[Atipical uremic hemolityc syndrome in pregnancy]

Cir Cir. 2016 Jul-Aug;84(4):344-9. doi: 10.1016/j.circir.2016.02.001. Epub 2016 Mar 19.
[Article in Spanish]

Abstract

Atypical haemolytic uraemic syndrome is one of the main variants of thrombotic microangiopathy, and is characterized by excessive complement activation in the microvasculature. It is also characterised by the clinical triad; non-immune haemolytic anaemia, thrombocytopenia, and acute renal failure. In addition, 60% of patients have mutations in the genes encoding complement regulators (factor H, factor I, membrane cofactor proteins, and thrombomodulin), activators (factor B and C3), as well as autoantibodies against factor H. Multiple factors are required for the disease to manifest itself, including a trigger and gene mutations with adequate penetration. Being one of the differential diagnoses of preeclampsia- eclampsia and HELLP syndrome means that the clinician must be familiar with the disease due to its high mortality, which can be modified with early diagnosis and comprehensive treatment.

Keywords: Atypical haemolytic uraemic syndrome; Embarazo; Microangiopatía trombótica; Pregnancy; Síndrome urémico hemolítico atípico; Thrombotic microangiopathy.

Publication types

  • Review

MeSH terms

  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Atypical Hemolytic Uremic Syndrome* / diagnosis
  • Atypical Hemolytic Uremic Syndrome* / epidemiology
  • Atypical Hemolytic Uremic Syndrome* / physiopathology
  • Atypical Hemolytic Uremic Syndrome* / therapy
  • Complement Activation / genetics
  • Diagnosis, Differential
  • Female
  • Genetic Predisposition to Disease
  • Humans
  • Mutation
  • Plasmapheresis
  • Pregnancy
  • Pregnancy Complications, Hematologic* / diagnosis
  • Pregnancy Complications, Hematologic* / epidemiology
  • Pregnancy Complications, Hematologic* / physiopathology
  • Pregnancy Complications, Hematologic* / therapy
  • Pregnancy Outcome
  • Prognosis

Substances

  • Antibodies, Monoclonal, Humanized
  • eculizumab