Wiel E, Marciniak B, Wibaut B
Département d'anesthésie-réanimation chirurgicale 2, hôpital Claude-Huriez, CHU Lille, France.
Ann Fr Anesth Reanim. 1998;17(1):61-4. doi: 10.1016/s0750-7658(97)80186-4.
The authors report the case of a 22-month-old boy experiencing a voluminous subcutaneous haematoma, 72 hours after a head trauma. Two subsequent drainages of this haematoma were required because of its recurrence. The child, whose parents had blood relations, suffered from recurrent bleeding since his birth. A standard haemostasis assessment including prothrombin time, activated partial thrombopiastin time, bleeding time, concentration of fibrinogen and platelet count was unremarkable. Therefore, coagulation factors were explored. An inherited factor XIII deficiency (less than 2%) was recognized. A new drain was inserted, after administration of factor XIII concentrate. The time course of the haematoma was favourable. After discharge, the prophylactic therapy consisted of an injection of factor XIII concentrate (50 Ul.kg-1) every 5 weeks.
作者报告了一名22个月大男童的病例，该男童在头部外伤72小时后出现大量皮下血肿。由于血肿复发，随后进行了两次引流。该患儿父母有血缘关系，自出生以来就反复出血。包括凝血酶原时间、活化部分凝血活酶时间、出血时间、纤维蛋白原浓度和血小板计数在内的标准止血评估无异常。因此，对凝血因子进行了检查。发现存在遗传性因子XIII缺乏（低于2%）。在注射因子XIII浓缩物后插入了一根新的引流管。血肿的病程进展顺利。出院后，预防性治疗包括每5周注射一次因子XIII浓缩物（50 Ul.kg-1）。
