Denosumab-induced autoimmune hepatitis: Case report.

This report described a patient not known to have a hepatic disease, found to have a drug-induced autoimmune hepatitis from denosumab. This is an unreported side effect, and here, we presented the possible predisposing factors and suggested monitoring parameters.

Saudi association for the study of liver diseases and transplantation position statement on the hepatology workforce in Saudi Arabia.

The field of hepatology has evolved significantly over the last two decades. Hepatology practice in Saudi Arabia (SA) was dominated by hepatitis B and C viruses but is now being overtaken by patients with non-alcoholic fatty liver disease. These patients require greater medical attention as their care is more complex compared to patients with viral hepatitis. In addition, liver transplantation (LT) has expanded significantly in SA over the last three decades. There is a necessity to increase the hepatology workforce to meet the demand in SA. The time has come to reinforce the transplant hepatology fellowship program, that was launched recently, and to develop a nurse practitioner practice model to meet these demands. In addition, SA is going through a health care reform to enhance health care delivery which may affect the financial compensation polices of various specialties including gastroenterology and hepatology. Therefore, the Saudi Association for the Study of Liver diseases and Transplantation (SASLT) established a task force to discuss the current and future demands in the hepatology workforce in SA, as well as to discuss different avenues of financial compensation for transplant hepatologists in LT centers.

Clinical Practice Guidelines for Liver Transplantation in Saudi Arabia.

The demand for liver transplantation in the Kingdom of Saudi Arabia (KSA) is associated with the country's high burden of liver disease. Trends in the epidemiology of liver transplantation indications among recipients in KSA have changed over 20 years. Non-alcoholic steatohepatitis has eclipsed the hepatitis C virus in the country due to the effective treatment strategies for HCV. Risk factors for NASH, like type 2 diabetes mellitus, obesity, and hyperlipidemia, are becoming a major concern and a leading indication for liver transplantation in the KSA. There is also a significantly increased prevalence and incidence of genetic adult familial liver diseases in KSA. New immunosuppressive agents and preservation solutions, improved surgical capabilities, and early disease recognition and management have increased the success rate of liver transplant outcome but concerns about the side effects of immunosuppressive therapy can jeopardise long-term survival outcomes. Despite this, indications for liver transplantation continue to increase, resulting in ongoing challenges to maximize the number of potential donors and reduce patient mortality rate while expecting to get transplanted. The Saudi Center of Organ Transplant is the recognized National Organ Donation Agency for transplantation, which renders important support for procurement and allocation of organs. This guidance document aims to help healthcare providers in managing patients in the liver transplant setting.

New paradigms of USP53 disease: normal GGT cholestasis, BRIC, cholangiopathy, and responsiveness to rifampicin.

Biallelic variants in the USP53 gene have recently been reported to segregate with normal gamma glutamyltransferase (GGT) cholestasis. Using whole-exome sequencing (WES), we detected two USP53 homozygous variants (c.951delT; p. Phe317fs and c.1744C>T; p. Arg582*) in five additional cases, including an unpublished cousin of a previously described family with intractable itching and normal GGT cholestasis. Three patients, a child and two adults, presented with recurrent episodes of normal GGT cholestasis, consistent with a diagnosis of benign recurrent intrahepatic cholestasis (BRIC). Cholangiopathic changes, possibly autoimmune in origin, were recognized in some patients. Additional phenotypic details in one patient included an enlarged left kidney, and speech/developmental delay. Notably, two patients exhibited a complete response to rifampicin, and one responded to ursodeoxycholic acid (UDCA). Two adult patients were suspected to have autoimmune liver disease and treated with steroids. This report describes new cases of USP53 disease presenting with normal GGT cholestasis or BRIC in three children and two adults. We also describe the novel finding of a dramatic response to rifampicin. The association of cholangiopathy with normal GGT cholestasis provides a diagnostic challenge and remains poorly understood.

A tool to measure the economic impact of Hepatitis B elimination: A case study in Saudi Arabia.

