The future of cystic fibrosis care: a global perspective

The role of respiratory viruses in cystic fibrosis.

Pseudomonas aeruginosa (PA) is associated with a distinctive smell produced by a combination of volatile compounds (VCs). Selected ion flow tube mass spectrometry (SIFT-MS) provides a novel and rapid methodology for rapid, accurate detection of trace quantities (parts per billion; ppb) of VCs in air. We studied the VCs produced by different isolates of PA cultures in vitro from patients with cystic fibrosis. Twenty-one patients with cystic fibrosis provided sputum and cough swab samples for culture. These were used to inoculate blood agar (BA) and Pseudomonas-selective media (PSM). These plates were incubated for 48 hr at 37°C inside sealed plastic bags. The air surrounding the samples after 48 hr (headspace) was analyzed using SIFT-MS. PA growth was commonly associated with the production of significant quantities of VCs, notably hydrogen cyanide gas (HCN). This was detectable in the headspace of 15/22 of PA-positive samples. In contrast, it was only seen in the headspace of 1/13 control samples (6 sterile plates and 7 plates with only mixed upper respiratory tract flora). The concentration of HCN was significantly higher above PA-positive samples than above other bacterial growth (P < 0.01), and in our study, levels of HCN greater than 100 ppb were a sensitive (68%) and highly specific (100%) biomarker of PA. SIFT-MS can detect a range of VCs from PA in vitro. HCN may be a specific indicator of PA infection in vivo, and offers promise as a biomarker for noninvasive detection of PA infection by breath analysis. Pediatr Pulmonol. © 2005 Wiley-Liss, Inc.

Detection of volatile compounds emitted by Pseudomonas aeruginosa using selected ion flow tube mass spectrometry.

Background Long-term benefits of newborn screening (NBS) for cystic fibrosis (CF) have been established with respect to nutritional status, but effects on pulmonary health remain unclear. Hypothesis With early diagnosis and commencement of standardised treatment, lung function at ∼3 months of age is normal in NBS infants with CF. Methods Lung clearance index (LCI) and functional residual capacity (FRC) using multiple breath washout (MBW), plethysmographic (pleth) FRC and forced expirations from raised lung volumes were measured in 71 infants with CF (participants in the London CF Collaboration) and 54 contemporaneous healthy controls age ∼3 months. Results Compared with controls, and after adjustment for body size and age, LCI, FRC MBW and FRC pleth were significantly higher in infants with CF (mean difference (95% CI): 0.5 (0.1 to 0.9), p=0.02; 0.4 (0.1 to 0.7), p=0.02 and 0.9 (0.4 to 1.3), p 0.5 ) and flows (FEF 25–75 ) were significantly lower (−0.9 (−1.3 to −0.6), p 1.96 z-scores) and 25% (17/68) an abnormally low FEV 0.5 (below −1.96 z-scores). While only eight infants with CF had abnormalities of LCI and FEV 0.5 , using both techniques identified abnormalities in 35% (24/68). Hyperinflation (FRC pleth >1.96 z-scores) was identified in 18% (10/56) of infants with CF and was significantly correlated with diminished FEF 25–75 (r=−0.43, p 0.5 . Conclusion Despite early diagnosis of CF by NBS and protocol-driven treatment in specialist centres, abnormal lung function, with increased ventilation inhomogeneity and hyperinflation and diminished airway function, is evident in many infants with CF diagnosed through NBS by 3 months of age.

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Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening

Since the late 1970s when the potential of the immunoreactive trypsinogen assay for early identification of infants with cystic fibrosis was first recognised, the performance of newborn blood spot screening (NBS) has been continually assessed and its use has gradually expanded. NBS for cystic fibrosis is a cost-effective strategy and, if standards of care are fully implemented and robust management pathways are in place, has a positive effect on clinical outcomes. In the past decade, NBS has undergone rapid expansion and an unprecedented number of infants with cystic fibrosis have access to early diagnosis and care. Cystic fibrosis NBS has now moved on from the development phase and is entering an era of consolidation. In the future, research should focus on the rationalisation and optimisation of existing programmes, with particular attention to bioethical implications such as unwanted detection of carriers and inconclusive diagnoses.

Newborn screening for cystic fi brosis

We audited prospectively 322 cough swabs taken from cystic fibrosis children and compared cough swabs with concomitant sputum samples in 30 expectorating patients. A positive cough swab is a strong predictor of sputum culture. However, a negative cough swab does not rule out infection. Persistent symptoms should be further investigated.

https://adc.bmj.com/content/archdischild/85/5/438.full.pdf

Use of cough swabs in a cystic fibrosis clinic

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Use of cough swabs in a cystic fibrosis clinic