SERS spectroscopy for detection of hydrogen cyanide in breath from children colonised with P. aeruginosa

There is a need for a fast and non-invasive tool to detect Pseudomonas aeruginosa airway colonisation in cystic fibrosis (CF) patients unable to expectorate.</p

Pulmonary function testing in children's interstitial lung disease

The use of pulmonary function tests (PFTs) has been widely described in airway diseases like asthma and cystic fibrosis, but for children's interstitial lung disease (chILD), which encompasses a broad spectrum of pathologies, the usefulness of PFTs is still undetermined, despite widespread use in adult interstitial lung disease. A literature review was initiated by the COST/Enter chILD working group aiming to describe published studies, to identify gaps in knowledge and to propose future research goals in regard to spirometry, whole-body plethysmography, infant and pre-school PFTs, measurement of diffusing capacity, multiple breath washout and cardiopulmonary exercise tests in chILD. The search revealed a limited number of papers published in the past three decades, of which the majority were descriptive and did not report pulmonary function as the main outcome.PFTs may be useful in different stages of management of children with suspected or confirmed chILD, but the chILD spectrum is diverse and includes a heterogeneous patient group in all ages. Research studies in well-defined patient cohorts are needed to establish which PFT and outcomes are most relevant for diagnosis, evaluation of disease severity and course, and monitoring individual conditions both for improvement in clinical care and as end-points in future randomised controlled trials

National, clinical cohort study of late effects among survivors of acute lymphoblastic leukaemia:The ALL-STAR study protocol

Introduction More than 90% of patients diagnosed with childhood acute lymphoblastic leukaemia (ALL) today will survive. However, half of the survivors are expected to experience therapy-related chronic or late occurring adverse effects, reducing quality of life. Insight into underlying risk trajectories is warranted. The aim of this study is to establish a Nordic, national childhood ALL survivor cohort, to be investigated for the total somatic and psychosocial treatment-related burden as well as associated risk factors, allowing subsequent linkage to nation-wide public health registers.Methods and analysis This population-based observational cohort study includes clinical follow-up of a retrospective childhood ALL survivor cohort (n=475), treated according to a common Nordic ALL protocol during 2008–2018 in Denmark. The study includes matched controls. Primary endpoints are the cumulative incidence and cumulative burden of 197 health conditions, assessed through self-report and proxy-report questionnaires, medical chart validation, and clinical examinations. Secondary endpoints include organ-specific outcome, including cardiovascular and pulmonary function, physical performance, neuropathy, metabolic disturbances, hepatic and pancreatic function, bone health, oral and dental health, kidney function, puberty and fertility, fatigue, and psychosocial outcome. Therapy exposure, acute toxicities, and host genome variants are explored as risk factors.Ethics and dissemination The study is approved by the Regional Ethics Committee for the Capital Region in Denmark (H-18035090/H-20006359) and by the Danish Data Protection Agency (VD-2018–519). Results will be published in peer-reviewed journals and are expected to guide interventions that will ameliorate the burden of therapy without compromising the chance of cure

Clinical value of measurement of pulmonary radioaerosol mucociliary clearance in the work up of primary ciliary dyskinesia

BACKGROUND: We aimed to evaluate and define the general clinical applicability and impact of pulmonary radioaerosol mucociliary clearance (PRMC) on the work up of patients suspected of having primary ciliary dyskinesia (PCD). In addition, we wanted to evaluate the accuracy of the reference values used in the PRMC test. METHODS: Measurement of PRMC after inhalation of (99m)Tc-albumin colloid aerosol was carried out on 239 patients (4–75 years of age) during a 9-year period. All were referred to the nuclear medicine department because of clinical suspicion of PCD. The results were compared primarily to results from nasal ciliary function testing, to electron microscopic (EM) examination of the ultrastructure of the cilia, and to the final clinical diagnosis. RESULTS: Of the 239 patients, 27 ended up with a final clinical diagnosis of definitive PCD. No patients with a PRMC test that was normal or otherwise not consistent with PCD ended up with PCD as final clinical diagnosis (though a minority of patients in this group ended up unresolved in regard to PCD). Forty percent of patients with an abnormal PRMC test ended up with PCD as final clinical diagnosis. Furthermore, the PRMC test had a high rate of conclusive results (90 %). Children <14 years of age with normal PRMC measurements showed significantly faster lung clearance than adults with similarly normal PRMC measurements. CONCLUSIONS: To this date, PRMC is the only test providing evaluation of the mucociliary clearance of the entire lung. Its greatest strength is its ability to reject a suspected PCD diagnosis with great certainty. In our material, this accounted for 2/3 of referred patients. In addition, the test has a high rate of conclusive results. According to our analyses, reference equations on children would benefit from updated data

