High rates of mortality and morbidity occur in infants with parenteral nutrition-associated cholestasis.

Background: Extremely few data are available about the natural history of parenteral nutrition (PN)-associated cholestasis. The authors evaluated a cohort of infants at a large center to determine the outcome of PN-associated cholestasis in infants with some gastrointestinal function.

Methods: The authors reviewed the records of all infants admitted to a level 3 neonatal intensive care unit over a 16-month period who had the diagnosis of PN-associated cholestasis. Records were reviewed in these infants for course of cholestasis, laboratory values, outcome, and infection rate.

Results: Sixty-six patients were admitted who met the study criteria. There were 10 deaths and 1 referral for liver transplant (Death/TPlant) (17%) in the first year of life. All Death/TPlant infants had at least 1 positive blood culture after the onset of cholestasis. Maximum conjugated bilirubin (MaxCB) in Death/TPlant infants was 15.7 +/- 2.2 (SEM) compared to 8.4 +/- 1.0 mg/dL in babies who recovered. Of 21 infants with a MaxCB > or =10.0, Death/TPlant occurred in 8/21 (38%). Of 40 babies with positive blood cultures, 11 were in the Death/TPlant group vs no deaths among the 25 without positive blood cultures. Average time to resolution from the MaxCB to a CB <2.0 mg/dL was 66 +/- 7 days (n = 49).

Conclusions: Infants with PN-associated cholestasis have high rates of mortality despite the presence of some gastrointestinal function. These data support further evaluation and the development of novel forms of therapy for babies with parenteral-associated CB > or =2 mg/dL with emphasis on interventions for infants with a CB >10 mg/dL.