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多次输血的青少年血友病患者患慢性肝病的高风险。

The high risk of chronic liver disease in multitransfused juvenile hemophiliac patients.

作者信息

Spero J A, Lewis J H, Fisher S E, Hasiba U, Van Thiel D H

出版信息

J Pediatr. 1979 Jun;94(6):875-8. doi: 10.1016/s0022-3476(79)80205-x.

Abstract

Eighty-seven asymptomatic children with either hemophilia A or B were treated before they were 21 years of age. Seventy-two received factor concentrates and 15 cryoprecipitate or fresh-frozen plasma only. Thirty-two of the 72 in the former group have persistently elevated alanine aminotransferase values compared to one of 15 in the latter group. In a subset of children treated with factor concentrates before five years of age, four of seven are chronically HBsAg positive. Liver biopsies were performed in 13 of the 32 asymptomatic patients with abnormal ALT values in the fraction group. It is recommended that until further data become available, children with mild hemophilia and all less than 5 years of age should receive only cryo or FFP.

摘要

87名患有甲型或乙型血友病的无症状儿童在21岁之前接受了治疗。72名接受了凝血因子浓缩物治疗,15名仅接受了冷沉淀或新鲜冰冻血浆治疗。前一组72名患者中有32名丙氨酸转氨酶值持续升高,而后一组15名患者中有1名丙氨酸转氨酶值持续升高。在5岁之前接受凝血因子浓缩物治疗的儿童亚组中,7名中有4名慢性乙肝表面抗原阳性。在凝血因子浓缩物组中,32名无症状且谷丙转氨酶值异常的患者中有13名进行了肝活检。建议在获得更多数据之前,轻度血友病儿童以及所有5岁以下儿童应仅接受冷沉淀或新鲜冰冻血浆治疗。

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