• Hiroshi Yagasaki, Maiko Kato, Katsuyoshi Shimozawa, Maiko Hirai, Eri Nishikawa, Hirotsugu Okuma, Wakako Ishii, Yuki Imai, Masaharu Matsumura, Ryuta Yonezawa, Kayo Yoshikawa, Hiroyuki Shichino, Motoaki Chin, and Hideo Mugishima.
• Department of Pediatrics, School of Medicine, Nihon University, Tokyo, Japan. yagasaki@med.nihon-u.ac.jp
• Thromb. Res. 2012 Dec 1;130(6):e289-93.

IntroductionRecombinant thrombomodulin (rTM), which degrades factors Va and VIIIa by activating protein C, has been developed as a new drug for treating disseminated intravascular coagulation (DIC).Materials And MethodsSince July 2009, we have treated 25 children with DIC using rTM (380 U/kg/day, or 130 U/kg/day for newborns) as a first-line therapy. Median duration of rTM administration was 5 consecutive days (range, 2-13 days). We employed DIC criteria of the Japan Welfare and Health Ministry. The first day on which rTM treatment was given was defined as day 1.ResultsMedian patients age was 3 years. Underlying diseases were hematological disorders (n=13) and severe infection (n=12). Overall, 20 of the 25 patients had recovered from DIC by day 7 and 22 of the 25 patients remained alive at day 28. Median Pediatric Logistic Organ Dysfunction score improved from 11 on day 1 to 2 on day 7 (p=0.009). Laboratory data (median) on day 7 (prothrombin time (PT) ratio, 1.15; fibrin and fibrinogen degradation products (FDP), 9.6 mg/l; D-dimer, 1.6 mg/l FEU; antithrombin, 112%; protein C, 105%) were significantly improved compared to results on day 1 (PT ratio, 1.39; FDP, 21.6 mg/l; D-dimer, 6.4 mg/l FEU; antithrombin, 86%; protein C, 54%). Whereas, 5 patients failed to respond and serious bleeding events were observed in 2 newborns.ConclusionThe efficacy of rTM cannot be assessed from the present dataset, due to several limitations such as the small heterogenous patient cohort, and the lack of age- and disease-matched controls. Nevertheless, this case-series remains important in terms of enabling further prospective control studies to evaluate the efficacy of rTM in children.Copyright © 2012 Elsevier Ltd. All rights reserved.

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