Introduction The diagnosis of neonatal cholestasis (NC) is urgent in order to define a specific diagnosis, as well as, for the immediate treatment with vitamins’ supplements.

Objectives Understand the modification of NC aetiology in a tertiary centre, throughout 17 years.

Determine age at diagnosis, aetiology, hepatic function, need of imaging studies and hepatic biopsy.

Methods Retrospective study of newborns and infants with NC diagnosed in a central hospital.

Results During 17 years, were diagnosed 100 cases of NC. Median age at diagnosis was 9,5 days. The most frequent causes of NC were premature newborns under prolonged parenteral nutrition (34%), neonatal sepsis (29%), alpha-1 antitrypsin deficiency (5%) and cytomegalovirus congenital infection (5%).

From those that due to jaundice, realised abdominal ultrasound to exclude biliary atresia (n = 59), 3 had biliary atresia and 9 had other alterations. Hepatic biopsy has been performed in 7 cases (biliary atresia n = 3; paucity of biliary ducts n = 1; Byler n = 1; Morsier’s syndrome n = 1, idiopathic n = 1).

Thirteen patients died, 5 developed chronic hepatitis and 2 were transplanted.

Death occurred in those with neonatal sepsis (n = 4), premature newborns with prolonged parenteral nutrition (n = 4), Zellweger syndrome (n = 2) and liver failure (n = 3).

Conclusions The majority of NC occurred in newborns and infants hospitalised in intensive neonatal unit care, due to neonatal sepsis/prolonged parenteral nutrition, in opposed to the classic aetiology of biliary atresia/ alpha-1 antitrypsin deficiency.

The celerity of NC diagnosis continues to be crucial, in order to avoid delay in a biliary atresia diagnosis.