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Orthotopic liver transplantation has been applied to the treatment of Wilson's disease (WD), living-related liver transplantation (LRLT) has also been indicated for WD with increasing frequency. Between January 2001 and November 2003, 22 LRLTs were performed on patients (19 pediatric, three adults) with WD in liver transplantation center. Two patients were transplanted because of a presentation coexistent with fulminant hepatic failure. Twenty presented with chronic advanced liver disease with ( n = 9) or without ( n = 11) associated neurologic manifestations. All the recipients had low serum ceruloplasmin levels with a mean value of 12.8 ± 3.2 mg/dl before transplantation and increased to an average of 26.0 ± 3.6 mg/dl after LRLT at the latest evaluation. The survival patients with neurologic manifestations such as tremor, dysarthia, dysphagia, dystonia and sialorrhea had improved after LRLT. This suggests that LRLT not only resolves the hepatic but also ameliorates the neurologic consequences of WD. [ABSTRACT FROM AUTHOR]

Although an accurate anatomical understanding of the hepatic arteries is the most and essential step in living-related liver transplantation (LRLT), the need to reduce the burden place on the donor should be considered in imaging diagnosis. The present study examined the reliability of intravenously enhanced three-dimensional (3D) angiography from multidetector-row computed tomography (MDCT) in evaluating the anatomical configuration of the hepatic arteries comparing with those from conventional angiography by Seldinger method. A total of 109 patients underwent MDCT and 3D images were reconstructed on arterial phase using the volume rendering (VR) method. In the case of 3D angiography, at an infusion rate of 4 ml/s, the extrahepatic hepatic arteries were visualized successfully in all cases (the right, left and middle hepatic artery). The aberrant hepatic arteries were successfully visualized in 23 of 24 cases. The 3D angiography is a reliable method of visualizing the extrahepatic and aberrant hepatic arteries. This minimally invasive examination procedure is useful in individual operative planning and is help to increase the safety of surgery. [ABSTRACT FROM AUTHOR]