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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 145 (1986), S. 80-83 
    ISSN: 1432-1076
    Keywords: Cystic fibrosis ; Dopamine-beta-hydroxylase ; Catecholamines
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The enzymatic activity of plasma dopamine-beta-hydroxylase (DBH) was determined in 92 children/adolescents with cystic fibrosis, in 25 parents (heterozygotes) of these patients and in 68 healthy controls. Statistically significant (P〈0.01) lower DBH levels were found in cystic fibrosis patients (17.9±1.4 μmol/min per 1, SEM) compared to the controls (25.5±2.1 μmol/min per 1). In 37 patients plasma norepinephrine and dopamine levels were compared to the enzymatic activity, no significant correlation between these variables being shown (r=-0.134 for norepinephrine; r=0.159 for dopamine). However, due to the large variability of DBH within the groups and due to the lack of relationship of this enzymatic activity to plasma catecholamines, it is concluded that these differences may neither account for the observed elevation of plasma dopamine in cystic fibrosis patients (0.94–6.73 nmol/l vs. 0.01–0.986 nmol/l for controls) nor for their putative autonomic dysfunction.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 152 (1993), S. 382-386 
    ISSN: 1432-1076
    Keywords: Infant whole-body plethysmography ; Wheezy bronchitis ; Infant respiratory distress syndrome ; Cystic fibrosis ; Bronchial hyperreactivity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract There are new techniques which have been developed in order to assess objective functional data concerning the severity and type of abnormalities in infants and children with lung disease. In the present review some applications of the various techniques are given in connection with deductions, which can be based on such functional findings. Insight into the mechanisms leading to lung disease and the institution of appropriate therapeutic guidelines largely depend on whether the degree of pulmonary hyperinflation or restriction, of bronchial obstruction, of bronchial hyperreactivity, of ventilation inequalities and the function of gas exchange can be objectively evaluated. Recent advances in the non-invasive assessment of lung function in infants and children promises progress in this task.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Cystic fibrosis ; Pseudomonas aeruginosa ; Lung involvement ; Survival ; Prognosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To evaluate the prognostic significance of the age at onset of chronicPseudomonas aeruginosa colonization (OPCP) with respect to pulmonary disease progression in patients with cystic fibrosis (CF), a retrospective long-term analysis using annual chest radiographs was performed on 54 CF patients. Thirtyseven patients (68%) were chronically colonized before the age of 12 years (group 1), 17 patients (32%) thereafter (group 2). These two groups did not significantly differ in terms of mean duration of follow up (16.2±5.9 years), sex, CF genotypes, colonization with other respiratory pathogens, supportive medical treatment and death rate during the study period. Chest radiographs were evaluated according to the Chrispin-Norman score, increasing scores representing increasing severity of respiratory disease. In both groups, progression of score means was not accelerated up to 6 years after OCPC (Scores at OCPC set 0; mean score ±SEM 6 years prior to OCPC −5.6±2.0; 10 years after OCPC +3.6±0.7 points). Patients chronically colonized prior to age 12 years (group 1) scored significantly higher between age 2 and 11 years (maximum difference at age 8 years [mean ±SEM]: 9.4±0.7 vs. 4.3±1.3 points;P= 0.002) as compared to group 2. After age 11 years, mean scores were similar in both groups, since in group 2 scores increased rapidly after age 8 years. We conclude that OCPC did not cause an immediate acceleration of CF lung disease judged by serial chest radiographs. Rapid progression in group 2 (OCPC after age 12 years) was independent of OCPC since it occurred earlier. These data indicate that OCPC may be a marker rather than the cause of respiratory disease progression.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 138 (1982), S. 172-175 
    ISSN: 1432-1076
    Keywords: Fusidic acid ; Cystic fibrosis ; Sputum penetration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report the sputum penetration and efficacy of fusidic acid in 16 children with cystic fibrosis (CF) who were hospitalized because of acute respiratory tract infection due to Staphylococcus aureus. Penetration into sputum and response to therapy were evaluated after intravenous and oral drug administration. Oral fusidic acid therapy (approximately 1,000 mg/m2/day) achieved highly satisfactory serum (10–50 μg/ml) and sputum (0.6–4.0 μg/ml) concentrations which were several fold greater than the MIC of the infecting S. aureus strains. The average percentage penetration into sputum during the steady state phase of the concentration-time curve was between 5 and 10%. The efficacy of treatment is reflected by improvement in clinical findings and laboratory data. Eradication of S. aureus from the sputum was documented in 14 of the 16 patients, although 6 months later this was shown to be transient in half of them (7 of 14 patients). No staphylococci resistant to fusidic acid were found. Nine of the 14 children with documented staphylococcal eradication from the sputum had received gentamicin in addition to fusidic acid therapy which could have added antistaphylococcal efficacy. Further investigations are needed to formulate the definitive role of fusidic acid in the antistaphylococcal therapy of patients with CF.
    Type of Medium: Electronic Resource
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