Estimates of primary ciliary dyskinesia prevalence: a scoping review.

Background: Primary ciliary dyskinesia (PCD) is a rare multisystem genetic disease caused by dysfunctional motile cilia. Despite PCD being the second most common inherited airway disease after cystic fibrosis, PCD continues to be under-recognised globally owing to nonspecific clinical features and the lack of a gold standard diagnostic test. Commonly repeated prevalence estimates range from one in 10 000 to one in 20 000, based on regional epidemiological studies with known limitations.

Mortality of Pediatric Surgical Lung Biopsies in Ontario, Canada, 2000-2019.

Surgical lung biopsies are often required for the definitive diagnosis of nonmalignant pediatric diffuse lung diseases; however, the literature on mortality after surgical lung biopsy in pediatric patients is sparse. To determine the 30-day postoperative mortality rate after surgical lung biopsies for nonmalignant lung disease in pediatric patients in Ontario, Canada, and to identify risk factors associated with mortality. We performed an observational cohort study using population-based health administrative data available from ICES in Ontario, Canada, from 2000 to 2019.

Bronchodilator responsiveness in children with primary ciliary dyskinesia.

Background: Reversible airway obstruction is common in children with primary ciliary dyskinesia. However, the diagnostic value of adding bronchodilator (BD) response testing to routine spirometry is unclear.

Methods: This is a retrospective analysis of pulmonary function test results obtained from children with primary ciliary dyskinesia seen as outpatients at the Hospital for Sick Children, Toronto.

Situs Ambiguus Is Associated With Adverse Clinical Outcomes in Children With Primary Ciliary Dyskinesia.

Background: Primary ciliary dyskinesia (PCD) is a rare disorder of motile cilia associated with situs abnormalities. At least 12% of patients with PCD have situs ambiguus (SA), including organ laterality defects falling outside normal arrangement (situs solitus [SS]) or mirror image inversion (situs inversus totalis [SIT]).

Research Question: Do patients with PCD and SA achieve worse clinical outcomes compared with those with SS or SIT?

Study Design And Methods: This cross-sectional, multicenter study evaluated participants aged 21 years or younger with PCD.

Inter- and intravisit repeatability of free-breathing MRI in pediatric cystic fibrosis lung disease.

Purpose: The purpose of this study is to assess the intra- and interscan repeatability of free-breathing phase-resolved functional lung (PREFUL) MRI in stable pediatric cystic fibrosis (CF) lung disease in comparison to static breath-hold hyperpolarized 129-xenon MRI (Xe-MRI) and pulmonary function tests.

Methods: Free-breathing 1-hydrogen MRI and Xe-MRI were acquired from 15 stable pediatric CF patients and seven healthy age-matched participants on two visits, 1 month apart. Same-visit MRI scans were also performed on a subgroup of the CF patients.

Granulomatosis with polyangiitis in an adolescent male with chronic cough and pulmonary nodules.

Granulomatosis with polyangiitis (GPA) is a small to medium vessel vasculitis that is uncommon in paediatrics. However, with chronic cough often being the initial symptom, a common complaint and a median age of diagnosis of 14 years, it is nevertheless an important condition for paediatricians to consider as it can otherwise go undiagnosed for a long period of time. In this case report, we discuss a paediatric patient with GPA that presented with non-specific respiratory symptoms for several months and was then found to have pulmonary nodules on chest imaging once a broader differential diagnosis was considered.

Association of Neonatal Hospital Length of Stay with Lung Function in Primary Ciliary Dyskinesia.

Primary ciliary dyskinesia (PCD), an inherited lung disease, is characterized by abnormal ciliary function leading to progressive bronchiectasis. There is wide variability in respiratory disease severity at birth and later in life. To evaluate the association between neonatal hospital length of stay (neonatal-LOS) and supplemental oxygen duration (SuppO) with lung function in pediatric PCD.

Timing of Antenatal Corticosteroids for Optimal Neonatal Outcomes: A Markov Decision Analysis Model.

Objective: Antenatal corticosteroids (ACSs) are administered to pregnant individuals at high risk of preterm delivery to reduce neonatal morbidity and mortality. ACSs have a limited timeframe of effectiveness, and timing of administration can be difficult because of uncertainty surrounding the likelihood of preterm delivery. The objective of the current study was to design a decision analysis model to optimize the timing of ACS administration and identify important model variables that impact administration timing preference.

Measurement of forces applied using a Macintosh direct laryngoscope compared with a Glidescope video laryngoscope in patients with predictors of difficult laryngoscopy: A randomised controlled trial.

Background: In patients with predictive features associated with easy direct laryngoscopy, videolaryngoscoy with the GlideScope has been shown to require less force when compared with Macintosh direct laryngoscopy.

Objective: The aim of this study was to compare forces applied with Glidescope vs. Macintosh laryngoscopes in patients with predictive features associated with difficult direct laryngoscopy.

Dry Powder Inhaler Delivery of Tobramycin in In Vitro Models of Tracheostomized Children.

Background: Pediatric tracheostomies are not uncommon and aerosols allow for targeted lung therapy. However, there is little literature that quantifies aerosol delivery through tracheostomies. Nebulizers are commonly used in delivering tobramycin, but there are drawbacks, for example, time burden.

Recurrent Diplopia in a Pediatric Patient with Bickerstaff Brainstem Encephalitis.

Introduction. Acute complete external ophthalmoplegia is a rare finding in clinical practice that is associated with diseases affecting the neuromuscular junction, the oculomotor nerves, or the brainstem. Ophthalmoplegia has been reported with acute ataxia in Miller Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE).

Modeling breath-enhanced jet nebulizers to estimate pulmonary drug deposition.

Background: Predictable delivery of aerosol medication for a given patient and drug-device combination is crucial, both for therapeutic effect and to avoid toxicity. The gold standard for measuring pulmonary drug deposition (PDD) is gamma scintigraphy. However, these techniques expose patients to radiation, are complicated, and are relevant for only one patient and drug-device combination, making them less available.