Inhaled mannitol for the treatment of cystic fibrosis

Hurt, Katharine; Bilton, Diana

doi:10.1586/ers.11.87

Cited by 24 publications

(17 citation statements)

References 40 publications

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“…Indeed, it may provide an alternative mechanism on how hypertonic saline or inhaled mannitol helps ameliorate lung inflammation in cystic fibrosis patients, 84,85 since this disease has been recognized recently to harbor defective autophagy and to benefit from its induction by other stimuli. 86 It may also be of relevance for kidney inflammation since immune cells recruited to the kidney medulla become exposed within seconds to a steep increase of environmental osmolality and since autophagy has been implicated in various immune cell functions including phagocytosis and antigen presentation.…”

Section: Discussionmentioning

confidence: 99%

Hypertonic stress promotes autophagy and microtubule-dependent autophagosomal clusters

et al. 2013

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Section: Discussionmentioning

confidence: 99%

Hypertonic stress promotes autophagy and microtubule-dependent autophagosomal clusters

et al. 2013

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“…Adverse events suggestive of local tolerability, such as cough, are not unexpected, given the dry powder formulation. 31 Subject satisfaction, as measured by convenience of use, was higher with TIP than with TIS in all age groups.…”

Section: Discussionmentioning

confidence: 83%

Tobramycin Inhalation Powder in Cystic Fibrosis Patients: Response by Age Group

et al. 2013

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BACKGROUND: Tobramycin powder for inhalation (TIP) is a drug-device combination designed to reduce treatment time and improve ease of use compared with tobramycin inhalation solution (TIS) in cystic fibrosis (CF) patients. However, the ability of patients to use dry powder inhalers, and the efficacy of the treatments, may vary by age. METHODS: The "Establish a New Gold Standard for Efficacy and Safety With Tobramycin in Cystic Fibrosis" (EAGER) trial was a randomized, 24-week, multicenter, open-label, parallel-group study designed to evaluate the safety of TIP versus TIS in 553 subjects, ages > 6 years, with CF and P. aeruginosa infection. The main efficacy end point was percent-of-predicted FEV 1 at week 20 (end of third cycle of treatment). A post hoc analysis was undertaken in 517 subjects who took > 1 dose of study medication, to evaluate the relative efficacy and safety of TIP and TIS by age group: > 6 to < 13 y (children, n ‫؍‬ 46); > 13 to < 20 y (adolescents, n ‫؍‬ 114); and > 20 y (adults, n ‫؍‬ 357). RESULTS: Improvements in percent-of-predicted FEV 1 from baseline to end of cycle 3 were greatest in the children for both TIP and TIS. The treatment differences (TIP ؊ TIS) were 4.7% (85% CI ؊1.2 to 10.6), 3.7% (85% CI ؊0.1 to 7.5), and ؊0.8% (85% CI ؊3.1 to 1.5) in children, adolescents, and adults, respectively. Sputum P. aeruginosa density decreased from baseline with both treatments, with comparable treatment differences across the age groups after 3 cycles: children ؊0.93 (85% CI ؊2.4 to 0.5), adolescents ؊0.17 (85% CI ؊1.2 to 0.8), and adults ؊0.89 (85% CI ؊1.3 to ؊0.4). Overall, subject satisfaction scores were greater in all subjects with TIP, irrespective of age group. With the exception of cough and dysphonia, the safety profile of TIP was comparable to TIS, irrespective of age. CONCLUSIONS: TIP is comparable to TIS in efficacy outcomes and safety profile but had greater patient satisfaction in all the age groups.

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“…Ten capsules of 40 mg each make up the standard dose of 400 mg, each requiring a single individual inhalation. This process takes less than 5 min [21]. …”

Section: Hyperosmolar Therapymentioning

confidence: 99%

Inhaled Interventions in Cystic Fibrosis: Mucoactive and Antibiotic Therapies

Hurt¹,

Bilton²

2014

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Treatment of cystic fibrosis lung disease has developed from an understanding of the abnormal airway surface liquid resulting from a lack of function of the cystic fibrosis transmembrane regulator protein. Mucus plugging resulting in infection and inflammation leads to airway wall destruction and bronchiectasis. Inhaled therapies have formed the backbone of treatments. In combination with antibiotics delivered direct to the airway, mucoactive drugs, including mucolytics and hyperosmolar agents, are utilised to improve mucociliary clearance to reduce infection and inflammation. Until recently, airway therapies were delivered as nebulisers, but we have now entered the era of dry powder inhalers for treatment of cystic fibrosis lung disease. The theory and practice of these therapies are discussed in this review.

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Inhaled mannitol for the treatment of cystic fibrosis

Cited by 24 publications

References 40 publications

Hypertonic stress promotes autophagy and microtubule-dependent autophagosomal clusters

Hypertonic stress promotes autophagy and microtubule-dependent autophagosomal clusters

Tobramycin Inhalation Powder in Cystic Fibrosis Patients: Response by Age Group

Inhaled Interventions in Cystic Fibrosis: Mucoactive and Antibiotic Therapies

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