pediatrics
Neonatal Cholestasis
Treatment of Neonatal Cholestasis
Specific cause treated Vitamin A, D, E, and K supplements Medium-chain triglycerides Sometimesursodeoxycholic acid For suspected biliary atresia, surgical exploration and sometimes portoenterostomy Specific treatment is directed at the cause. If there is no specific therapy, treatment is supportive and consists primarily of nutritional therapy, including supplements of vitamins A, D, E, and K. For formula-fed infants, a formula that is high in medium-chain triglycerides should be used because it is absorbed better in the presence of bile salt deficiency. Adequate calories are required; infants may need>130 calories/kg/day. In infants with some bile flow,ursodeoxycholic acidonce or twice a day may relieve itching. Infants with presumed biliary atresia require surgical exploration with an intraoperative cholangiogram. If biliary atresia is confirmed, a portoenterostomy (Kasai procedure) should be done. Ideally, this procedure should be done in the first 1 to 2 months of life. After this period, the short-term prognosis significantly worsens. Postoperatively, many patients have significant chronic problems, including persistent cholestasis, recurrent ascending cholangitis, and failure to thrive. Prophylactic antibiotics (eg,sulfamethoxazole/trimethoprim, SMX-TMP, cotrimoxazole) are frequently prescribed for a year postoperatively in an attempt to prevent ascending cholangitis. Even with optimal therapy, most infants develop cirrhosis and require liver transplantation. Cholestasis caused by parenteral nutrition resolves if the parenteral nutrition is stopped or sometimes if a newer-generation lipid emulsion is substituted for an older one before the infant develops severe liver disease. Because gestational alloimmune liver disease has no definitive marker and/or test, treatment with IV immune globulin (IVIG) or exchange transfusion needs to be considered early to reverse the ongoing liver injury if no definite diagnosis has been made ( Treatment reference ). 1.Fischer HS, Staufner C, Sallmon H, et al: Early exchange transfusion to treat neonates with gestational alloimmune liver disease: An 11-year cohort study.J Pediatr Gastroenterol Nutr70(4):444–449, 2020. doi: 10.1097/MPG.0000000000002593 