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

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.