Liver disease in cystic fibrosis

C. Colombo, M. G. Apostolo, M. Assaisso, B. Roman, P. Bottani

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Liver disease associated with cystic fibrosis (CF) is considered a secondary effect of the basic defect of the disease, leading to obstruction of bile ductules by abnormal mucoid secretions; additional factors have been involved in the pathogenesis, such as abnormalities in bile acid metabolism, nutritional deficiencies, drug hepatotoxicity, stenosis of the common bile duct by the fibrotic pancreas. Clinical presentation of liver disease in CF is rare during the first few years of life, although neonatal cholestasis can be occasionally the first manifestation of the disease. Isolated massive steatosis has been reported in less than 5% of cases as a consequence of malnutrition. Focal biliary cirrhosis is the pathognomonic hepatic lesion and is present in 25-30% of CF patients, most of whom are asymptomatic. The focally distributed lesions can extend leading to multi-lobular biliary cirrhosis with occurrence of signs and symptoms of cirrhosis and portal hypertension. Early diagnosis of CF-associated liver disease is difficult since liver function tests may be normal even in cases of overt cirrhosis: no test has proved to be sufficiently sensitive and specific and even liver biopsy is of questionable relevance due to the focal distribution of hepatic lesions. Clinical examination is of major importance, since the presence of hepatomegaly seems to correlate well with the histologic finding of fibrosis. The rationale for the use of the choleretic non-toxic bile acid ursodeoxycholic acid in CF-associated liver disease is to reduce the viscosity of bile and to replace toxic bile acids which accumulate in the hepatocyte. Pilot studies have reported beneficial effects of short-term treatment on liver function tests, quantitative liver function, scintigraphic parameters related to biliary drainage and nutritional status. The effects of treatment on survival and on clinically relevant events are currently under investigation through long-term controlled trials.

Original languageEnglish
Pages (from-to)119-122
Number of pages4
JournalNetherlands Journal of Medicine
Volume41
Issue number3
Publication statusPublished - 1992

Fingerprint

Cystic Fibrosis
Liver Diseases
Bile Acids and Salts
Fibrosis
Biliary Liver Cirrhosis
Liver
Liver Function Tests
Bile
Malnutrition
Cholagogues and Choleretics
Ursodeoxycholic Acid
Hepatomegaly
Poisons
Cholestasis
Common Bile Duct
Portal Hypertension
Nutritional Status
Viscosity
Signs and Symptoms
Early Diagnosis

Keywords

  • cystic fibrosis
  • liver disease
  • ursodeoxycholic acid

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Colombo, C., Apostolo, M. G., Assaisso, M., Roman, B., & Bottani, P. (1992). Liver disease in cystic fibrosis. Netherlands Journal of Medicine, 41(3), 119-122.

Liver disease in cystic fibrosis. / Colombo, C.; Apostolo, M. G.; Assaisso, M.; Roman, B.; Bottani, P.

In: Netherlands Journal of Medicine, Vol. 41, No. 3, 1992, p. 119-122.

Research output: Contribution to journalArticle

Colombo, C, Apostolo, MG, Assaisso, M, Roman, B & Bottani, P 1992, 'Liver disease in cystic fibrosis', Netherlands Journal of Medicine, vol. 41, no. 3, pp. 119-122.
Colombo C, Apostolo MG, Assaisso M, Roman B, Bottani P. Liver disease in cystic fibrosis. Netherlands Journal of Medicine. 1992;41(3):119-122.
Colombo, C. ; Apostolo, M. G. ; Assaisso, M. ; Roman, B. ; Bottani, P. / Liver disease in cystic fibrosis. In: Netherlands Journal of Medicine. 1992 ; Vol. 41, No. 3. pp. 119-122.
@article{1849909d01e14a4ba85c5bb6a0cbdf52,
title = "Liver disease in cystic fibrosis",
abstract = "Liver disease associated with cystic fibrosis (CF) is considered a secondary effect of the basic defect of the disease, leading to obstruction of bile ductules by abnormal mucoid secretions; additional factors have been involved in the pathogenesis, such as abnormalities in bile acid metabolism, nutritional deficiencies, drug hepatotoxicity, stenosis of the common bile duct by the fibrotic pancreas. Clinical presentation of liver disease in CF is rare during the first few years of life, although neonatal cholestasis can be occasionally the first manifestation of the disease. Isolated massive steatosis has been reported in less than 5{\%} of cases as a consequence of malnutrition. Focal biliary cirrhosis is the pathognomonic hepatic lesion and is present in 25-30{\%} of CF patients, most of whom are asymptomatic. The focally distributed lesions can extend leading to multi-lobular biliary cirrhosis with occurrence of signs and symptoms of cirrhosis and portal hypertension. Early diagnosis of CF-associated liver disease is difficult since liver function tests may be normal even in cases of overt cirrhosis: no test has proved to be sufficiently sensitive and specific and even liver biopsy is of questionable relevance due to the focal distribution of hepatic lesions. Clinical examination is of major importance, since the presence of hepatomegaly seems to correlate well with the histologic finding of fibrosis. The rationale for the use of the choleretic non-toxic bile acid ursodeoxycholic acid in CF-associated liver disease is to reduce the viscosity of bile and to replace toxic bile acids which accumulate in the hepatocyte. Pilot studies have reported beneficial effects of short-term treatment on liver function tests, quantitative liver function, scintigraphic parameters related to biliary drainage and nutritional status. The effects of treatment on survival and on clinically relevant events are currently under investigation through long-term controlled trials.",
keywords = "cystic fibrosis, liver disease, ursodeoxycholic acid",
author = "C. Colombo and Apostolo, {M. G.} and M. Assaisso and B. Roman and P. Bottani",
year = "1992",
language = "English",
volume = "41",
pages = "119--122",
journal = "Netherlands Journal of Medicine",
issn = "0300-2977",
publisher = "Van Zuiden Communications BV",
number = "3",

