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World J Clin Pediatr. Jul 9, 2022; 11(4): 321-329
Published online Jul 9, 2022. doi: 10.5409/wjcp.v11.i4.321
Hereditary fructose intolerance: A comprehensive review
Sumit Kumar Singh, Moinak Sen Sarma
Sumit Kumar Singh, Department of Pediatrics, Sri Aurobindo Medical College and PGI, Indore 453555, Madhya Pradesh, India
Moinak Sen Sarma, Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, India
Author contributions: Sarma MS conception, final drafting of the manuscript; Singh SK conception, primary drafting of the manuscript.
Conflict-of-interest statement: All the authors declare that they have no conflict of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Moinak Sen Sarma, MD, Associate Professor, Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow 226014, India. moinaksen@yahoo.com
Received: November 27, 2021
Peer-review started: November 27, 2021
First decision: April 13, 2022
Revised: May 8, 2022
Accepted: June 20, 2022
Article in press: June 20, 2022
Published online: July 9, 2022
Core Tip

Core Tip: Hereditary Fructose Intolerance is a rare autosomal recessive inherited disorder due to the mutation of enzyme aldolase B. Awareness regarding its diverse manifestations is required to clinically suspect and diagnose this condition. Genetic testing clinches the diagnosis. Treatment is simple and involves only the dietary exclusion of fructose, sucrose and sorbitol. The prognosis is favourable. This review provides a comprehensive understanding of the disease.