The Prognosis of Cystic Fibrosis - A Clinician's Perspective

Lebecque, Patrick

doi:10.5772/30660

Cited by 2 publications

(2 citation statements)

References 207 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“… 3 , 49 , 50 Over the long-term clinical course of CF, FEV 1 is considered a measure of irreversible damage to the large airways 3 and, once it is below 30% predicted, a significant predictor of mortality. 51 Improved survival and lack of deterioration in FEV 1 over time 4 , 48 , 52 – 54 make reliance on FEV 1 as a primary outcome measure in clinical practice and research challenging. 55 Clinically, FEV 1 represents the influence of multiple treatments on various components of CF disease, and therefore it cannot measure the specific influence of a particular intervention, such as airway clearance, over time.…”

Section: Discussionmentioning

confidence: 99%

Effects of Autogenic Drainage on Sputum Recovery and Pulmonary Function in People with Cystic Fibrosis: A Systematic Review

Morgan¹,

Osterling²,

Gilbert

et al. 2015

Physiotherapy Canada

View full text Add to dashboard Cite

Purpose: To determine the effects of short- and long-term use of autogenic drainage (AD) on pulmonary function and sputum recovery in people with cystic fibrosis (CF). Methods: The authors conducted a systematic review of randomized and quasi-randomized clinical trials in which participants were people with CF who use AD as their sole airway clearance technique. Results: Searches in 4 databases and secondary sources using 5 key terms yielded 735 articles, of which 58 contained the terms autogenic drainage and cystic fibrosis. Ultimately, 4 studies, 2 of which were long term, were included. All measured forced expiratory volume in 1 second (FEV1) and found no change. The long-term studies were underpowered to detect change in FEV1; however, the short-term studies found a clinically significant sputum yield (≥4 g). Conclusion: AD has been shown to produce clinically significant sputum yields in a limited number of investigations. The effect of AD on the function of the pulmonary system remains uncertain, and questions have emerged regarding the appropriateness of FEV1 as a valid measure of airway clearance from peripheral lung regions. Further consideration should be given to the use of FEV1 as a primary measure of the effect of AD.

show abstract

Section: Discussionmentioning

confidence: 99%

Effects of Autogenic Drainage on Sputum Recovery and Pulmonary Function in People with Cystic Fibrosis: A Systematic Review

Morgan¹,

Osterling²,

Gilbert

et al. 2015

Physiotherapy Canada

View full text Add to dashboard Cite

show abstract

“…Several factors influencing the prognosis in a child with CF can be divided into following three groups, i.e., factors associated with quality of care, genetics and environment [74]. In Indian perspective, factors related to quality of care stands out with delayed diagnosis and advanced stage of the disease at diagnosis being adversely related to outcome.…”

Section: Prognosis Of Cfmentioning

confidence: 99%

Cystic Fibrosis in India: Past, Present and Future

Kabra¹

2015

PMRR

View full text Add to dashboard Cite

Cystic Fibrosis (CF), a genetic, life-limiting, multisystem disease of the Caucasians, does exist in India. Though the exact prevalence is unknown, it is perhaps more common than once thought of. The children with CF in India are diagnosed late, but the clinical features are similar to the patients from rest of the world. Indian patients differ from their counterparts from developed world in being frequently malnourished, having clinical evidence of fat soluble vitamin deficiencies and more chances of being colonized with Pseudomonas. Diagnostic facilities in India are scarce. Mutation profile is different with a lower prevalence of ΔF508. Management of CF in India is difficult due to inadequately trained manpower, lack of financial support, limited availability and high cost of pharmacologic agents. The determinants of early death in Indian children with CF include: severe malnutrition, colonization with Pseudomonas at the time of diagnosis, more than four episodes of lower respiratory infection per year and age of onset of symptoms before 2 months of age. There is need to create awareness amongst pediatricians, develop diagnostic facilities and management protocols based on locally available resources.

show abstract

The Prognosis of Cystic Fibrosis - A Clinician's Perspective

Cited by 2 publications

References 207 publications

Effects of Autogenic Drainage on Sputum Recovery and Pulmonary Function in People with Cystic Fibrosis: A Systematic Review

Effects of Autogenic Drainage on Sputum Recovery and Pulmonary Function in People with Cystic Fibrosis: A Systematic Review

Cystic Fibrosis in India: Past, Present and Future

Contact Info

Product

Resources

About