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Clinical presentations of cystic fibrosis-related liver disease

Clinical presentations of cystic fibrosis-related liver disease
Category Approximate frequency in individuals with CF Typical characteristics
Neonatal cholestasis <10% Conjugated hyperbilirubinemia in a neonate, often with hepatomegaly. Usually regresses within months and is not a predictor of later CFLD, unless there is prolonged TPN exposure or surgery related to MI.
Focal biliary cirrhosis 20 to 40% May or may not be associated with persistently elevated aminotransferases and hepatomegaly, usually asymptomatic and developing within the first 12 years of life.
Multilobular cirrhosis 5 to 10% Advanced stage of focal biliary cirrhosis. May be complicated by portal hypertension (causing splenomegaly, ascites, and esophageal varices), gastrointestinal bleeding, and nutritional deficiencies. Hepatic synthetic dysfunction (coagulopathy and hypoalbuminemia) is a late and rare occurrence.
Noncirrhotic portal hypertension Unknown, but this entity is well described Portal hypertension with esophageal varices but often more than expected for the observed degree of fibrosis. If biopsy is performed, it typically shows nodular regenerative hyperplasia with missing portal veins or obliterative portal venopathy.
Hepatic steatosis 10 to 60%

Incidental finding on ultrasound or liver biopsy, with transient hepatomegaly. The relationship between hepatic steatosis and the development of focal biliary cirrhosis in CF is unclear. May be seen from infancy to adolescence, and may be associated with stunting or wasting.

Steatosis may be caused by iatrogenic or environmental factors such as malnutrition, medications, essential fatty acid deficiency, and ethanol ingestion.
CF: cystic fibrosis; CFLD: cystic fibrosis-related liver disease; TPN: total parenteral nutrition; MI: meconium ileus.
References:
  1. ​Colombo C, Battezzati PM, Crosignani A, et al. Liver disease in cystic fibrosis: A prospective study on incidence, risk factors, and outcome. Hepatology 2002; 36:1374.
  2. Lamireau T, Monnereau S, Martin S, et al. Epidemiology of liver disease in cystic fibrosis: a longitudinal study. J Hepatol 2004; 41:920.
  3. Debray D, Kelly D, Houwen R, et al. Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease. J Cyst Fibros 2011; 10 Suppl 2:S29.
  4. Wu H, Vu M, Dhingra S, et al. Obliterative Portal Venopathy Without Cirrhosis Is Prevalent in Pediatric Cystic Fibrosis Liver Disease With Portal Hypertension. Clin Gastroenterol Hepatol 2019; 17:2134.
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