Cargando…
Maturity-onset diabetes of the young (MODY): current perspectives on diagnosis and treatment
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...
Autor principal: | |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Dove
2019
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6625604/ https://www.ncbi.nlm.nih.gov/pubmed/31360071 http://dx.doi.org/10.2147/DMSO.S179793 |
_version_ | 1783434446556364800 |
---|---|
author | Urakami, Tatsuhiko |
author_facet | Urakami, Tatsuhiko |
author_sort | Urakami, Tatsuhiko |
collection | PubMed |
description | 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). |
format | Online Article Text |
id | pubmed-6625604 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Dove |
record_format | MEDLINE/PubMed |
spelling | pubmed-66256042019-07-29 Maturity-onset diabetes of the young (MODY): current perspectives on diagnosis and treatment Urakami, Tatsuhiko Diabetes Metab Syndr Obes Review 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). Dove 2019-07-08 /pmc/articles/PMC6625604/ /pubmed/31360071 http://dx.doi.org/10.2147/DMSO.S179793 Text en © 2019 Urakami. http://creativecommons.org/licenses/by-nc/3.0/ This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (https://www.dovepress.com/terms.php). |
spellingShingle | Review Urakami, Tatsuhiko Maturity-onset diabetes of the young (MODY): current perspectives on diagnosis and treatment |
title | 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_short | 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 |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6625604/ https://www.ncbi.nlm.nih.gov/pubmed/31360071 http://dx.doi.org/10.2147/DMSO.S179793 |
work_keys_str_mv | AT urakamitatsuhiko maturityonsetdiabetesoftheyoungmodycurrentperspectivesondiagnosisandtreatment |