Coexistence of Maturity-Onset Diabetes of the Young Type 3 (MODY 3) and Diabetic Ketoacidosis: A Report of a Rare Case

Maturity-onset diabetes of the young (MODY) is a monogenic form of diabetes often misclassified as type 1 or type 2 diabetes. We report a young woman initially diagnosed with type 1 diabetes following gestational hyperglycaemia. Autoimmune testing was negative, and genetic analysis confirmed an <i>HNF1A</i> mutation consistent with maturity-onset diabetes of the young type 3 (MODY 3). Despite sulfonylurea therapy, she required insulin and experienced recurrent diabetic ketoacidosis (DKA) due to poor adherence. Improved engagement and continuous glucose monitoring led to marked glycaemic improvement. This case highlights the diagnostic challenge of MODY presenting with DKA, a rare occurrence in this condition. Early genetic testing is essential for accurate diagnosis, personalised management, and family counselling.

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Journal
Cureus
Published
2025-11-08
DOI
https://doi.org/10.7759/cureus.96360
Primary Topic
Diabetes and associated disorders
Type
article
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article

Coexistence of Maturity-Onset Diabetes of the Young Type 3 (MODY 3) and Diabetic Ketoacidosis: A Report of a Rare Case

DU Ekwueme, Mohammad Mustafizur Rahman, Kyi Min Sann
Cureus
Diabetes and associated disorders
article

Coexistence of Maturity-Onset Diabetes of the Young Type 3 (MODY 3) and Diabetic Ketoacidosis: A Report of a Rare Case

DU Ekwueme, Mohammad Mustafizur Rahman, Kyi Min Sann
article en

Abstract

Maturity-onset diabetes of the young (MODY) is a monogenic form of diabetes often misclassified as type 1 or type 2 diabetes. We report a young woman initially diagnosed with type 1 diabetes following gestational hyperglycaemia. Autoimmune testing was negative, and genetic analysis confirmed an <i>HNF1A</i> mutation consistent with maturity-onset diabetes of the young type 3 (MODY 3). Despite sulfonylurea therapy, she required insulin and experienced recurrent diabetic ketoacidosis (DKA) due to poor adherence. Improved engagement and continuous glucose monitoring led to marked glycaemic improvement. This case highlights the diagnostic challenge of MODY presenting with DKA, a rare occurrence in this condition. Early genetic testing is essential for accurate diagnosis, personalised management, and family counselling.

CureusVol. 17(11)
Wrexham Maelor Hospital (GB)
Openalex Percentile: Top 65%
Diabetes and associated disorders
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