NEONATAL JAUNDICE – A RARE FORM OF CYSTIC FIBROSIS

Background. Cystic fibrosis is an autosomal recessive disorder that results in damage to organs containing secretory epithelial cells. It predominantly affects respiratory tract and pancreatic function. While hepatobiliary system is frequently affected in older children with cystic fibrosis, in neonatal period jaundice caused by cystic fibrosis is a rare condition. Two cases were identified in the last ten years (1988–98) in the Department of Pediatrics University Medical Centre Ljubljana.Patients and methods. We present two patients with neonatal jaundice as one of the rare features of cystic fibrosis. Laboratory evidences of cholestasis and liver involvement were present in both cases. In the first case, an improvement of a liver disease occured spontaneously and in the second case the resolution of clinical and laboratory evidence of liver disease followed after she had been treated with ursodeoxycholic acid.Conclusions. Neonatal jaundice and cholestasis can be caused by cystic fibrosis. The treatment is conservative with ursodeoxycholic acid. Only in the cases when the disease is not improving conservatively, the surgical procedure can be considered.</p