Background To date, few data on paediatric COVID-19 have been published, and most reports originate from China. This study aimed to capture key data on children and adolescents with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection across Europe to inform physicians and health-care service planning during the ongoing pandemic. Methods This multicentre cohort study involved 82 participating health-care institutions across 25 European countries, using a well established research network-the Paediatric Tuberculosis Network European Trials Group (ptbnet)-that mainly comprises paediatric infectious diseases specialists and paediatric pulmonologists. We included all individuals aged 18 years or younger with confirmed SARS-CoV-2 infection, detected at any anatomical site by RT-PCR, between April 1 and April 24, 2020, during the initial peak of the European COVID-19 pandemic. We explored factors associated with need for intensive care unit (ICU) admission and initiation of drug treatment for COVID-19 using univariable analysis, and applied multivariable logistic regression with backwards stepwise analysis to further explore those factors significantly associated with ICU admission. Findings 582 individuals with PCR-confirmed SARS-CoV-2 infection were included, with a median age of 5•0 years (IQR 0•5-12•0) and a sex ratio of 1•15 males per female. 145 (25%) had pre-existing medical conditions. 363 (62%) individuals were admitted to hospital. 48 (8%) individuals required ICU admission, 25 (4%) mechanical ventilation (median duration 7 days, IQR 2-11, range 1-34), 19 (3%) inotropic support, and one (<1%) extracorporeal membrane oxygenation. Significant risk factors for requiring ICU admission in multivariable analyses were being younger than 1 month (odds ratio 5•06, 95% CI 1•72-14•87; p=0•0035), male sex (2•12, 1•06-4•21; p=0•033), pre-existing medical conditions (3•27, 1•67-6•42; p=0•0015), and presence of lower respiratory tract infection signs or symptoms at presentation (10•46, 5•16-21•23; p<0•0001). The most frequently used drug with antiviral activity was hydroxychloroquine (40 [7%] patients), followed by remdesivir (17 [3%] patients), lopinavir-ritonavir (six [1%] patients), and oseltamivir (three [1%] patients). Immunomodulatory medication used included corticosteroids (22 [4%] patients), intravenous immunoglobulin (seven [1%] patients), tocilizumab (four [1%] patients), anakinra (three [1%] patients), and siltuximab (one [<1%] patient). Four children died (case-fatality rate 0•69%, 95% CI 0•20-1•82); at study end, the remaining 578 were alive and only 25 (4%) were still symptomatic or requiring respiratory support. Interpretation COVID-19 is generally a mild disease in children, including infants. However, a small proportion develop severe disease requiring ICU admission and prolonged ventilation, although fatal outcome is overall rare. The data also reflect the current uncertainties regarding specific treatment options, highlighting that additional data on antiviral and immunomodulatory drugs...

BackgroundNontuberculous mycobacteria (NTM) are an emerging threat to cystic fibrosis (CF) patients but their epidemiology is not well described.MethodsIn this retrospective observational study we identified all Scandinavian CF patients with a positive NTM culture from airway secretions from 2000 to the end of 2012 and used national CF databases to describe microbiological and clinical characteristics.ResultsDuring the 13-year period 157 (11%) CF patients were culture positive for NTM at least once. Mycobacterium abscessus complex (MABSC) (45%) and Mycobacterium avium complex (MAC) (32%) were the predominant species with geographical differences in distribution. Younger patients were more prone to MABSC (p < 0.01). Despite treatment, less than one-third of MABSC patients with repeated positive cultures cleared their infection and a quarter had a lung transplant or died.ConclusionNTM are significant CF pathogens and are becoming more prevalent in Scandinavia. MABSC and MAC appear to target distinct patient groups. Having multiple positive cultures despite treatment conveys a poor outcome.

