%0 Journal Article
%T Response to oral gliclazide in a pre-pubertal child with hepatic nuclear factor-1 alpha maturity onset diabetes of the young
%A Habeb Abdelhadi
%A George Elizabeth
%A Mathew Verghese
%A Hattersley Andrew
%J Annals of Saudi Medicine
%D 2011
%I King Faisal Specialist Hospital and Research Centre
%X The term "maturity onset diabetes of the young" (MODY) describes a heterogeneous group of monogenic diabetes of which hepatic nuclear factor-1 alpha (HNF-1¦Á) MODY is the most common. Patients with HNF-1¦Á mutations typically present after puberty, and oral sulfonylureas (SU) have been shown to be effective in adults with this condition. A 7-year-old boy presented with asymptomatic hyperglycemia ranging between 6.2 and 10.1 mmol/L and glycosuria for nearly a year. The child¡äs initial HbA 1c was 6.9% and the pancreatic Islet cell autoantibodies were negative. His response to the oral glucose tolerance test (OGTT) showed a large increment of glucose from basal level of 7.7 to 21.1 mmol/L in 120 min. The mild presentation, family history, and negative autoantibodies were suggestive of HNF-1¦Á MODY, which was confirmed by mutation analysis. Initial management with diet alone was not sufficient, but he responded well to 20 mg oral gliclazide once a day with an improvement of HbA 1C from 7.2% to 6.5% within 3 months of treatment. The case is an illustration of the clinical utility of molecular genetic tests in the management of childhood diabetes.
%U http://www.saudiannals.net/article.asp?issn=0256-4947;year=2011;volume=31;issue=2;spage=190;epage=193;aulast=Habeb