Hemolytic-Uremic Syndrome in childhood

J Bras Nefrol. Apr-Jun 2014;36(2):208-20. doi: 10.5935/0101-2800.20140032.
[Article in English, Portuguese]

Abstract

There is a group of diseases that may manifest with thrombotic microangiopathy and present clinical overlap. Among these we emphasize the thrombotic thrombocytopenic purpura and Hemolytic Uremic Syndrome, and the latter can occur by the action of toxins, systemic diseases, overactivation of the alternative complement system pathway, which can occur due to changes in regulatory proteins (atypical HUS) and finally, idiopathic. You must carry out a series of tests to differentiate them. aHUS is a diagnosis of exclusion of other causes of MAT. The treatment of aHUS with plasma therapy, results in most cases with good shortterm response, especially hematological; however, it is a progressive and devastating disease and can lead to death and terminal chronic renal disease. Treatment with plasma displays great recurrence of long-term disease and renal insufficiency. Eculizumab, a monoclonal antibody anti-C5, has been associated with hematological remission, benefits on renal function and no need of plasma therapy.

Publication types

  • Review

MeSH terms

  • Child
  • Complement Activation
  • Diagnosis, Differential
  • Hemolytic-Uremic Syndrome* / diagnosis
  • Hemolytic-Uremic Syndrome* / immunology
  • Hemolytic-Uremic Syndrome* / therapy
  • Humans
  • Purpura, Thrombotic Thrombocytopenic / diagnosis