Background and objective Cryotherapy in interventional bronchoscopy is a new treatment modality, which has recently been made available for the paediatric airway. Lack of experience and safety concerns have led to hesitant adaptation. The aim of this study was to assess indications, success rates and complications of airway cryotherapy in children. Methods Bronchoscopists from medical centre performing cryotherapy in patients between 0 and 18 years were invited to participate in a prospective study based on an online questionnaire. Patient and participant data were collected between June 2020 and June 2021. Results A total of 69 cryotherapy procedures were performed in 57 patients a for three main indications: Biopsy (30), restoration of airway patency (23) and foreign body aspiration (16). The overall success rate was 93%, the remaining 7% were performed for foreign body removal and required a switch of technique. Restoration of airway patency was successfully applied in various pathologies, including mucus plugs, bronchial casts and post traumatic stenosis. The diagnostic yield of transbronchial biopsies was 96%. No severe complications were encountered; one pneumothorax following a cryobiopsy required a chest drain for 48 h. No child was admitted to intensive care or died from a procedural complication. Conclusion In this largest paediatric case collection to date, cryotherapy was safe and carried a high success rate. Cryobiopsy compares favourably to the widely used forceps biopsy and could replace it in the future. Paediatric bronchoscopists are encouraged to add cryotherapy to their armamentarium of airway interventions.

Genetic predisposition and environmental factors modulate the expression of allergic phenotypes. The frequent allergic phenotype 'immediate cutaneous hypersensitivity' was established in mice as a model for atopy. Genetic dissection of this trait requires a robust procedure to assess the allergic phenotype. To this end, different mouse strains were immunized with birch pollen extract. Immediate cutaneous hypersensitivity reactions were induced through intradermal allergen exposure. Wheel formation was quantitated and expressed as a hypersensitivity score according to the bonitur method. This procedure identified A/J and C57BL/6 mice as high– and low–responder strains, respectively. Crosses of A/J and C57BL/6 mice should allow the characterization of mendelian factors responsible for the two extreme phenotypes identified here.

Patients with initially successful treatment of central nervous system tuberculosis should undergo an alert follow-up for the development of late cerebral tuberculomas. Treatment should consist of prolonged courses of antituberculous chemotherapy and high-dose corticosteroids.

Primary ciliary dyskinesia, a rare disease causing bronchiectasis, lacks a sound evidence base for treatment. @beatpcd proposes 1) forming a PCD European clinical trial network to address this situation and 2) conducting n-of-1 trials to access medication. https://bit.ly/3j5blfM Cite this article as: Crowley S, Azevedo I, Boon M et al. Access to medicines for rare diseases: beating the drum for primary ciliary dyskinesia.

LGEA is a severe congenital malformation, with severe morbidity. No mortality was seen. MBW could be a useful tool for the early detection of progressive pulmonary damage.

BackgroundImmigrants from countries with high incidence of tuberculosis (TB) are usually offered screening when they arrive to low incidence countries. The tuberculin skin test (TST) is often used. The interferon gamma release assays (IGRAs) are more specific and not affected by BCG vaccination. The aims of this study were 1. To see if there if there is a correlation between a positive IGRA (QFT) and presence of a BCG scar in children with TST ≥10 mm, 2. To compare the TST diameter with QFT result, 3. To see if chest X-ray can be omitted in QFT negative children despite TST ≥10 mm.Methods762 healthy children/adolescents (median age 14 years) arriving to Gothenburg and surroundings with TST ≥10 mm were tested with QFT.ResultsA total of 163/492 (33 %) children with BCG scar had positive QFT, whereas 205/270 (76 %) without BCG scar had positive QFT (p < 0.0001). The median TST was 12 mm in QFT negative and 18 mm in QFT positive children (p < 0.0001) but with considerable overlap. Median TST was the same (12 mm) in QFT negative children with and without BCG scar. Among the QFT positive children 25/368 had chest X-ray changes compared to 2/393 among the QFT negative children (p < 0.0007).ConclusionsPrevious BCG vaccination had an effect on the TST diameter so an IGRA is recommended to diagnose latent TB. Using only TST for screening of latent TB would lead to overdiagnosis. The TST diameter was larger in QFT positive than in QFT negative children but could not predict QFT in the individual patient. Chest X ray contributes little to the diagnosis of TB in QFT negative children but can not be omitted because of late seroconversion of QFT in some patients.Trial registrationNot applicable.