Total parenteral nutrition-associated intrahepatic cholestasis in infants: 25 years' experience

BACKGROUND/PURPOSE: There are few long-term chronological reviews examining the incidence of total parenteral nutrition (TPN)-associated intrahepatic cholestasis (TPNAC) in infants. The authors therefore reviewed TPNAC in their 25-year series, and also looked at the current problems associated with TPN in infants. METHODS: Two hundred seventy-three surgical neonates who received TPN for more than 2 weeks were divided into 3 groups chronologically: group A (1971 through 1982, n = 77), group B (1983 through 1987, n = 72), and group C (1992 through 1996, n = 124). TPNAC was defined as serum direct bilirubin (DB) level greater than 2.0 mg/dL during the neonatal period. RESULTS: The incidence of TPNAC in groups A, B and C was 57%, 31%, and 25% (P< .01), respectively, and the mortality rate from TPN-associated complications was 13%, 3%, and 3% (P< .05), respectively. Over the last 5 years, severe TPNAC developed in 20 patients (16%). Four of 20 died of TPN-associated sepsis with hepatic failure; 2 had hypoganglionosis with intractable stagnant enteritis and subsequent sepsis, and 2 had fatal respiratory or cardiac disease. CONCLUSIONS: The incidence of TPNAC in surgical neonates and TPN-associated mortality rates have decreased significantly. The mortality rate, however, still remains at 3%. Two of 4 fatal cases had hypoganglionosis, which were totally dependent on TPN. In patients who require long-term TPN, TPN still has unsolved problems, and small bowel transplantation may be indicated.