Management and pregnancy outcomes of women with GCK-MODY enrolled in the US Monogenic Diabetes Registry

• Published in: Acta diabetologica 2019-04, Vol.56 (4), p.405-411
• Main Authors: Dickens, Laura T., Letourneau, Lisa R., Sanyoura, May, Greeley, Siri Atma W., Philipson, Louis H., Naylor, Rochelle N.
• Format: Article
• Language: English
• Subjects: Abortion, Spontaneous - epidemiology; Adolescent; Adult; Birth weight; Birth Weight - drug effects; Births; Cross-Sectional Studies; Diabetes; Diabetes mellitus; Diabetes Mellitus, Type 2 - epidemiology; Diabetes Mellitus, Type 2 - genetics; Diabetes Mellitus, Type 2 - therapy; Female; Fetuses; Gestational age; Glucokinase - genetics; Humans; Hyperglycemia; Hyperglycemia - epidemiology; Hyperglycemia - genetics; Hyperglycemia - therapy; Hypoglycemia; Infant, Newborn; Infants; Insulin; Insulin - therapeutic use; Internal Medicine; Management; Medicine; Medicine & Public Health; Metabolic Diseases; Miscarriage; Mutation; Neonates; Original Article; Pregnancy; Pregnancy and diabetes; Pregnancy in Diabetics - epidemiology; Pregnancy in Diabetics - genetics; Pregnancy in Diabetics - therapy; Pregnancy Outcome - epidemiology; Premature birth; Registries; Treatment Outcome; Ultrasound; Young Adult
• ISSN: 0940-5429; 1432-5233
• DOI: 10.1007/s00592-018-1267-z

Aims GCK-MODY is characterized by mild hyperglycemia. Treatment is not required outside of pregnancy. During pregnancy, insulin treatment is recommended if second trimester fetal ultrasound monitoring shows macrosomia, suggesting the fetus has not inherited the GCK gene. There are limited data about GCK-MODY management in pregnancy. The aim of this study was to examine clinical management and pregnancy outcomes amongst women with a known diagnosis of GCK-MODY. Methods In this observational, cross-sectional study, a survey was distributed via Redcap to women ≥ 18 years enrolled in the University of Chicago Monogenic Diabetes Registry ( n  = 94). All or part of the survey was completed by 54 women (128 pregnancies). Results There were 78 term births (61%), 15 pre-term births (12%), and 24 miscarriages (19%). Of the 39 pregnancies where insulin was given, 22 (56%) had occasional or frequent hypoglycemia including 9 with severe hypoglycemia. Average birth weight for full-term GCK-affected infants was significantly less in cases of maternal insulin treatment versus no treatment (2967 and 3725 g, p  = 0.005). For GCK-unaffected infants, conclusions are limited by small sample size but large for gestational age (LGA) was common with maternal insulin treatment (56%) and no treatment (33%), p  = 0.590. Conclusions The observed miscarriage rate was comparable to the background US population rate (15–20%). Patients treated with insulin experienced a 23% incidence of severe hypoglycemia and lower birth weights were observed in the insulin-treated, GCK-affected neonates. These data support published guidelines of no treatment if the fetus is suspected to have inherited GCK-MODY and highlight the importance of additional studies to determine optimal pregnancy management for GCK-MODY, particularly among unaffected fetuses.