Presentation, diagnosis and outcome of predominantly hepatic wilson's disease in adult Saudi patients: a single centre experience

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.

Saudi gelines for the diagnosis and management of hepatocellular carcinoma: technical review and practice guidelines created and endorsed by the saudi association for the study of liver diseases and transplantation and the Saudi oncology society

Recognizing the significant prevalence of hepatocellular carcinoma [HCC] in Saudi Arabia, and the difficulties often faced in early and accurate diagnoses, evidence-based management, and the need for appropriate referral of HCC patients, the Saudi Association for the study of liver diseases and transplantation [SASLT] formed a multi-disciplinary task force to evaluate and update the previously published guidelines by the Saudi Gastroenterology Association. These guidelines were later reviewed, adopted and endorsed by the Saudi Oncology Society [SOS] as its official HCC guidelines as well. The committee assigned to revise the Saudi HCC guidelines was composed of hepatologists, oncologists, liver surgeons, transplant surgeons, and interventional radiologists. Two members of the task force served as guidelines editors. A wide based search on all published reports on all aspects of the epidemiology, natural history, risk factors, diagnosis, and management of HCC was performed. All available literature was critically examined and available evidence was then classified according to its strength. The whole document and the recommendations were then discussed in details by members and consensus was obtained. All recommendations in these guidelines were based on the best available evidence, but were tailored to the patients treated in Saudi Arabia. We hope that these guidelines will improve HCC patient care and enhance the multidisciplinary care needed for these patients