ABSTRACT
To evaluate the clinical manifestations, diagnostic features, disease course and response to treatment among Saudi adults with predominantly hepatic Wilson's disease. A retrospective cohort study of 40 adult patients diagnosed with predominantly hepatic Wilson's disease between 1994 and 2008 at King Abdulaziz Medical City, Riyadh was carried out. The diagnosis was based on varying combinations of clinical and laboratory evidence of liver disease, presence of Kayser Fleisher rings, low serum ceruloplasmin levels, elevated 24 hour urinary copper excretion and histopathological findings on liver biopsy. The most frequent clinical presentation was decompensated chronic liver disease in 19 [47.5%], followed by chronic hepatitis in 15 [37.5%] and fulminant hepatic failure [FHF] in 5 [12.5%] patients. Eight [20%] patients with end-stage liver disease had liver transplantation, while 24 [60%] patients followed up on medical treatment for a variable period of 1-12 years showed clinical and laboratory improvement. One patient was lost early in follow up. Eight [20%] patients died during the study period, 5 with FHF, and 2 with advanced hepatic and neurological disease and one seven years after liver transplantation. Mortality rate was 100% in FHF without liver transplantation. A predominantly hepatic Wilson's disease has varied clinical presentations with decompensated chronic liver disease being the most common among adult patients. Majority of the patients show stabilization of the disease on medical treatment. FHF in Wilson's disease has a grave prognosis without liver transplantation, the later remains a definitive treatment option for decompensated cirrhotics and patients with FHF.
ABSTRACT
The aim of this study is to compare the response of hepatitis C virus [HCV] genotype 4 with other genotypes to anti-viral treatment among Saudi patients in a prospective randomized trial. The study was conducted in the Department of Hepatobiliary Sciences at King Abdul-Aziz Medical City, King Fahad National Guard Hospital, Riyadh, Kingdom of Saudi Arabia from March 1997 to January 2000. Sixty-two patients [33 males and 29 females] aged >/= 18 with chronic hepatitis C not treated previously were tested for HCV genotype and randomly assigned to receive interferon [IFN] alfa 2b 3 million units 3 times per week alone or in combination with ribavirin 1000-1200mg orally per day for 48 weeks. All patients were monitored for safety and efficacy of the therapy at 4 week intervals during treatment and followed up for at least 24 weeks after completion of treatment. The primary end point was loss of detectable HCV-RNA 24 weeks after treatment completion, defined as sustained virological response [SVR]. Hepatitis C virus genotype 4 was seen among [64.5%] HCV Saudi patients. Hepatitis C virus genotype 1 was the next most common [30.6%]. A SVR of 42.8% [9 out of 21] was seen in HCV genotype 4 and 40% [4 out of 10] among other HCV genotypes with combination therapy of IFN and ribavirin [p>0.1]. With IFN alone the sustained response rate was 15.7% for genotype 4 and 16.6% for other genotypes mainly genotype 1 [p>0.1]. We concluded that HCV genotype 4 is the most prevalent genotype among HCV infected Saudi patients. Genotype 1 was the next most common while genotypes 2, 3 and 5 were least prevalent. There is no statistically significant difference in response rate of patients with HCV genotype 4 to either IFN alone or IFN plus ribavirin when compared with genotype 1 of HCV