Mortality from cystic fibrosis in Europe: 1994–2010

Quintana‐Gallego, Esther; Ruíz-Ramos, Miguel; Delgado-Pecellín, Isabel; Calero, Carmen; Soriano, Joan B.; Lόpez-Campos, José Luís

doi:10.1002/ppul.23337

Cited by 18 publications

(26 citation statements)

References 29 publications

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“…Additionally, Hispanic patients had lower FEV1 percent predicted at age 6 but no difference in rate of decline compared to non‐Hispanics. Mortality secondary to CF in the European Union is decreasing with an improvement in average age of death over the time period 1994‐2010, although a higher mortality rate in females and variation in rates between countries was seen . It is important to understand that each study may have used different definitions and statistical methods to calculate mortality rates.…”

Section: Epidemiologymentioning

confidence: 99%

Cystic fibrosis year in review 2016

Savant

McColley

2017

Pediatr Pulmonol

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Section: Epidemiologymentioning

confidence: 99%

Cystic fibrosis year in review 2016

Savant

McColley

2017

Pediatr Pulmonol

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“…In Europe a significant reduction in potential years life lost (PYLL) for patients between 0 and 30 years old has been described. In males, the standardised PYLL decreased from 14.65 (11.37–17.93) lost years per million persons per year in 1994 to 7.49 (6.14–8.84) in 2010 [13]. In Canada, median survival age increased from 31.9 years (95% CI 28.3–35.2 years) in 1990 to 49.7 years (95% CI 46.1–52.2 years) in the 5-year window ending in 2012 [14].…”

Section: Epidemiologymentioning

confidence: 99%

A Practical Approach to Glucose Abnormalities in Cystic Fibrosis

Jones

Sainsbury

2016

Diabetes Ther

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Cystic fibrosis is a common genetic condition and abnormal glucose handling leading to cystic fibrosis-related diabetes (CFRD) is a frequent comorbidity. CFRD is mainly thought to be the result of progressive pancreatic damage resulting in beta cell dysfunction and loss of insulin secretion. Whilst Oral Glucose Tolerance Testing is still recommended for diagnosing CFRD, the relationship between glucose abnormalities and adverse outcomes in CF is complex and occurs at stages of dysglycaemia occurring prior to diagnosis of diabetes by World Health Organisation criteria. Insulin remains the mainstay of treatment of CF-related glucose abnormalities but the timing of insulin commencement, optimum insulin regime and targets of glycaemic control are not clear. These complexities are compounded by common issues with nutritional status, need for enteral feeding, steroid use and high disease burden on CF patients.

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“…Therapeutic advances and specialist units have contributed towards this disease ceasing to be lethal in childhood [1], [2], [3]. However, although current survival time can exceed 40 years, mostly in the case of patients from pediatric units, a diagnosis at over 40 years of age is still unusual, but the possibility of diagnosis at over 60 years of age still exists [4], [5].…”

Section: Introductionmentioning

confidence: 99%

“…Awareness of the clinical characteristics of patients diagnosed at advanced ages could be important in order to consider CF in adults of 60 years, since few studies have been carried out so far. Diagnosis of CF is important because it implies a prognosis and provision of appropriate treatment, not to mention genetic counselling in families [1], [4].…”

Section: Introductionmentioning

confidence: 99%

How are the ancient cystic fibrosis patients? Cystic fibrosis diagnosed over 60 years-old

Prados

Lerín

Cabanillas

et al. 2017

Respiratory Medicine Case Reports

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Background and aimsTo specify the prevalence of patients diagnosed with CF at age of ≥60 year-old and to analyze their characteristics.Patients and methodsObservational study of CF patients which were diagnosed at age ≥60 year-old. The analyzed variables were: age, sex, nationality, lung function parameters, conditions present at diagnosis, microbiological characteristics and genetic findings.Resultseight patients were included. 7 patients were female (87.5%) with a mean age of 70.6 years (median 71.5 years, range 60–78 years). The most important findings were: sweat test >60 mEq/l; heterozygotes F508del; bronchiectasis in CT; methicillin-sensitive Staphylococcus aureus (50%) in sputum. The most patients presented a normal or mild obstructive lung function.ConclusionsCF must also be considered a disease diagnosed in adulthood, incorporating the sweat test within the usual techniques of differential diagnosis in patients with different diseases associated with CF, because genetic counselling is esencial.

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Mortality from cystic fibrosis in Europe: 1994–2010

Abstract: Our data suggest a continued downward trend of CF mortality throughout the EU, with differences by country and gender.

Cited by 18 publications

References 29 publications

Cystic fibrosis year in review 2016

Cystic fibrosis year in review 2016

A Practical Approach to Glucose Abnormalities in Cystic Fibrosis

How are the ancient cystic fibrosis patients? Cystic fibrosis diagnosed over 60 years-old

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