Liver involvement in cystic fibrosis

Catherine Brigman, Andrew Feranchak

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

The hepatobiliary manifestations of cystic fibrosis (CF) encompass a broad clinical spectrum, from mild steatosis, associated with poor nutrition, to multilobular cirrhosis and the complications of portal hypertension. The factor(s) responsible for the development and progression of liver disease in a subset of patients with CF are unknown. Liver disease can be silent and progressive, manifesting only with complications associated with cirrhosis and portal hypertension. Clinical evaluation for detecting and monitoring the progression of liver disease includes the following: physical examination of the liver, biochemical tests of Liver function and injury, and radiological imaging with abdominal ultrasonography. Careful monitoring should take place in all patients with CF, as currently, there are no sensitive and/or specific historical or biochemical markers to predict who is at risk for the development of Liver disease. Current treatment options for CF-associated liver disease are very Limited. The bile acid ursodeoxycholic acid may improve biochemical parameters of liver disease, but its tong-term efficacy in preventing the progression of liver disease in CF is unproven. Treatment therefore rests on optimizing nutritional status; correcting fat-soluble vitamin, essential fatty acid, and other mineral deficiencies; and treating complications of end-stage liver disease, such as pruritis, ascites, and portal hypertension.

Original languageEnglish (US)
Pages (from-to)484-496
Number of pages13
JournalCurrent Treatment Options in Gastroenterology
Volume9
Issue number6
DOIs
StatePublished - Dec 2006

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Cystic Fibrosis
Liver Diseases
Liver
Portal Hypertension
Fibrosis
Ursodeoxycholic Acid
Essential Fatty Acids
End Stage Liver Disease
Liver Function Tests
Pruritus
Nutritional Status
Bile Acids and Salts
Ascites
Vitamins
Physical Examination
Minerals
Ultrasonography
Biomarkers
Fats
Wounds and Injuries

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Liver involvement in cystic fibrosis. / Brigman, Catherine; Feranchak, Andrew.

In: Current Treatment Options in Gastroenterology, Vol. 9, No. 6, 12.2006, p. 484-496.

Research output: Contribution to journalArticle

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