Risk of toxic megacolon
Disposition
- Admit
See Also
References
- 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