小儿肝豆状核变性CT脑扫描的临床价值(附13例分析)

本文报告了13例小儿肝豆状核变性病例,年龄8——12岁。以神经症状及体征首发6例,以肝脏症状及体征首发3例,溶血首发2例,2例查体发现。13例眼角膜后弹力层均查见K—F氏环。血清铜蓝蛋白均低于正常值,其父母及部分同胞亦做了血清铜及铜蓝蛋白测定,且铜蓝蛋白也低于正常。CT改变为脑变性及继发性脑萎缩,表现为形状及大小不一的低密度灶。脑CT的改变通常与临床症状及体征相一致。且CT作为症状前患者的早期诊断及与慢性肝病患者的鉴别均有价值。此外,定期CT扫描有助于修订治疗方案、观察疗效及判断预后。

Clinical Value of CT Scanning in Children Hapatolenticular Degeneration (Wilson''''s disease)(P. S. 13 cases analysis)

13 cases of children hepatolenticular degeneration are reported in this paper, age from 8 to 12. 6 cases began With nervous symptoms and signs, 3 cases began With hepatic symptoms and signs, 2 cases began With hemolysis, 2 cases are found in physical examination. K-F's ring is seen in 13 cases with postelastic membrane of cornea.Serum ceruloplasmin is lower than normal. The serum copper and serum ceruloplasmin of their parents and some relatives are also examined, and their serum copper and ceruloplasmin is lower than normal. The CT changes are cerebral degeneration and secondary cerebral atrophy, manifested as low density focus different in both shape and size. The CT changes of cerebrum were always compatible with clinical symptoms and signs. CT scanning was valuable in early diagnosis of symptomless patients and also in differential diagnosis with chronic hepatic disease. Also, periodical CT scanning can help to revise the therapeutic plan, observe the treatment result and decide the prognosis.