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A case of atypical hemolytic uremic syndrome with a transient decrease in complement factor H.

Hyewon Hahn1, Eun Young Um, Young Seo Park

  • 1Department of Pediatrics, Eulji University School of Medicine, Daejeon, Korea. petercat67@empal.com

Pediatric Nephrology (Berlin, Germany)
|December 20, 2005
PubMed
Summary

This case study describes a child with atypical hemolytic uremic syndrome (HUS) who experienced a temporary drop in complement factor H (FH). Treatment led to recovery of FH levels and full renal function, with no HUS recurrence.

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Area of Science:

  • Nephrology
  • Immunology
  • Pediatrics

Background:

  • Atypical hemolytic uremic syndrome (HUS) is a rare, severe thrombotic microangiopathy.
  • It is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury.
  • Genetic factors and complement dysregulation are implicated in HUS pathogenesis.

Observation:

  • A 31-month-old boy presented with hemolysis and azotemia, lacking a diarrhea prodrome.
  • Laboratory tests revealed decreased levels of complement 3 (C3) and complement factor H (FH).
  • No mutation in the factor H gene (HF1) was identified, suggesting a non-genetic cause or a novel mutation.

Findings:

  • Plasma infusion therapy successfully controlled the hemolytic uremic syndrome.
  • Following treatment, complement C3 and FH levels normalized.

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  • The patient achieved full renal function recovery with no HUS relapse.

    • Implications:

    • This case highlights a sporadic form of atypical HUS potentially linked to transient complement factor H deficiency.
    • It underscores the importance of assessing complement levels in unexplained hemolytic uremic syndrome.
    • Prompt management with plasma infusion can lead to favorable long-term renal outcomes in such cases.