Cystic Fibrosis Liver Disease: Know More
Journal Title: Oman Medical Journal - Year 2019, Vol 34, Issue 6
Abstract
Cystic fibrosis (CF) is a multisystem disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. CFTR is expressed in the apical surface of cholangiocytes. Homozygous CFTR gene mutation results in viscous and acidic bile secretions secondary to deficient surface fluid and bicarbonate efflux. Viscous, inspissated bile causes ductular obstruction and hepatotoxicity from retained bile components, leading to fibrosis and ultimately cirrhosis, known as CF liver disease (CFLD). CFLD is the third leading cause of death in CF patients. CFLD manifestations can take many forms. They range from asymptomatic elevation of transaminases to cirrhosis and end-stage liver disease. CFLD is diagnosed after excluding other causes of chronic liver disease. To date, there is no effective therapy to prevent or treat CFLD. Management of CFLD emphasizes on optimizing nutritional status. Ursodeoxycholic acid is the only available treatment that may prevent progression of CFLD at present. All CF patients with CFLD need annual investigations and follow-up for early detection of the disease. Liver transplantation should be considered in patients with decompensated cirrhosis and portal hypertension, with acceptable long-term outcomes. Novel therapies of CFLD are promising. This review article aims to summarize the published literature on CFLD, its pathophysiology, clinical features and complications, and management including new therapeutic options.
Authors and Affiliations
Siham Al Sinani, Sharef Al-Mulaabed, Khalid Al Naamani, Rabab Sultan
Keywords
Related Articles
- EP ID EP671224
- DOI 10.5001/omj.2019.90
- Views 73
- Downloads 0
Delayed Start Protocol with Gonadotropin-releasing Hormone Antagonist in Poor Responders Undergoing In Vitro Fertilization: A Randomized, Double-blinded, Clinical Trial
Objectives: We sought to determine the effects of the delayed start protocol with gonadotropin-releasing hormone (GnRH) antagonists in poor responders undergoing in vitro fertilization (IVF). Methods: This randomized cli...
HBV Transmission Risk Assessment in Healthcare Workers, Household and Sexual Contacts of HBV Infected Patients in the Southwest Region of Cameroon
Objectives: Hepatitis B virus (HBV) is known to be highly transmissible via the body fluids of an infected person. We investigated the transmission risks, awareness, and prevalence among healthcare workers (HCWs), househ...
Epidemiological and Clinical Features of Salmonella Typhi Infection Among Adult Patients in Qatar: A Hospital-based Study
Objectives: We sought to describe the epidemiological and clinical features of typhoid fever in Qatar. Methods: We conducted a retrospective study of adult patients treated for typhoid fever at Hamad General Hospital and...
Screening Questionnaires for Obstructive Sleep Apnea: An Updated Systematic Review
Obstructive sleep apnea (OSA) is the most common sleep-related breathing disorder and is associated with significant morbidity. We sought to present an updated systematic review of the literature on the accuracy of scree...
Interprofessional Competency Framework for Health Service Managers in Oman: An e-Delphi Study
Objectives: This study aimed to develop the required interprofessional competencies for health service managers in Oman. Methods: Experts (n = 20) were selected based on their years’ experience, position, fluency in Engl...