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

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

“…In those with MI 7.2% developed CFLD with PH, a proportion virtually identical to the 6.3% of CF infants without MI, indicating that MI is unlikely to have had a contributing role to the later development of CFLD. This is in contrast with earlier reports suggesting infants with MI have an increased risk of developing CFLD 18 21 29–32. However, broad definitions and criteria for the diagnosis of CFLD have been used in previous studies, which could explain these different results.…”

Cirrhosis and other liver disease in cystic fibrosis

“…William's score of ultrasound abnormalities ≥4 [11] Both definitions include many disparate types of liver disease variable impact on outcome. Recent reports on the prevalence of CFLD using the broader criteria listed above, found a 30–40% prevalence of liver involvement in children and adults with CF [9,10,12]. However, longitudinal studies found that only 20–30% of individuals with CF develop focal biliary cirrhosis and at most 5–10% will develop multilobular cirrhosis [4,5,10].…”

Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology

“…CF-related liver disease (CFLD) was recently shown to be an independent risk factor for mortality and lung transplantation, and has become one of the leading causes of death among patients with CF [6]. …”