Effects of ursodeoxycholic acid therapy for liver disease associated with cystic fibrosis

Carla Colombo, Kenneth D R Setchell, Mauro Podda, Andrea Crosignani, Aldo Roda, Laura Curcio, Mariangela Ronchi, Annamaria Giunta

Research output: Contribution to journalArticle

119 Citations (Scopus)

Abstract

The hydrophilic bile acid ursodeoxycholic acid (UDCA) has recently been shown to improve indexes of liver function in adult patients with various liver diseases. The clinical and biochemical responses to UDCA administration (10 to 15 mg/kg body weight per day) were therefore investigated in nine patients with cystic fibrosis and evidence of liver disease. All patients were receiving pancreatic enzymes and taurine supplementation. Liver function tests were done and serum bile acid concentrations and biliary bile acid composition were determined before and during UDCA therapy; fat balance studies and fecal bile acid excretion were carried out before and 6 months after UDCA treatment. After 2 months of bile acid therapy, biliary bile acid composition was enriched in UDCA from approximately 5% before treatment to 25%, at the expense of cholic and chenodeoxycholic acids, thus making the pool more hydrophilic. This enrichment is lower than that reported for adults with chronic liver diseases. Serum concentrations of UDCA increased significantly but variably. UDCA became the predominant fecal bile acid excreted (12% to 67%), indicating a variable absorption of the administered bile acid. Liver function improved in all patients after 2 to 6 months of therapy, although the degree of improvement (aspartate aminotransferase, -34%; alanine aminotransferase, -41%; γ-glutamyltranspeptidase, -41%; alkaline phosphatase, -19%) was lower than that observed in adults with chronic liver diseases. Mean coefficient of fat absorption and growth rate were, on average, unaffected by UDCA therapy, although an improvement was noted for three patients with greater severity of steatorrhea. The study indicates that UDCA can be used safely in this patient population but that higher doses of UDCA may be of greater benefit in the treatment of the liver disease associated with cystic fibrosis.

Original languageEnglish
Pages (from-to)482-489
Number of pages8
JournalJournal of Pediatrics
Volume117
Issue number3
DOIs
Publication statusPublished - 1990

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Ursodeoxycholic Acid
Cystic Fibrosis
Liver Diseases
Bile Acids and Salts
Therapeutics
Cholic Acids
Chronic Disease
Fats
Steatorrhea
Liver
Liver Function Tests
Taurine
Aspartate Aminotransferases
Serum
Alanine Transaminase
Alkaline Phosphatase
Body Weight

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Effects of ursodeoxycholic acid therapy for liver disease associated with cystic fibrosis. / Colombo, Carla; Setchell, Kenneth D R; Podda, Mauro; Crosignani, Andrea; Roda, Aldo; Curcio, Laura; Ronchi, Mariangela; Giunta, Annamaria.

In: Journal of Pediatrics, Vol. 117, No. 3, 1990, p. 482-489.

Research output: Contribution to journalArticle

Colombo, Carla ; Setchell, Kenneth D R ; Podda, Mauro ; Crosignani, Andrea ; Roda, Aldo ; Curcio, Laura ; Ronchi, Mariangela ; Giunta, Annamaria. / Effects of ursodeoxycholic acid therapy for liver disease associated with cystic fibrosis. In: Journal of Pediatrics. 1990 ; Vol. 117, No. 3. pp. 482-489.
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abstract = "The hydrophilic bile acid ursodeoxycholic acid (UDCA) has recently been shown to improve indexes of liver function in adult patients with various liver diseases. The clinical and biochemical responses to UDCA administration (10 to 15 mg/kg body weight per day) were therefore investigated in nine patients with cystic fibrosis and evidence of liver disease. All patients were receiving pancreatic enzymes and taurine supplementation. Liver function tests were done and serum bile acid concentrations and biliary bile acid composition were determined before and during UDCA therapy; fat balance studies and fecal bile acid excretion were carried out before and 6 months after UDCA treatment. After 2 months of bile acid therapy, biliary bile acid composition was enriched in UDCA from approximately 5{\%} before treatment to 25{\%}, at the expense of cholic and chenodeoxycholic acids, thus making the pool more hydrophilic. This enrichment is lower than that reported for adults with chronic liver diseases. Serum concentrations of UDCA increased significantly but variably. UDCA became the predominant fecal bile acid excreted (12{\%} to 67{\%}), indicating a variable absorption of the administered bile acid. Liver function improved in all patients after 2 to 6 months of therapy, although the degree of improvement (aspartate aminotransferase, -34{\%}; alanine aminotransferase, -41{\%}; γ-glutamyltranspeptidase, -41{\%}; alkaline phosphatase, -19{\%}) was lower than that observed in adults with chronic liver diseases. Mean coefficient of fat absorption and growth rate were, on average, unaffected by UDCA therapy, although an improvement was noted for three patients with greater severity of steatorrhea. The study indicates that UDCA can be used safely in this patient population but that higher doses of UDCA may be of greater benefit in the treatment of the liver disease associated with cystic fibrosis.",
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