Location: Children's Nutrition Research CenterTitle: High rates of mortality and morbidity occur in infants with perenteral nutrition - associated cholestasis) Author
Submitted to: Journal of Parenteral and Enteral Nutrition
Publication Type: Peer reviewed journal
Publication Acceptance Date: 9/24/2008
Publication Date: 1/5/2010
Citation: Willis, T.C., Carter, B.A., Rogers, S.P., Hawthorne, K.M., Hicks, P.D., Abrams, S.A. 2010. High rates of mortality and morbidity occur in infants with perenteral nutrition - associated cholestasis. Journal of Parenteral and Enteral Nutrition. 34(1):32-37. Interpretive Summary: There is very little data available about the natural history of intravenous nutrition-associated liver disease, called cholestasis. We therefore, evaluated a group of infants at our hospital to determine the outcome of cholestasis in infants. We reviewed the records of all infants admitted to our neonatal intensive care unit over a 16-month period, who were diagnosed with cholestasis due to intravenous nutrition. We found 66 patients were admitted who met the study criteria. There were 10 deaths and one referral for liver transplant (17%) in the first year of life among these patients. All of these babies had at least one blood infection, and those who died had much more severe liver failure. If the level of liver failure was such that the test for it, called the conjugated bilirubin was greater than or equal to 10.0, then 38% of the infants died, or needed a liver transplant. It took an average of 66 days for those who got better to recover from their liver disease. We concluded that infants with cholestasis from intravenous nutrition have high rates of death. Our results support further evaluation and the development of novel forms of therapy for babies with this condition.
Technical Abstract: There is very little data 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. 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. 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 greater than or equal to 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 less than 2.0 mg/dL was 66 +/- 7 days (n = 49). 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 greater than or equal to 2 mg/dL with emphasis on interventions for infants with a CB greater than 10 mg/dL.