[Childhood-onset diabetes treated with sulphonylureas].
MODY 3 type diabetes belongs to the group of monogenic diabetes and is caused by mutations in the gene for hepatocyte nuclear factor 1-alpha (HNF1-alpha). Although MODY 2 type diabetes is the most frequent form of MODY diabetes in childhood, type 3 is the most frequent in the general population. We report the case of a 12 year old child with basal and post-prandrial hyperglycaemia. No cardinal symptoms of type 1 diabetes mellitus were present. There are numerous cases of diabetes in his family. C-Peptide was 1.13 ng/ml and pancreatic autoimmunity markers were negative. HNF-1alpha gene mutation was found in the patient as well as in his father and sister. Treatment with glibenclamide was started at a dose of 2.5 mg/day in order to reduce the risk of microvascular disease, as this as high in MODY 3 type diabetes as in type 1 diabetes mellitus. Blood glucose returned to normal and glycosylated haemoglobin was maintained between 4.9 and 5.6 %. Side-effects were not observed except some mild hypoglycaemias.
Palomo Atance
E
E
Unidad de Endocrinología Pediátrica, Servicio de Pediatría, Hospital General de Ciudad Real, Ciudad Real, España. kikepalomo@wanadoo.es
Ballester Herrera
M J
MJ
Giralt Muiña
P
P
López Garrido
P
P
D002648:Child; D003924:Diabetes Mellitus, Type 2 / Q000188:drug therapy* / Q000235:genetics; D005905:Glyburide / Q000627:therapeutic use*; D006801:Humans; D007004:Hypoglycemic Agents / Q000627:therapeutic use*; D008297:Male; D010375:Pedigree
D007004:Hypoglycemic Agents; D005905:Glyburide