Merchant R H, Agarwal B R, Currimbhoy Z, Pherwani A, Avasthi B
Division of Neonatology, B.J. Wadia Hospital for Children, Parel, Bombay.
Indian Pediatr. 1992 Jul;29(7):831-6.
Clinical and hematological data of 9 cases with factor XIII deficiency is highlighted. The age at first bleed ranged from 3 days of life to 1 year. Seven of these 9 cases had bleeding from the umbilicus, 3 had recurrent subcutaneous and muscle hematomas, while 4 cases had CNS bleeds of which 3 expired. Routine coagulogram was normal, while clot solubility in 5 molar urea solution was abnormal in all cases. Factor XIII assay was not done in any. Patients were treated with plasma transfusion during episodes of bleeding. No patient received plasma transfusion as prophylactic therapy. The cumulative Indian data so far documented, inclusive of this series, shows a very high incidence of CNS bleeds (33%) in patients with this inherited coagulation disorder.
重点介绍了9例因子 XIII 缺乏症患者的临床和血液学数据。首次出血的年龄范围从出生3天至1岁。这9例患者中有7例出现脐部出血，3例有复发性皮下和肌肉血肿，4例发生中枢神经系统出血，其中3例死亡。常规凝血检查正常，但所有病例在5摩尔尿素溶液中的凝块溶解度均异常。所有病例均未进行因子 XIII 检测。患者在出血发作期间接受血浆输注治疗。没有患者接受预防性血浆输注治疗。迄今为止记录的包括本系列在内的印度累积数据显示，这种遗传性凝血障碍患者的中枢神经系统出血发生率非常高（33%）。
