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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 157 (1998), S. 422-426 
    ISSN: 1432-1076
    Keywords: Key words Primary ciliary dyskinesia ; Lung function ; Bronchodilators
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Pulmonary function tests were obtained in 11 patients with primary ciliary dyskinesia. Their mean age was 15 years (range 6–32). Their pulmonary function was obstructive, with a vital capacity (mean ± SD) of 75% ± 20% predicted, a forced expiratory volume in 1s (FEV1) of 63% ± 20% predicted and a raised residual volume of 169% ± 50% predicted. After inhalation of 200 μg of salbutamol the mean change in FEV1 was +13.2% ± 9.6% of the baseline value. In the 10 oldest patients, lung function had been measured at regular intervals during 3–20 years. Interestingly, during childhood and adolescence the evolution was not unfavourable: vital capacity increased by 8% ± 20% and FEV1 remained stable (mean change 0.3% ± 12%). Only 2 patients had an unfavourable evolution. Conclusion At time of diagnosis, patients with primary ciliary dyskinesia have partially reversible obstructive airway disease. During regular follow up and therapy, there is no evidence of a further decline in lung function. Patients with associated immunodeficiency or important damage at the start of therapy may have a worse prognosis.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Key words Cystic fibrosis ; Pseudomonas aeruginosa ; IgA ; antibodies ; IgG antibodies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In patients with cystic fibrosis (CF) and chronic colonisation with Pseudomonas aeruginosa, specific anti-pseudomonal IgG and IgA, as well as serum immunoreactive protein C, WBC and differential count, ESR, pulmonary function and chest radiograph score were determined before and after a 2 week intravenous course of anti-pseudomonal antibiotics in 32 cases of acute exacerbation of pulmonary infection. Specific anti-pseudomonal IgA but not specific anti-pseudomonal IgG decreased significantly after treatment. Log of anti-pseudomonal IgA but not log anti-pseudomonal IgG correlated well with disease severity as assessed by the Brasfield chest radiograph score (r 0.57), forced expiratory volume in 1 s (r 0.6) as well as C-reactive protein (r 0.62).
    Type of Medium: Electronic Resource
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