Background: Cystic fibrosis is a hereditary disease of mucus and
sweat glands characterized by respiratory infections and pancreatic
insufficiency. Case presentation: We describe a girl infant with
cholestasis as of the first clinical presentation at the age of 1
month. She developed severe anemia which required transfusion for
several times. High level of direct bilirubin, low level of albumin and
positive sweat tests were detected in laboratory tests. Histopathology
report of liver biopsy indicated giant cell neonatal hepatitis.
Conclusion: Although neonatal hepatitis is common cause of neonatal
cholestasis, such condition is quite rare in cystic fibrosis, which
easily could be misdiagnosed. Early diagnosis and appropriate treatment
could prevent further complications of the disease