Vitamin A concentration in the liver decreases with age in patients with cystic fibrosis

J Pediatr Gastroenterol Nutr. 1997 Mar;24(3):264-70. doi: 10.1097/00005176-199703000-00006.

Abstract

Background: Vitamin A deficiency is a common manifestation in cystic fibrosis (CF), but high levels of vitamin A in the liver have also been described. High levels of vitamin A in the liver are toxic, while normal levels might be protective against liver damage. In order to investigate whether liver damage in patients with CF is related to vitamin A content of the liver, vitamin A status was investigated in 15 patients with CF aged 8 to 34 years.

Methods: Liver biopsy was performed on clinical indication and the vitamin A concentration in the liver was determined as retinylpalmitate. Serum levels of retinol and retinol-binding protein were investigated on the morning of the biopsy. Eight patients had morphologic signs of cirrhosis. Eight patients had been on treatment with ursodeoxycholic acid for 1 to 3 years. All but three patients had been on vitamin A supplementation for years.

Results: Five patients had serum concentrations of retinol below the reference range and seven patients had decreased serum levels of retinol-binding protein. There was a strong correlation between serum levels of retinol and retinol-binding protein (rs = 0.90, p = 0.01), but no correlations with age, Shwachamn score, or genotype. Six of the patients had vitamin A concentrations in the liver < 40 micrograms/g wet weight, and the concentrations decreased significantly with age (rs = 0.77, p = 0.01), without correlation to clinical score or liver disease. There was no indication of hypervitaminosis, although younger patients had been or were being treated with vitamin A in fat-water emulsion.

Conclusions: Our results indicate that the risk of vitamin a deficiency in cystic fibrosis increases with age. The data do not support the view that patients are at risk for hypervitaminosis by long-term supplementation with vitamin A. No correlation was found between the severity of liver disease and the vitamin A content in the liver.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aging*
  • Biopsy
  • Child
  • Cystic Fibrosis / metabolism*
  • Diterpenes
  • Female
  • Humans
  • Liver / metabolism*
  • Male
  • Reference Values
  • Retinol-Binding Proteins / metabolism
  • Retinyl Esters
  • Vitamin A / analogs & derivatives
  • Vitamin A / blood
  • Vitamin A / metabolism*

Substances

  • Diterpenes
  • Retinol-Binding Proteins
  • Retinyl Esters
  • Vitamin A
  • retinol palmitate