Maturity-onset diabetes of the young (MODY): current perspectives on diagnosis and treatment

Tatsuhiko UrakamiDepartment of Pediatrics, Nihon University School of Medicine, Tokyo, JapanAbstract: Maturity-onset diabetes of the young (MODY) is characterized by autosomal dominant inheritance, onset before 25 years of age, absence of β-cell autoimmunity, and sustained pancreatic &b...

Full description

Bibliographic Details
Main Author: Urakami T
Format: Article
Language:English
Published: Dove Medical Press 2019-07-01
Series:Diabetes, Metabolic Syndrome and Obesity : Targets and Therapy
Subjects:
Online Access:https://www.dovepress.com/maturity-onset-diabetes-of-the-young-mody-current-perspectives-on-diag-peer-reviewed-article-DMSO
id doaj-acb9bb5fe34f40b5be29d8425ccd4c11
record_format Article
spelling doaj-acb9bb5fe34f40b5be29d8425ccd4c112020-11-25T00:17:28ZengDove Medical PressDiabetes, Metabolic Syndrome and Obesity : Targets and Therapy1178-70072019-07-01Volume 121047105646918Maturity-onset diabetes of the young (MODY): current perspectives on diagnosis and treatmentUrakami TTatsuhiko UrakamiDepartment of Pediatrics, Nihon University School of Medicine, Tokyo, JapanAbstract: Maturity-onset diabetes of the young (MODY) is characterized by autosomal dominant inheritance, onset before 25 years of age, absence of β-cell autoimmunity, and sustained pancreatic β-cell function. To date, mutations have been identified in at least 14 different genes, including six genes encoding proteins that, respectively, correspond to MODY subtypes 1–6: hepatocyte nuclear factor (HNF) 4α (HNF4α), glucokinase (GCK), HNF1α (HNF1α), pancreatic and duodenal homeobox 1 (PDX1), HNF1β (HNF1β), and neurogenic differentiation 1 (NEUROD1). Diagnostic tools based on currently available genetic tests can facilitate the correct diagnosis and appropriate treatment of patients with MODY. Candidates for genetic testing include nonobese subjects with hyperglycemia, no evidence of β-cell autoimmunity, sustained β-cell function, and a strong family history of similar-type diabetes among first-degree relatives. Moreover, identification of the MODY subtype is important, given the subtype-related differences in the age of onset, clinical course and progression, type of hyperglycemia, and response to treatment. This review discusses the current perspectives on the diagnosis and treatment of MODY, particularly with regard to the six major subtypes (MODY 1–6).Keywords: MODY, molecular diagnosis, genetic testing, pharmacological treatmenthttps://www.dovepress.com/maturity-onset-diabetes-of-the-young-mody-current-perspectives-on-diag-peer-reviewed-article-DMSOMODYmolecular diagnosisgenetic testingpharmacological treatment
collection DOAJ
language English
format Article
sources DOAJ
author Urakami T
spellingShingle Urakami T
Maturity-onset diabetes of the young (MODY): current perspectives on diagnosis and treatment
Diabetes, Metabolic Syndrome and Obesity : Targets and Therapy
MODY
molecular diagnosis
genetic testing
pharmacological treatment
author_facet Urakami T
author_sort Urakami T
title Maturity-onset diabetes of the young (MODY): current perspectives on diagnosis and treatment
title_short Maturity-onset diabetes of the young (MODY): current perspectives on diagnosis and treatment
title_full Maturity-onset diabetes of the young (MODY): current perspectives on diagnosis and treatment
title_fullStr Maturity-onset diabetes of the young (MODY): current perspectives on diagnosis and treatment
title_full_unstemmed Maturity-onset diabetes of the young (MODY): current perspectives on diagnosis and treatment
title_sort maturity-onset diabetes of the young (mody): current perspectives on diagnosis and treatment
publisher Dove Medical Press
series Diabetes, Metabolic Syndrome and Obesity : Targets and Therapy
issn 1178-7007
publishDate 2019-07-01
description Tatsuhiko UrakamiDepartment of Pediatrics, Nihon University School of Medicine, Tokyo, JapanAbstract: Maturity-onset diabetes of the young (MODY) is characterized by autosomal dominant inheritance, onset before 25 years of age, absence of β-cell autoimmunity, and sustained pancreatic β-cell function. To date, mutations have been identified in at least 14 different genes, including six genes encoding proteins that, respectively, correspond to MODY subtypes 1–6: hepatocyte nuclear factor (HNF) 4α (HNF4α), glucokinase (GCK), HNF1α (HNF1α), pancreatic and duodenal homeobox 1 (PDX1), HNF1β (HNF1β), and neurogenic differentiation 1 (NEUROD1). Diagnostic tools based on currently available genetic tests can facilitate the correct diagnosis and appropriate treatment of patients with MODY. Candidates for genetic testing include nonobese subjects with hyperglycemia, no evidence of β-cell autoimmunity, sustained β-cell function, and a strong family history of similar-type diabetes among first-degree relatives. Moreover, identification of the MODY subtype is important, given the subtype-related differences in the age of onset, clinical course and progression, type of hyperglycemia, and response to treatment. This review discusses the current perspectives on the diagnosis and treatment of MODY, particularly with regard to the six major subtypes (MODY 1–6).Keywords: MODY, molecular diagnosis, genetic testing, pharmacological treatment
topic MODY
molecular diagnosis
genetic testing
pharmacological treatment
url https://www.dovepress.com/maturity-onset-diabetes-of-the-young-mody-current-perspectives-on-diag-peer-reviewed-article-DMSO
work_keys_str_mv AT urakamit maturityonsetdiabetesoftheyoungmodycurrentperspectivesondiagnosisandtreatment
_version_ 1725379594344726528