Liver disease in children with cystic fibrosis: observational study

Introduction: Cystic fibrosis (CF) is a genetic disease of autosomal recessive inheritance, characterized by dysfunction of the exocrine secretion glands. The liver is an affected organ, which causes an increase in early morbidity and mortality.Objective: To evaluate liver disease in a group of children with CF.Material and methods: A total of 82 children with CF confirmed with genetic testing were included.Biochemical liver function tests and liver ultrasound were evaluated.The presence of fibrosis was estimated using the aspartato aminotransferasa to platelet ratio index (APRI) and correlation tests were performed.Results: 59.8% (n = 49) of patients had elevated alanine aminotransferase.30.5% (n = 25) showed an APRI suggestive of fibrosis.The correlation of APRI with alanino aminotransferase was 0.685 (p < 0.001) and with GGT 0.385 (p < 0.001).The prevalence of alterations in hepatic echogenicity was lower than biochemical alterations in transaminases.Conclusions: There is a high prevalence of liver disease at the diagnosis of CF and even a third of children could present with liver fibrosis.In this study, we found no difference in liver function tests according to liver ultrasound.