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Management and Outcomes of Maturity-Onset Diabetes of the Young in Pregnancy

Published:August 02, 2019DOI:https://doi.org/10.1016/j.jcjd.2019.07.004

      Abstract

      Maturity-onset diabetes of the young (MODY) is a group of monogenic disorders that accounts for 1% to 5% of diabetes. The most common mutations are those in the hepatocyte nuclear factor-1-alpha (HNF-1-alpha) and in the glucokinase (GCK) genes. Although management of MODY is well established, no guidelines currently exist for management during pregnancy. Both maternal glycemic control and fetal mutation status are factors that may influence outcomes during pregnancy. The primary aim of this project was to describe cases of MODY during pregnancy to highlight the clinical implications of management of this disorder during pregnancy. The Ottawa Hospital is the primary referral centre for high-risk obstetrical patients, including those with diabetes in pregnancy, in Ottawa, Canada. Referrals between 2008 and 2018 were reviewed and a case series of three women and five pregnancies is described. Together with the illustrative cases, a literature review of MODY in pregnancy is used to highlight clinical considerations unique to MODY in pregnancy. We describe 5 pregnancies with MODY-2 (GCK mutation) and MODY 3 (HNF-1-alpha mutation). Important issues identified included monitoring of fetal growth and individualization of maternal glycemic control, particularly in cases where fetal mutation status is unknown. Management of MODY in pregnancy is challenging and there is little evidence to guide recommendations. Fetal growth can be used to guide management of maternal glycemic targets when fetal mutation status is unknown.

      Résumé

      Le diabète de la maturité apparaissant chez le jeune (MODY) constitue un groupe d'anomalies monogéniques qui représente de 1 % à 5 % des types de diabète. Les mutations les plus fréquentes sont celles des gènes codant pour le hépatocyte nucléaire facteur 1-alpha (HNF-1-alpha) et pour la glucokinase (GCK). Bien que la prise en charge du MODY soit bien établie, il n'existe actuellement aucune ligne directrice sur sa prise en charge durant la grossesse. La régulation de la glycémie maternelle et le statut mutationnel du fœtus sont des facteurs qui peuvent influencer les issues de la grossesse. L'objectif principal de la présente étude était de décrire les cas de MODY durant la grossesse pour démontrer les conséquences cliniques de la prise en charge de cette anomalie durant la grossesse. L’Hôpital d’Ottawa est le principal centre, à Ottawa, au Canada, qui offre des services de consultation en obstétrique aux patientes exposées à un risque élevé, y compris aux patientes diabétiques durant leur grossesse. Nous avons passé en revue les consultations entre 2008 et 2018, et nous avons décrit des séries de cas de 3 femmes et de 5 grossesses. Conjointement aux cas cités en exemple, une revue de littérature du MODY durant la grossesse est utilisée pour faire valoir les considérations cliniques propres au MODY durant la grossesse. Nous décrivons 5 grossesses de femmes ayant le MODY-2 (mutation codant pour la GCK) et le MODY-3 (mutation codant pour le HNF-1-alpha). Les points importants sont la surveillance de la croissance fœtale et l'individualisation de la régulation de la glycémie maternelle, notamment dans les cas où l'on ignore le statut mutationnel du fœtus. La prise en charge du MODY durant la grossesse est difficile. Il existe peu de données probantes pour mener à des recommandations. La croissance fœtale peut être utilisée pour orienter la prise en charge des valeurs cibles de la glycémie maternelle lorsqu'on ignore l’état mutationnel du fœtus.

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      Mots clés

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