A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis

Eigen, Howard; Rosenstein, Beryl J.; FitzSimmons, Stacey C.; Schidlow, Daniel V.

doi:10.1016/s0022-3476(95)70343-8

Cited by 317 publications

(205 citation statements)

References 14 publications

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“…Conventional treatment of pulmonary disease in CF is directed at managing infection, using airway clearance techniques and administering systemic and nebulized antibiotics to reduce bacterial stimulation of inflammation in the lungs (22). However, neutrophilic airway inflammation may be an important therapeutic target in CF, and antiinflammatory agents may slow pulmonary deterioration (23,24), measured as a reduced annual rate of decline in the FEV 1 .…”

Section: Discussionmentioning

confidence: 99%

Quantifying Pulmonary Inflammation in Cystic Fibrosis with Positron Emission Tomography

Chen

Ferkol

Mintun

et al. 2006

Am J Respir Crit Care Med

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Section: Discussionmentioning

confidence: 99%

Quantifying Pulmonary Inflammation in Cystic Fibrosis with Positron Emission Tomography

Chen

Ferkol

Mintun

et al. 2006

Am J Respir Crit Care Med

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“…Examination of spontaneously expectorated sputum reveals a predominantly neutrophilic profile similar to in CF [98]. Antiinflammatory strategies such as alternate-day prednisolone have been shown to be effective in CF [99], but need to be formally trialled in PCD before they could be recommended. There are no data on which to recommend or avoid inhaled corticosteroids; by analogy with CF [100], they are probably best avoided unless they can be shown to be of definite benefit in an individual patient.…”

Section: Anti-inflammatory Strategiesmentioning

confidence: 96%

Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children

Barbato¹,

Frischer²,

Kuehni³

et al. 2009

Eur Respir J

465

587

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Primary ciliary dyskinesia (PCD) is associated with abnormal ciliary structure and function, which results in retention of mucus and bacteria in the respiratory tract, leading to chronic oto-sino-pulmonary disease, situs abnormalities and abnormal sperm motility.The diagnosis of PCD requires the presence of the characteristic clinical phenotype and either specific ultrastructural ciliary defects identified by transmission electron microscopy or evidence of abnormal ciliary function.Although the management of children affected with PCD remains uncertain and evidence is limited, it remains important to follow-up these patients with an adequate and shared care system in order to prevent future lung damage.This European Respiratory Society consensus statement on the management of children with PCD formulates recommendations regarding diagnostic and therapeutic approaches in order to permit a more accurate approach in these patients. Large well-designed randomised controlled trials, with clear description of patients, are required in order to improve these recommendations on diagnostic and treatment approaches in this disease.

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“…Another anti-inflammatory therapy, oral glucocorticoids administered every other day, was evaluated in a 4 year prospective, double-blind, randomized, placebo controlled study of 285 patients. Glucocorticoids, 1 mg/kg every other day, improved pulmonary function in patients colonized with P. aeruginosa at baseline, and decreased IgG levels (Eigen et al, 1995). However, glucocorticoid therapy was not recommended as a routine therapy because persistent growth impairment was noted 6-7 years after this study was completed in boys 18 years of age or older (Lai et al, 2000).…”

Section: Anti-inflammatory and Antiprotease Therapies In Cfmentioning

confidence: 97%

Proteases and cystic fibrosis

Voynow

Fischer

Zheng

2008

The International Journal of Biochemistry & Cell Biology

175

159

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Cystic fibrosis is the most common, inherited fatal disease in Caucasians. The major cause of morbidity and mortality is chronic lung disease due to infection and inflammation in the airways leading to bronchiectasis and respiratory failure. The signature pathologic features of CF lung disease including abnormal mucus obstructing airways, chronic infection with S. aureus, P. aeruginosa and other gram negative bacteria, and a robust neutrophil-dominant airway inflammation, are exacerbated by unopposed proteases present at high concentrations in the ASL. There is strong evidence that proteases, particularly neutrophil elastase, contribute to the pathology of CF by impairing mucociliary clearance, interfering with innate immune functions, and perpetuating neutrophilic inflammation. The mechanisms employed by proteases to impact airway function in CF will be reviewed.

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A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis

Cited by 317 publications

References 14 publications

Quantifying Pulmonary Inflammation in Cystic Fibrosis with Positron Emission Tomography

Quantifying Pulmonary Inflammation in Cystic Fibrosis with Positron Emission Tomography

Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children

Proteases and cystic fibrosis

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