肝豆状核变性又名wilson氏病,是一种常染色体隐性遗传性进行性疾病。多在儿童和青年期发病,早的4～5岁即可出现症状。目前认为本病系全身性铜代谢障碍引起的疾病。主要病理改变是豆状核退行性变和结节性肝硬化。由于本病常被看作是神经系统变性疾病,对以肝病为首发症状的腹型患者常漏诊,以致影响疾病预后,为吸取经验教训,现将我院儿科1978年收治的2例患儿,结合文献复习作一报道。 Wilson’s disease, also known as wilson’s disease, is an autosomal recessive inherited progressive disease. More incidence in children and adolescence, as early as 4-5 years old can be symptoms. At present, the disease is caused by systemic copper metabolism disorders. The main pathological changes are somatic degeneration and nodular cirrhosis. As the disease is often seen as a degenerative disease of the nervous system, the first symptom of liver disease in abdominal patients often missed diagnosis, which affects the prognosis of the disease, to learn from experience, now our hospital pediatric patients admitted in 1978 2 cases, Combined with literature review for a report.