BACKGROUND: No virologic cure exists for hepatitis B virus (HBV) infection, and existing therapies are designed to control viral replication. We aimed to estimate the national prevalence of HBsAg in 2017 and study the impact of an enhanced diagnosis rate and universal treatment administration on HBV-related outcomes in Saudi Arabia. MATERIALS AND METHODS: A dynamic transmission and disease burden model was developed to estimate the future economic burden of HBV infection. The infected population was tracked by age and gender-defined cohorts; direct costs (healthcare, screening, diagnostics and treatment) and indirect costs (disability-adjusted life years and the value of a statistical life year) were calculated. The impact of two intervention scenarios (Achieve WHO Targets: diagnose 90% of infections and treat 80% of high viral load patients by 2030; and Diagnose and Treat All: diagnose and treat all infected patients by 2022) were compared against the Base Case scenario (no policy action), with near-universal vaccination coverage rates held constant. A sensitivity analysis of future treatment cost was also conducted. RESULTS: In 2017, HBsAg prevalence was estimated at 1.7%, corresponding to 574,000 infections. The same year, there was an estimated incidence of 490 cases of decompensated cirrhosis, 1500 cases of hepatocellular carcinoma (HCC) and 1740 liver-related deaths (LRD). HBsAg prevalence was 0.1% among 5-year-olds and <0.1% among infants. Disease burden outcomes by 2030, as compared with 2015, were as follows - Base Case: LRDs and HCC incidence were projected to increase by 70%. WHO Targets: A 30-35% decline in both HCC incidence and LRDs. Diagnose and Treat All: A 50-55% decline in HCC incidence and LRDs. In all scenarios, HBsAg prevalence among infants and 5-year-olds declined to <0.1% with the Diagnose and Treat all scenario resulting in a prevalence approaching zero in this age group. Annual direct costs are projected to increase and peak by 2022 in both intervention scenarios due to expansion of treatment and diagnostics. However, these are offset by the reduction of indirect economic costs, starting immediately in the WHO Targets scenario and by 2023 in the strategy to diagnose and treat all. Achieving WHO Targets is estimated to achieve a positive return on investment (ROI) by 2021 when examining direct costs and indirect economic losses at a treatment price of $2700 USD per patient yearly. Diagnosing and treating all patients, however, would require at least a 50% reduction in the unit cost of treatment to achieve a positive ROI by 2029. CONCLUSIONS: Increased diagnosis and treatment rates of HBV would lead to substantial declines in HCC and LRD. This effect would be dramatically enhanced by administering treatment to all HBV cases regardless of viral load and estimated to be highly cost-effective if treatment prices can be substantially reduced.

The impact of metabolic syndrome and prevalent liver disease on living donor liver transplantation: a pressing need to expand the pool.

BACKGROUND AND AIMS: Organ shortage has been the ongoing obstacle to expanding liver transplantation worldwide. Living donor liver transplantation (LDLT) is hoped to improve this shortage. The aim of the present study is to analyze the impact of metabolic syndrome and prevalent liver disease on living donations. METHODS: From July 2007 to May 2012, 1065 potential living donors were evaluated according to a stepwise evaluation protocol. The age of the worked-up donors ranged from 18 to 45 years. RESULTS: Only 190 (18%) were accepted for donation, and 875 (82%) were rejected. In total, 265 (24.9%) potential donors were excluded because of either diabetes or a body mass index >28. Some potential donors were excluded at initial screening because of incompatible blood groups (115; 10.8%), social reasons (40; 3.8%), or elevated liver enzymes (9; 1%). Eighty-five (8%) donors were excluded because of positive hepatitis serology. Steatosis resulted in the exclusion of 84 (8%) donors. In addition, 80 (7.5%) potential donors were rejected because of variations in biliary anatomy, and 20 (2%) were rejected because of aberrant vascular anatomy. Rejection due to biliary-related aberrancy decreased significantly in the second half of our program (11 vs. 4%, p = 0.001). In total, 110 (10.3%) potential donors were rejected because of insufficient remnant volume (<30%) as determined by CT volumetry, whereas 24 (2.2%) were rejected because of a graft-to-recipient body weight ratio less than 0.8%. CONCLUSION: Metabolic syndrome and viral hepatitis negatively impacted our living donor pool. Expanding the donor pool requires the implementation of new strategies.