Hemolytic–uremic syndrome

Hemolytic–uremic syndrome
Other namesHaemolytic–uraemic syndrome
Schistocytes as seen in a person with hemolytic–uremic syndrome
SpecialtyPediatrics, nephrology
SymptomsEarly: Bloody diarrhea, vomiting, fever Later: Low platelets, low red blood cells, kidney failure[1]
ComplicationsNeurological problems, heart failure[1]
TypesShiga toxin–producing E. coli HUS (STEC HUS),
S. pneumoniae-associated HUS (SP-HUS),
Atypical hemolytic uremic syndrome (aHUS),
Cobalamin C HUS[1]
CausesInfection by E coli O157:H7, shigella, salmonella[2]
Risk factorsYounger age, female, immunocompromised, or existing renal, urinary, or lower GI disease (because these are the systems involved in the disease)[1]
Diagnostic methodBlood tests (to monitor levels of platelets, red blood cells, and white blood cells), stool tests (especially to check for microscopic or macroscopic levels of fresh or old blood), urinalysis (to help monitor kidney function, like blood urea nitrogen, or BUN, levels, pH, and for blood in the urine- hematuria)[3]
Differential diagnosisThrombotic thrombocytopenic purpura (TTP), disseminated intravascular coagulation (DIC), certain problems with an artificial heart valve[4]
TreatmentSupportive care, dialysis, steroids, blood transfusions, plasmapheresis[2][1]
Prognosis<25% long-term kidney problems, which for some of these, could include chronic kidney dysfunction or even failure (which could ultimately need dialysis or transplantation to treat);[1] 5% risk of death during the illness in developed countries with treatment
Frequency1.5 per 100,000 per year[5]
Deaths<5% risk of death[1]

Hemolytic–uremic syndrome (HUS) is a syndrome characterized by low red blood cells, acute kidney injury (previously called acute renal failure), and low platelets.[1][3] Initial symptoms typically include bloody diarrhea, fever, vomiting, and weakness.[1][2] Kidney problems and low platelets then occur as the diarrhea progresses.[1] Children are more commonly affected, but most children recover without permanent damage to their health, although some children may have serious and sometimes life-threatening complications.[6] Adults, especially the elderly, may show a more complicated presentation.[2][6] Complications may include neurological problems and heart failure.[1]

Most cases occur after infectious diarrhea due to a specific type of E. coli called O157:H7.[2] Other causes include S. pneumoniae, Shigella, Salmonella, and certain medications.[1][2][3] The underlying mechanism typically involves the production of Shiga toxin by the bacteria.[1][2] Atypical hemolytic uremic syndrome (aHUS) is often due to a genetic mutation and presents differently.[1][2] However, both can lead to widespread inflammation and multiple blood clots in small blood vessels, a condition known as thrombotic microangiopathy.[7]

Treatment involves supportive care and may include dialysis, steroids, blood transfusions, or plasmapheresis.[1][2] About 1.5 per 100,000 people are affected per year.[5][1] Less than 5% of those with the condition die.[1] Of the remainder, up to 25% have ongoing kidney problems.[1] HUS was first defined as a syndrome in 1955.[1][8]

  1. ^ a b c d e f g h i j k l m n o p q r s Cody, EM; Dixon, BP (February 2019). "Hemolytic Uremic Syndrome". Pediatric Clinics of North America. 66 (1): 235–246. doi:10.1016/j.pcl.2018.09.011. PMID 30454746. S2CID 53875876.
  2. ^ a b c d e f g h i "Hemolytic uremic syndrome". Genetic and Rare Diseases Information Center (GARD). Archived from the original on 29 January 2020. Retrieved 21 November 2018.
  3. ^ a b c Salvadori, M; Bertoni, E (6 August 2013). "Update on hemolytic uremic syndrome: Diagnostic and therapeutic recommendations". World Journal of Nephrology. 2 (3): 56–76. doi:10.5527/wjn.v2.i3.56. PMC 3832913. PMID 24255888.
  4. ^ Ferri, Fred F. (2010). Ferri's Differential Diagnosis E-Book: A Practical Guide to the Differential Diagnosis of Symptoms, Signs, and Clinical Disorders. Elsevier Health Sciences. p. 219. ISBN 978-0-323-08163-4.
  5. ^ a b Noris, M; Remuzzi, G (2009). "Atypical hemolytic–uremic syndrome". N Engl J Med. 361 (17): 1676–1687. doi:10.1056/NEJMra0902814. PMID 19846853.
  6. ^ a b Chu, P; Hemphill, RR (2004). "222: Acquired hemolytic anemia". Emergency Medicine: A Comprehensive Study Guide (6th ed.). New York, NY: McGraw-Hill. ISBN 978-0-07-138875-7.
  7. ^ Benz, K; Amann, K (2010). "Thrombotic microangiopathy: new insights". Current Opinion in Nephrology and Hypertension. 19 (3): 242–247. doi:10.1097/MNH.0b013e3283378f25. PMID 20186056. S2CID 25429151.
  8. ^ Gasser C, Gautier E, Steck A, Siebenmann RE, Oechslin R (September 1955). "Hemolytic–uremic syndrome: bilateral necrosis of the renal cortex in acute acquired hemolytic anemia". Schweiz Med Wochenschr (in German). 85 (38–39): 905–9. PMID 13274004.
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