Rate of improvement of CF life expectancy exceeds that of general population—Observational death registration study

Hurley, Margaret M.; McKeever, Tricia M.; Prayle, Andrew; Fogarty, Andrew; Smyth, Alan

doi:10.1016/j.jcf.2013.12.002

Cited by 70 publications

(48 citation statements)

References 25 publications

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“…Recent decades have seen a great improvement in survival of individuals with CF and this trend supports the assertion that median survival for those born in 2000 is to be expected beyond 50 years . The universal implementation of NBS and of evidence‐based guidelines, as well as the introduction of drugs aimed at the modulation of CFTR, could further improve the projections, also considering that the survival of patients with CF is more rapidly improving compared to the general population …”

Section: Discussionsupporting

confidence: 53%

“…The gender gap in survival is still up for debate, although several studies highlight the survival disadvantage of women . Latest registry reports describe even now that males continue to have a higher median age of survival compared to females (in UK 47.96 years in males vs. 42.62 years in females and in Canada around 53 years in males vs. around 47 years in females).…”

Section: Discussionmentioning

confidence: 99%

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Update of literature from cystic fibrosis registries 2012–2015. Part 6: Epidemiology, nutrition and complications

Salvatore

Buzzetti

Mastella

2016

Pediatric Pulmonology

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Patient registries provide useful information to afford more knowledge on rare diseases like Cystic Fibrosis (CF). Twenty-two studies originating from national CF registries, focusing on demographics, survival, genetics, nutritional status, and non-pulmonary complications, were published between December 2011 and March 2015. The purpose of this review article is to examine these reports, aiming attention to the clinical characteristics of CF patients included in the registries, current, and estimated future epidemiological data, the role of gender gap, the increasing survival in different countries. Some studies offer insights into pubertal growth and non-pulmonary complications, such as liver disease, nephropathy, and cancer. Pediatr Pulmonol. 2017;52:390-398. © 2016 Wiley Periodicals, Inc.

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Section: Discussionsupporting

confidence: 53%

Section: Discussionmentioning

confidence: 99%

Update of literature from cystic fibrosis registries 2012–2015. Part 6: Epidemiology, nutrition and complications

Salvatore

Buzzetti

Mastella

2016

Pediatric Pulmonology

View full text Add to dashboard Cite

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“…The current median life expectancy in United States and Great Britain is 37 years and is increasing due to improved treatments and physiotherapy. It is estimated children born in the 21st century will have a life expectancy of over 50 years [4]. Nevertheless, as the main cause of mortality in CF is lung disease, it is critical that we improve pulmonary diagnosis in patients to better predict adverse events and monitor therapies [5].…”

Section: Introductionmentioning

confidence: 99%

Evaluation of high resolution computed tomography findings of cystic fibrosis

Şaşihüseyinoğlu

Altıntaş

Soyupak

et al. 2019

Korean J Intern Med

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Monitoring of pulmonary physiology is fundamental to the clinical management of patients with Cystic Fibrosis. The current standard clinical practise uses spirometry to assess lung function which delivers a clinically relevant functional readout of total lung function, however does not supply any visible or localised information. High Resolution Computed Tomography (HRCT) is a well-established current 'gold standard' method for monitoring lung anatomical changes in Cystic Fibrosis patients. HRCT provides excellent morphological information, however, the X-ray radiation dose can become significant if multiple scans are required to monitor chronic diseases such as cystic fibrosis. X-ray phase-contrast imaging is another emerging X-ray based methodology for Cystic Fibrosis lung assessment which provides dynamic morphological and functional information, albeit with even higher X-ray doses than HRCT. Magnetic Resonance Imaging (MRI) is a non-ionising radiation imaging method that is garnering growing interest among researchers and clinicians working with Cystic Fibrosis patients. Recent advances in MRI have opened up the possibilities to observe lung function in real time to potentially allow sensitive and accurate assessment of disease progression. The use of hyperpolarized gas or non-contrast enhanced MRI can be tailored to clinical needs. While MRI offers significant promise it still suffers from poor spatial resolution and the development of an objective scoring system especially for ventilation assessment.

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“…Life expectancy of CF patients continues to steadily improve over time . As it does so, more emphasis is placed on understanding adult manifestations of the disease, and focus has shifted to preserving quality of life.…”

Section: Introductionmentioning

confidence: 99%

Impact of high‐ versus low‐risk genotype on sinonasal radiographic disease in cystic fibrosis

et al. 2018

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Objective Understanding of how specific mutations impact the cystic fibrosis transmembrane conductance regulator (CFTR) protein has given rise to the classification of CF patients into low‐risk and high‐risk genotypes. Few prior studies have investigated differences in sinonasal disease between low‐risk and high‐risk CF genotypes. This multi‐institutional review aimed to evaluate radiographic sinus disease severity based on genotype. Methods A review was conducted on adult patients with CF evaluated between 2005 to 2017 at three academic institutions. Data including age, gender, CFTR mutation, and presence of a maxillofacial/sinus computed tomography scan was collected. A modified Lund‐Mackay score (MLMS) was assigned to each scan, and the presence of sinus aplasia or hypoplasia was determined. Patients were further grouped depending on genotype into low‐ or high‐risk for comparison. Results A total of 126 patients were included with 99 patients in the high‐risk and 21 in the low‐risk groups. The high‐risk group had significantly higher MLMS than the low‐risk group (mean 13.88 vs. 8.06, P < 0.0001, 95% CI −8.196 to −3.462) The rate of frontal (P < 0.01), maxillary (P = 0.04), and sphenoid (P < 0.001) hypoplasia/aplasia was significantly higher in high‐risk patients compared to low‐risk. Conclusion This is one of the largest studies to date evaluating the impact of CF genotype on paranasal sinus development and disease. Genotype appears to impact sinonasal disease severity and also potentially paranasal sinus cavity development to a degree, although the exact mechanism is unknown. Level of Evidence 4 Laryngoscope, 129:788–793, 2019

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Rate of improvement of CF life expectancy exceeds that of general population—Observational death registration study

Cited by 70 publications

References 25 publications

Update of literature from cystic fibrosis registries 2012–2015. Part 6: Epidemiology, nutrition and complications

Update of literature from cystic fibrosis registries 2012–2015. Part 6: Epidemiology, nutrition and complications

Evaluation of high resolution computed tomography findings of cystic fibrosis

Impact of high‐ versus low‐risk genotype on sinonasal radiographic disease in cystic fibrosis

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