Title

Early thiamine therapy in Rogers syndrome: Systematic review of hematologic, auditory and glycemic outcomes

Authors

Zalak Upadhyay^1,*, Shailesh Sudani¹ & Abhay Jain²

Affiliation

¹Department of Pediatrics, Shantabaa Medical College and General Hospital, Amreli, Gujarat, India; ²Department of Pediatrics, American International Institute of Medical Sciences, Udaipur, Rajasthan, India; *Corresponding author

Email

Zalak Upadhyay - E-mail: drzmupadhyay@yahoo.co.in
Shailesh Sudani - E-mail: drshaileshsudani263@gmail.com
Abhay Jain - E-mail: abhaytanu77@gmail.com

Article Type

Review

Date

Received January 1, 2026; Revised January 31, 2026; Accepted January 31, 2026, Published January 31, 2026

Abstract

Rogers syndrome (thiamine-responsive megaloblastic anemia, TRMA) is a rare autosomal recessive disorder caused by mutations in SLC19A2 and is characterized by megaloblastic anemia, diabetes and sensorineural deafness. Therefore, it is of interest to review the effect of early thiamine therapy on recovery of hematologic function, preservation of hearing and glycemic control. Available data shows that anemia reversed consistently with early thiamine, glycemic control improved when treatment was initiated before β-cell exhaustion and hearing loss almost never improved once it had occurred. Thus, early administration of thiamine is critical to hematologic recovery and to partial metabolic benefit, but is not uniformly preventive of deafness.

Keywords

Rogers syndrome, mutations, megaloblastic anemia, glycemic control.

Citation

Upadhyay et al. Bioinformation 22(1): 26-30 (2026)

Edited by

Vini Mehta

ISSN

0973-2063

Publisher

Biomedical Informatics

License

This is an Open Access article which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. This is distributed under the terms of the Creative Commons Attribution License.