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...

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Autor principal:	Urakami T
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2019
Materias:	MODY molecular diagnosis genetic testing pharmacological treatment Specialties of internal medicine RC581-951
Acceso en línea:	https://doaj.org/article/acb9bb5fe34f40b5be29d8425ccd4c11
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spelling	oai:doaj.org-article:acb9bb5fe34f40b5be29d8425ccd4c112021-12-02T03:38:38ZMaturity-onset diabetes of the young (MODY): current perspectives on diagnosis and treatment1178-7007https://doaj.org/article/acb9bb5fe34f40b5be29d8425ccd4c112019-07-01T00:00:00Zhttps://www.dovepress.com/maturity-onset-diabetes-of-the-young-mody-current-perspectives-on-diag-peer-reviewed-article-DMSOhttps://doaj.org/toc/1178-7007Tatsuhiko 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 treatmentUrakami TDove Medical PressarticleMODYmolecular diagnosisgenetic testingpharmacological treatmentSpecialties of internal medicineRC581-951ENDiabetes, Metabolic Syndrome and Obesity: Targets and Therapy, Vol Volume 12, Pp 1047-1056 (2019)
institution	DOAJ
collection	DOAJ
language	EN
topic	MODY molecular diagnosis genetic testing pharmacological treatment Specialties of internal medicine RC581-951
spellingShingle	MODY molecular diagnosis genetic testing pharmacological treatment Specialties of internal medicine RC581-951 Urakami T Maturity-onset diabetes of the young (MODY): current perspectives on diagnosis and treatment
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
format	article
author	Urakami T
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
publishDate	2019
url	https://doaj.org/article/acb9bb5fe34f40b5be29d8425ccd4c11
work_keys_str_mv	AT urakamit maturityonsetdiabetesoftheyoungmodycurrentperspectivesondiagnosisandtreatment
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Maturity-onset diabetes of the young (MODY): current perspectives on diagnosis and treatment

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