Popis: |
Wilson disease (WD) can be effectively treated with zinc: the mechanisms of action are still debated and probably involve induction of metallothionein (MT) synthesis. We measured MT, zinc and copper concentrations in the duodenal mucosa and in the hepatic tissue of WD pts on long term treatment either with zinc sulphate or D-penicillamine. Fifteen WD pts (3F, 12M, mean age: 25.8 years) underwent endoscopy with duodenal biopsies and liver biopsy as part of the diagnostic or follow up protocol. 12 pts were receiving zinc sulphate (ZnSO4, 220 mg po rid), 2 D-PCA (1200 mg/day) and one patient was studied at diagnosis. Two groups of patients were included as controls: 17 pts (8F, 9M, mean age 32 years) undergoing endoscopy for dyspepsia and 8 pts (5F, 3M, mean age 41 years) undergoing liver biopsy for chronic viral hepatitis. MT concentration (ug/mg protein) was measured by the Silver saturation method, zinc (ug/mg dry wt) and copper (ug/mg dry wt) by atomic absorption spectrophotometry. Duodenal findings: MT concentration was significantly increased in WD pts on zinc therapy (11.66 _+ 9) with respect to controls with negative endoscopy (0.76 _+ 0.38), pts with duodenitis (1.4 _+ 0.5) and pts on D-PCA (1.2 + 0.04) (p |