Current Diabetes Reports

, 18:58 | Cite as

Monogenic Diabetes in Children and Adolescents: Recognition and Treatment Options

  • May Sanyoura
  • Louis H. Philipson
  • Rochelle Naylor
Pediatric Type 2 and Monogenic Diabetes (PS Zeitler and O Pinhas-Hamiel, Section Editors)
Part of the following topical collections:
  1. Topical Collection on Pediatric Type 2 and Monogenic Diabetes


Purpose of Review

We provide a review of monogenic diabetes in young children and adolescents with a focus on recognition, management, and pharmacological treatment.

Recent Findings

Monogenic forms of diabetes account for approximately 1–2% of diabetes in children and adolescents, and its incidence has increased in recent years due to greater awareness and wider availability of genetic testing. Monogenic diabetes is due to single gene defects that primarily affect beta cell function with more than 30 different genes reported. Children with antibody-negative, C-peptide-positive diabetes should be evaluated and genetically tested for monogenic diabetes. Accurate genetic diagnosis impacts treatment in the most common types of monogenic diabetes, including the use of sulfonylureas in place of insulin or other glucose-lowering agents or discontinuing pharmacologic treatment altogether.


Diagnosis of monogenic diabetes can significantly improve patient care by enabling prediction of the disease course and guiding appropriate management and treatment.


Monogenic diabetes Maturity-onset diabetes of the young Syndromic diabetes Type 1 diabetes Type 2 diabetes Genetic testing 


Compliance with Ethical Standards

Conflict of Interest

May Sanyoura, Louis H. Philipson, and Rochelle Naylor declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major Importance

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • May Sanyoura
    • 1
  • Louis H. Philipson
    • 1
  • Rochelle Naylor
    • 1
  1. 1.Section of Adult and Pediatric Endocrinology, Diabetes, and MetabolismThe University of ChicagoChicagoUSA

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