Diagnosing cystic fibrosis-related diabetes: current methods and challenges

Prentice, Bernadette; Hameed, Shihab A.; Verge, Charles F.; Ooi, Chee Y.; Jaffé, Adam; Widger, John

doi:10.1080/17476348.2016.1190646

Cited by 18 publications

(16 citation statements)

References 116 publications

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“…Furthermore, there is increasing evidence that early insulin deficiency in CF has a significant impact on clinical status, prior to the 120-min OGTT diagnosis of diabetes ( 102 ). Thus, a model that predicts early insulin deficiency glucose abnormalities should be sought, which will be a more proactive and cost-effective strategy.…”

Section: Diagnosis Of Cfrdmentioning

confidence: 99%

Cystic Fibrosis-Related Diabetes

2018

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Cystic fibrosis (CF) is the most common autosomal recessive disorder in Caucasian populations. Individuals with CF have seen significant increases in life expectancy in the last 60 years. As a result, previously rare complications are now coming to light. The most common of these is cystic fibrosis-related diabetes (CFRD), which affects 40–50% of CF adults. CFRD significantly impacts the pulmonary function and longevity of CF patients, yet a lack of consensus on the best methods to diagnose and treat CFRD remains. We begin by reviewing our understanding of the pathogenesis of CFRD, as emerging evidence shows the cystic fibrosis transmembrane conductance regulator (CFTR) also has important roles in the release of insulin and glucagon and in the protection of β cells from oxidative stress. We then discuss how current recommended methods of CFRD diagnosis are not appropriate, as continuous glucose monitoring becomes more effective, practical, and cost-effective. Finally, we evaluate emerging treatments which have narrowed the mortality gap within the CF patient group. In the future, pharmacological potentiators and correctors directly targeting CFTR show huge promise for both CFRD and the wider CF patient groups.

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Section: Diagnosis Of Cfrdmentioning

confidence: 99%

Cystic Fibrosis-Related Diabetes

2018

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“…76 The prevalence in older patients is around 40%. 76,77 The difficulties of dealing with a new additional disease that is associated with a less positive outcome and a huge additional burden of treatment is not straightforward for most adults. The negative impact on survival has been highlighted in the recent lung transplant guidelines as an important cofactor indicating consideration for early referral in individuals with FEV1< 40% predicted.…”

Section: Other Organ Diseasesmentioning

confidence: 99%

Adult Care in Cystic Fibrosis

Elborn

2019

Semin Respir Crit Care Med

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Cystic fibrosis (CF) is now more common in adults than children in countries with well-developed health care systems. The number of adults continues to increase and will further increase if the new cystic fibrosis transmembrane conductance regulator (CFTR) modulators are disease modifying. Most of the complex morbidity and almost all the mortality of CF occur in adults and will increasingly follow this pattern even with new effective modulator therapies. Maintaining good quality of life including social functioning and maximizing survival for adults are the key priorities. This requires a highly knowledgeable and adaptable multidisciplinary team, which, though focused on maintaining lung health, requires an increasing range of other disciplines and specialties to maximize well-being. Changes in health care systems will require current models of care to adapt to provide care for the large number of adult patients. With increasing survival and age, many are likely to have both CF morbidities and additional diseases of aging. New models are needed for health care delivery for this expanding population with complex medical conditions.

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“…Increased fluctuation (or variability) in highly regulated biological systems suggests impaired homeostasis (10) and assessments of variability in peak expiratory flow rate have been demonstrated to aid assessments of asthma control (11) and predict future risk (12). The underlying aetiology of CFRD is incompletely understood, but is considered to be a combination of beta cell dysfunction secondary to exocrine pancreatic insufficiency (13) and, possibly, a direct effect of abnormal CFTR function on insulin secretion (14,15). Decreased insulin secretion results in a catabolic state associated with impaired nutritional status, decreased lung function, and increased risk of bacterial infections (13).…”

Section: Introductionmentioning

confidence: 99%

“…The underlying aetiology of CFRD is incompletely understood, but is considered to be a combination of beta cell dysfunction secondary to exocrine pancreatic insufficiency (13) and, possibly, a direct effect of abnormal CFTR function on insulin secretion (14,15). Decreased insulin secretion results in a catabolic state associated with impaired nutritional status, decreased lung function, and increased risk of bacterial infections (13).…”

Section: Introductionmentioning

confidence: 99%

“…There are no data to support diagnosis with HbA1c.Therefore some CF centres have switched to using a continuous glucose monitoring system (CGMS) in order to diagnose CFRD at an earlier stage (21). The criteria for diagnosis of CFRD are still largely based on discrete cut-off points extrapolated from previous guidelines (13,22), and objective measures are needed to aid clinician's decisions when looking at raw traces that may fall within the range of current normal limits but appear inherently pathologicalrepresenting the so-called "toxic threshold" (23).…”

Section: Introductionmentioning

confidence: 99%

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