Outcome in Cystic Fibrosis Liver Disease

Liver disease in adult patients with cystic fibrosis: A frequent and independent prognostic factor associated with death or lung transplantation

“…In this study, BMI at baseline was not associated with the presence of liver disease. Other nutritional parameters such as mid upper arm circumference, skinfold thickness have been described as a factor of liver disease [21,22]. Those parameters were not evaluated in this study as they are not currently measured in our center.…”

Cystic fibrosis: An update for clinicians. Part 2: Hepatobiliary and pancreatic manifestations

“…This has been suspected since earliest autopsy descriptions of children dying with CF with 10–40% demonstrating significant hepatic fibrosis, increasing with age, ranging from “focal biliary fibrosis” to established multilobular cirrhosis. Recent prospective studies by Rowland and Lewindon indicate coexisting significant disease, in the form of portal hypertension or fibrosis–cirrhosis, to be an additional risk factor for early mortality. Past and current mortality statistics likely underestimate the true burden of cystic fibrosis liver disease (CFLD) as it is often silent until an advanced stage and most diagnostic modalities lack sensitivity, specificity, or are too invasive.…”

“…Steatosis has been found in up to 70% of liver biopsies from children with suspected CFLD and represents the commonest identified histological abnormality . This has been thought to be secondary to selective nutritional deficiencies and altered phospholipid metabolism . The significance of steatosis in CF biopsies is unknown; however, it is known that nonalcoholic steatohepatitis can lead to cirrhosis .…”

Cholestasis and meconium ileus in infants with cystic fibrosis and their clinical outcomes

“…The latter studies are important contributions as MBC and PH can be recognised clinically and confirmed by non-invasive imaging. Moreover, literature in the last two decades has indicated that MBC is a phenomenon of childhood with de novo occurrence in adult CF patients being very uncommon,17 19 21 22 and thus, longitudinal studies should be able to determine the true outcome of those originally with neonatal cholestasis within a 20-year span from birth.…”