Diabetes Is Associated With Dramatically Decreased Survival in Female but Not Male Subjects With Cystic Fibrosis

Milla, Carlos; Billings, Joanne; Moran, Antoinette

doi:10.2337/diacare.28.9.2141

Cited by 183 publications

(179 citation statements)

References 30 publications

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“…An association between poor pulmonary function and CFRD has been reported from cross-sectional studies (4 -6), and other studies have demonstrated accelerated decline in pulmonary function before diagnosis of CFRD (2,25). In cystic fibrosis, it is possible that prediabetic (or prediagnostic) states worsen pulmonary function.…”

Section: Risk Factorsmentioning

confidence: 96%

Genetic Determinants and Epidemiology of Cystic Fibrosis–Related Diabetes

et al. 2008

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OBJECTIVE -Longer survival of patients with cystic fibrosis has increased the occurrence of cystic fibrosis-related diabetes (CFRD). In this study we documented the incidence of CFRD and evaluated the association between mutations responsible for cystic fibrosis and incident CFRD, while identifying potential risk factors.RESEARCH DESIGN AND METHODS -This was a population-based longitudinal study of 50 cystic fibrosis speciality clinics in the U.K. Subjects included 8,029 individuals aged 0 -64 years enrolled in the U.K. Cystic Fibrosis Registry during 1996 -2005. Of these, 5,196 with data and without diabetes were included in analyses of incidence, and 3,275 with complete data were included in analyses of risk factors. Diabetes was defined by physician diagnosis, oral glucose tolerance testing, or treatment with hypoglycemic drugs.RESULTS -A total of 526 individuals developed CFRD over 15,010 person-years. The annual incidence was 3.5%. The incidence was higher in female patients and in patients with mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene in classes I and II. In a multivariate model of 377 cases of 3,275 patients, CFTR class (relative risk 1.70 [95% CI 1.16 -2.49], class I or II versus others), increasing age, female sex, worse pulmonary function, liver dysfunction, pancreatic insufficiency, and corticosteroid use were independently associated with incident diabetes.CONCLUSIONS -The incidence of CFRD is high in Britain. CFTR class I and II mutations increase the risk of diabetes independent of other risk factors including pancreatic exocrine dysfunction.

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Section: Risk Factorsmentioning

confidence: 96%

Genetic Determinants and Epidemiology of Cystic Fibrosis–Related Diabetes

et al. 2008

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“…It is not clear whether or not these two facts are connected. Milla et al 57 found that the median age of survival was 30.7 years for females with CFRD, and for males it was 47.4 years. It must be noted that this difference in age survival may be due to CFRD or it may arise from other factors (e.g.…”

Section: Prognosismentioning

confidence: 99%

Screening for cystic fibrosis-related diabetes: a systematic review.

