Maturity-onset diabetes of the young (MODY): a new challenge for pediatric diabetologists.

Abstract	The differential diagnosis of hyperglycemia in childhood and adolescence has to take into consideration early-onset non-insulin-dependent diabetes, defined as maturity onset diabetes of the young (MODY). To date, mutations in genes of five proteins have been shown to cause MODY: glucokinase (MODY2), hepatic nuclear factor-1 alpha (HNF-1 alpha) (MODY3), hepatic nuclear factor-4 alpha (HNF-4 alpha) (MODY1), insulin promoter factor 1 (IPF-1) (MODY4) and hepatic nuclear factor-1 beta (HNF-1 beta) (MODY5), but other MODY genes still await elucidation. Clinical and metabolic heterogeneity of these subtypes of type 2 diabetes need to be defined, as deficiency of each factor has its own phenotype. Pediatric diabetologists should be aware of the increasing importance of MODY as a possible cause of hyperglycemia in children and adolescents. This will allow for the early diagnosis of these metabolic conditions and for the appropriate follow-up and treatment.
Authors	L Guazzarotti, E Bartolotta, F Chiarelli
Journal	Journal of pediatric endocrinology & metabolism : JPEM (J Pediatr Endocrinol Metab) 1999 Jul-Aug Vol. 12 Issue 4 Pg. 487-97 ISSN: 0334-018X [Print] Germany
PMID	10417964 (Publication Type: Journal Article, Review)
Chemical References	Basic Helix-Loop-Helix Leucine Zipper Transcription Factors DNA-Binding Proteins HNF1A protein, human HNF1B protein, human HNF4A protein, human Hepatocyte Nuclear Factor 1-alpha Hepatocyte Nuclear Factor 4 Homeodomain Proteins Insulin MLX protein, human Nuclear Proteins Phosphoproteins Trans-Activators Transcription Factors pancreatic and duodenal homeobox 1 protein Hepatocyte Nuclear Factor 1 Hepatocyte Nuclear Factor 1-beta Glucokinase
Topics	Adolescent Basic Helix-Loop-Helix Leucine Zipper Transcription Factors Child DNA-Binding Proteins Diabetes Mellitus, Type 2 (diagnosis, genetics, immunology) Glucokinase (genetics) Hepatocyte Nuclear Factor 1 Hepatocyte Nuclear Factor 1-alpha Hepatocyte Nuclear Factor 1-beta Hepatocyte Nuclear Factor 4 Homeodomain Proteins Humans Insulin (metabolism, pharmacology) Insulin Secretion Mutation Nuclear Proteins Pediatrics Phosphoproteins (genetics) Trans-Activators (genetics) Transcription Factors (genetics)

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