www.controlled-trials.com (ISRCTN 93153804, ISRCTN 73047833) and www.clinicaltrials.gov (NCT01332175 & NCT02050425).

We explore whether real-time breath analysis by high resolution mass spectrometry is suitable to monitor changes at the metabolic level due to inhaling bronchodilator medication. We compared the breath levels of metabolites in a group of patients (n = 50) at baseline and 10 and 30 min after inhalation of 200 μg salbutamol. The same procedure was performed with a group of controls (n = 48) inhaling a placebo spray. A total of 131 mass spectral features were significantly altered as a result of inhaling medication, but not after inhaling placebo. We found that homologous series of chemical classes correlated strongly with each other, strengthening the notion that certain biochemical processes can be monitored. For example, a series of fatty acids was found to be increased after salbutamol intake, suggesting lipolysis stimulation. Peaks corresponding to salbutamol, its main metabolite salbutamol-4-O-sulfate and formoterol were found to be generally increased in patients inhaling the drugs on an as-needed basis, as compared to non-medicated volunteers. Overall, these results suggest such real-time breath analysis is a useful tool for non-invasive therapeutic drug monitoring.

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Background: In Ehlers-Danlos syndrome (EDS), a group of monogenic disorders affecting connective tissues, obstructive sleep apnoea (OSA) is highly prevalent in adults. The prevalence of OSA in children with EDS is unknown. Objectives: This prospective cross-sectional study aimed at determining the prevalence of OSA in paediatric EDS patients. Methods: Children with EDS (n = 24) were recruited from the Children’s Hospital Zurich and matched to healthy controls. Participants completed home respiratory polygraphy and questionnaires (Sleep-Related Breathing Disorder Scale [SRBD], Epworth Sleepiness Scale [ESS], and Child Health Questionnaire [CHQ]). The American Academy of Sleep Medicine criteria were applied for OSA diagnosis (obstructive apnoea-hypopnoea index [oAHI] ≥1/h). Conditional logistic regression was used to compare the prevalence of OSA and to adjust for possible confounding. Results: OSA was found in 42% of paediatric EDS patients and in 13% of matched controls (OR = 4.5, 95% CI = 0.97–20.83, p = 0.054). The median oAHI was higher in EDS patients than in controls (0.77/h, IQR = 0.19–1.76, vs. 0.24/h, IQR = 0.0–0.60, p < 0.001 adjusted for age, sex, and BMI z-score). EDS patients had lower scores in most CHQ scales and higher SRBD and ESS scores than controls (0.26, IQR = 0.1–0.35, vs. 0.07, IQR = 0–0.19, p = 0.004); 7 ± 4 vs. 5 ± 4, p = 0.033, respectively). Conclusion: OSA is a previously underestimated EDS-related complication increasing disease burden.

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