Medical Triads Codexery

Hemolytic–uremic syndrome

A syndrome of kidney injury, low platelets, and red blood cell destruction.

Last updated

Most cases occur after infectious diarrhea due to E. coli O157:H7, and the underlying mechanism typically involves Shiga toxin production.[1] The triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury defines Hemolytic–uremic syndrome (HUS).[2] Supportive management for HUS can incorporate dialysis, corticosteroids, erythrocyte transfusions, or plasma exchange.[3]

Key facts and history

Atypical HUS (aHUS) represents 5–10% of cases and results from genetic mutations causing uncontrolled complement activation.[4] Argentina has the greatest prevalence of HUS, with up to 7.9 cases per 100,000 children annually.[5][6] Bloody diarrhea, fever, vomiting, and weakness are among the first manifestations of the condition.[2] Conrad Gasser provided the initial description of HUS as a distinct syndrome in 1955.[3][7]

A classification system for E. coli strains that cause human illness was created by Karmali.[8][9] The familial variant of aHUS was localized by Paul Warwicker to a segment of chromosome 1 that holds complement regulatory genes.[10] In September 2011, the FDA granted approval for eculizumab, a monoclonal antibody that inhibits complement, as a treatment for aHUS.[11] Annually, roughly 1.5 individuals per 100,000 population are diagnosed with HUS, and fewer than 5% of those affected succumb to the illness.[3]

What the research sources say

The UNC Kidney Center article describes HUS as one of the thrombotic microangiopathies, alongside TTP.[12] The same source indicates that Karmali devised the method for categorizing E. coli strains that are pathogenic to humans.[9]

References

  1. Wikipedia, "Hemolytic–uremic syndrome" (CC BY-SA 4.0), which cites: "Hemolytic uremic syndrome", Genetic and Rare Diseases Information Center (GARD); Cody, "Hemolytic Uremic Syndrome.", Pediatric Clinics of North America, February 2019, p. 235–246; "Hemolytic uremic syndrome", Genetic and Rare Diseases Information Center (GARD); Salvadori, "Update on hemolytic uremic syndrome: Diagnostic and therapeutic recommendations.", World Journal of Nephrology, 6 August 2013, p. 56–76; Cody, "Hemolytic Uremic Syndrome.", Pediatric Clinics of North America, February 2019, p. 235–246; "Hemolytic uremic syn en.wikipedia.org
  2. Wikipedia, "Hemolytic–uremic syndrome" (CC BY-SA 4.0), which cites: Cody, "Hemolytic Uremic Syndrome.", Pediatric Clinics of North America, February 2019, p. 235–246; Salvadori, "Update on hemolytic uremic syndrome: Diagnostic and therapeutic recommendations.", World Journal of Nephrology, 6 August 2013, p. 56–76; Cody, "Hemolytic Uremic Syndrome.", Pediatric Clinics of North America, February 2019, p. 235–246; "Hemolytic uremic syndrome", Genetic and Rare Diseases Information Center (GARD); Cody, "Hemolytic Uremic Syndrome.", Pediatric Clinics of North America, February 2019, p. 235–246; Chu, en.wikipedia.org
  3. Wikipedia, "Hemolytic–uremic syndrome" (CC BY-SA 4.0), which cites: Cody, "Hemolytic Uremic Syndrome.", Pediatric Clinics of North America, February 2019, p. 235–246; "Hemolytic uremic syndrome", Genetic and Rare Diseases Information Center (GARD); Noris, "Atypical hemolytic–uremic syndrome", N Engl J Med, 2009, p. 1676–1687; Cody, "Hemolytic Uremic Syndrome.", Pediatric Clinics of North America, February 2019, p. 235–246; Cody, "Hemolytic Uremic Syndrome.", Pediatric Clinics of North America, February 2019, p. 235–246; Cody, "Hemolytic Uremic Syndrome.", Pediatric Clinics of North America, Fe en.wikipedia.org
  4. Wikipedia, "Hemolytic–uremic syndrome" (CC BY-SA 4.0), which cites: Noris, "Atypical hemolytic–uremic syndrome", N Engl J Med, 2009, p. 1676–1687; Noris, "Atypical hemolytic–uremic syndrome", N Engl J Med, 2009, p. 1676–1687 (original not read by us) en.wikipedia.org
  5. Wikipedia, "Hemolytic–uremic syndrome" (CC BY-SA 4.0), which cites: Rivero, "Factors associated with sporadic verotoxigenic Escherichia coli infection in children with diarrhea from the Central Eastern Area of Argentina", Foodborne Pathogens and Disease, 2011, p. 901–6; Rivas, "Home-prepared hamburger and sporadic hemolytic uremic syndrome, Argentina", Emerging Infectious Diseases, 2003, p. 1184–6; Rivero, "Enterohemorrhagic Escherichia coli and hemolytic–uremic syndrome in Argentina", Medicina, 2004, p. 352–6 (original not read by us) en.wikipedia.org
  6. Wikipedia, "Hemolytic–uremic syndrome" (CC BY-SA 4.0), which cites: Ruggenenti, "Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura", Kidney Int, 2001, p. 831–846 (original not read by us) en.wikipedia.org
  7. Wikipedia, "Hemolytic–uremic syndrome" (CC BY-SA 4.0), which cites: Gianantonio, "Acute Renal Failure", Springer US, 1984, p. 327–339 (original not read by us) en.wikipedia.org
  8. Wikipedia, "Hemolytic–uremic syndrome" (CC BY-SA 4.0), which cites: OVC Communications, "Dr. Mohamed Karmali – Solving the Mystery of the Haemolytic-Uremic Syndrome - June 10", Tumblr (we also read that source) en.wikipedia.org
  9. OVC Communications, "Dr. Mohamed Karmali – Solving the Mystery of the Haemolytic-Uremic Syndrome - June 10", Tumblr. facts only, no text reused. bulletin.ovc.uoguelph.ca
  10. Wikipedia, "Hemolytic–uremic syndrome" (CC BY-SA 4.0), which cites: "Dr Paul Warwicker: General (internal) medicine, Renal medicine", finder.bupa.co.uk; Warwicker, "Genetic studies into inherited and sporadic hemolytic uremic syndrome", Kidney International, April 1998, p. 836–844 (original not read by us) en.wikipedia.org
  11. Wikipedia, "Hemolytic–uremic syndrome" (CC BY-SA 4.0), which cites: "2011 Notifications", FDA}}{{dead link, May 2025 (original not read by us) en.wikipedia.org
  12. "Thrombotic Microangiopathy (TMA)", UNC Kidney Center, 2025. facts only, no text reused. unckidneycenter.org

Each numbered sentence above was checked against the source it cites before publication. Sentences citing Wikipedia adapt text from the English Wikipedia article Hemolytic–uremic syndrome (contributors), used under CC BY-SA 4.0; this entry is available under the same licence. Other sources were used for facts only; no text or images are copied from them.

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Sources

Compiled from Wikipedia and the sources listed below. Text from Wikipedia is available under CC BY-SA 4.0; this entry is adapted from it.

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