INTRODUCTION: Cholestasis associated with long-term total parenteral nutrition (TPN) occurs commonly in very low birth weight (VLBW) infants. Indeed, the majority of infants with TPN-associated cholestasis (TPNAC) respond very well to TPN withdrawal and full enteral feeding, yet some of them do not respond and have the potential for development of intractable cholestasis. It has been demonstrated that ursodeoxycholic acid (UDCA) has beneficial effects in treating TPNAC in various age groups. Nevertheless, the clinical data of UDCA use in VLBW infants, the most vulnerable group, are limited. We report the results of administration of UDCA therapy to VLBW infants with intractable TPNAC. METHODS: Medical records of VLBW infants who were treated with oral UDCA, at dose of 15-20 mg/kg/day, for intractable TPNAC were reviewed from 1999-2001. Treatment effectiveness was evaluated by monitoring the biochemical hepatic markers, including total bilirubin, direct bilirubin, alkaline phosphatase (ALP), alanine aminotransferase (ALT), aspartate aminotransferase (AST) and gamma-glutamyl transferase (GGT). RESULTS: A total of 13 infants were identified with the diagnosis of intractable TPNAC and they were treated with UDCA therapy. There was a significant reduction in serum levels of direct bilirubin, total bilirubin (p-value equals 0.0001) and AST (p-value equals 0.001). However, the serum levels of ALP, ALT and GGT showed a trend of improvement, yet none of them was statistically significant. Serum direct bilirubin was noted as the first marker to respond to UDCA therapy. It declined steadily during the course of therapy except in two intervals at the sixth and twelfth week of therapy that apparently associated with severe sepsis. There were no serious side effects noted. CONCLUSION: Our series data suggest that UDCA is safe and may be a potential treatment for intractable TPNAC if used within two weeks after TPN withdrawal and full enteral feeding. Sepsis may alter the effectiveness of UDCA therapy.
INTRODUCTION:Cholestasis associated with long-term total parenteral nutrition (TPN) occurs commonly in very low birth weight (VLBW) infants. Indeed, the majority of infants with TPN-associated cholestasis (TPNAC) respond very well to TPN withdrawal and full enteral feeding, yet some of them do not respond and have the potential for development of intractable cholestasis. It has been demonstrated that ursodeoxycholic acid (UDCA) has beneficial effects in treating TPNAC in various age groups. Nevertheless, the clinical data of UDCA use in VLBW infants, the most vulnerable group, are limited. We report the results of administration of UDCA therapy to VLBW infants with intractable TPNAC. METHODS: Medical records of VLBW infants who were treated with oral UDCA, at dose of 15-20 mg/kg/day, for intractable TPNAC were reviewed from 1999-2001. Treatment effectiveness was evaluated by monitoring the biochemical hepatic markers, including total bilirubin, direct bilirubin, alkaline phosphatase (ALP), alanine aminotransferase (ALT), aspartate aminotransferase (AST) and gamma-glutamyl transferase (GGT). RESULTS: A total of 13 infants were identified with the diagnosis of intractable TPNAC and they were treated with UDCA therapy. There was a significant reduction in serum levels of direct bilirubin, total bilirubin (p-value equals 0.0001) and AST (p-value equals 0.001). However, the serum levels of ALP, ALT and GGT showed a trend of improvement, yet none of them was statistically significant. Serum direct bilirubin was noted as the first marker to respond to UDCA therapy. It declined steadily during the course of therapy except in two intervals at the sixth and twelfth week of therapy that apparently associated with severe sepsis. There were no serious side effects noted. CONCLUSION: Our series data suggest that UDCA is safe and may be a potential treatment for intractable TPNAC if used within two weeks after TPN withdrawal and full enteral feeding. Sepsis may alter the effectiveness of UDCA therapy.