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Early impairment of glucose tolerance and β-cell function in obese children.

AbstractINTRODUCTION:
A clinical criterion of the Wolfram syndrome is the coexistence of diabetes and optic atrophy recognized before the age of 15. Diabetes present in Wolfram syndrome is a result of the selective β cell loss and failed insulin secretion which is probably associated with non-autoimmune pathogenesis.
AIM OF THE STUDY:
The aim of the study was an evaluation of HLA subtypes and presence of β-cell autoantibodies in patients with molecularly confirmed Wolfram syndrome.
MATERIAL AND METHODS:
9 patients with Wolfram syndrome aged 10-24 years were examined. We also studied 218 patients with type 1 diabetes as a reference group. A control group of 176 healthy individuals was included in the study. Besides the clinical assessment the HLA typing by PCR-SSO was performed. Islet cell antibodies (ICA), antibodies to glutamic acid decarboxylase (GADA), thyrosine phosphatase antibodies (IA2A) and insulin antibodies (IAA) were also detected.
RESULTS:
In all nine patients the coexistence of diabetes with optic atrophy was observed and in 8/9 individuals additional symptoms were recognized. In patients with Wolfram syndrome a significantly lower age of diagnosis of diabetes (Me=5.0 years) than in type 1 diabetic children (Me=10.4; p=0.002) was observed. Studies of HLA subtypes demonstrated an increased prevalence of HLA-DQw1, DRB1⋅03 and/or 04 and DR2. A comparison of the frequency of the HLA alleles in patients with Wolfram syndrome with type 1 diabetic children showed a more frequent presence of the DRB1⋅1501 (p=0.03; OR=13.28 (2.44-72.12)) and DQB1⋅06 (p=0.016; OR=10.15 (2.49-41.35)) alleles in patients with Wolfram syndrome.
CONCLUSIONS:
Polish patients with Wolfram syndrome have a different profile of the HLA antigens with the presence of DR2, DQw1 and DRB3/4 allele and are negative for diabetes-related autoantibodies, which may confirm non-autoimmune β-cell destruction in this syndrome.
AuthorsPiotr Fichna, Bogda Skowrońska, Katarzyna Majewska, Witold Stankiewicz
JournalPediatric endocrinology, diabetes, and metabolism (Pediatr Endocrinol Diabetes Metab) Vol. 16 Issue 4 Pg. 255-61 ( 2010) ISSN: 2081-237X [Print] Poland
PMID21447266 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Autoantibodies
  • HLA-DQ Antigens
  • HLA-DR Antigens
  • HLA-DR2 Antigen
  • HLA-DRB1 Chains
  • HLA-DRB3 Chains
  • islet cell antibody
Topics
  • Adolescent
  • Autoantibodies (immunology)
  • Child
  • Female
  • Glucose Tolerance Test
  • HLA-DQ Antigens (immunology)
  • HLA-DR Antigens (immunology)
  • HLA-DR2 Antigen (immunology)
  • HLA-DRB1 Chains
  • HLA-DRB3 Chains
  • Humans
  • Insulin-Secreting Cells (immunology)
  • Male
  • Obesity (complications, metabolism)
  • Wolfram Syndrome (immunology)

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