Cholestasis in the newborn: experience of a level III Neonatal Intensive Care Unit during 19 years

Carolina Carneiro, Susana Pissarra, Filipa Flor-de-Lima, Sandra Costa, Hercília Guimarães


Introduction: Neonatal cholestasis is a rare and always pathological condition that must be distinguished from physiologic jaundice of the newborn. It is characterized by a conjugated hyperbilirubinemia with accumulation of biliary products due to multiple causes, some of which need prompt treatment. The objectives of this study are to characterize a population of neonates with neonatal cholestasis in a level III Neonatal Intensive Care Unit (NICU) and to identify predictors of mortality.

Materials and methods: All patients presenting cholestasis and admitted to “Centro Hospitalar São João” NICU from January 1996 to December 2014 were included.

Results: A total of 83 newborns were included with a prevalence of prematurity of 78.3% and of major malformations of 30.1%. Sixty-seven newborns developed sepsis and 71 needed total parenteral nutrition for a median length of 32 days. A multifactorial etiology for cholestasis was found in 57.8%; extra- and intrahepatic diseases accounted for 19.3% and 22.9% of the cases, respectively. Maximum values for total bilirubin (TB) and direct bilirubin (DB) were significantly higher in newborns who died (TB: median 22.9 versus 13.5 mg/dl, p = 0.005; DB: median 15.0 versus 6.4 mg/dl, p = 0.009). The same was observed for minimum values of albumin and total proteins. Ursodeoxycholic acid (UDCA) was more often used in patients that survived than in those that died (50.9% versus 19.2%) and this difference was statistically significant (p = 0.007).

Conclusions: Cholestasis in our NICU has a multifactorial etiology and a prevalence of 1%. TB and total proteins can be used as predictors of mortality in newborns with cholestasis. Higher levels of DB and lower levels of albumin were also associated with worse prognosis with a statistically significant difference between the groups. UDCA is a possible agent in this context and clinicians should be reminded of its utility.


cholestasis; conjugated hyperbilirubinemia; newborns; neonatal intensive care unit; neonatal jaundice; prognosis

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