Background: Wilson's disease (WD), a disorder of copper metabolism characterized by copper overload and autosomal recessive inheritance was first described in 1912. Mutation in ATP7B causes dysfunction of ATP7B protein and reduction in copper excretion into bile in hepatocytes and excess copper accumulation leads to liver injury. D-Pencillamine primarily can inhibit fibrogenesis and preventing the appearance of scar lesions in liver, we studied this phenomenon in our patients retrospectively.
Material & Method: Pathologic slides from explanted liver of 26 patients diagnosed as having WD with hepato-neurological manifestation between 2000-2008 who treated with liver transplantation (LTx) at Namazi hospital were investigated retrospectively. Patients divided into two groups according to history of D-Penicillamine consumption before transplantation. Degree of fibrosis and inflammation classified as: mild (I), moderate (II) and severe (III) and reviewed by an impartial hepatopathologist.
Results: Of 26 cases (20 male, 6 female) with mean age of 17.6±8.6 years of WD, 69%(18/26) had history of D- Pencillamine use before LTx from 6 months to 9 years (mean 3.4±2.7 years). In Penicillamine group, 14 patients (77%) had grade I fibrosis. Grade II and III fibrosis were seen in 5.6 and 16% of patients, respectively. In this group inflammation was as : grade III 44%(8/18), grade II 44%(8/18), and grade I 11%(2/18). In non-Penicillamine group (8 cases), grades of fibrosis and inflammation were as follows: grade III 62%, grade II 25%, inflammation: grade I 12%, grade III 87%, grade I 13%, respectively. In Penicillamine group the degree of fibrosis was significantly lower than non-Penicillamine (p<0.05).
Conclusion: D-Penicillamine may reduce the rate of liver fibrogenesis in patients with WD as found in this study.
Volume : 6
Issue : 4
Pages : 33
Shiraz Transplant Center, Namazi Hospital, and Transplantation Research Center, Shiraz University of Medical Science, Shiraz, Iran