Fourteen Years of Experience of Liver Transplantation for Wilson's Disease; a Report on 107 Cases from Shiraz, Iran.

BACKGROUND AND AIM: Liver transplantation is a potential cure for liver damage from Wilson's disease but the course of neuropsychiatric manifestations after transplantation remains undetermined. MATERIAL AND METHODS: In this study, data on all patients who'd received a liver transplant for Wilson's disease at the Shiraz Organ Transplantation Center between December 2000 and March 2014 were reviewed and compared to data on a control group who'd received a liver transplant over the same period but due to other causes. RESULTS: Out of 2198 patients who'd received a liver transplant in the period; 107 patients were diagnosed with Wilson's disease (21 with fulminant hepatic failure); age of patient ranged from 5 to 59 years; 56.07% of patients in this series had some type of neuropsychiatric manifestation before transplantation, of which 66.67% showed improvement after the procedure. 18 patients had aggravation of neuropsychiatric symptoms after transplantation. These neuropsychiatric symptoms were mostly for anxiety, tremor and depression but there were four cases of new onset dysarthria, rigidity and ataxia in various combinations. Survival rates of 1-month, 1-year, and 5-years for patients with Wilson's disease were 88%, 86%, 82%, respectively, evaluations were not statistically different from that of the control group. CONCLUSIONS: Liver transplantation showed good long-term results in patients with Wilson's disease, even in those presenting fulminant hepatic failure. Neuropsychiatric manifestations normally show improvement after transplantation but in some cases new onset of manifestations occurred after successful liver transplantation.

Hepatocellular carcinoma in explanted livers of patients with genotype d HBV cirrhosis: report of the first experience from Iran.

BACKGROUND: This study was conducted to determine the impact of hepatitis B virus (HBV) as a cause of hepatocellular carcinoma (HCC) in a single liver transplant center in Iran.  METHODS: We included all hepatectomy specimens from patients with HBV-related cirrhosis who underwent transplants from May 1993 until January 2012 in this study. From these, we determined the number that had HBV-induced HCC. Nested PCR results were used to determine the HBV genotype from sections of the hepatectomy pathology specimens. RESULTS: During this time period there were 1361 cirrhotic livers transplanted in our center. Of these, 249 were attributed to HBV cirrhosis. Overall, HCC was detected in 40 (2.9%) subjects, of which 29 (1.2%) had HBV-related HCC.  Genotype D was only genotype observed in all HBV subjects.  CONCLUSIONS: The results revealed that although HBV-related cirrhosis was the most frequent single cause for liver transplant, the frequency of HBV-induced HCC was very low among transplant recipients. Out of 1361 transplant recipients, only 29 (2.1%) were diagnosed with HBV-related HCC. All HBV subjects had genotype D.

Primary glomus tumor of the liver: a rare case report and review of the literature.

Glomus tumor is a rare neoplasm of the distal extremities. It occurs very rarely in the deep visceral organs such as stomach, lung, pancreas, ovary, and liver. Herein, we report a very rare case of glomus tumor of the liver in a 50-year-old woman presenting with abdominal mass which was diagnosed after surgery. The tumor was large and cystic; however, the morphology was similar to the ordinary soft-tissue glomus tumor. Tumor cells were reactive with CD34 and SMA. The patient's follow up failed to show any evidence of malignant behavior or tumor recurrence. This report is the third primary glomus tumor of the liver in the English literature.