The aim of this study was to evaluate how lung function and growth changed over time in children with cystic fibrosis (CF) colonized with pseudomonas aeruginosa (Pa) compared with those free of the organism. A total of 192 children attended our cystic fibrosis clinic between 1982 and 1992. Sixty-two of these had three or more annual assessments for lung function, and 117 had three or more annual assessments for height and weight. When lung function was expressed as a standard deviation score (SDS), forced expiratory volume in 1 second (FEV1) and forced expiratory flow at 25% of vital capacity (FEF25) decreased significantly more with respect to height in colonized compared with noncolonized children: FEV1, -0.052 verses -0.015 SDS/cm (P < 0.05); FEF25, -0.060 verses -0.007 SDS/cm (P < 0.05); forced vital capacity (FVC), -0.034 versus -0.012 (NS). In actual values those patients colonized with Pa increased their FEV1 by 16.4 versus 31.6 mL/cm (P < 0.01); FVC by 28.8 versus 41.4 mL/cm, P < 0.01; and FEF25 by -0.001 versus 0.015 L/s/cm, P < 0.01. In terms of height, colonized children grew at 5.63 versus 6.96 cm/yr, P < 0.001, and height SDS decreased in colonized compared with noncolonized children at -0.031 verses 0.08 SDS/yr, P < 0.05. Clinically, most children with CF, with or without Pa, grew within +/- 1 SD of the norm for weight and height. However, in terms of lung function despite optimum pulmonary management colonized children deteriorated significantly faster.

Voriconazole may be a useful adjunctive therapy for ABPA in CF. Voriconazole monotherapy appears to be an alternative treatment strategy when oral corticosteroids may not be suitable.

To explore the hypothesis that there is an increased metabolic rate in cystic fibrosis, resting energy expenditure was measured by indirect calorimetry in 23 subjects with cystic fibrosis in a stable clinical state and in 42 normal control subjects. Resting energy expenditure was found to be elevated by an average of 0.45 MJ/24 h [95% confidence interval (CI) = 0.26-0.64, t = 4.91, P less than 0.001] (108 kcal/24 h), or 9.2% above expected values derived from the regression relating resting energy expenditure to whole body weight and sex in control subjects. When related to lean body mass, values were still elevated by 0.36 MJ/24 h (95% CI = 0.18-0.53, t = 4.15, P less than 0.001) (86 kcal/24 h), or 7.2%. The increased values were found to be independent of age, sex, or body size. There were significant correlations between increased values and poor pulmonary function as measured by the ratio of the forced expiratory volume in 1 s to forced vital capacity (r = -0.44, P less than 0.05) and subclinical infection as indicated by the blood leukocyte count (r = 0.40, P less than 0.05). However, the correlations were low, suggesting that other factors may contribute to the increased resting energy expenditure, possibly including the putative metabolic defect in cystic fibrosis.

Background: Diagnostic error is a significant problem in specialities characterised by diagnostic uncertainty such as primary care, emergency medicine and paediatrics. Despite wide-spread availability, computerised aids have not been shown to significantly improve diagnostic decision-making in a real world environment, mainly due to the need for prolonged system consultation. In this study performed in the clinical environment, we used a Web-based diagnostic reminder system that provided rapid advice with free text data entry to examine its impact on clinicians' decisions in an acute paediatric setting during assessments characterised by diagnostic uncertainty.

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