HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) All Versions of this Article: dc06-1915v1 30/5/1187    most recent Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Preumont, V. Articles by Buysschaert, M. Search for Related Content PubMed PubMed Citation Articles by Preumont, V. Articles by Buysschaert, M. Social Bookmarking                What's this?

GLUCOSE HOMEOSTASIS AND GENOTYPE--PHENOTYPE INTERPLAY IN CYSTIC FIBROSIS PATIENTS WITH GENE CFTR F 508 MUTATION

¹Department of Endocrinology and Nutrition, Université Catholique de Louvain, Cliniques universitaires St-Luc, UCL 5474, B-1200 Brussels, Belgium
²Cystic Fibrosis Unit, Université Catholique de Louvain, Cliniques universitaires St-Luc, UCL 5474, B-1200 Brussels, Belgium

buysschaert{at}diab.ucl.ac.be

ABSTRACT

Objective: To determine the clinical phenotype of adolescent/adult patients with cystic fibrosis (CF), according to heterozygosity or homozygosity for CFTR F508 mutation, and to analyse their characteristics according to glucose tolerance status.

Design: 76 CF patients with CFTR F508 mutation (33 heterozygous, 43 homozygous) stratified according to normal (NGT, n=51) or abnormal glucose homeostasis (AGH; IFG-IGT or diabetes, n=25) had their HOMA ß-cell function (B), insulin sensitivity (S), and [BxS] hyperbolic product measured. Pancreatic exocrine insufficiency was inferred from pancreatine requirements. Clinical effects of insulin therapy on weight and lung function were recorded.

Results: AGH was observed in 24 and 40% of heterozygous and homozygous subjects. AGH patients were older than NGT (29±10 vs. 23±8 years, p=0.006) and their B function was lower (93±49 vs. 125±51%, p=0.011). S values were comparable in NGT and AGH. A lower BxS product was observed in AGH, although non-significant when adjusted for error propagation. Pancreatic insufficiency was observed in 52 and 100% of hetero- and homozygous patients (p=0.001).

Conclusions: Prediabetes and diabetes represent frequent co-morbidities in CFTR gene F 508 mutation in the homozygous or heterozygous states. Impairment of insulin secretion, as shown by HOMA, is an important determinant when compared with the magnitude of compensation from insulin sensitivity. Given the high prevalence of abnormal glucose tolerance, screening for (pre) diabetes is mandatory. Insulin supplementation in diabetic CFTR gene F 508 subjects seems a rational therapy for consideration, although this does not preclude that therapy directed toward insulin resistance could also interact.

CiteULike    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

				A. I. Adler, B. S.F. Shine, P. Chamnan, C. S. Haworth, and D. Bilton Genetic Determinants and Epidemiology of Cystic Fibrosis-Related Diabetes: Results from a British cohort of children and adults Diabetes Care, September 1, 2008; 31(9): 1789 - 1794. [Abstract] [Full Text] [PDF]