Success rates and feasibility of nasal Nitric Oxide sampling during tidal breathing and velum closure modality, using hand-held and stationary analysers in patients with primary ciliary dyskinesia, cystic fibrosis and in healthy subjects.

<p>Hand-held nNO was measured by sampling rate of both 2 ml/s (MINO2) and 5 ml/s (MINO5). Two stationary analysers (CLD 88sp and NIOX Flex) were employed. Both non-velum closure (nVC) and velum closure (VC) results are shown. §: VC accomplished by standard manoeuvre during exhalation against resistance in a mouthpiece. The fraction of cooperative measurements and according success rates are shown for each test modality, with age and diagnoses given for uncooperative individuals. TB: Tidal Breathing, BH: Breath Hold, PCD: Primary Ciliary Dyskinesia, CF: Cystic Fibrosis, HS: Healthy Subjects.</p

Characteristics, diagnostic tests and respiratory infections in participating subjects, cystic fibrosis patients and patients with primary ciliary dyskinesia.

§<p>: Pulmonary Radioaerosol Mucociliary Clearance – number of abnormal results in number of tested patients – 2 out of 11 were inconclusive. #:Chronic infection defined when bacteria were isolated in more than 50% of respiratory cultures within the last year. <i>P. aeruginosa: Pseudomonas aeruginosa, A. xylosoxidans: Achromobacter xylosoxidans, S. maltophilia: Stenotrophomonas maltophilia, S. aureus: Staphylococus aureus, A. Fumigatus: Aspergillus fumigatus, H. Influenzae: Haemophilus influenzae, M. Catharhalis: Morexella catharhalis.</i> Irr: Irrelevant.</p

Cut-off values, sensitivity and specificity in the discrimination between patients with primary ciliary dyskinesia and healthy subjects by nasal Nitric Oxide concentrations (ppb), during tidal breathing and velum closure modality using hand-held and stationary nNO analysers.

<p>Hand-held nNO was measured by sampling rate of both 2 ml/s (MINO2) and 5 ml/s (MINO5). Two stationary analysers (CLD 88 sp and NIOX Flex) were employed. Both non-velum closure (nVC) and velum closure (VC) results are shown. §: VC accomplished by standard manoeuvre during exhalation against resistance in a mouthpiece. Cut-off values (ppb) with associated sensitivity (%) and specificity (%) were calculated from Receiver Operating Characteristic analyses.</p><p>BH: Breath Hold, TB: Tidal Breathing, VC: Velum Closure, nVC: non-Velum Closure.</p

Discrimination as reflected by mean nasal Nitric Oxide concentrations in parts per billion during tidal breathing and velum closure modality, using hand-held and stationary analysers in patients with primary ciliary dyskinesia, cystic fibrosis and in healthy subjects.

<p>Hand-held nNO was measured by sampling rate of both 2 ml/s (MINO2) and 5 ml/s (MINO5). Two stationary analysers (CLD 88 sp and NIOX Flex) were employed. Both non-velum closure (nVC) and velum closure (VC) results are shown. §: VC accomplished by standard manoeuvre during exhalation against resistance in a mouthpiece. TB: Tidal Breathing, BH: Breath Hold. *: Primary Ciliary Dyskinesia versus Healthy Subjects; **: Primary Ciliary Dyskinesia versus Cystic Fibrosis.</p