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Cystic fibrosis related diabetes (CFRD)-the end stage of progressive insulin deficiency.
MedLine Citation:
PMID:  21626717     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
In cystic fibrosis, gradual pancreatic destruction causes progressive insulin deficiency, culminating in cystic fibrosis related diabetes (CFRD). As a consequence of insulin deficiency, elevated glucose levels can be detected (well before the diagnosis of CFRD), by continuous ambulatory subcutaneous interstitial fluid glucose monitoring or 30-min sampled oral glucose tolerance test (OGTT). Current diagnostic criteria for CFRD (based on 0 and 120-min OGTT blood glucose levels) were originally designed to forecast microvascular disease in type 2 diabetes, rather than CF-specific outcomes such as declining weight or lung function. In CF, decline in either weight or lung function predicts early mortality. Both may precede the diagnosis of CFRD by several years. Insulin, a potent anabolic hormone, is recommended treatment for CFRD, but use in earlier stages of insulin deficiency is not established. Conventional dosing (with four or more insulin injections per day) is burdensome and carries substantial risk of hypoglycemia. However, recent uncontrolled trials suggest that once-daily injection of intermediate or long-acting insulin improves weight and lung function, with minimal hypoglycemia risk, in CFRD and also in early insulin deficiency. It is plausible that insulin may be of greater benefit to respiratory function when given prior to the diagnosis of CFRD, after which structural lung disease may be irreversible. It is also plausible that early insulin treatment may prolong the lifespan of the remaining insulin-secreting β-cells. Randomized controlled trials are now needed to determine whether or not current clinical practice should be altered toward the earlier commencement of insulin in CF. Pediatr Pulmonol. © 2011 Wiley-Liss, Inc.
Authors:
Shihab Hameed; Adam Jaffé; Charles F Verge
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-5-27
Journal Detail:
Title:  Pediatric pulmonology     Volume:  -     ISSN:  1099-0496     ISO Abbreviation:  -     Publication Date:  2011 May 
Date Detail:
Created Date:  2011-5-31     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8510590     Medline TA:  Pediatr Pulmonol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011 Wiley-Liss, Inc.
Affiliation:
Department of Endocrinology, Sydney Children's Hospital, Randwick, NSW, Australia; School of Women's and Children's Health, University of New South Wales, Australia. s.hameed@unsw.edu.au.
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