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本文旨在通过对近一年来遇到的6例肝豆状核变性患儿的描写。指出该病的某些早期临床表现,从而早诊断,早治疗,改善愈后。 本组6例中男性1例,女性5例。年龄6—12岁。起病类型:神经系统症状者2例,肝炎者1例,溶血性贫血者1例,无症状者2例。6例中有1例的临床经过与暂时性血管内溶血伴急性肝脏衰竭相符。5例K-F环(+),全部病例的血清铜氧化酶活力测定均明显降低。病程半月至5年不等。2例死亡,1例症状改善,治疗后能上学,1例因家长放
The aim of this article is to describe the 6 children with hepatolenticular degeneration encountered in the past year. Pointed out some of the early clinical manifestations of the disease, which early diagnosis and early treatment, improve the prognosis. The group of 6 patients in 1 male and 5 female. Age 6-12 years old. Type of onset: 2 cases of neurological symptoms, 1 case of hepatitis, hemolytic anemia in 1 case, asymptomatic in 2 cases. One of six patients had clinical evidence of transient intravascular hemolysis with acute liver failure. 5 cases of K-F ring (+), all cases of serum copper oxidase activity were significantly lower. Duration of half a month to 5 years. Two patients died, one patient had symptoms improved, he could go to school after treatment, and one patient was released by parents