This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: None, Subject name: Surgery
[{'content': 'Which of the following are histopathological features of Extra hepatic Biliary Atresia -a) Bile lakesb) Hepatocyte ballooning degenerationc) Marked bile duct proliferationd) Fibrosis of hepatic ducte) Parenchymal cholestasis \nSelect and explain. \nA. cde, B. acd, C. bde, D. ade', 'role': 'user'}, {'content': 'Answer: A \nExplanation: • Characterized by progressive obliteration of the extrahepatic and intrahepatic bile ducts.\n• Etiology is unknown; incidence 1 in 12,000 live births.\n• Presently, there is no medical therapy to reverse the obliterative process\n• Patients who are not offered surgical treatment uniformly develop biliary cirrhosis, portal hypertension, and death by 2 years of age.\n• MC indication for pediatric liver transplantation\nPathology\n• Bile duct proliferation, severe cholestasis with plugging, and inflammatory cell infiltrate are the pathologic hallmarks of this\xa0disease.\n• Over time, these changes progress to fibrosis with end-stage cirrhosis.\n• Positive for neural cell adhesion molecule (CD56) staining\nVariants of biliary atresia\n• Patency to the level of CBD\n• Patency to the level of common hepatic duct\n• Left and right hepatic duct at porta involved, solid porta hepatis (90%)\nClinical Features\n• Infants with biliary atresia present with jaundice at birth or shortly thereafter.\n• Infants with biliary atresia characteristically have acholic, pale gray stools, secondary to obstructed bile flow.\n• With passage of time, progressive failure to thrive and, if untreated, develop stigmata of liver failure and portal hypertension (splenomegaly and esophageal varices)\n• Associated malformations in 25%: Polysplenia, malrotation, preduodenal portal vein, and intrahepatic vena cava.\nDiagnosis\n• USG of the liver and GB is important in the evaluation of the infant with cholestasis.\n• USG: GB is shrunken and CBD is not visible. A triangle cord sign\xa0found on ultrasound has a predictive accuracy of 95%, the gallbladder ghost triad\xa0in which the gallbladder is short (<1.9 cm) and irregular and lacks an echogenic inner lining also got good sensitivity.\n• Next diagnostic step: Percutaneous liver biopsy\xa0if the hepatic synthetic function is normal (diagnostic accuracy 90%).\n• Hepatobiliary scintigraphy: In cases in which the USG and biopsy findings are inconclusive (absent excretion into the intestine)\nTreatment\n• Exploratory laparotomy: If the needle biopsy or abdominal ultrasound is consistent with BA\n• Intra-operative cholecystocholangiography: To confirm the diagnosis, demonstration of the fibrotic biliary remnant and definition of absent proximal and distal bile duct patency\n• Treatment of choice: Kasai hepatoportoenterostomy\xa0(Roux-en-Y hepaticojejunostomy)\nPostoperative Management\n• Ursodeoxycholic acid (facilitate bile flow) + Methylprednisolone (anti-inflammatory agent) + TMP-SMX (antimicrobial prophylaxis)\n• Cholangitis is the MC post-operative complication.\nPrognosis\n• About 30% of infants undergoing hepatoportoenterostomy before 60 days of age will have a long-term successful outcome and not require liver transplantation.\n• Liver transplantation in the patients who develop progressive hepatic fibrosis with resultant portal hypertension and progressive cholestasis.\n• Serum bilirubin at 3 months after surgery seems to be strongly predictive of long-term survival.', 'role': 'assistant'}]