BPG is committed to discovery and dissemination of knowledge
Retrospective Cohort Study
Copyright: ©Author(s) 2026. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution-NonCommercial (CC BY-NC 4.0) license. No commercial re-use. See permissions. Published by Baishideng Publishing Group Inc.
World J Clin Pediatr. Dec 9, 2026; 15(4): 121263
Published online Dec 9, 2026. doi: 10.5409/wjcp.121263
UGT1A1-related disorders in Bahrain: A genetic and clinical overview of Crigler-Najjar and Gilbert syndromes
Hasan M Isa, Fatema A Abdulaal, Maryam Y Busehail, Maryam H Kamal, Hawra A Alaswad, Fatima Y Alshaikh, Aysha A Aljassmi, Anfal J Hijris
Hasan M Isa, Maryam Y Busehail, Department of Pediatrics, College of Medicine and Medical Sciences, Arabian Gulf University, Manama 26671, Bahrain
Hasan M Isa, Fatema A Abdulaal, Maryam Y Busehail, Maryam H Kamal, Hawra A Alaswad, Aysha A Aljassmi, Anfal J Hijris, Department of Pediatrics, Salmaniya Medical Complex, Manama 26671, PO Box 12, Bahrain
Fatima Y Alshaikh, Department of Internal Medicine, Salmaniya Medical Complex, Manama 26671, PO Box 12, Bahrain
Author contributions: Isa HM contributed to the study design, literature review, data analysis, manuscript drafting, and oversight of all project phases and final approval of the version to be published; Abdulaal FA contributed to the study design, literature review, data analysis, and manuscript drafting; Busehail MY was responsible for data collection and manuscript drafting; Kamal MH conducted the literature review and drafted the manuscript; Alaswad HA conducted the literature review, collected data, and drafted the manuscript; Alshaikh FY, Aljassmi AA, and Hijris AJ contributed to the literature review and data collection.
Institutional review board statement: This study was conducted in accordance with the principles of the Declaration of Helsinki (1964), as revised in 2000, and was approved by the Research and Research Ethics Committee, Salmaniya Medical Complex, Government Hospitals, Manama, Bahrain (No. 42080523).
Informed consent statement: Informed consent was waived because the study was retrospective and did not involve direct patient contact or identifiable patient data.
Conflict-of-interest statement: All authors declare that they have no relevant conflicts of interest related to this article.
STROBE statement: The authors have read the STROBE Statement-checklist of items, and the manuscript was prepared and revised according to the STROBE Statement- checklist of items.
Data sharing statement: The data supporting the findings of this study are available from the corresponding author upon reasonable request.
Corresponding author: Hasan M Isa, Associate Professor, Consultant, Principal Investigator, Senior Researcher, Department of Pediatrics, College of Medicine and Medical Sciences, Arabian Gulf University, No. 2904 Al Salmaniya Area, Manama 26671, Bahrain. halfaraj@hotmail.com
Received: March 23, 2026
Revised: April 17, 2026
Accepted: May 19, 2026
Published online: December 9, 2026
Processing time: 182 Days and 16.1 Hours
Core Tip

Core Tip: This first study from Bahrain demonstrated that UGT1A1-related disorders represent important causes of persistent unconjugated hyperbilirubinemia, affecting 2.2 per 100000 individuals. Gilbert syndrome (GS) was the most common phenotype, followed by Crigler–Najjar syndrome (CNS) type I and type II. Early genetic testing is crucial for diagnosis, particularly in patients with parental consanguinity and those with associated hematological disorders, mainly glucose-6-phosphate dehydrogenase deficiency. Homozygous (TA)7/7 was the most common variant. CNS-I requires lifelong phototherapy or liver transplantation, whereas CNS-II is treated with phenobarbital; however, treatment adherence remains challenging. No treatment is required for GS.

Write to the Help Desk