Department of Pediatrics, SMGS Hospital, Shalamar, Jammu, India.
Indian J Pediatr. 2013 Jul;80(7):607-8. doi: 10.1007/s12098-012-0796-7. Epub 2012 Jun 14.
Factor X deficiency is an extremely rare coagulation defect inherited as an autosomal recessive disorder with variable bleeding manifestations. The authors report case of a 16 y-old girl born from a consanguineous marriage who presented with excessive bleeding at the start of menarche. Investigations revealed severe anemia, prolongation of both prothrombin time and activated partial thromboplastin time and moderate deficiency of factor X (1 %). She was given multiple transfusions including packed cells and fresh frozen plasma and was advised to remain under regular follow up.
X 因子缺乏症是一种极为罕见的遗传性凝血缺陷疾病,呈常染色体隐性遗传,具有不同的出血表现。作者报告了一例 16 岁女孩的病例,她出生于近亲婚姻,初潮时出现过度出血。检查发现严重贫血,凝血酶原时间和激活部分凝血活酶时间延长,X 因子中度缺乏(1%)。她接受了多次输血,包括浓缩红细胞和新鲜冷冻血浆,并被建议定期随访。