A 31-year-old woman presents with recurrent jaundice during pregnancy (BP 118/76, HR 82, RR 16, Temp 37°C, SpO2 98%). Laboratory studies reveal elevated direct bilirubin at 3.2 mg/dL with normal AST/ALT (<40 U/L). Abdominal ultrasound shows no gallstones or biliary obstruction. Liver biopsy demonstrates dark brown pigmentation within hepatocytes. She denies pruritus or acholic stools. Which enzyme defect best explains these findings?
- A)Autoimmune destruction of intrahepatic bile ducts
- B)Impaired bilirubin conjugation in hepatocytes
- C)Deficiency of glucose 6 phosphate dehydrogenase
- D)Excess heme breakdown due to occult hemolysis
- E)Defective canalicular secretion of conjugated bilirubinGABARITO
Explicação
Dubin Johnson syndrome is caused by defective canalicular transport of conjugated bilirubin into bile, producing a predominantly conjugated hyperbilirubinemia. The condition is benign and classically associated with a darkly pigmented liver. Pregnancy can acce... Ver explicação completa e trilha adaptativa →