Publication:
Tapentadol withdrawal, a newer trend in opioid overuse in Nepal: A case report

Research Projects

Organizational Units

Journal Issue

Abstract

Abstract Crigler–Najjar Syndrome Type 2 (CNS2) is a rare autosomal recessive disorder characterized by unconjugated hyperbilirubinemia due to partial deficiency of the enzyme uridine diphosphate glucuronosyltransferase 1A1 (UGT1A1). We present a case of a 13-month-old male admitted to Kanti Children’s Hospital with persistent jaundice since birth. Diagnostic evaluation accompanied by gene sequencing confirmed CNS2 and the patient was effectively managed with orally administered phenobarbitone. CNS2 can be distinguished from other potential causes of unconjugated hyperbilirubinemia based on bilirubin concentration and the affected patient’s response to phenobarbitone. Genetic counselling is essential for the recognition and prevention of severe hyperbilirubinemia which, in the absence of timely medical intervention, may lead to neurotoxicity. Keywords: Case report; crigler-Najjar syndrome; genetic counseling; phenobarbitone; unconjugated hyperbilirubinemia.

Description

Hemant Chand Department of Emergency Medicine, Nova Hospital, Nepal Om Prakash Bhatta Department of Emergency Medicine, Nova Hospital, Nepal Sabita Chand Department of Emergency Medicine, Nova Hospital, Nepal Neha Dangol Department of Emergency Medicine, Nova Hospital, Nepal Sachin Awasthi Department of Emergency Medicine, Yashoda Hospital, Nepal Ram Chandra Poudel Department of Internal Medicine, Nova Hospital, Nepal Ram Prasad Lamichhane Department of Psychiatry, Nova Hospital, Nepal

Keywords

Citation

Collections