Improved survival at low lung function in cystic fibrosis: cohort study from 1990 to 2007

George, PM; Banya, Winston; Pareek, Nilesh; Bilton, Diana; Cullinan, Paul; Hodson, Margaret E.; Simmonds, Nicholas J.

doi:10.1136/bmj.d1008

Cited by 130 publications

(103 citation statements)

References 27 publications

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“…Pulmonary transplantation is an important therapy for end-stage CF lung disease. Survival once patients reach an FEV 1 of 30% was only 2 years in 1989 [69], but improved to 5.3 years in 2003 mainly due to the impact of lung transplantation [70]. The adult CF physician must be skilled in recognising end-stage disease and timing discussion and referral for transplantation appropriately.…”

Section: Transplantation and Terminal Carementioning

confidence: 99%

Adult cystic fibrosis care in the 21st century

Garattini

Bilton

Cremona

et al. 2015

Monaldi Arch Chest Dis

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Cystic fibrosis (CF) is the most common autosomal recessive inherited disease of Caucasian populations. As a result of a variety of diagnostic and therapeutic strategies there has been a dramatic increase in the life expectancy of patients with CF in the last decades and 50% of patients are now adults. This review will focus on the disease in adults and the provision of appropriate care. The complex care required to improve the survival and quality of life in the adult patients can best be provided in a dedicated adult cystic fibrosis unit. These units currently exist in many European countries, but more are needed in Italy.

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Section: Transplantation and Terminal Carementioning

confidence: 99%

Adult cystic fibrosis care in the 21st century

Garattini

Bilton

Cremona

et al. 2015

Monaldi Arch Chest Dis

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“…Long-term use of rhDNase reduces the frequency of pulmonary exacerbations and delays decline in pulmonary function [6,44,45].…”

Section: Rhdnasementioning

confidence: 99%

“…In the past 25 years, several factors relating to preservation of pulmonary function have been implemented in the standard of care among CF patients, focusing on two main aspects [6,7]. The first aspect is the need to keep the airways clear of mucus secretions using physiotherapy, nebulized recombinant human DNase (rhDNase) and/or hypertonic saline (HS).…”

Section: Introductionmentioning

confidence: 99%

“…The first aspect is the need to keep the airways clear of mucus secretions using physiotherapy, nebulized recombinant human DNase (rhDNase) and/or hypertonic saline (HS). rhDNase in particular has been widely used since the mid-1990s and has participated to achieve an improved airway clearance, improved pulmonary function, fewer exacerbations and, most importantly, an improved survival [6]. The second aspect is the suppression of bacterial colonization and infection with intravenous and/or inhaled antibiotics, [6] such as tobramycin, colistin and aztreonam lysine for inhalation (AZLI).…”

Section: Introductionmentioning

confidence: 99%

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Bronchial Hyperreactivity Related to Inhalation Therapy in Cystic Fibrosis Patients

Eyns¹

2014

J Pulm Respir Med

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It is presumed that bronchial hyperreactivity (BHR) can occur with any inhaled agent and may be a reason for discontinuation of inhalation therapy in cystic fibrosis (CF) patients. On the other hand, inhalation of antibiotics is being increasingly used to eradicate or treat infections.This review focuses on identifying the mechanisms of BHR for a better understanding of its impact on inhalation treatment. BHR in CF is suggested to be secondary to underlying airway disease (associated to poor pulmonary function, chronic inflammation and aerosol distribution) or to be a separate condition occurring more in CF. Furthermore, certain characteristics of the aerosol solution itself, such as the active molecule, chemical additives and particle size, can cause BHR.Recombinant human DNase (rhDNase), hypertonic saline (HS) and the antibiotics tobramycin, colistin and aztreonam lysine for inhalation (AZLI) are frequently used inhalation drugs in the treatment of CF. Prevalence of BHR related to both short and long-term inhalation of these drugs as reported in the literature was investigated. Acute BHR is documented in up to two thirds of CF patients. Despite the widespread use of rhDNase, HS, tobramycin, colistin and AZLI, only one long-term trial looked for, but did not demonstrate, BHR at the end of the trial period.

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“…У особо тяжелых больных с показа-телями объема форсированного выдоха за первую секунду (ОФВ 1 ) менее 30% от должного значения медиана выжи-ваемости увеличилась с 1,2 до 5,3 лет (рис. 3) [7].…”

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Dornase Alfa in Russia: 15 Years Later. Efficacy of the Drug in the Basic Treatment in Children With Cystic Fibrosis

Simonova¹,

Лукина²

2012

Vopr. sovr. pediatr.

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Improved survival at low lung function in cystic fibrosis: cohort study from 1990 to 2007

Abstract: A marked improvement has occurred in the survival of patients with cystic fibrosis with an FEV(1) less than 30% predicted. Secondary analyses suggest that some of this improvement may be due to use of recombinant human DNase.

Cited by 130 publications

References 27 publications

Adult cystic fibrosis care in the 21st century

Adult cystic fibrosis care in the 21st century

Bronchial Hyperreactivity Related to Inhalation Therapy in Cystic Fibrosis Patients

Dornase Alfa in Russia: 15 Years Later. Efficacy of the Drug in the Basic Treatment in Children With Cystic Fibrosis

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