Document Detail


The progression from obesity to type 2 diabetes in Alström syndrome.
MedLine Citation:
PMID:  21722283     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Alström syndrome (ALMS) is a rare autosomal recessive monogenic disease associated with obesity, hyperinsulinemia, and alterations of glucose metabolism that often lead to the development of type 2 diabetes at a young age.
OBJECTIVE: To study the relationship between weight and metabolism in a group of ALMS patients and matched controls.
RESEARCH DESIGN AND METHODS: Fifteen ALMS patients (eight males, seven females; aged 3-51) were compared in a cross-sectional study with an age- and weight-matched control population. Anthropometric parameters, fat mass, glucose and insulin secretion in basal and dynamic oral glucose tolerance test (OGTT) conditions were measured. Furthermore, anthropometric and body composition data were obtained from an international group of 27 ALMS patients (13 males, 14 females, age range: 4-29 yr).
RESULTS: In ALMS we observed an inverse correlation between age and standard deviation scores for height, weight, and body mass index. The OGTT glycemic curves of ALMS subjects were similar to those of age-matched controls, whereas insulin response was clearly greater. In ALMS individuals the insulin response showed a reduction with age. We documented pathologic values of the derived indices homeostasis model assessment of insulin resistance (HOMA-IR), insulin sensitivity index, HOMA%β-cell and insulinogenic index in ALMS, but unlike the insulin-resistance indices, the β-cell function indices showed a significant reduction with age.
CONCLUSIONS: In ALMS the progression from the early onset obesity toward the impaired fasting glucose or impaired glucose tolerance and overt diabetes is mostly because of a progressive failure of β-cell insulin secretion without any further worsening of insulin resistance with age, even in the presence of weight reduction.
Authors:
Vera Bettini; Pietro Maffei; Claudio Pagano; Sara Romano; Gabriella Milan; Francesca Favaretto; Jan D Marshall; Richard Paisey; Francesco Scolari; Nella A Greggio; Ilaria Tosetto; Jürgen K Naggert; Nicola Sicolo; Roberto Vettor
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2011-07-03
Journal Detail:
Title:  Pediatric diabetes     Volume:  13     ISSN:  1399-5448     ISO Abbreviation:  Pediatr Diabetes     Publication Date:  2012 Feb 
Date Detail:
Created Date:  2012-01-27     Completed Date:  2012-06-01     Revised Date:  2014-09-17    
Medline Journal Info:
Nlm Unique ID:  100939345     Medline TA:  Pediatr Diabetes     Country:  Denmark    
Other Details:
Languages:  eng     Pagination:  59-67     Citation Subset:  IM    
Copyright Information:
© 2011 John Wiley & Sons A/S.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Alstrom Syndrome / complications*,  epidemiology
Body Composition / genetics,  physiology
Case-Control Studies
Child
Child, Preschool
Diabetes Mellitus, Type 2 / epidemiology,  etiology*
Disease Progression
Female
Humans
Male
Middle Aged
Obesity / complications*,  epidemiology
Young Adult
Grant Support
ID/Acronym/Agency:
HD-036878/HD/NICHD NIH HHS; R01 HD036878/HD/NICHD NIH HHS; R01 HD036878-13/HD/NICHD NIH HHS
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