Cystic fibrosis-associated liver disease is the third leading cause of morbidity and mortality in patients with cystic fibrosis (CF). Liver damage in the course of CF ranges from biochemical abnormalities to full-blown cirrhosis and may involve complicated processes like inflammation, fibrogenesis, remodeling, apoptosis, and cholestasis. Despite robust research in the field of CF, its complex pathogenesis is not fully understood. Because of the unknown pathogenesis, it is difficult to develop a highly sensitive and specific test or technology that is standardized, acceptable, and available at most pediatric institutions. The Cystic Fibrosis Foundation (CFF) recommends annual blood tests to screen for liver pathology, which often fails to identify early-onset liver disease. In this review article, we present the use of different liver indices and imaging modalities that can help identify liver disease at the onset and may help in its prevention. Although the disease is commonly diagnosed in the pediatric population, due to increased life expectancy, there is increasing evidence of liver disease in adults too. We believe that the tools we present in this review will help in the prevention of liver disease and thereby reduce the associated morbidity and mortality.
Keywords: cystic fibrosis; cystic fibrosis complication; liver and gall bladder disease; pediatrics; prevention.
Copyright © 2022, Shukla et al.