68例肝豆状核变性肾脏声像图观察

OBSERVATION ON ULTRASONOGRAM OF KIDNEY IN 68 CASES OF HEPATOLENTICULAR DISEASE

肝豆状核变性（ＨＬＤ），又称Ｗｉｌｓｏｎ病，是一种常染色体隐性遗传的铜代谢障碍引起的家族性疾病。通常于青少年期发病，由体内过剩的铜积蓄于肝、肾、脑、角膜等器官，除引起锥体外系症状外，往往早期出现肝、肾症状，易被误诊为肝炎、肝硬化或肾病延误治疗。本文报...

The Kidney was studied with Ultrasonography in 68 cases of HePatolenticular Disease(HLD)and its sonograms were classified into three types:normal (50 cases),coxtex damaged(14 cases)and pyramids calcified(4 cases).These different types demonstrated the pathological changes resulting from copper accumulation in kidney from mild to severe degree.Moreover,the cause of misdiagnosis was analysed and differentiation from other renal disease was explored.Hence it has an important clinical value.