Long-Term Inhaled Dry Powder Mannitol in Cystic Fibrosis

Aitken, Moira L.; Bellon, G.; Boeck, K. De; Flume, Patrick A.; Fox, Howard; Geller, David E.; Haarman, Eric G.; Hebestreit, Helge; Lapey, Allen; Schou, I. Manjula; Zuckerman, Jonathan B.; Charlton, Brett

doi:10.1164/rccm.201109-1666oc

Cited by 122 publications

(92 citation statements)

References 24 publications

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“…It is difficult to compare results with those published in the literature since results are presented in terms of relative rather than absolute differences and sometimes with absolute changes in lung volume data rather than percent-predicted data. In two recent identical trials of mannitol in CF in which FEV 1 was expressed in the same way as in our trial (difference versus placebo in percent-predicted FEV 1 was 2.4%, 95% CI 0.9 to 3.9 in one trial [12] and 1.9%, 95% CI -0.02 to 3.8 in the other), changes were similar in magnitude to those demonstrated for tiotropium [13]. Treatment differences must also be considered in light of the study design and short duration of the trials.…”

Section: Discussionsupporting

confidence: 80%

“…However, in the phase 3 trial, patients ≤11years in the placebo group showed an improvement in percent-predicted FEV 1 AUC 0-4h over time, which suggests a learning effect during the trial among the younger patients, who were less skilled in performing spirometry. In a recent study of inhaled mannitol, a sustained, significant (p<0.001) improvement in FEV 1 was seen in the control group [12], but the improvement was not significant in another similarly designed study (p=0.059) in similar populations [13]. Interestingly, in the pooled analysis, significant improvements in the mannitol versus control group in FEV 1 occurred in patients aged ≥18 years old but not in younger participants [14].…”

Section: Discussionmentioning

confidence: 93%

See 1 more Smart Citation

Tiotropium Respimat® in cystic fibrosis: Phase 3 and Pooled phase 2/3 randomized trials

Ratjen

Koker

Geller

et al. 2015

Journal of Cystic Fibrosis

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Section: Discussionsupporting

confidence: 80%

Section: Discussionmentioning

confidence: 93%

Tiotropium Respimat® in cystic fibrosis: Phase 3 and Pooled phase 2/3 randomized trials

Ratjen

Koker

Geller

et al. 2015

Journal of Cystic Fibrosis

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“…Although mannitol is currently used as stimulus in bronchial provocation tests (Figure 1), and thus may cause bronchoconstriction, BHR was reported in only two patients by Bilton et al [115] and was not observed by Aitken et al [116].…”

Section: Mannitolmentioning

confidence: 98%

“…This will increase the volume of the airway surface liquid and improves mucociliary clearance [115][116][117]. To date, two long-term trials (26 weeks) investigated the efficacy and safety of inhaled dry powder mannitol in CF patients [115,116] and a third long-term trial is running (NCT02134353).…”

Section: Mannitolmentioning

confidence: 99%

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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“…Prve raziskave o vplivu na pljučno funkcijo so bile obetavne, saj je manitol izboljšal FEV 1 za 7 % v primerjavi s placebom.34 Podobne zaključke je imela raziskava CF301.35 V nedavno objavljeni raziskavi (CF302) pa ugotavljajo relativno izboljšanje FEV 1 v skupini, ki je prejemala manitol v odmerku 2 × 400mg, le za 3,7 % v primerjavi s kontrolno skupino, ki pa je prejemala manitol v odmerku 2 × 50 mg. Med obema skupina ni bilo statistično pomembne razlike v številu poslabšanj. 36 Raziskovali so tudi učinek zmanjševanja aktivnosti natrijevih kanalčkov z amiloridom, tako bi zmanjšali absorbcijo natrija in vode iz površine dihalnih poti v epitelne celice ter preprečili izsuševanje sluzi na površini dihalnih poti. Metaanaliza petih randomiziranih s placebom kontroliranih raziskav o amiloridu ni pokazala izboljšanja pljučne funkcije ali čiščenja sluzi pri ljudeh s CF.37…”

Section: Izboljševanje Vlažnosti Sluzi V Dihalnih Potehunclassified

Novosti v Obravnavi Pljučne Bolezni Pri Otrocih in Mladostnikih S Cistično Fibrozo

Praprotnik¹,

Pirs²,

Salobir³

et al. 2015

SlovMedJour

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Cystic fibrosis (CF) is a multi-organ disease, affecting mostly lungs and gastrointestinal tract. Data from patient registries show that the survival of patients with CF has progressively improved over the past several decades, as a result of advances in antibiotic treatment, supplementation of pancreatic enzymes, better nutrition and a holistic approach to treatment in CF centres.The purpose of this review is to survey recent developments in the treatment of lung disease in children and adolescents with CF.We describe newborn screening for CF.When chronic respiratory insufficiency occurs, lung transplantation becomes a very important issue.Lung disease is the most common cause of morbidity and mortality in CF patients. Emerging new therapies are targeted at all points in the pathogenesis of lung disease, from drugs that treat infection and inflammation in the airways to gene transfer studies and to drugs that augment airway surface liquid height. A number of antibacterial agents formulated for inhalation are at various stages of study and there are several anti-inflammatory candidate drugs in clinical trials. The most important development in the recent years is modulation of the abnormal protein that causes CF, the cystic fibrosis transmembrane regulator (CFTR), where drugs are targeted at specific defects in the transcription, processing or functioning.When chronic respiratory insufficiency occurs, lung transplantation becomes a very important issue. The role of the CF nurse, who has responsibilities in educating and teaching clinical skills to patients and families, is described.

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Long-Term Inhaled Dry Powder Mannitol in Cystic Fibrosis

Cited by 122 publications

References 24 publications

Tiotropium Respimat® in cystic fibrosis: Phase 3 and Pooled phase 2/3 randomized trials

Tiotropium Respimat® in cystic fibrosis: Phase 3 and Pooled phase 2/3 randomized trials

Bronchial Hyperreactivity Related to Inhalation Therapy in Cystic Fibrosis Patients

Novosti v Obravnavi Pljučne Bolezni Pri Otrocih in Mladostnikih S Cistično Fibrozo

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