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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.

Increased cough frequency is a common symptom associated with infective pulmonary exacerbations of cystic fibrosis (CF), but subjective assessment of cough is very unreliable. The aims of this study were: 1) to validate a modification of our previously described ambulatory cough recording device (LR 100); 2) to determine how accurately children with CF assess levels of cough; and 3) to assess the change in cough in children with CF when treated with intravenous antibiotics for a respiratory exacerbation, and whether the children themselves were able accurately to perceive any change. Fourteen CF children (aged 13.6 +/- 2.6 years) were included in the study. All 14 children were simultaneously recorded with the LR 100 cough recorder and a conventional tape recorder during a chest physiotherapy session on the first or second day of admission for an infective exacerbation diagnosed by standard criteria. The difference between the two was an underestimate of 0.5 epoch/session by the tape recorder. Ten children were recorded on admission with the LR 100 cough recorder for a complete cycle (17 hr and 40 min), and we also assessed their day and night-time cough with cough scores and visual analogue scores (VAS). In 8 of these children, the same assessments were repeated on discharge. There was no significant correlation between any of the admission or discharge cough scores, daytime or night-time cough scores, and daytime or night-time VAS scores, with the actual number of coughs recorded on the LR 100 cough recorder. For the 8 children who had cough monitoring on admission and on discharge, there was no significant improvement in daytime or night-time cough scores or VAS on discharge, despite significant improvements in spirometry. There was also no significant improvement on daytime and night-time cough counts with the cough monitor on discharge, and no significant correlation with changes in lung function. There were weak correlations only between change in daytime VAS scores and change in forced expired volume in 1 sec (r = -0.794, P = 0.019) and forced vital capacity (r = -0.723, P = 0.04). In conclusion, we describe a reliable and well-tolerated method for obtaining cough counts objectively. The use of this objective method showed that CF children did not assess their cough frequency well. In addition, treatment of respiratory exacerbation improved neither subjective nor objective measures of cough in CF children.

Osteopenia is increasingly recognized in adults with cystic fibrosis (CF), and is potentially related to vitamin D deficiency in both adulthood and childhood. Vitamin D supplements are recommended and prescribed to all pancreatic-insufficient patients. We aimed to ascertain whether vitamin D deficiency in children with CF was prevalent. 25-hydroxyvitamin D (25-OHD) was measured in 290 children attending a specialist pediatric CF clinic for annual assessment. 25-OHD levels were compared with reference values and to other biochemical markers, lung function, and growth. Levels were also analyzed by pancreatic status and by the presence of CF-related liver disease. Median 25-OHD was 65 (range, 9-190) nmol/l. One percent had levels below 15 nmol/l, and 6% had levels less than 25 nmol/l. Levels were lower in adolescents (P < 0.001) and during the "winter" months (P < 0.001). No relationship was found with pancreatic status or liver disease. In conclusion, the majority of children had normal 25-OHD levels. Interpretation is difficult due to a lack of knowledge of optimal levels of 25-OHD required for healthy bone accretion. Lower levels in adolescents may be a precursor to low levels in adulthood, and did not seem to be simply related to poor compliance with supplementation. This may reflect normal physiology.