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 104
- Downloads 0
Characteristics and Correlates of Psychiatric Problems in Wives of Men with Substance-related Disorders, Kermanshah, Iran
Objectives: We sought to evaluate the characteristics and correlates of psychiatric problems in the wives of men with substance-related disorders. Methods: Four-hundred and fifty women whose husbands had substance-relate...
Prevalence and Determinants of Depression and Anxiety Symptoms in Surgical Patients
Objectives: Mood disorders are prevalent in hospitalized patients. However, risk factors for early diagnosis have not been studied exclusively in surgical patients. Our study aimed to investigate the prevalence and deter...
Characteristics and Outcomes of Patients with Cerebral Venous Sinus Thrombosis
Objectives: Cerebral venous sinus thrombosis (CVST) is a disease with potentially serious consequences. The clinical presentation and outcomes of these patients have not been described in Oman. We sought to describe the...
Impact of Blood Transfusion on Troponin I Levels and Outcomes after Cardiac Surgery: A Cohort Study
Objectives: Increased cardiac troponin I (TI) has been suggested to be a sensitive indicator of intraoperative myocardial injury. We investigated the association of transfusion on TI levels post-surgery and outcomes in p...
Epidemiology of Lung Cancer in Oman: 20-Year Trends and Tumor Characteristics
Objectives: We sought to investigate the epidemiology of lung cancer and its trends in Oman over 20 years. Methods: We analyzed all cases of primary lung cancer reported to the Oman National Cancer Registry between 1996...