Congenital disorders of the liver, biliary tract, and pancreas

Congenital disorders of the liver, biliary tract, and pancreas are rare or very rare. Biliary atresias present with cholestatic jaundice starting after the first 2 weeks of life and may eventually cause biliary cirrhosis and liver failure. Prognosis depends on the type of atresia: (1) intrahepatic—both nonsyndromic (with cirrhosis usually developing in late childhood, and fatal without liver transplantation) and syndromic (e.g. Alagille’s syndrome, due to mutation in the ...

Talon Cusps Presenting in a Child with Alagille's Syndrome –A Case Report

Talon cusp is a rare dental anomaly often associated with systemic conditions such as Rubenstein-Taybi Syndrome,Ellis-van Crevald Syndrome and Incontentia pigmenti achromians. It has not been previously reported as occurring in Alagille Syndrome. Early diagnosis of talon cusps is crucial for the correct management and avoidance of complications. This case highlights the need for careful dental examination when treating children with syndromes, as new phenotypes that can have an effect on the dentition.