Cystic fibrosis: Hepatobiliary disease
- Daniel H Leung, MD
Daniel H Leung, MD
- Assistant Professor of Pediatrics
- Baylor College of Medicine
- Drucy Borowitz, MD
Drucy Borowitz, MD
- Department of Pediatrics
- State University of New York at Buffalo
- Section Editors
- George B Mallory, MD
George B Mallory, MD
- Section Editor — Pediatric Pulmonology
- Associate Professor of Pediatrics
- Baylor College of Medicine
- Elizabeth B Rand, MD
Elizabeth B Rand, MD
- Section Editor — Pediatric Hepatology
- Professor of Pediatrics
- University of Pennsylvania School of Medicine
Cystic fibrosis-related liver disease (CFLD) is becoming increasingly recognized due to early diagnosis of cystic fibrosis (CF), improved life expectancy, and more vigilant screening and monitoring by CF providers. Patients with severe CFLD usually present during childhood, and the disease tends to progress rapidly. Milder forms of CFLD can be difficult to diagnose because patients may remain asymptomatic until late in the disease process, and currently available screening tests correlate poorly with disease severity. Early identification of CFLD permits anticipation and treatment of its complications, which include malnutrition, variceal bleeding due to portal hypertension, and occasionally liver failure. Other hepatobiliary complications of CF include cholelithiasis (gallstones), cholecystitis, and microgallbladder.
The clinical manifestations, diagnosis, and management of CFLD will be discussed in this topic review, and associated disorders of the gallbladder will be discussed briefly. Other topic reviews relevant to the management of a patient with CF and hepatobiliary disease include:
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- EPIDEMIOLOGY AND NATURAL HISTORY
- CLINICAL MANIFESTATIONS
- Progressive CFLD
- Other manifestations of liver disease
- - Neonatal cholestasis
- - Hepatic steatosis
- Further evaluation
- Risk reduction
- Ursodeoxycholic acid
- Management of specific complications
- - Portal hypertension
- - Hepatopulmonary syndrome
- - Portopulmonary hypertension
- - Liver failure
- Liver transplantation
- GALLBLADDER DISEASE
- SUMMARY AND RECOMMENDATIONS