Generic placeholder image

Current Diabetes Reviews


ISSN (Print): 1573-3998
ISSN (Online): 1875-6417

Review Article

Maturity-onset Diabetes of the Young (MODY) in Pregnancy: A Review

Author(s): Agata Majewska*, Paweł Stanirowski, Mirosław Wielgoś and Dorota Bomba-Opoń

Volume 19, Issue 1, 2023

Published on: 06 April, 2022

Article ID: e280122200657 Pages: 5

DOI: 10.2174/1573399818666220128124043

Price: $65


Hyperglycaemia in pregnancy is one of the most common complications of pregnancy and is generally diagnosed as gestational diabetes mellitus (GDM). Nevertheless, clinical symptoms of hyperglycaemia in pregnancy in some cases do not match the clinical manifestations of GDM. It is suspected that 1-2 % of women diagnosed with GDM are misdiagnosed maturity-onset diabetes of the young (MODY). MODY often has a subclinical course; thus, it is challenging for clinicians to aptly diagnose monogenic diabetes in pregnancy. Proper diagnosis is crucial for the effective treatment of hyperglycaemia in pregnancy. Many studies revealed that misdiagnosis of MODY increases the rate of complications for both mother and fetus. This literature review reports the current knowledge regarding diagnosis, treatment, and complications of the most common types of MODY in pregnancy.

Keywords: Maturity-onset diabetes of the young, gestational diabetes mellitus, monogenic diabetes, hyperglycaemia in pregnancy, GCK-MODY, HNF1A-MODY.

