Transcatheter Occlusion of Left-To-Right Shunts in Premature Infants with Bronchopulmonary Dysplasia.

Introduction: Patent ductus arteriosus (PDA) and atrial septal defects (ASDs) cause pulmonary overcirculation, potentially worsening bronchopulmonary dysplasia (BPD) in premature infants. Transcatheter device occlusion of these defects is feasible and safe, though no case-controlled studies have assessed respiratory outcomes in infants with BPD. We hypothesized infants with BPD and ASDs or PDAs would experience improved respiratory outcomes following device occlusion of these lesions as compared to those who did not.

Novel Treatment-Refractory Preschool Wheeze Phenotypes Identified by Cluster Analysis of Lung Lavage Constituents.

Background: Preschool children with treatment-refractory wheeze often require unscheduled acute care. Current guidelines advise treatment of persistent wheeze with inhaled corticosteroids. Alternative treatments targeting structural abnormalities and specific inflammatory patterns could be more effective.

Lung Lavage Granulocyte Patterns and Clinical Phenotypes in Children with Severe, Therapy-Resistant Asthma.

Background: Children with severe asthma have frequent exacerbations despite guidelines-based treatment with high-dose corticosteroids. The importance of refractory lung inflammation and infectious species as factors contributing to poorly controlled asthma in children is poorly understood.

Objective: To identify prevalent granulocyte patterns and potential pathogens as targets for revised treatment, 126 children with severe asthma underwent clinically indicated bronchoscopy.

Respiratory motile cilia dysfunction in a patient with cranioectodermal dysplasia.

Ciliopathies such as cranioectodermal dysplasia, Sensenbrenner syndrome, short-rib polydactyly, and Jeune syndrome are associated with respiratory complications arising from rib cage dysplasia. While such ciliopathies have been demonstrated to involve primary cilia defects, we show motile cilia dysfunction in the airway of a patient diagnosed with cranioectodermal dysplasia. While this patient had mild thoracic dystrophy not requiring surgical treatment, there was nevertheless newborn respiratory distress, restrictive airway disease with possible obstructive airway involvement, repeated respiratory infections, and atelectasis.

Sirolimus-induced interstitial lung disease following pediatric stem cell transplantation.

Sirolimus-induced ILD is a known but rare complication in adults who have undergone SOT. However, little is known about this adverse effect in children. Diagnosis of sirolimus-induced ILD can be challenging, especially in patients who have difficulty participating in lung function testing.

Airway ciliary dysfunction and sinopulmonary symptoms in patients with congenital heart disease.

Rationale: Patients with congenital heart disease with heterotaxy exhibit a high prevalence of abnormal airway ciliary motion and low nasal nitric oxide, characteristics associated with primary ciliary dyskinesia, a reflection of the role of motile cilia in airway clearance and left-right patterning.

Objectives: To assess the potential broader clinical significance of airway ciliary dysfunction in congenital heart disease, we assessed the prevalence of ciliary dysfunction versus respiratory symptoms in patients with congenital heart disease with or without heterotaxy.

Methods: Patients with a broad spectrum of congenital heart disease were recruited (n = 218), 39 with heterotaxy.