2008, Number 4
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Rev Endocrinol Nutr 2008; 16 (4)
Persistant neonatal hyperglycemia
Rojas-Zacarías A, Medina-Serrano JM, Nishimura-Meguro E
Language: Spanish
References: 12
Page: 172-176
PDF size: 105.32 Kb.
ABSTRACT
Transient or permanent neonatal diabetes mellitus (NDM) is rare disorder which requires subcutaneous insulin for management. The recently discovered mutations in the KCNJ11 and ABCC8 genes, encoding the Kir6.2 and SUR1 subunits of the pancreatic K(ATP) channel involved in regulation of insulin secretion, account for a third to a half of the PNDM cases. Patients with these mutations may be converted from insulin therapy to sulphonylureas. We describe the transition from insulin to glibenclamide therapy in a 4 year old girl with permanent NDM in the outpatient setting. She had a normal abdominal ultrasound and no pancreatic antibodies or phenotypic alterations. Sulphonylurea treatment resulted in increase in basal C-peptide levels from 0.01 to 0.8 ng/mL, HbA1c levels were reduced from 7.5 to 5.2% and insulin treatment discontinuation was possible at 12 weeks of treatment.
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