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Pediatric Nephrology

, Volume 21, Issue 11, pp 1769–1771 | Cite as

Secondary failure of plasma therapy in factor H deficiency

  • Sylvie NathansonEmail author
  • Tim Ulinski
  • Véronique Frémeaux-Bacchi
  • Georges Deschênes
Brief Report

Abstract

We report a patient with homozygous factor H deficiency leading to permanent alternate complement activation and early onset of the hemolytic uremic syndrome. He was successfully treated with weekly infusions of fresh frozen plasma over 4 years, displaying normal blood pressure while only treated with an angiotensin converting enzyme (ACE) inhibitor, a steady level of haptoglobin, low-range proteinuria and normal creatinine clearance. By the end of the fourth year of treatment, he dramatically developed a relapse of hemolytic and uremic syndrome, displaying undetectable haptoglobin, nephrotic range proteinuria and progressive renal failure. Despite a ten-fold increase in the dosage of plasma infusion through daily plasma exchange, haptoglobin remained undetectable while circulating antigenic factor H levels reached 22–24% (normal values 65–140%). Three months following the biological onset of the relapse, a bilateral nephrectomy was performed owing to uncontrolled hypertension and rapidly progressive renal failure. The molecular mechanism of plasma resistance remained unclear while antifactor H antibodies were not detected in the plasma. We suggest that protracted administration of exogenous factor H might not be a long-term strategy in homozygous factor H deficiency.

Keywords

Hemolytic uremic syndrome Factor H fresh frozen plasma 

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Copyright information

© IPNA 2006

Authors and Affiliations

  • Sylvie Nathanson
    • 1
    Email author
  • Tim Ulinski
    • 2
  • Véronique Frémeaux-Bacchi
    • 3
  • Georges Deschênes
    • 2
  1. 1.Hôpital André MignotLe ChesnayFrance
  2. 2.Hôpital Armand-TrousseauService de Néphrologie PédiatriqueParisFrance
  3. 3.Hôpital Européen Georges PompidouService d’Immunologie BiologiqueParisFrance

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