哈桑·萨迪金医院的先天性无纤维蛋白原血症
Congenital afibrinogenemia in Hasan Sadikin Hospital.
作者信息
Supandiman I, Sumantri R
机构信息
Department of Internal Medicine, School of Medicine, Padjadjaran University, Hasan Sadikin Hospital, Bandung, Indonesia.
出版信息
Southeast Asian J Trop Med Public Health. 1993;24 Suppl 1:267-9.
A case of a 24-year-old male with congenital afibrinogenemia has been discussed. The diagnosis was made based upon history, physical examination, umbilical cord bleeding, bleeding history and similar cases were found being the patient's grandfather and brother. Laboratory tests supported the diagnosis with a prolonged bleeding time, prothrombin time (PT), undetected partial thromboplastin time (PTT), flat line on thromboelastogram, and undetected fibrinogen (less than 78 mg/dl), with substitution test which showed fibrinogen deficiency.
讨论了一例24岁先天性无纤维蛋白原血症男性患者。根据病史、体格检查、脐带出血、出血史做出诊断，发现患者的祖父和兄弟有类似病例。实验室检查支持诊断，出血时间、凝血酶原时间（PT）延长，活化部分凝血活酶时间（PTT）未测出，血栓弹力图呈平线，纤维蛋白原未测出（低于78mg/dl），替代试验显示纤维蛋白原缺乏。
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