Dental management of a patient with progressive familial intrahepatic cholestasis

Abstract

Progressive familial intrahepatic cholestasis refers to a heterogenous group of autosomal recessive disorders in which children develop severe intrahepatic cholestasis progressing to biliary cirrhosis and chronic liver failure, usually during the first decade of life. The clinical features include jaundice, hepatomegaly, splenomegaly, growth retardation and severe pruritus. The laboratory tests demonstrate elevated bilirubin, bile acids and liver function enzymes. The only curative treatment of progressive familial intrahepatic cholestasis is liver transplantation.This article presents the medical and dental history along with a comprehensive dental management and prognosis of a 6years old male patient with progressive familial intrahepatic cholestasis type I and liver cirrhosis 5years post living related liver transplant in Riyadh, Saudi Arabia. The patient demonstrated improved oral hygiene performance during the course of treatment, and continued to demonstrate a low caries rate up to 7months following treatment. Based upon the apparent success of the preventive programme, the patient was judged to have a very good prognosis.