Monogenetisk diabetes

Mathilde Svendstrup; Johanne Skov Jensen; Anne Cathrine Baun Thuesen; Henrik Maagensen; Torben Hansen; Julie Støy; Tina Vilsbøll

doi:10.61409/V10250809

Monogenetisk diabetes

Mathilde Svendstrup
, Johanne Skov Jensen
, Anne Cathrine Baun Thuesen
, Henrik Maagensen
, Torben Hansen
, Julie Støy^*
, Tina Vilsbøll

^*Corresponding author af dette arbejde

Steno Diabetes Center Copenhagen

Abstract

Monogenic diabetes covers single-variant inherited subtypes of diabetes, of which the most prevalent are HNF4A-MODY (MODY1), GCK-MODY (MODY2), HNF1A-MODY (MODY3), HNF1B-MODY (MODY5) and mitochondrial diabetes. Together, they account for about 1% of diabetes cases, but more than 70% of affected individuals are classified with other forms of diabetes. The classical phenotype is young-onset, non-autoimmune diabetes with partially preserved insulin secretion and a family history of diabetes. Correct genetic diagnosis is crucial for family counselling and optimal diabetes management, as reviewed in this article.

Bidragets oversatte titel	Osteodystrophy in chronic liver disease
Originalsprog	Dansk
Artikelnummer	V10250809
Tidsskrift	Ugeskrift for Laeger
Vol/bind	188
Udgave nummer	26
ISSN	0041-5782
DOI	https://doi.org/10.61409/V10250809
Status	Udgivet - 22 jun. 2026

Emneord

Humans
Diabetes Mellitus, Type 2/genetics
Liver Diseases/complications
Phenotype
Osteochondrodysplasias/genetics
Chronic Disease

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10.61409/V10250809Licens: CC BY-NC-ND

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Citationsformater

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title = "Monogenetisk diabetes",

abstract = "Monogenic diabetes covers single-variant inherited subtypes of diabetes, of which the most prevalent are HNF4A-MODY (MODY1), GCK-MODY (MODY2), HNF1A-MODY (MODY3), HNF1B-MODY (MODY5) and mitochondrial diabetes. Together, they account for about 1\% of diabetes cases, but more than 70\% of affected individuals are classified with other forms of diabetes. The classical phenotype is young-onset, non-autoimmune diabetes with partially preserved insulin secretion and a family history of diabetes. Correct genetic diagnosis is crucial for family counselling and optimal diabetes management, as reviewed in this article.",

keywords = "Humans, Diabetes Mellitus, Type 2/genetics, Liver Diseases/complications, Phenotype, Osteochondrodysplasias/genetics, Chronic Disease",

author = "Mathilde Svendstrup and Jensen, \{Johanne Skov\} and Thuesen, \{Anne Cathrine Baun\} and Henrik Maagensen and Torben Hansen and Julie St{\o}y and Tina Vilsb{\o}ll",

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year = "2026",

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doi = "10.61409/V10250809",

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T1 - Monogenetisk diabetes

AU - Svendstrup, Mathilde

AU - Jensen, Johanne Skov

AU - Thuesen, Anne Cathrine Baun

AU - Maagensen, Henrik

AU - Hansen, Torben

AU - Støy, Julie

AU - Vilsbøll, Tina

N1 - Published under Open Access CC-BY-NC-ND 4.0. https://creativecommons.org/licenses/by-nc-nd/4.0/.

PY - 2026/6/22

Y1 - 2026/6/22

N2 - Monogenic diabetes covers single-variant inherited subtypes of diabetes, of which the most prevalent are HNF4A-MODY (MODY1), GCK-MODY (MODY2), HNF1A-MODY (MODY3), HNF1B-MODY (MODY5) and mitochondrial diabetes. Together, they account for about 1% of diabetes cases, but more than 70% of affected individuals are classified with other forms of diabetes. The classical phenotype is young-onset, non-autoimmune diabetes with partially preserved insulin secretion and a family history of diabetes. Correct genetic diagnosis is crucial for family counselling and optimal diabetes management, as reviewed in this article.

AB - Monogenic diabetes covers single-variant inherited subtypes of diabetes, of which the most prevalent are HNF4A-MODY (MODY1), GCK-MODY (MODY2), HNF1A-MODY (MODY3), HNF1B-MODY (MODY5) and mitochondrial diabetes. Together, they account for about 1% of diabetes cases, but more than 70% of affected individuals are classified with other forms of diabetes. The classical phenotype is young-onset, non-autoimmune diabetes with partially preserved insulin secretion and a family history of diabetes. Correct genetic diagnosis is crucial for family counselling and optimal diabetes management, as reviewed in this article.

KW - Humans

KW - Diabetes Mellitus, Type 2/genetics

KW - Liver Diseases/complications

KW - Phenotype

KW - Osteochondrodysplasias/genetics

KW - Chronic Disease

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DO - 10.61409/V10250809

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SN - 0041-5782

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JO - Ugeskrift for Laeger

JF - Ugeskrift for Laeger

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Monogenetisk diabetes

Abstract

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