Dramatic Extrahepatic Cholestasis Improvement after Steroid Therapy

Biliary atresia remains a health concern and the leading cause of liver transplantation despite the establishment of Kasai surgery. This case report describes an infant with extrahepatic cholestasis leading to biliary atresia that improved with steroids. A one-month-24-day-old girl presented with the chief complaint of jaundice accompanied by pale stools, dark urine, and a distended abdomen since she was two weeks of age. The laboratory findings showed elevated liver function tests (AST 99 U/L, ALT 87 U/L, GGT 100.9 U/L, and ALP 968 U/L) and cholestasis (total bilirubin 10.02 mg/dL and direct bilirubin 7.34 mg/dL) with anemia (Hb 9.0 g/dL), accompanied by reactive CMV IgG and reactive CMV IgM. A two-phase abdominal ultrasound examination revealed a gallbladder length of ±2.54 cm, increased HA/PV, and a gallbladder contractility index of ±76%. A percutaneous liver biopsy revealed extrahepatic cholestasis. After eight weeks of steroid and ursodeoxycholic acid treatment, improvements were observed in her clinical condition, laboratory biomarkers (AST 41 U/L, ALT 77 U/L, GGT 115 U/L, ALP 383 U/L, total bilirubin 0.3 mg/dL, direct bilirubin 0.1 mg/dL, and Hb 13.8 g/dL) and gallbladder contractility index. Hence, steroid administration in extrahepatic cholestasis leading to biliary atresia may improve inflammation in the biliary system and prevent the progression of biliary obstruction to biliary atresia.