Waugh

Royle²,

Craigie³

et al. 2012

Health Technol Assess

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How to obtain copies of this and other HTA programme reports An electronic version of this title, in Adobe Acrobat format, is available for downloading free of charge for personal use from the HTA website (www.hta.ac.uk). A fully searchable DVD is also available (see below).Printed copies of HTA journal series issues cost £20 each (post and packing free in the UK) to both public and private sector purchasers from our despatch agents.Non-UK purchasers will have to pay a small fee for post and packing. For European countries the cost is £2 per issue and for the rest of the world £3 per issue. How to order:-fax (with credit card details) -post (with credit card details or cheque) -phone during office hours (credit card only).Additionally the HTA website allows you to either print out your order or download a blank order form. Contact details are as follows:Synergie UK (HTA Department) Digital House, The Loddon Centre Wade Road Basingstoke Hants RG24 8QW Email: orders@hta.ac.uk Tel: 0845 812 4000 -ask for 'HTA Payment Services' (out-of-hours answer-phone service) Fax: 0845 812 4001 -put 'HTA Order' on the fax header Payment methods Paying by chequeIf you pay by cheque, the cheque must be in pounds sterling, made payable to University of Southampton and drawn on a bank with a UK address.Paying by credit card You can order using your credit card by phone, fax or post. SubscriptionsNHS libraries can subscribe free of charge. Public libraries can subscribe at a reduced cost of £100 for each volume (normally comprising 40-50 titles). The commercial subscription rate is £400 per volume (addresses within the UK) and £600 per volume (addresses outside the UK). Please see our website for details. Subscriptions can be purchased only for the current or forthcoming volume.How do I get a copy of HTA on DVD?Please use the form on the HTA website (www.hta.ac.uk/htacd/index.shtml). HTA on DVD is currently free of charge worldwide.The website also provides information about the HTA programme and lists the membership of the various committees. HTA NIHR Health Technology Assessment programmeThe Health Technology Assessment (HTA) programme, part of the National Institute for Health Research (NIHR), was set up in 1993. It produces high-quality research information on the effectiveness, costs and broader impact of health technologies for those who use, manage and provide care in the NHS. 'Health technologies' are broadly defined as all interventions used to promote health, prevent and treat disease, and improve rehabilitation and long-term care. The research findings from the HTA programme directly influence decision-making bodies such as the National Institute for Health and Clinical Excellence (NICE) and the National Screening Committee (NSC). HTA findings also help to improve the quality of clinical practice in the NHS indirectly in that they form a key component of the 'National Knowledge Service' . The HTA programme is needs led in that it fills gaps in the evidence needed by the NHS. There are three routes to the start of projects...

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“…CFRD has a burden of complications, chiefly the deterioration of pulmonary function (7)(8)(9), but also classic diabetes complications, as recently shown (10,11). Overall, CFRD increases the mortality risk, particularly in females (12).…”

Section: Introductionmentioning

confidence: 99%

Spontaneous hypoglycemia in patients with cystic fibrosis

Battezzati¹,

Battezzati²,

Costantini³

et al. 2007

eur j endocrinol

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Objective: Diabetes frequently complicates cystic fibrosis (CF) without fasting hyperglycemia or despite spontaneous hypoglycemia (anecdotally ascribed to malnutrition), whose prevalence, clinical meaning, and relationship with glucose tolerance and clinical/nutritional status were not previously investigated. The relationship of CF genotype with insulin secretion control is also unclear. Design and methods: A total of 129 CF patients without stable diabetes received 188 oral glucose tolerance tests. Distribution of fasting plasma glucose (FPG), glucose, insulin and C-peptide responses, clinical/nutritional variables, and their relationships were analyzed. Results: FPG!60 mg/dl (3.3 mmo/l) was detected in 14% of studies and reactive hypoglycemia (PG!50 mg/dl (2.8 mmo/l)) in 15%. OGTT-based diabetes frequency was similar in the lowest quartile (Q1) and Q2-3 for FPG (10 and 8%), with higher glucose increment and area under the curve in Q1. Insulin and C-peptide levels were similar among FPG quartiles. Class I cystic fibrosis transmembrane conductance regulator mutation carriers had higher insulin concentrations than class II, especially in Q1 for FPG. Age, sex, nutritional, and anthropometric parameters including fat and lean body mass were unrelated to FPG. Lower FPG was associated with more frequent hospitalization rates (PZ0.002) and lower Shwachman scores (PZ0.041). Steroids weaning was accurately evaluated but then excluded as a possible cause of hypoglycemia. Conclusions/interpretation: Fasting asymptomatic hypoglycemia is frequent and possibly related to inappropriate insulin secretion control in class I mutation carriers. Low FPG does not exclude impaired glucose tolerance (IGT) and diabetes in CF and reflects worse clinical status.

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Diabetes Is Associated With Dramatically Decreased Survival in Female but Not Male Subjects With Cystic Fibrosis

Cited by 183 publications

References 30 publications

Genetic Determinants and Epidemiology of Cystic Fibrosis–Related Diabetes

Genetic Determinants and Epidemiology of Cystic Fibrosis–Related Diabetes

Screening for cystic fibrosis-related diabetes: a systematic review.

Spontaneous hypoglycemia in patients with cystic fibrosis

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