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Rare subtypes of diabetes mellitus MODY: Analysis of the registry of monogenic diabetes mellitus in children
https://doi.org/10.25557/2073-7998.2025.06.70-71
Abstract
MODY (Maturity onset diabetes of the young) is a group of non–immune genetic disorders of carbohydrate metabolism caused by variants of genes associated with the synthesis, secretion or action of insulin. There are 14 subtypes of MODY. GCK-MODY (69.7%) and HNF1A-MODY (10.1%) are the most common subtypes according to the registry of the Institute of Pediatric Endocrinology of the Endocrinology Research Centre, Moscow, Russia. Rare subtypes of MODY were used in 9.4% of cases. 1.1% patients have variants in MODY candidate genes. 6.7% of patients were diagnosed with MODYX. Verification of the genetic cause plays a key role in the choice of therapeutic tactics and opens up prospects for the use of gene therapy in such patients in the future.
Supporting agencies: This research was supported by the Ministry of Science and Higher Education of the Russian Federation (Agreement No. 075-15-2024-645).
About the Authors
M. P. Koltakova
Endocrinology Research Centre
Russian Federation
Russian Federation
11, Dm.Ulyanova st., Moscow, 117292
E. A. Sechko
Endocrinology Research Centre
Russian Federation
Russian Federation
11, Dm.Ulyanova st., Moscow, 117292
T. L. Kuraeva
Endocrinology Research Centre
Russian Federation
Russian Federation
11, Dm.Ulyanova st., Moscow, 117292
I. A. Eremina
Endocrinology Research Centre
Russian Federation
Russian Federation
11, Dm.Ulyanova st., Moscow, 117292
D. N. Laptev
Endocrinology Research Centre
Russian Federation
Russian Federation
11, Dm.Ulyanova st., Moscow, 117292
O. B. Bezlepkina
Endocrinology Research Centre
Russian Federation
Russian Federation
11, Dm.Ulyanova st., Moscow, 117292
V. A. Peterkova
Endocrinology Research Centre
Russian Federation
Russian Federation
11, Dm.Ulyanova st., Moscow, 117292
References
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2. Aarthy R., Aston-Mourney K., Mikocka-Walus A., et al. Clinical features, complications and treatment of rarer forms of maturity-onset diabetes of the young (MODY) – A review. J Diabetes Complications. 2021;35(1):107640. doi: 10.1016/j.jdiacomp.2020.107640.
3. Delvecchio M., Pastore C., Giordano P. Treatment Options for MODY Patients: A Systematic Review of Literature. Diabetes Ther. 2020;11(8):1667-1685. doi: 10.1007/s13300-020-00864-4.
Review
For citations:
Koltakova M.P., Sechko E.A., Kuraeva T.L., Eremina I.A., Laptev D.N., Bezlepkina O.B., Peterkova V.A. Rare subtypes of diabetes mellitus MODY: Analysis of the registry of monogenic diabetes mellitus in children. Medical Genetics. 2025;24(6):70-71. (In Russ.) https://doi.org/10.25557/2073-7998.2025.06.70-71
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