先天性纤维蛋白稳定因子[因子 XIII]缺乏症
[Congenital deficiency of fibrin stabilizing factor [factor XIII)].
作者信息
Blanckaert D, Oueidat I, Chelala J, Loeuille G A, Delepoulle F
机构信息
Service de pédiatrie et médecine de l'adolescence, CHG, Dunkerque, France.
出版信息
Pediatrie. 1993;48(6):451-3.
PMID:8247644
Abstract
The authors report a new case of congenital deficiency of fibrin stabilizing factor. The defect is transmitted as an autosomal recessive trait. The earliest symptom is umbilical bleeding when the cord separates. The diagnosis is confirmed by the rapid dispersion of the clot in urea 5 M or monochloroacetic acid.
摘要
作者报告了一例新的纤维蛋白稳定因子先天性缺乏病例。该缺陷以常染色体隐性性状遗传。最早的症状是脐带脱落时脐部出血。通过在5M尿素或一氯乙酸中凝块迅速溶解来确诊。
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