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Pediatric Nephrology
Published in: Pediatric Nephrology 2/2007

01-02-2007 | Brief Report

Intravenous gamma globulin for thrombotic microangiopathy of unknown etiology

Authors: Shuichi Ito, Kenichi Okuyama, Tomoko Nakamura, Jun-ichi Tetanishi, Kazuo Saito, Masanori Matsumoto, Yoshihiro Fujimura, Yukoh Aihara, Shumpei Yokota

Published in: Pediatric Nephrology | Issue 2/2007

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Abstract

We encountered the case of a 4-year-old boy with thrombotic microangiopathy (TMA) of unknown etiology. Verotoxin-induced hemolytic uremic syndrome (HUS), Streptococcus-pneumoniae-related HUS, factor H deficiency, drug-induced thrombotic thrombocytopenic purpura (TTP), and ADAMTS13 (von Willebrand factor-cleaving protease; a disintegrin-like and metalloprotease with thrombospondin type 1 repeats)-related TTP were excluded. His condition was refractory to anticoagulants and plasma exchange, and his clinical course was catastrophic, with central nervous system symptoms and progressive renal failure. However, factual treatment of intravenous gamma globulin (IVIG) ended the hemolysis and resulted in a rise in platelet count. He fully recovered except for end-stage renal failure, but he underwent a successful renal transplant after peritoneal dialysis. He has not suffered a relapse of TMA or an allograft rejection for 4 years. IVIG might be an option for some patients with TMA of unknown etiology refractory to conventional treatment.
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Metadata
Title
Intravenous gamma globulin for thrombotic microangiopathy of unknown etiology
Authors
Shuichi Ito
Kenichi Okuyama
Tomoko Nakamura
Jun-ichi Tetanishi
Kazuo Saito
Masanori Matsumoto
Yoshihiro Fujimura
Yukoh Aihara
Shumpei Yokota
Publication date
01-02-2007
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 2/2007
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-006-0326-9

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