Background:

although the chronic hepatic illness as complication of the cystic fibrosis has been eclipsed by other signs and more obvious symptoms at breathing and pancreatic level, is the second cause of death in the patients with cystic fibrosis.

Objective:

to present the case of a three year-old transitional one with hepatomegaly and hepatic analytic tests altered as initial manifestations of the cystic fibrosis.

Clinical case:

a three-year old, female patient with history of abdominal distension, hepatomegaly, and malnutrition. For this symptoms, she is remitted to the Gastroenterology consultation.

Conclusions:

the cystic fibrosis should be suspected in pediatric patient with chronic hepatic diseases. Clinical improvement of the breathing manifestations was observed and of the nutritional state, however the chronic hepatic disease evolved to the established hepatic cirrhosis.

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