Maturity-onset Diabetes of the Young in Children with Incidental Hyperglycemia: a Multicenter Italian Study of 172 Families

Overview

Journal Diabetes Care

Specialty Endocrinology

Date 2009 Jul 1

PMID 19564454

Citations 34

Authors

Renata Lorini

Catherine Klersy

Giuseppe DAnnunzio

Ornella Massa

Nicola Minuto

Dario Iafusco

Christine Bellanne-Chantelot

Anna Paola Frongia

Sonia Toni

Franco Meschi

Franco Cerutti

Fabrizio Barbetti

Affiliations

Soon will be listed here.

Abstract

Objective: To investigate the prevalence of maturity-onset diabetes of the young (MODY) in Italian children with incidental hyperglycemia.

Research Design And Methods: Among 748 subjects age 1-18 years with incidental hyperglycemia, minimal diagnostic criteria for MODY were met by 172 families. Mutational analyses of the glucokinase (GCK) and hepatocyte nuclear factor 1alpha (HNF1A) genes were performed.

Results: We identified 85 GCK gene mutations in 109 probands and 10 HNF1A mutations in 12 probands. In GCK patients, the median neonatal weight and age at the first evaluation were lower than those found in patients with HNF1A mutations. Median fasting plasma glucose and impaired fasting glucose/impaired glucose tolerance frequency after oral glucose tolerance testing were higher in GCK patients, who also showed a lower frequency of diabetes than HNF1A patients.

Conclusions: GCK mutations are the prevailing cause of MODY (63.4%) when the index case is recruited in Italian children with incidental hyperglycemia.

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