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Erschienen in: Acta Diabetologica 4/2019

11.12.2018 | Original Article

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

verfasst von: Laura T. Dickens, Lisa R. Letourneau, May Sanyoura, Siri Atma W. Greeley, Louis H. Philipson, Rochelle N. Naylor

Erschienen in: Acta Diabetologica | Ausgabe 4/2019

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Abstract

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.
Literatur
1.
Zurück zum Zitat Fajans SS, Bell GI, Polonsky KS (2001) Molecular mechanisms and clinical pathophysiology of maturity-onset diabetes of the young. N Engl J Med 345(13):971–980CrossRefPubMed Fajans SS, Bell GI, Polonsky KS (2001) Molecular mechanisms and clinical pathophysiology of maturity-onset diabetes of the young. N Engl J Med 345(13):971–980CrossRefPubMed
5.
Zurück zum Zitat Spyer G, Hattersley AT, Sykes JE, Sturley RH, MacLeod KM (2001) Influence of maternal and fetal glucokinase mutations in gestational diabetes. Am J Obstet Gynecol 185(1):240–241CrossRefPubMed Spyer G, Hattersley AT, Sykes JE, Sturley RH, MacLeod KM (2001) Influence of maternal and fetal glucokinase mutations in gestational diabetes. Am J Obstet Gynecol 185(1):240–241CrossRefPubMed
9.
Zurück zum Zitat Greeley SA, Naylor RN, Cook LS, Tucker SE, Lipton RB, Philipson LH (2011) Creation of the Web-based University of Chicago Monogenic Diabetes Registry: using technology to facilitate longitudinal study of rare subtypes of diabetes. J Diabetes Sci Technol 5(4):879–886CrossRefPubMedPubMedCentral Greeley SA, Naylor RN, Cook LS, Tucker SE, Lipton RB, Philipson LH (2011) Creation of the Web-based University of Chicago Monogenic Diabetes Registry: using technology to facilitate longitudinal study of rare subtypes of diabetes. J Diabetes Sci Technol 5(4):879–886CrossRefPubMedPubMedCentral
11.
Zurück zum Zitat Paul A, Harris R, Taylor R et al (2009) Research electronic data capture (REDCap)—a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform 42(2):377–381CrossRef Paul A, Harris R, Taylor R et al (2009) Research electronic data capture (REDCap)—a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform 42(2):377–381CrossRef
16.
Zurück zum Zitat Hsu LYF (1998) Prenatal diagnosis of chromosomal abnormalities through amniocentesis. In: Milunsky A (ed) Genetic disorders and the fetus, 4th edn. The Johns Hopkins University Press, Baltimore, p 179 Hsu LYF (1998) Prenatal diagnosis of chromosomal abnormalities through amniocentesis. In: Milunsky A (ed) Genetic disorders and the fetus, 4th edn. The Johns Hopkins University Press, Baltimore, p 179
18.
Zurück zum Zitat Nielsen LR, Pedersen-Bjergaard U, Thorsteinsson B, Johansen M, Damm P, Mathiesen ER (2008) Hypoglycemia in pregnant women with type 1 diabetes: predictors and role of metabolic control. Diabetes Care 31(1):9–14 (Epub 2007 Oct 1) CrossRefPubMed Nielsen LR, Pedersen-Bjergaard U, Thorsteinsson B, Johansen M, Damm P, Mathiesen ER (2008) Hypoglycemia in pregnant women with type 1 diabetes: predictors and role of metabolic control. Diabetes Care 31(1):9–14 (Epub 2007 Oct 1) CrossRefPubMed
20.
Zurück zum Zitat Shields BM, Hicks S, Shepherd MH et al (2010) Maturity-onset diabetes of the young (MODY): how many cases are we missing? Diabetologia 53:2504–2508CrossRefPubMed Shields BM, Hicks S, Shepherd MH et al (2010) Maturity-onset diabetes of the young (MODY): how many cases are we missing? Diabetologia 53:2504–2508CrossRefPubMed
21.
Zurück zum Zitat Chakera AJ, Spyer G, Vincent N, Ellard S, Hattersley AT, Dunne FP (2014) 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 37(5):1230–1236. https://doi.org/10.2337/dc13-2248 CrossRefPubMed Chakera AJ, Spyer G, Vincent N, Ellard S, Hattersley AT, Dunne FP (2014) 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 37(5):1230–1236. https://​doi.​org/​10.​2337/​dc13-2248 CrossRefPubMed
Metadaten
Titel
Management and pregnancy outcomes of women with GCK-MODY enrolled in the US Monogenic Diabetes Registry
verfasst von
Laura T. Dickens
Lisa R. Letourneau
May Sanyoura
Siri Atma W. Greeley
Louis H. Philipson
Rochelle N. Naylor
Publikationsdatum
11.12.2018
Verlag
Springer Milan
Erschienen in
Acta Diabetologica / Ausgabe 4/2019
Print ISSN: 0940-5429
Elektronische ISSN: 1432-5233
DOI
https://doi.org/10.1007/s00592-018-1267-z

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