Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome

Gema Ariceta, Nesrin Besbas, Sally Johnson, Diana Karpman, Daniel Landau, Christoph Licht, Chantal Loirat, Carmine Pecoraro, C. Mark Taylor, Nicole Van der Kar, Johan VandeWalle, Lothar B. Zimmerhackl

Research output: Contribution to journalEditorial

265 Scopus citations

Abstract

This guideline for the investigation and initial treatment of atypical hemolytic uremic syndrome (HUS) is intended to offer an approach based on opinion, as evidence is lacking. It builds on the current ability to identify the etiology of specific diagnostic sub-groups of HUS. HUS in children is mostly due to infection, enterohemorrhagic Escherichia coli (EHEC), Shigella dysenteriae type 1 in some geographic regions, and invasive Streptococcus pneumoniae. These sub-groups are relatively straightforward to diagnose. Their management, which is outside the remit of this guideline, is related to control of infection where that is necessary and supportive measures for the anemia and acute renal failure. A thorough investigation of the remainder of childhood HUS cases, commonly referred to as "typical" HUS, will reveal a risk factor for the syndrome in approximately 60% of cases. Disorders of complement regulation are, numerically, the most important. The outcome for children with atypical HUS is poor, and, because of the rarity of these disorders, clinical experience is scanty. Some cases of complement dysfunction appear to respond to plasma therapy. The therapeutic part of this guideline is the consensus of the contributing authors and is based on limited information from uncontrolled studies. The guideline proposes urgent and empirical plasmapheresis replacement with whole plasma fraction for the first month after diagnosis. This should only be undertaken in specialized pediatric nephrology centers where appropriate medical and nursing skills are available. The guideline includes defined terminology and audit points so that the early clinical effectiveness of the strategy can be evaluated.

Original languageEnglish
Pages (from-to)687-696
Number of pages10
JournalPediatric Nephrology
Volume24
Issue number4
DOIs
StatePublished - 1 Jan 2009
Externally publishedYes

Keywords

  • A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13)
  • Atypical HUS
  • Complement C3
  • Complement factor B
  • Complement factor H
  • Complement factor I
  • Enterohemorrhagic Escherichia coli
  • Hemolytic uremic syndrome
  • Membrane co-factor protein MCP (CD46)
  • Plasmapheresis
  • Thrombotic thrombocytopenic purpura

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Nephrology

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