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A 13-year-old boy presented with a 4-day history of vomiting, jaundice, pruritus, pale stools and dark urine. He was apyrexic and had right-upper-quadrant abdominal tenderness. He had raised alkaline-phosphatase (620 U/L), gamma-glutamyl-transferase (435 U/L), total bilirubin (102 μmol/L), direct bilirubin (94 μmol/L), alanine-transaminase (123 U/L) and lipase (68 U/L), but normal amylase (37 U/L), faecal elastase (232 µg/g) and serum IgG4 levels.
Transabdominal ultrasound showed a distended gallbladder and dilated common bile duct (15 mm). He received prophylactic pancreatic enzyme supplementation, vitamin K, cholestyramine, rifampicin and …
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