Hemolytic uremic syndrome (HUS) is a life-threatening infectious disease in childhood for which there is no confirmed therapeutic strategy. Endothelial inflammation leading to microthrombosis formation via complement activation is the main pathology of HUS. Thrombomodulin is an endothelial membrane protein that has anticoagulation and anti-inflammatory effects, including the suppression of complement activity. Recombinant human soluble thrombomodulin (rTM) is a novel therapeutic medicine for disseminated intravascular coagulation. We administered rTM to 3 patients with HUS for 7 days and investigated the outcomes in view of the patients’ prognoses, changes in biochemical markers, complications, and adverse effects of rTM. Symptoms and laboratory data improved after initiation of rTM in all 3 patients. Abnormal activation of complements was also dramatically suppressed in 1 patient. The patients recovered without any complications or adverse effects of rTM. They were discharged having normal neurologic status and with no renal dysfunction. To our knowledge, this is the first report of rTM being used to treat HUS. These case reports show the positive effect of rTM in patients with HUS. Randomized controlled studies should be performed to assess the efficacy and safety of rTM for children with HUS.
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March 2013
Case Report|
March 01 2013
A Novel Strategy for Hemolytic Uremic Syndrome: Successful Treatment With Thrombomodulin α
Takashi Honda, MD;
Takashi Honda, MD
Department of Pediatrics, Kitasato University School of Medicine, Sagamihara, Japan
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Shohei Ogata, MD;
Shohei Ogata, MD
Department of Pediatrics, Kitasato University School of Medicine, Sagamihara, Japan
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Eri Mineo, MD;
Eri Mineo, MD
Department of Pediatrics, Kitasato University School of Medicine, Sagamihara, Japan
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Yukako Nagamori, MD;
Yukako Nagamori, MD
Department of Pediatrics, Kitasato University School of Medicine, Sagamihara, Japan
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Shinya Nakamura, MD;
Shinya Nakamura, MD
Department of Pediatrics, Kitasato University School of Medicine, Sagamihara, Japan
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Yuki Bando, MD;
Yuki Bando, MD
Department of Pediatrics, Kitasato University School of Medicine, Sagamihara, Japan
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Masahiro Ishii, MD
Department of Pediatrics, Kitasato University School of Medicine, Sagamihara, Japan
Address correspondence to Masahiro Ishii, MD, Department of Pediatrics, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0329, Japan. E-mail: ishiim@med.kitasato-u.ac.jp
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Address correspondence to Masahiro Ishii, MD, Department of Pediatrics, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0329, Japan. E-mail: ishiim@med.kitasato-u.ac.jp
FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.
Pediatrics (2013) 131 (3): e928–e933.
Article history
Accepted:
October 29 2012
Citation
Takashi Honda, Shohei Ogata, Eri Mineo, Yukako Nagamori, Shinya Nakamura, Yuki Bando, Masahiro Ishii; A Novel Strategy for Hemolytic Uremic Syndrome: Successful Treatment With Thrombomodulin α. Pediatrics March 2013; 131 (3): e928–e933. 10.1542/peds.2012-1466
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