Pathology · Hepatobiliary and Pancreatic Pathology

A 3-week-old term boy has had jaundice since day 5 of life. Total bilirubin is 11 mg/dL with a direct fraction of 8 mg/dL. Stools are pale and urine stains the diaper yellow. Ultrasound shows an absent or atretic gallbladder. What is the most appropriate next step?

  • A Ursodeoxycholic acid therapy with reassessment after 4 weeks
  • B High-dose corticosteroid trial for suspected neonatal hepatitis
  • C Liver transplantation as initial definitive treatment
  • D Hepatoportoenterostomy (Kasai procedure)
Correct answer: D. Hepatoportoenterostomy (Kasai procedure)

Explanation

Conjugated jaundice with acholic stools and a small or absent gallbladder in the first weeks of life indicates extrahepatic biliary atresia. Kasai portoenterostomy performed before 60 days of age restores bile flow in many infants and delays cirrhosis; success falls sharply after this window. Transplantation is reserved for failed Kasai or late presentation, not the initial step. Steroids or observation waste irreplaceable time.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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