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. 2022 Dec;23(8):1188-1211.
doi: 10.1111/pedi.13426.

ISPAD Clinical Practice Consensus Guidelines 2022: The diagnosis and management of monogenic diabetes in children and adolescents

Siri Atma W Greeley  1 , Michel Polak  2 , Pål R Njølstad  3 , Fabrizio Barbetti  4 , Rachel Williams  5 , Luis Castano  6 , Klemens Raile  7 , Dung Vu Chi  8   9 , Abdelhadi Habeb  10 , Andrew T Hattersley  11 , Ethel Codner  12
Affiliations

ISPAD Clinical Practice Consensus Guidelines 2022: The diagnosis and management of monogenic diabetes in children and adolescents

Siri Atma W Greeley et al. Pediatr Diabetes. 2022 Dec.
No abstract available

Keywords: MODY; diabetes mellitus classification; genetics; monogenic; neonatal diabetes.

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Conflict of interest statement

Dr Michel Polak MD, PhD has acted as scientific advisor for the development of the glibenclamide‐glyburide suspension named AMGLIDIA in the European Union. The other authors have declared no conflicts of interest.

Figures

FIGURE 1
FIGURE 1
Insulin secretion from the pancreatic beta cell in (A) normal cell in a high plasma glucose environment and (B) in a cell with a K‐ATP channel mutation. Source: adapted from Reference . (A) Glucose enters the cell and is metabolized, causing an increase in ATP, K‐ATP channel closure is induced via ATP binding, the membrane is depolarized, and calcium influx is triggered resulting in the release of insulin from its storage vesicles. (B) A gain of function mutation in the K‐ATP channel results in the failure of ATP to bind to the channel, causing the channel to remain open, the membrane stays hyperpolarized and no insulin is released
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