Kashani A A
Trans Ophthalmol Soc U K (1962). 1977 Apr;97(1):18-9.
Within the last 5 years, 25 proved cases of hepato-lenticular degeneration (Wilson's disease) have been seen at the Children's Hospital Medical Center affiliated to Tehran University. The disease manifested abnormal copper metabolism in the following respects: (1) Hypoceruloplasminaemia was present: (2) 24-hr urinary copper excretion was low; (3) Tissue concentration of copper was high; (4) Treatment with penicillamine led to increased copper excretion in the urine and usually to relief of symptoms. The ophthalmologist cannot always assist the paediatrician in diagnosis, but fourteen of the 25 patients showed a Kayser-Fleischer ring and these were all in the older age groups. The following conclusions were drawn: (1) The incidence of Wilson's disease in Iran is high; (2) Penicillamine treatment may be successful; (3) Any young person with kidney, liver, or neurological disease of uncertain aetiology should have a detailed ophthalmological examination.
在过去5年里，德黑兰大学附属儿童医院共确诊了25例肝豆状核变性（威尔逊氏病）病例。该病在以下方面表现出铜代谢异常：（1）存在低铜蓝蛋白血症；（2）24小时尿铜排泄量低；（3）组织铜浓度高；（4）用青霉胺治疗可使尿铜排泄增加，且通常能缓解症状。眼科医生并非总能协助儿科医生进行诊断，但25例患者中有14例出现了凯泽尔-弗莱舍尔环，且这些患者均为年龄较大的群体。得出了以下结论：（1）威尔逊氏病在伊朗的发病率很高；（2）青霉胺治疗可能会取得成功；（3）任何患有病因不明的肾脏、肝脏或神经系统疾病的年轻人都应接受详细的眼科检查。
