Liver function and morphology during long-term fatty acid supplementation in cystic fibrosis.

Liver function tests and liver morphology were studied in 18 patients with cystic fibrosis (CF) for a period of 3 years. The patients were 5-19 years of age at the start of the study. Nine patients received regular supplementation of essential fatty acids (Intralipid) and nine sex- and age-matched patients, with as similar clinical status as possible but without fat emulsion treatment, were followed as controls. There were no significant differences between the two groups with regard to the parameters of liver function, including determinations of bile acids in serum and urine. Histological examinations of liver biopsies suggested less progression of liver disease in the fatty acid supplemented group, including fatty infiltration of the hepatocytes, evaluated morphometrically. The results show that regular supplementation of fat emulsions has no negative effects in the liver of patients with CF, and the lessening of progression of liver damage suggests that essential fatty acid deficiency might contribute to the liver damage in this disease.