Maturity-Onset Diabetes of the Young (MODY): Genetic Causes, Clinical Characteristics, Considerations for Testing, and Treatment Options

Zoltan Antal

doi:10.3390/endocrines2040043

journal article Open Access Dec 03, 2021

Maturity-Onset Diabetes of the Young (MODY): Genetic Causes, Clinical Characteristics, Considerations for Testing, and Treatment Options

Zoltan Antal

Endocrines Vol. 2 No. 4 pp. 485-501 · MDPI AG

View at Publisher Save 10.3390/endocrines2040043

Abstract

Maturity Onset Diabetes of the Young (MODY) encompasses a group of rare monogenic forms of diabetes distinct in etiology and clinical presentation from the more common forms of Type 1 (autoimmune) and Type 2 diabetes. Since its initial description as a clinical entity nearly 50 years ago, the underlying genetic basis for the various forms of MODY has been increasingly better elucidated. Clinically, the diagnosis may be made in childhood or young adulthood and can present as overt hyperglycemia requiring insulin therapy or as a subtle form of slowly progressive glucose impairment. Due to the heterogeneity of clinical symptoms, patients with MODY may be misdiagnosed as possessing another form of diabetes, resulting in potentially inappropriate treatment and delays in screening of affected family members and associated comorbidities. In this review, we highlight the various known genetic mutations associated with MODY, clinical presentation, indications for testing, and the treatment options available.

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Details

Published: Dec 03, 2021
Vol/Issue: 2(4)
Pages: 485-501
License: View

Authors

Z

Zoltan Antal

Division of Pediatric Endocrinology, Department of Pediatrics, Weill Cornell Medicine, New York Presbyterian Hospital, 505 East 70 Street, New York, NY 10021, USA

Cite This Article

Zoltan Antal (2021). Maturity-Onset Diabetes of the Young (MODY): Genetic Causes, Clinical Characteristics, Considerations for Testing, and Treatment Options. Endocrines, 2(4), 485-501. https://doi.org/10.3390/endocrines2040043

Maturity-Onset Diabetes of the Young (MODY): Genetic Causes, Clinical Characteristics, Considerations for Testing, and Treatment Options

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