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Multicenter Study
. 2024 Nov;16(11):e70028.
doi: 10.1111/1753-0407.70028.

Clinical characteristics, treatment, and treatment switch after molecular-genetic classification in individuals with maturity-onset diabetes of the young: Insights from the multicenter real-world DPV registry

Stefanie Lanzinger  1   2 , Katharina Laubner  3 , Katharina Warncke  4 , Julia K Mader  5 , Sebastian Kummer  6 , Claudia Boettcher  7 , Torben Biester  8 , Angela Galler  9 , Daniela Klose  10 , Reinhard W Holl  1   2
Affiliations
Multicenter Study

Clinical characteristics, treatment, and treatment switch after molecular-genetic classification in individuals with maturity-onset diabetes of the young: Insights from the multicenter real-world DPV registry

Stefanie Lanzinger et al. J Diabetes. 2024 Nov.

Abstract

Background: Individuals with maturity-onset diabetes of the young (MODY) are often misdiagnosed as type 1 or type 2 diabetes and receive inappropriate care. We aimed to investigate the characteristics and treatment of all MODY types in a multicenter, real-world setting.

Methods: Individuals with MODY from the diabetes prospective follow-up (DPV) registry were studied. We compared clinical parameters during the first year of diabetes and the most recent treatment year after MODY diagnosis.

Results: A total of 1640 individuals were identified with GCK-MODY (n = 941) and HNF1A-MODY (n = 417) as the most frequent types. Among these, 912 individuals were available with information during the first and the most recent treatment year (median duration of follow-up: 4.2 years [2.6-6.6]). Positive beta cell autoantibodies were present in 20.6% (15.2% IAA). Median age at diagnosis ranged from 9.9 years in GCK-MODY (Q1-Q3: 6.2-13.1 years) and INS-MODY (2.7-13.7 years) to 14.3 years (5.0-17.1) in KCNJ11-MODY. Frequency of oral antidiabetic agents (OAD) use increased and insulin decreased in HNF4A-MODY (OAD: 18% to 39%, insulin: 34% to 23%) and in HNF1A-MODY (OAD: 18% to 31%, insulin: 35% to 25%). ABCC8-MODY was characterized by a decrement in nonpharmacological treatment (26% to 16%) and "insulin only" treatment (53% to 42%), while the proportion of individuals treated with OAD but no insulin increased from 0% to 21%.

Conclusions: Our results indicate that some teams caring for individuals with MODY are hesitant with regard to current recommendations. Registries are an essential source of information and provide a basis for discussing treatment guidelines for MODY.

Keywords: MODY; diabetes prospective follow‐up (DPV) registry; monogenic diabetes; oral antidiabetic drugs; real‐world data.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

FIGURE 1
FIGURE 1
Temporal trend in the proportion of individuals classified with “other specific types of diabetes” (solid line) and MODY (dashed line) by year of diabetes diagnosis. MODY, maturity‐onset diabetes of the young.
FIGURE 2
FIGURE 2
Sankey plots showing changes in treatment from first year after diagnosis to the most recent year in (A) HNF4A‐MODY, (B) GCK‐MODY, (C) HNF1A‐MODY, (D) HNF1B‐MODY, and (E) ABCC8‐MODY. MODY, maturity‐onset diabetes of the young.
See this image and copyright information in PMC

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