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血友病的家庭治疗。一项随访研究。

Home treatment of haemophilia. A follow-up study.

作者信息

Ekert H, Moorehead M, Williamson G

出版信息

Med J Aust. 1981 Jul 11;2(1):21-3.

PMID:6792476
Abstract

Twenty-four children and adolescents who have been receiving home treatment for haemophilia A and B, and were followed up for a median period of five years, have been assessed for physical activity, social adjustment, range of joint movement and infection with hepatitis viruses. They were treated with cryoprecipitate from 1972 to 1977, and since then with factor-VIII concentrates. The average dose of factor VIII was 20 units/kg body mass. It was found that there was near normal range of physical activity and school performance, and, in virtually all families, near normal family function could be preserved. Approximately one-third of the patients showed impairment of the normal range of joint movement in flexion and extension. Although there was no clinical evidence of liver disease, elevated aspartate aminotransferase (AST) levels were found in 14 patients. Evidence of past, or present, infection with hepatitis B was found in 19 patients, and of infection with hepatitis A in seven patients. Home treatment is associated with a reduced level of disability from haemophilia, but transfusion therapy continues to be associated with a high rate of liver function abnormalities, probably of infectious origin.

摘要

对24名接受甲型和乙型血友病家庭治疗的儿童及青少年进行了评估,评估内容包括身体活动、社会适应、关节活动范围以及肝炎病毒感染情况。这些患者接受了中位时间为五年的随访。他们在1972年至1977年期间接受冷沉淀治疗,从那以后接受凝血因子VIII浓缩物治疗。凝血因子VIII的平均剂量为每千克体重20单位。结果发现,患者的身体活动和学业表现接近正常范围,而且几乎在所有家庭中,家庭功能都能维持在接近正常的水平。约三分之一的患者在屈伸关节时出现关节活动范围受损。虽然没有肝病的临床证据,但在14名患者中发现天门冬氨酸氨基转移酶(AST)水平升高。19名患者有既往或目前感染乙型肝炎的证据,7名患者有感染甲型肝炎的证据。家庭治疗与血友病导致的残疾程度降低有关,但输血治疗仍然与较高的肝功能异常发生率相关,肝功能异常可能源于感染。

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