Clinical features, complications and treatment of rarer forms of maturity-onset diabetes of the young (MODY) - A review

Maturity onset diabetes of the young (MODY) is the most common form of monogenic diabetes and is currently believed to have 14 subtypes. While much is known about the common subtypes of MODY (MODY-1, 2, 3 and 5) little is known about its rare subtypes (MODY4, 6–14). With the advent of next-generatio...

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Published in:Journal of diabetes and its complications Vol. 35; no. 1; p. 107640
Main Authors: Aarthy, Ramasamy, Aston-Mourney, Kathryn, Mikocka-Walus, Antonina, Radha, Venkatesan, Amutha, Anandakumar, Anjana, Ranjit Mohan, Unnikrishnan, Ranjit, Mohan, Viswanathan
Format: Journal Article
Language:English
Published: United States Elsevier Inc 01.01.2021
Elsevier Limited
Subjects:
Clinical characteristics
Complications
Cysts
Diabetes
Diabetes Mellitus, Type 2 - complications
Diabetes Mellitus, Type 2 - diagnosis
Diabetes Mellitus, Type 2 - drug therapy
Diabetic ketoacidosis
Genes
Humans
Hyperglycemia
Insulin
Maturity onset diabetes of the young
Monogenic diabetes
Mutation
Pharmaceutical Preparations
Software
Subtypes types of MODY
Sulfonylurea Compounds
Systematic review
Treatment
Subtypes types of MODY
Clinical characteristics
Treatment
Maturity onset diabetes of the young
Complications
Monogenic diabetes
ISSN:1056-8727, 1873-460X, 1873-460X
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Summary:Maturity onset diabetes of the young (MODY) is the most common form of monogenic diabetes and is currently believed to have 14 subtypes. While much is known about the common subtypes of MODY (MODY-1, 2, 3 and 5) little is known about its rare subtypes (MODY4, 6–14). With the advent of next-generation sequencing (NGS) there are several reports of the rarer subtypes of MODY emerging from across the world. Therefore, a greater understanding on these rarer subtypes is needed. A search strategy was created, and common databases were searched, and 51 articles finally selected. INS-(MODY10) and ABCC8-(MODY12) mutations were reported in relatively large numbers compared to the other rare subtypes. The clinical characteristics of the rare MODY subtypes exhibited heterogeneity between families reported with the same mutation. Obesity and diabetic ketoacidosis (DKA) were also reported among rarer MODY subtypes which presents as a challenge as these are not part of the original description of MODY by Tattersal and Fajans. The treatment modalities of the rarer subtypes included oral drugs, predominantly sulfonylureas, insulin but also diet alone. Newer drugs like DPP-4 and SGLT2 inhibitors have also been tried as new modes of treatment. The microvascular and macrovascular complications among the patients with various MODY subtypes are less commonly reported. Recently, there is a view that not all the 14 forms of ‘MODY’ are true MODY and the very existence of some of these rarer subtypes as MODY has been questioned. This scoping review aims to report on the clinical characteristics, treatment and complications of the rarer MODY subtypes published in the literature. •Maturity Onset Diabetes of the Young (MODY) was first identified by Fajans and Tattersall in 1975.•Clinical features of MODY 1, 2 ,3 and 5 are well described while that of rarer types are unclear.•Not all 14 forms are true MODY. This review provides guidelines on how we should classify the MODY subtypes in the future.
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ISSN:1056-8727
1873-460X
1873-460X
DOI:10.1016/j.jdiacomp.2020.107640