Skip to main content

Long-term Follow-up of Glycemic and Neurological Outcomes in an International Series of Patients With Sulfonylurea-Treated Permanent Neonatal Diabetes.

Citation
Bowman, P., et al. “Long-Term Follow-Up Of Glycemic And Neurological Outcomes In An International Series Of Patients With Sulfonylurea-Treated Permanent Neonatal Diabetes.”. Diabetes Care, pp. 35-42.
Center University of Chicago
Author Pamela Bowman, Frances Mathews, Fabrizio Barbetti, Maggie H Shepherd, Janine Sanchez, Barbara Piccini, Jacques Beltrand, Lisa R Letourneau-Freiberg, Michel Polak, Siri Atma W Greeley, Eamon Rawlins, Tarig Babiker, Nicholas J Thomas, Elisa De Franco, Sian Ellard, Sarah E Flanagan, Andrew T Hattersley, Neonatal Diabetes International Collaborative Group
Abstract

OBJECTIVE: mutations cause neonatal diabetes mellitus that can be transient (TNDM) or, less commonly, permanent (PNDM); ∼90% of individuals can be treated with oral sulfonylureas instead of insulin. Previous studies suggested that people with PNDM require lower sulfonylurea doses and have milder neurological features than those with PNDM. However, these studies were short-term and included combinations of -PNDM and -TNDM. We aimed to assess the long-term glycemic and neurological outcomes in sulfonylurea-treated -PNDM.

RESEARCH DESIGN AND METHODS: We studied all 24 individuals with PNDM diagnosed in the U.K., Italy, France, and U.S. known to transfer from insulin to sulfonylureas before May 2010. Data on glycemic control, sulfonylurea dose, adverse effects including hypoglycemia, and neurological features were analyzed using nonparametric statistical methods.

RESULTS: Long-term data were obtained for 21 of 24 individuals (median follow-up 10.0 [range 4.1-13.2] years). Eighteen of 21 remained on sulfonylureas without insulin at the most recent follow-up. Glycemic control improved on sulfonylureas (presulfonylurea vs. 1-year posttransfer HbA 7.2% vs. 5.7%, = 0.0004) and remained excellent long-term (1-year vs. 10-year HbA 5.7% vs. 6.5%, = 0.04), = 16. Relatively high doses were used (1-year vs. 10-year dose 0.37 vs. 0.25 mg/kg/day glyburide, = 0.50) without any severe hypoglycemia. Neurological features were reported in 13 of 21 individuals; these improved following sulfonylurea transfer in 7 of 13. The most common features were learning difficulties (52%), developmental delay (48%), and attention deficit hyperactivity disorder (38%).

CONCLUSIONS: Sulfonylurea treatment of -PNDM results in excellent long-term glycemic control. Overt neurological features frequently occur and may improve with sulfonylureas, supporting early, rapid genetic testing to guide appropriate treatment and neurodevelopmental assessment.

Year of Publication
2021
Journal
Diabetes care
Volume
44
Issue
1
Number of Pages
35-42
Date Published
01/2021
ISSN Number
1935-5548
DOI
10.2337/dc20-1520
Alternate Journal
Diabetes Care
PMID
33184150
PMCID
PMC7783935
Download citation