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我科于1994年7月～2000年3月,共收治肝豆状核变性患儿8例,现将其合并症的表现与治疗报道如下。 1 临床资料 8例中,男3例,女5例;年龄6～10岁,平均7.9岁;病程15天～5年;住院天数3～40天,平均25.6天。治疗后转归:未愈4例,好转3例,死亡1例。5例以黄疸型肝炎、2例以肾病入院。8例均无家族遗传病史。1例父母系近亲结婚。其母患甲亢,肝功不良者各1例。8例均经化验、跟科检查证实为肝豆状核变性。
My department in July 1994 ~ March 2000, a total of 8 cases of children with Wilson’s disease, now the performance of the complication and treatment reported as follows. 1 Clinical data 8 cases, 3 males and 5 females; aged 6 to 10 years, mean 7.9 years; duration of 15 days to 5 years; hospital stay days 3 to 40 days, an average of 25.6 days. After treatment outcome: unhealed in 4 cases, improved in 3 cases, 1 case of death. 5 cases of jaundice hepatitis, 2 cases of renal disease hospitalized. All 8 cases had no family history. One parent was married. The mother suffering from hyperthyroidism, liver dysfunction in 1 case. 8 cases were tested, with the examination confirmed liver degeneration.