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 109
- Downloads 0
Tuberculous Mastitis Presenting as Breast Abscess
Tuberculous mastitis is a rare clinical entity and usually affects women from the Indian sub-continent and Africa. It often mimics breast carcinoma and pyogenic breast abscess clinically and radiologically, may both co-e...
Neurobrucellosis Presenting as Pseudotumor Cerebri: First Report from Oman
A ten-year-old boy presented to the hospital with body ache and joint pains for two months and headache, vomiting, and skin rash for three days. He was drowsy and lethargic at admission. Physical examination revealed bil...
Shift Work and the Risk of Cardiovascular Diseases and Metabolic Syndrome Among Jordanian Employees
Objectives: We sought to evaluate the effect of night shift working on increasing the risk of developing cardiovascular disease (CVD) using three different predictors. Methods: One hundred and forty adult Jordanian emplo...
Home Ventilation for Children in Oman, Are We Prepared for this New Reality?
Transitioning the complex care of children with different chronic disorders to their home environment has gained significant interest over the past three decades. The high cost of prolonged hospital stay, both financiall...
An Accurate Diagnostic Pathway Helps to Correctly Distinguish Between the Possible Causes of Acute Scrotum
Objectives: We sought to identify a simplified approach for the rapid differential diagnosis of patients presenting with acute scrotum. Methods: A total of 440 patients referred to the emergency department of the Univers...