Diagnostic screening of liver disease in cystic fibrosis

• Published in: Jornal de pediatria 2002-09, Vol.78 (5), p.389-96
• Main Authors: Fagundes, Eleonora D T, Roquete, Mariza L V, Penna, Francisco J, Reis, Francisco J C, Duque, Cristiano G
• Format: Article
• Language: eng ; por
• ISSN: 0021-7557
• DOI: 10.2223/JPED.886

To determine the prevalence of clinical and biochemical abnormalities suggestive of liver disease in the population of patients with cystic fibrosis (CF) seen at the CF Outpatient Clinic of the Federal University of Minas Gerais; to describe the group of patients with liver disease and to compare it with the group without liver disease, regarding several clinical and laboratory variables. Descriptive study, resultant of a prospective, and partially retrospective assessment of 106 patients with CF. Liver disease was defined by the presence of firm hepatomegaly (>2.5 cm from the right costal margin), and/or splenomegaly, and/or persistent and significant increase (>1.5 times the upper limit of normality, over six months) of at least two serum liver enzymes (alanine aminotransferase--ALT, aspartate aminotransferase--AST, alkaline phosphatase--ALP, gamma-glutamyl transpeptidase--GGT). Hepatomegaly was verified in seven patients (6.6%) and splenomegaly in five (4.7%). AST activity was altered in 18.9% of the patients, ALT in 9.4%, GGT in 11.3%, and ALP in 46.2%. Significant and persistent increase in liver enzyme activities was verified in nine patients (8.5%). Ten patients with CF (9.4%) fulfill the criteria for liver disease. Other causes of liver disease were excluded. The mean age of the patients with liver disease was 7.7 years. There was absolute predominance of the male sex. All the patients are without symptoms. The prevalence of liver disease associated with CF was 9.4%. The frequent and transitory insignificant elevations of liver enzymes are well documented in the literature. Its significance as a predictor of liver disease has not been determined yet. However, the results of this study enhance the need for longitudinal assessment in order to define cases of liver disease in CF.