Hepatobiliary involvement in children with cystic fibrosis Kistik fibrozisli çocuklarda hepatobiliyer etkilenme

Çakar S., Kuyum P., Aksoy B., ÖZTÜRK Y.

Cocuk Sagligi ve Hastaliklari Dergisi, vol.58, no.2, pp.51-56, 2015 (Scopus) identifier

  • Publication Type: Article / Article
  • Volume: 58 Issue: 2
  • Publication Date: 2015
  • Journal Name: Cocuk Sagligi ve Hastaliklari Dergisi
  • Journal Indexes: Scopus
  • Page Numbers: pp.51-56
  • Keywords: Child, Cystic fibrosis, Hepatobiliary involvement
  • Dokuz Eylül University Affiliated: Yes


The rate of hepatobiliary involvement in patients with cystic fibrosis (CF) is given at varying percentages. The reason for this may be the lack of clear diagnostic criteria for hepatobiliary involvement in CF. The aim of this study was to investigate the frequency and characteristics of hepatobiliary involvement in CF patients. Records of CF cases were examined retrospectively; age at diagnosis, gender, nutritional status, liver function test results, ultrasound findings, drug therapy and follow-up were noted. Fifty-five patients diagnosed with CF were included in the study; their ages ranged from two months to 16 years, and 54.5% were female. The follow-up period was between six and 144 months (mean, 65 months). We detected disturbances in liver function tests during follow-up in 15 cases (27.3%). In none of these fifteen patients was hypoalbuminemia, hyperbilirubinemia or prolongation of prothrombin time observed. Among the fifteen cases, one had hepatic steatosis, one had an increase in the echogenicity of the liver parenchyma and granular pattern, and two had hepatomegaly. Hepatomegaly was detected in one patient without elevated transaminase levels, and cholelithiasis in another. In 80% of the patients with elevated transaminase levels, the records showed antibiotic treatment in the previous two weeks. At the time of diagnosis, 2 of the 17 patients with hepatobiliary disorders were over 10 years of age (11 and 12); all of the others were under 10. Age at diagnosis, gender and malnutrition were not associated with the presence of hepatobiliary involvement. In the CF patients in our study, the rate of hepatobiliary involvement was found to be 30.9%. Most patients (88.2%) were <10 years old; the most frequent finding was transaminase elevation (88.2%). In over a third of patients, pathological sonographic findings were observed (35.3%). Most CF cases with elevated transaminases had a history of drug use and infection. Although a high frequency of hepatobiliary involvement was observed in children with cystic fibrosis, it is difficult to say whether this was due to primary or secondary effects of the disease.