Murphy R. Monogenic diabetes and pregnancy. Obstet Med 2015; 8(3): 114-20.
[] [PMID: 27512465]
Spyer G, Macleod KM, Shepherd M, Ellard S, Hattersley AT. Pregnancy outcome in patients with raised blood glucose due to a heterozygous glucokinase gene mutation. Diabet Med 2009; 26(1): 14-8.
[] [PMID: 19125755]
Gjesing AP, Rui G, Lauenborg J, et al. High prevalence of diabetes-predisposing variants in MODY genes among danish women with gestational diabetes mellitus. J Endocr Soc 2017; 1(6): 681-90.
[] [PMID: 29264522]
Aarthy R, Aston-Mourney K, Mikocka-Walus A, et al. Clinical features, complications and treatment of rarer forms of maturity-onset diabetes of the young (MODY) - A review. J Diabetes Complications 2021; 35(1): 107640.
[] [PMID: 32763092]
Rudland VL. Diagnosis and management of glucokinase monogenic diabetes in pregnancy: Current perspectives. Diabetes Metab Syndr Obes 2019; 12: 1081-9.
[] [PMID: 31372018]
Rudland VL, Hinchcliffe M, Pinner J, et al. Identifying glucokinase monogenic diabetes in a multiethnic gestational diabetes mellitus cohort: New pregnancy screening criteria and utility of HbA1c. Diabetes Care 2016; 39(1): 50-2.
[] [PMID: 26109503]
Monsonego S, Clark H, Karovitch A, O’Meara P, Shaw T, Malcolm J. Management and outcomes of maturity-onset diabetes of the young in pregnancy. Can J Diabetes 2019; 43(8): 647-54.
[] [PMID: 31564623]
Rengaraj S, Thiyagalingam S, Kathirvel V, Delhikumar CG. Raised blood glucose due to heterozygous glucokinase gene mutation (GCK-MODY) diagnosed for the first time in pregnancy: The dilemmas and successful management - Case report and review of literature. Obstet Med 2021; 14(1): 53-6.
[] [PMID: 33995576]
Chakera AJ, Spyer G, Vincent N, Ellard S, Hattersley AT, Dunne FP. The 0.1% of the population with glucokinase monogenic diabetes can be recognized by clinical characteristics in pregnancy: The atlantic diabetes in pregnancy cohort. Diabetes Care 2014; 37(5): 1230-6.
[] [PMID: 24550216]
Dickens LT, Naylor RN. Clinical management of women with monogenic diabetes during pregnancy. Curr Diab Rep 2018; 18(3): 12.
[] [PMID: 29450745]
Kopacz-Petranyuk K, Brandt-Varma A, Buraczewska M, et al. Neonatal outcome and diabetes course in children with GCK-MODY born from women with GCK-MODY. Pediatr Endocrinol Diabetes Metab 2018; 24(4): 167-73.
[] [PMID: 30963753]
Freathy RM, Hayes MG, Urbanek M, et al. Hyperglycemia and adverse pregnancy outcome (HAPO) study: Common genetic variants in GCK and TCF7L2 are associated with fasting and postchallenge glucose levels in pregnancy and with the new consensus definition of gestational diabetes mellitus from the international association of diabetes and pregnancy study groups. Diabetes 2010; 59(10): 2682-9.
[] [PMID: 20682688]
Hattersley AT, Beards F, Ballantyne E, Appleton M, Harvey R, Ellard S. Mutations in the glucokinase gene of the fetus result in reduced birth weight. Nat Genet 1998; 19(3): 268-70.
[] [PMID: 9662401]
Colclough K, Bellanne-Chantelot C, Saint-Martin C, Flanagan SE, Ellard S. Mutations in the genes encoding the transcription factors hepatocyte nuclear factor 1 alpha and 4 alpha in maturity-onset diabetes of the young and hyperinsulinemic hypoglycemia. Hum Mutat 2013; 34(5): 669-85.
[] [PMID: 23348805]
Shaat N, Groop L. Genetics of gestational diabetes mellitus. Curr Med Chem 2007; 14(5): 569-83.
[] [PMID: 17346148]
Bacon S, Schmid J, McCarthy A, et al. The clinical management of hyperglycemia in pregnancy complicated by maturity-onset diabetes of the young. Am J Obstet Gynecol 2015; 213(2): 236-7.
Stanescu DE, Hughes N, Kaplan B, Stanley CA, De León DD. Novel presentations of congenital hyperinsulinism due to mutations in the MODY genes: HNF1A and HNF4A. J Clin Endocrinol Metab 2012; 97(10): E2026-30.
[] [PMID: 22802087]
Rochon M, Rand L, Roth L, Gaddipati S. Glyburide for the management of gestational diabetes: Risk factors predictive of failure and associated pregnancy outcomes. Am J Obstet Gynecol 2006; 195(4): 1090-4.
[] [PMID: 17000241]
Poolsup N, Suksomboon N, Amin M. Efficacy and safety of oral antidiabetic drugs in comparison to insulin in treating gestational diabetes mellitus: A meta-analysis. PLoS One 2014; 9(10): e109985.
[] [PMID: 25302493]
Delvecchio M, Pastore C, Giordano P. Treatment options for MODY patients: A systematic review of literature. Diabetes Ther 2020; 11(8): 1667-85.
[] [PMID: 32583173]
Bitterman O, Iafusco D, Torcia F, Tinto N, Napoli A. A dizygotic twin pregnancy in a MODY 3-affected woman. Acta Diabetol 2016; 53(5): 849-52.
[] [PMID: 26997508]
Klupa T, Warram JH, Antonellis A, et al. Determinants of the development of diabetes (maturity-onset diabetes of the young-3) in carriers of HNF-1alpha mutations: Evidence for parent-of-origin effect. Diabetes Care 2002; 25(12): 2292-301.
[] [PMID: 12453976]
Stride A, Shepherd M, Frayling TM, Bulman MP, Ellard S, Hattersley AT. Intrauterine hyperglycemia is associated with an earlier diagnosis of diabetes in HNF-1alpha gene mutation carriers. Diabetes Care 2002; 25(12): 2287-91.
[] [PMID: 12453975]
Edghill EL, Bingham C, Slingerland AS, et al. Hepatocyte nuclear factor-1 beta mutations cause neonatal diabetes and intrauterine growth retardation: Support for a critical role of HNF-1beta in human pancreatic development. Diabet Med 2006; 23(12): 1301-6.
[] [PMID: 17116179]
Tarnowski M, Malinowski D, Safranow K, Dziedziejko V, Pawlik A. HNF1B, TSPAN8 and NOTCH2 gene polymorphisms in women with gestational diabetes. J Matern Fetal Neonatal Med 2018; 31(7): 837-42.
[] [PMID: 28274157]
Pearson ER, Boj SF, Steele AM, et al. Macrosomia and hyperinsulinaemic hypoglycaemia in patients with heterozygous mutations in the HNF4A gene. PLoS Med 2007; 4(4): e118.
[] [PMID: 17407387]
Shepherd M, Brook AJ, Chakera AJ, Hattersley AT. Management of sulfonylurea-treated monogenic diabetes in pregnancy: Implications of placental glibenclamide transfer. Diabet Med 2017; 34(10): 1332-9.
[] [PMID: 28556992]
Kapoor RR, Locke J, Colclough K, et al. Persistent hyperinsulinemic hypoglycemia and maturity-onset diabetes of the young due to heterozygous HNF4A mutations. Diabetes 2008; 57(6): 1659-63.
[] [PMID: 18268044]
Colom C, Corcoy R. Maturity onset diabetes of the young and pregnancy. Best Pract Res Clin Endocrinol Metab 2010; 24(4): 605-15.
[] [PMID: 20832739]
Gragnoli C, Stanojevic V, Gorini A, Von Preussenthal GM, Thomas MK, Habener JF. IPF-1/MODY4 gene missense mutation in an Italian family with type 2 and gestational diabetes. Metabolism 2005; 54(8): 983-8.
[] [PMID: 16092045]

Rights & Permissions Print Export Cite as
© 2024 Bentham Science Publishers | Privacy Policy