Remission of Severe Neonatal Diabetes With Very Early Sulfonylurea Treatment

Marshall, Bess A.; Green, Rebecca P.; Wambach, Jennifer A.; White, Neil H.; Remedi, Marı́a S.; Nichols, Colin G.

doi:10.2337/dc14-2124

Cited by 16 publications

(21 citation statements)

References 5 publications

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“…This is consistent with murine data indicating that early treatment with sulfonylureas preserves pancreatic β‐cells in KATP‐dependent diabetes . Very early sulfonylurea treatment may prevent progression to severe NDM and preserve sufficient glucose‐modulated insulin secretion to render the diabetes mild and reduce acute and long‐term complications . Therefore, treatment with glyburide very early in life enables good glycemic control with minimal dosing.…”

Section: Discussionsupporting

confidence: 88%

“…Moreover, it has been reported that early sulfonylurea treatment could ameliorate neurodevelopmental disabilities . An earlier age at the initiation of sulfonylurea treatment is associated with an improved response to sulfonylurea therapy and could also lead to a lower maintenance dose . Declining sensitivity to sulfonylurea with increasing age or duration of diabetes may be due to a loss of beta cell mass over time in those treated with insulin.…”

Section: Discussionmentioning

confidence: 99%

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Early transition from insulin to sulfonylureas in neonatal diabetes and follow-up: Experience from China

Sheng

et al. 2017

Pediatr Diabetes

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Section: Discussionsupporting

confidence: 88%

Section: Discussionmentioning

confidence: 99%

Early transition from insulin to sulfonylureas in neonatal diabetes and follow-up: Experience from China

Sheng

et al. 2017

Pediatr Diabetes

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“…In such patients, β‐cell mass is not initially lost, and insulin therapy is not necessarily appropriate. Indeed, most NDM patients who have mutations in 1 of the 2 subunits of the ATP sensitive K+ (K ATP ) channel (Kir6.2 or SUR1) will robustly and repeatedly respond to sulfonylureas, which circumvents the insulin secretory defect …”

Section: Introductionmentioning

confidence: 99%

“…The dramatic demonstration of sulfonylurea dependent insulin secretion in NDM, as well as in carriers of HNF1A mutations makes a compelling argument for further investigation of the potential action of these drugs in relevant groups of patients with diabetes. Sulfonylureas may provide not only a suitable tool for identification of such individuals but also a potentially appropriate therapy, as is clear in humans and mice with NDM . The pathway of GSIS is complex and defects at any step could result in failure of insulin secretion .…”

Section: Introductionmentioning

confidence: 99%

Sulfonylurea challenge test in subjects diagnosed with type 1 diabetes mellitus

et al. 2017

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Patients with early onset diabetes due to defects in glucose-stimulated insulin secretion (GSIS) may respond better to sulfonylureas than insulin treatment. Such patients include those with monogenic disorders, who can be differentiated from autoimmune type 1 diabetes mellitus (T1DM) by genetic testing. Genetic testing is expensive and unknown defects in GSIS would not be diagnosed. We propose a sulfonylurea challenge test to identify patients that have been clinically diagnosed with T1DM, but who maintain a preferentially sulfonylurea-responsive insulin secretion. Three healthy controls, two neonatal diabetes (NDM) subjects, three antibody-positive (Ab+T1DM), and twelve antibody-negative (Ab-T1DM) subjects with Type 1 diabetes, were given an intravenous bolus of glucose followed by an oral dose of glipizide. Healthy controls showed a robust C-peptide increase after both glucose and glipizide, but NDM subjects showed a large increase in C-peptide only following glipizide. As expected, two of three Ab+T1DM, as well as eleven of twelve Ab-T1DM showed no response to either glucose or glipizide. However, one Ab-T1DM and one Ab+T1DM showed a small C-peptide response to glucose and a marked positive response to glipizide, suggesting defects in GSIS rather than typical autoimmune diabetes. These data demonstrate the feasibility of the sulfonylurea challenge test, and suggest that responder individuals may be identified. We propose that this sulfonylurea challenge test should be explored more extensively, as it may prove useful as a clinical and scientific tool.

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“…Approximately 50% of the neonatal diabetes cases require lifelong treatment to control glycemia (permanent diabetes), and for the remaining half of the cases, a spontaneous remission is observed, usually within the first 3 months (transient diabetes), and a clinical relapse is always possible (1). …”

Section: Introductionmentioning

confidence: 99%

6q24 Transient Neonatal Diabetes – How to Manage while Waiting for Genetic Results

et al. 2016

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Diabetes, rare in the neonatal period, should be evoked in every newborn presenting with unexplained intrauterine and early postnatal growth retardation. This case report illustrates the clinical course and therapeutic approach of a newborn diagnosed with transient diabetes. The baby was born at 37 weeks of gestation with a severe intrauterine growth restriction. Except a mild macroglossia and signs of growth restriction, physical examination was normal. On the fifth day of life, hyperglycemia (180 mg/dl) was noted, and the next day, the diagnosis of diabetes was confirmed (high blood sugar, glucosuria, undetectable levels of insulin and C-peptide). Insulin infusion, initially intravenously and then subcutaneously, was started, tailored to assure the growth catch-up and normalize the blood sugar levels. At the age of 4 weeks, the baby returned at home under pump. At 8 weeks, the clinical impression of evolution to a transient diabetes (decreasing needs of insulin with very satisfactory weight gain) was genetically confirmed (paternal uniparental disomy of chromosome 6). There is no screening for neonatal diabetes, but the clinical suspicion avoids the metabolic decompensation and allows early initiation of insulin therapy. The genetic approach (for disease itself and its associated features) relies on timely clinical updates.

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Remission of Severe Neonatal Diabetes With Very Early Sulfonylurea Treatment

Cited by 16 publications

References 5 publications

Early transition from insulin to sulfonylureas in neonatal diabetes and follow-up: Experience from China

Early transition from insulin to sulfonylureas in neonatal diabetes and follow-up: Experience from China

Sulfonylurea challenge test in subjects diagnosed with type 1 diabetes mellitus

6q24 Transient Neonatal Diabetes – How to Manage while Waiting for Genetic Results

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