}

TY - JOUR

T1 - Liver disease in cystic fibrosis

AU - Colombo, C.

AU - Apostolo, M. G.

AU - Assaisso, M.

AU - Roman, B.

AU - Bottani, P.

PY - 1992

Y1 - 1992

N2 - Liver disease associated with cystic fibrosis (CF) is considered a secondary effect of the basic defect of the disease, leading to obstruction of bile ductules by abnormal mucoid secretions; additional factors have been involved in the pathogenesis, such as abnormalities in bile acid metabolism, nutritional deficiencies, drug hepatotoxicity, stenosis of the common bile duct by the fibrotic pancreas. Clinical presentation of liver disease in CF is rare during the first few years of life, although neonatal cholestasis can be occasionally the first manifestation of the disease. Isolated massive steatosis has been reported in less than 5% of cases as a consequence of malnutrition. Focal biliary cirrhosis is the pathognomonic hepatic lesion and is present in 25-30% of CF patients, most of whom are asymptomatic. The focally distributed lesions can extend leading to multi-lobular biliary cirrhosis with occurrence of signs and symptoms of cirrhosis and portal hypertension. Early diagnosis of CF-associated liver disease is difficult since liver function tests may be normal even in cases of overt cirrhosis: no test has proved to be sufficiently sensitive and specific and even liver biopsy is of questionable relevance due to the focal distribution of hepatic lesions. Clinical examination is of major importance, since the presence of hepatomegaly seems to correlate well with the histologic finding of fibrosis. The rationale for the use of the choleretic non-toxic bile acid ursodeoxycholic acid in CF-associated liver disease is to reduce the viscosity of bile and to replace toxic bile acids which accumulate in the hepatocyte. Pilot studies have reported beneficial effects of short-term treatment on liver function tests, quantitative liver function, scintigraphic parameters related to biliary drainage and nutritional status. The effects of treatment on survival and on clinically relevant events are currently under investigation through long-term controlled trials.

AB - Liver disease associated with cystic fibrosis (CF) is considered a secondary effect of the basic defect of the disease, leading to obstruction of bile ductules by abnormal mucoid secretions; additional factors have been involved in the pathogenesis, such as abnormalities in bile acid metabolism, nutritional deficiencies, drug hepatotoxicity, stenosis of the common bile duct by the fibrotic pancreas. Clinical presentation of liver disease in CF is rare during the first few years of life, although neonatal cholestasis can be occasionally the first manifestation of the disease. Isolated massive steatosis has been reported in less than 5% of cases as a consequence of malnutrition. Focal biliary cirrhosis is the pathognomonic hepatic lesion and is present in 25-30% of CF patients, most of whom are asymptomatic. The focally distributed lesions can extend leading to multi-lobular biliary cirrhosis with occurrence of signs and symptoms of cirrhosis and portal hypertension. Early diagnosis of CF-associated liver disease is difficult since liver function tests may be normal even in cases of overt cirrhosis: no test has proved to be sufficiently sensitive and specific and even liver biopsy is of questionable relevance due to the focal distribution of hepatic lesions. Clinical examination is of major importance, since the presence of hepatomegaly seems to correlate well with the histologic finding of fibrosis. The rationale for the use of the choleretic non-toxic bile acid ursodeoxycholic acid in CF-associated liver disease is to reduce the viscosity of bile and to replace toxic bile acids which accumulate in the hepatocyte. Pilot studies have reported beneficial effects of short-term treatment on liver function tests, quantitative liver function, scintigraphic parameters related to biliary drainage and nutritional status. The effects of treatment on survival and on clinically relevant events are currently under investigation through long-term controlled trials.

KW - cystic fibrosis

KW - liver disease

KW - ursodeoxycholic acid

UR - http://www.scopus.com/inward/record.url?scp=0026655893&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0026655893&partnerID=8YFLogxK

M3 - Article

VL - 41

SP - 119

EP - 122

JO - Netherlands Journal of Medicine

JF - Netherlands Journal of Medicine

SN - 0300-2977

IS - 3

ER -