Lack of Correlation of Sinonasal and Otologic Reported Symptoms With Objective Measurements Among Patients With Primary Ciliary Dyskinesia: An International Study.

peer reviewedSinonasal and otologic symptoms are common among patients with primary ciliary dyskinesia (PCD) of all ages. We used baseline data from the ENT Prospective International Cohort of PCD patients (EPIC-PCD), the first PCD cohort focused on ENT disease manifestations. We assessed agreement between patient- or parent-reported symptoms and relevant examination findings, and calculated unweighted Cohen’s kappa to adjust for agreement by chance. We included 404 participants, from 12 centres. We found no correlation between patient-reported sinonasal symptoms and relevant clinical examination findings. Otologic symptoms correlated poorly or weakly with otoscopy and audiometry findings, with age and centre identified as determinants of agreement

Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020

Background Viral infections can cause significant morbidity in cystic fibrosis (CF). The current Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic could therefore have a serious impact on the health of people with CF (pwCF). Methods We used the 38-country European Cystic Fibrosis Society Patient Registry (ECFSPR) to collect case data about pwCF and SARS-CoV-2 infection. Results Up to 30 June 2020, 16 countries reported 130 SARS-CoV-2 cases in people with CF, yielding an incidence of 2.70/1000 pwCF. Incidence was higher in lung-transplanted patients (n=23) versus non-transplanted patients (n=107) (8.43 versus 2.36 cases/1000). Incidence was higher in pwCF versus the age-matched general population in the age groups <15, 15-24, and 25-49 years (p<0.001), with similar trends for pwCF with and without lung transplant. Compared to the general population, pwCF (regardless of transplantation status) had significantly higher rates of admission to hospital for all age groups with available data, and higher rates of intensive care, although not statistically significant. Most pwCF recovered (96.2%), however 5 died, of whom 3 were lung transplant recipients. The case fatality rate for pwCF (3.85%, 95% CI: 1.26-8.75) was non-significantly lower than that of the general population (7.46%; p=0.133). Conclusions SARS-CoV-2 infection can result in severe illness and death for pwCF, even for younger patients and especially for lung transplant recipients. PwCF should continue to shield from infection and should be prioritized for vaccination

Sleep disordered breathing in childhood

Sleep disordered breathing (SDB) in childhood is linked with significant end-organ dysfunction across various systems, particularly with cardiovascular, neurocognitive and metabolic consequences. If we understand the pathophysiology of SDB, diagnose it promptly and treat appropriately, we may be able to prevent morbidity associated with SDB and also save health resources around the world. In this article, we highlight articles on this topic published in medical journals in the past year