Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Diabetes. Jan 15, 2024; 15(1): 15-23
Published online Jan 15, 2024. doi: 10.4239/wjd.v15.i1.15
Management of monogenic diabetes in pregnancy: A narrative review
Mohammad Sadiq Jeeyavudeen, Sarah R Murray, Mark W J Strachan
Mohammad Sadiq Jeeyavudeen, Mark W J Strachan, Metabolic Unit, Western General Hospital, Edinburgh EH4 2XU, United Kingdom
Sarah R Murray, MRC Centre for Reproductive Health, University of Edinburgh Queen’s Medical Research Institute, Edinburgh EH16 4TJ, United Kingdom
Author contributions: Jeeyavudeen MS performed the literature search, interpreted the relevant literature and drafted the initial manuscript, conceived the idea and designed the paper, prepared the figures and supervised the revision of the article critically for important intellectual content; Strachan MWJ and Murray SR contributed to the manuscript drafting and the idea of this manuscript; and all authors have read and approved the final version of the manuscript.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Mohammad Sadiq Jeeyavudeen, MD, Doctor, Metabolic Unit, Western General Hospital, Crewe Road South, Edinburgh EH4 2XU, United Kingdom.
Received: August 29, 2023
Peer-review started: August 29, 2023
First decision: October 24, 2023
Revised: November 13, 2023
Accepted: December 8, 2023
Article in press: December 8, 2023
Published online: January 15, 2024

Pregnancy in women with monogenic diabetes is potentially complex, with significant implications for both maternal and fetal health. Among these, maturity-onset diabetes of the young (MODY) stands out as a prevalent monogenic diabetes subtype frequently encountered in clinical practice. Each subtype of MODY requires a distinct approach tailored to the pregnancy, diverging from management strategies in non-pregnant individuals. Glucokinase MODY (GCK-MODY) typically does not require treatment outside of pregnancy, but special considerations arise when a woman with GCK-MODY becomes pregnant. The glycemic targets in GCK-MODY pregnancies are not exclusively dictated by the maternal/paternal MODY genotype but are also influenced by the genotype of the developing fetus. During pregnancy, the choice between sulfonylurea or insulin for treating hepatocyte nuclear factor 1-alpha (HNF1A)-MODY and HNF4A-MODY depends on the mother’s specific circumstances and the available expertise. Management of other rarer MODY subtypes is individualized, with decisions made on a case-by-case basis. Therefore, a collaborative approach involving expert diabetes and obstetric teams is crucial for the comprehensive management of MODY pregnancies.

Keywords: Diabetes, Pregnancy, Maturity-onset diabetes of the young, Insulin, Sulphonylurea, Glucokinase, Hepatocyte nuclear factor 1-alpha, hepatocyte nuclear factor 1-beta, and hepatocyte nuclear factor 4-alpha

Core Tip: Management of monogenic diabetes in pregnancy, particularly maturity-onset diabetes of the young (MODY), requires tailored approaches due to the unique challenges encountered in pregnancy. While glucokinase MODY often doesn’t require treatment outside pregnancy, managing it during pregnancy is complex due to its impact on fetal growth. Monitoring fetal genotype and growth patterns is essential for adjusting treatment. Non-invasive methods for fetal genotype determination, such as cell-free DNA analysis, hold promise but require further research.