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Cited by 3 publications
(8 citation statements)
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References 12 publications
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“…Not only is treatment with oral agents significantly easier for families, but years later patients continue to maintain normal or near normal hemoglobin A1c levels [54, 60, 61]. Although some patients continue to limit carbohydrate intake, it appears that many with no specific dietary plan report only transient glucose elevations that normalize without further intervention [53, 59, 60]. To the best of our knowledge, there has not been a single episode of severe hypoglycemia (altered mental status and/or inability to assist in care [62]) in any patient, even in those taking very high doses (>2 mg/kg/day of glyburide).…”
Section: Sulfonylurea-responsive Ndm: Revolutionized Care Through Molmentioning
confidence: 99%
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“…Not only is treatment with oral agents significantly easier for families, but years later patients continue to maintain normal or near normal hemoglobin A1c levels [54, 60, 61]. Although some patients continue to limit carbohydrate intake, it appears that many with no specific dietary plan report only transient glucose elevations that normalize without further intervention [53, 59, 60]. To the best of our knowledge, there has not been a single episode of severe hypoglycemia (altered mental status and/or inability to assist in care [62]) in any patient, even in those taking very high doses (>2 mg/kg/day of glyburide).…”
Section: Sulfonylurea-responsive Ndm: Revolutionized Care Through Molmentioning
confidence: 99%
“…The minority of patients who do not readily respond to sulfonylureas tend to be either older at the time of transition, or exhibit the most severe neurodevelopmental disability, termed DEND (developmental delay, epilepsy, neonatal diabetes) syndrome [44, 49, 53-55, 59]. In a 2006 report, two out of the five patients who failed to transition completely off of insulin (out of 49 total) were adult parents carrying the same mutations as their children who did successfully transition to exclusive sulfonylurea therapy [44].…”
Section: Sulfonylurea-responsive Ndm: Revolutionized Care Through Molmentioning
confidence: 99%
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“…The family kept daily communication with the Pediatric Endocrinology service as dose adjustments were made to a maximum of 80 mg/d (1.45 mg/kg/d), and by the 8th week of therapy, insulin therapy was discontinued entirely. Although many patients who have been switched to SU before the onset of puberty are typically able to discontinue insulin at a glyburide dose of approximately 1.0 mg/kg/d, this higher dose is similar to that of an 18-yr-old male who required 1.39 mg/kg/d at the end of his transition (15).…”
Section: Impact Of Su Treatment On Diabetesmentioning
confidence: 99%