Pediatric liver transplant

Q  ) Most common indication for Pediatric liver transplant (DNB SS) 
A. Biliary atresia
B. Metabolic diseases
C. Alagille syndrome
D. HCV

Free answer 

Biliay atresia

MOst common cause or liver transplant in children. Presents as persistent jaundice after birth. Etiology is not clear

Findings include absent extrahepatic ducts and gallbladder. Biopsy is diagnostic

Recurrent Pyogenic Cholangitis

Q) Not true about  recurrent pyogenic cholangitis :

a) Mostly there are intrahepatic strictures with involvement of the left side duct

b) It can present as choledochoduodenal fistula

c) In it there is complete biliary obstruction which  leads to marked jaundice and pruritis

d) MRCP and other other cholangiography can be diagnostic

Answer c

In recurrent pyogenic cholangitis (RPC)  complete obstruction does not occur and jaundice and pruritis is not marked. RPC is a disease commonly seen in young Asians (also known as oriental cholangiohepatitis) which leads to multiple strictures in extra or intrahepatic ducts.

Association with Ascaris lumbricoides and Clonorchis sinensis has been noted.

It can present as choledocholithiasis  with stricture, choledochoduodenal fistula, acute pancreatitis, secondary biliary cirrhosis and can lead to cholangiocarcinoma.

MRCP can be diagnostic and is preferred because of its non invasive nature.