262: Pilot study assessing the feasibility and safety of virtual oxygen assessments in the adult cystic fibrosis population

Objective. As part of a study to determine population-based frequencies of CFTR mutations in an ethnically diverse, midwestern cystic fibrosis (CF) population, clinical histories were studied in 119 CF patients. Methodology. We sought to examine the association between genotype as characterized by the ΔF508 and 11 other commonly occurring mutations and clinical parameters including age at diagnosis, clinical presentation, sweat chloride level, chest roentgenogram score, clinical scores, pulmonary function test results, percent weight for height, and presence of associated CF complications. Results. Age at diagnosis of CF was significantly associated with homozygosity for ΔF508 (mean age at diagnosis ± SE: 1.7 ± 0.3 years for ΔF508/ΔF508 vs 3.9 ± 0.9 years for ΔF508/other and other/other; P = .03). No other age-adjusted clinical parameter was significantly associated with ΔF508 or any other genotype. Conclusion. These data suggest that in this sample of CF patients, ΔF508 genotype is not predictive of disease severity. The lack of association between disease severity and genotype in this ethnically diverse sample may reflect the presence of more severe undetected mutations in our sample, or the effects of modifying genes at other, non-CF loci.

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The Prevalence of the Deletion F508 in a Belgian Cystic Fibrosis Population

Advances in Experimental Medicine and Biology - The Identification of the CF (Cystic Fibrosis) Gene ◽

10.1007/978-1-4684-5934-0_40 ◽

1991 ◽

pp. 369-370

Author(s):

W. Lissens ◽

M. Bonduelle ◽

A. Malfroot ◽

I. Dab ◽

I. Liebaers

Keyword(s):

Cystic Fibrosis ◽

Cystic Fibrosis Population

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A pilot study comparing two physiotherapy techniques in patietnts with cystic fibrosis

South African Journal of Physiotherapy ◽

10.4102/sajp.v60i2.183 ◽

2004 ◽

Vol 60 (2) ◽

Cited By ~ 7

Author(s):

S. M. Milne ◽

C. J. Eales

Keyword(s):

Cystic Fibrosis ◽

Pilot Study ◽

Lung Function ◽

Statistical Difference ◽

Positive Pressure ◽

Lung Function Tests ◽

Bronchial Secretions ◽

Group A ◽

Group B ◽

Minimal Research

The flutter is a simple hand held device designed to facilitate the mobilisation of excess bronchial secretions by means of oscillating positive pressure. Traditionally patients at the Johannesburg Hospital Cystic Fibrosis clinic used the active cycle of breathing technique as a means of facilitating secretion mobilisation and clearance. When the flutter became available in South Africa in 1999 many cystic fibrosis patients wanted to change to this technique. Minimal research has been conducted comparing these two techniques. The aim of this pilot study was therefore to determine which technique is more effective in the mobilisation of secretions in cystic fibrosis patients. The pilot study was conducted on seven cystic fibrosis patients (mean age 28 years, range 16-42 years) admitted to the Johannesburg Hospital for antibiotic therapy. The study lasted four days and consisted of two treatment days separated by a washout day on which no physiotherapy was performed. Patients randomised into Group A performed the flutter technique on day two and the active cycle of breathing technique on day four. Group B performed the active cycle of breathing technique on day two and the flutter on day four. The techniques were performed twice a day for 15 minutes. The measurements taken were daily 24-hour sputum samples and daily lung function tests. A questionnaire to determine patient preference to a technique concluded the study. The results showed no statistical difference between the two techniques with regard to sputum weight or lung function (p<0.05). The questionnaire indicated that on a whole, patients had no preference for a technique.

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