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凝血因子II、VII、IX和X联合缺乏:一例可能为先天性病因的病例。

Combined deficiency of coagulation factors II, VII, IX, and X: a case of probable congenital origin.

作者信息

Ekelund H, Lindeberg L, Wranne L

机构信息

Department of Paediatrics, Ostra Sjukhuset, Göteborg, Sweden.

出版信息

Pediatr Hematol Oncol. 1986;3(2):187-93. doi: 10.3109/08880018609031217.

Abstract

Combined deficiency of coagulant activity of the vitamin K-dependent factors was found in a 14-year-old boy suffering from severe hemorrhages. Immunoassays revealed the presence of acarboxyprothrombin. The bleedings could be controlled, but the coagulation defects persisted during more than 2 years' follow-up and could not be corrected by oral or parenteral vitamin K. No intoxication or underlying disease was found. The abnormality was considered a congenital disorder of the carboxylation of prothrombin.

摘要

在一名患有严重出血的14岁男孩中发现维生素K依赖因子的凝血活性联合缺乏。免疫测定显示存在脱羧基凝血酶原。出血可以得到控制,但在超过2年的随访期间凝血缺陷持续存在,口服或胃肠外补充维生素K均无法纠正。未发现中毒或潜在疾病。这种异常被认为是凝血酶原羧化的先天性障碍。

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