Clinical Management of Women with Monogenic Diabetes During Pregnancy

Diabetes and Pregnancy (M-F Hivert and CE Powe, Section Editors)
Part of the following topical collections:
  1. Topical Collection on Diabetes and Pregnancy


Purpose of Review

Monogenic diabetes accounts for 1–2% of all diabetes cases, but is frequently misdiagnosed as type 1, type 2, or gestational diabetes. Accurate genetic diagnosis directs management, such as no pharmacologic treatment for GCK-MODY, low-dose sulfonylureas for HNF1A-MODY and HNF4A-MODY, and high-dose sulfonylureas for KATP channel-related diabetes. While diabetes treatment is defined for the most common causes of monogenic diabetes, pregnancy poses a challenge to management. Here, we discuss the key issues in pregnancy affected by monogenic diabetes.

Recent Findings

General recommendations for pregnancy affected by GCK-MODY determine need for maternal insulin treatment based on fetal mutation status. However, a recent study suggests macrosomia and miscarriage rates may be increased with this strategy. Recent demonstration of transplacental transfer of sulfonylureas also raises questions as to when insulin should be initiated in sulfonylurea-responsive forms of monogenic diabetes.


Pregnancy represents a challenge in management of monogenic diabetes, where factors of maternal glycemic control, fetal mutation status, and transplacental transfer of medication must all be taken into consideration. Guidelines for pregnancy affected by monogenic diabetes will benefit from large, prospective studies to better define the need for and timing of initiation of insulin treatment.


Monogenic diabetes MODY Glucokinase gene mutation Hepatocyte nuclear factor-1A Pregnancy 


Funding information

This work was supported by the National Institute of Diabetes and Digestive and Kidney Diseases of the National Institutes of Health (grant numbers R01 DK104942, P30 DK020595) and the CTSA (grant number UL1 TR002389).

Compliance with Ethical Standards

Conflict of Interest

Laura T. Dickens and Rochelle N. Naylor declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article contains unpublished data from retrospective studies with human subjects performed by Laura T. Dickens and Rochelle N. Naylor. Informed consent was obtained from all subjects.


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Copyright information

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

Authors and Affiliations

  1. 1.Department of Medicine, Section of Adult and Pediatric Endocrinology, Diabetes, and MetabolismUniversity of ChicagoChicagoUSA
  2. 2.Department of Pediatrics, Section of Adult and Pediatric Endocrinology, Diabetes, and MetabolismUniversity of ChicagoChicagoUSA

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