MODY and incidental hyperglycemia Maturity-Onset Diabetes of the Young (MODY) in Children with Incidental Hyperglycemia. A Multicenter Italian Study on 172 families Running title: MODY and incidental hyperglycemia
نویسندگان
چکیده
* Equally contributed to the paper Department of Pediatrics, IRCCS Gaslini Children’s Hospital, University of Genoa, Genova, Italy; Department of Biometry and Clinical Epidemiology Services, IRCCS Policlinico San Matteo, Pavia, Italy; IRCCS Bambino Gesu Pediatric Hospital, Rome, Italy; Department of Pediatrics, 2nd University of Naples, Naples, Italy; Pediatric Division, Brotzu Hospital, Cagliari, Italy, Meyer Pediatric Hospital, Florence, Italy; Department of Pediatrics, Scientific Institute San Raffaele Hospital, Milan, Italy; Department of Pediatrics, University of Turin, Turin, Italy; Department of Genetics, AP-HP Hopital Pitie-Salpetriere, Universite Pierre et Marie Curie, Paris, France; Department of Laboratory Medicine, Tor Vergata University Hospital, Rome, Italy; San Raffaele Biomedical Park Foundation, Rome, Italy. ** A full list of participating investigators is available in the acknowledgements.
منابع مشابه
Maturity-Onset Diabetes of the Young in Children With Incidental Hyperglycemia:
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 ...
متن کاملIncidental mild hyperglycemia in children: two MODY 2 families identified in Brazilian subjects.
Maturity-onset diabetes of the young (MODY) is characterized by an autosomal dominant mode of inheritance, early onset of hyperglycemia, and defects of insulin secretion. MODY subtypes described present genetic, metabolic, and clinical differences. MODY 2 is characterized by mild asymptomatic fasting hyperglycemia, and rarely requires pharmacological treatment. Hence, precise diagnosis of MODY ...
متن کاملRecognition and Management of Individuals With Hyperglycemia Because of a Heterozygous Glucokinase Mutation.
Glucokinase-maturity-onset diabetes of the young (GCK-MODY), also known as MODY2, is caused by heterozygous inactivating mutations in the GCK gene. GCK gene mutations are present in ∼1 in 1,000 of the population, but most are not diagnosed. They are common causes of MODY (10-60%): persistent incidental childhood hyperglycemia (10-60%) and gestational diabetes mellitus (1-2%). GCK-MODY has a uni...
متن کاملMild fasting hyperglycemia in children: high rate of glucokinase mutations and some risk of developing type 1 diabetes mellitus.
BACKGROUND Incidental hyperglycemia in children generates concern about the presence of preclinical type 1 diabetes mellitus (T1DM). OBJECTIVE To genetically evaluate two common forms of maturity-onset diabetes of youth (MODY), the short-term prognosis in children with mild hyperglycemia, and a positive family history of diabetes mellitus. SUBJECTS Asymptomatic children and adolescents (n =...
متن کاملLow prevalence of HNF1A mutations after molecular screening of multiple MODY genes in 58 Italian families recruited in the pediatric or adult diabetes clinic from a single Italian hospital.
Maturity-onset diabetes of the young (MODY; MIM# 606391) is a genetically and clinically heterogeneous form of diabetes, accounting for 1–2% of all diabetes cases (1). MODY is characterized by mild hyperglycemia or overt diabetes usually detected in three consecutive generations, with onset before the age of 25 years and absence of type 1 diabetes autoantibodies. Among the thirteen MODY genes i...
متن کامل