Congenital lung malformations (CLM) comprise a spectrum of anatomical anomalies of the lungs and respiratory tree. The prenatal growth pattern of CLMs is unpredictable with larger lesions causing life-threatening complications, such as hydrops fetalis, and smaller lesions remaining asymptomatic and potentially regressing. The most common CLMs are congenital pulmonary adenomatoid malformations, bronchopulmonary sequestrations, congenital lobar emphysema, and bronchogenic cysts. All these lesions have clinically similar presentations when symptomatic, but pathophysiologic differences that must be considered when evaluating and treating. This article reviews the most common CLMs, their pathophysiology, clinical presentation, diagnostic considerations, and current literature on the controversies surrounding CLM management. [ Pediatr Ann . 2019;48(4):e169–e174.]
Paradoxical vocal cord motion (PVCM), also termed vocal cord dysfunction, is a poorly understood disorder of episodic dyspnea characterized by inappropriate vocal cord adduction during inspiration and potentially during expiration. It can coexist or be confused with asthma, so appropriate diagnosis is key to optimizing treatment success. Although many patients with PVCM may have underlying psychologic issues, there is emerging evidence to suggest that this entity is not psychogenic in every patient. Both laryngeal irritants and exercise have been identified as additional contributing factors in PVCM. Diagnosis of PVCM requires awake laryngoscopic confirmation. However, many patients do not exhibit signs of PVCM during this examination, despite provocation during testing. Therefore, clinical history remains key in determining which patients should proceed with behavioral therapy under the guidance of a speech pathologist. In addition, treatment may include limiting patient exposure to potential sources of laryngeal irritation. Refractory patients may benefit from psychologic assessment and treatment. [Pediatr Ann. 2016;45(5):e184-e188.].
Purpose of review The purpose of this review is to discuss the most recent data describing the impact of coronavirus disease 2019 (COVID-19) on the pediatric population with chronic pulmonary disease. We specifically focus on children with asthma, cystic fibrosis (CF), and lung transplant recipients. Recent findings Children with asthma, CF, and lung transplant recipients do not appear to have an increased risk of morbidity or mortality with COVID-19 infection compared to the general pediatric population. Data does not support the change or withdrawal of any asthma or CF maintenance medications; however, does advocate for the cessation of aerosolized medications whenever possible to minimize transmission risk. It may not be necessary to adjust immunosuppressive therapy when managing COVID-19 in pediatric lung transplant patients. Mechanisms of infection in airway epithelial cells in children may differ from adults, resulting in a milder phenotype. Summary Current data about pediatric patients with chronic lung disease infected with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) is promising but remains scarce. Additional study is needed to definitively understand the complex interplay of the SARS-CoV-2 virus in the airway of children with chronic lung disease, how it differs from adults, and how best to manage the symptoms of acute infection.
Cystic fibrosis (CF) has seen a multitude of therapeutic advances targeting its downstream effects. This has led to a steady increase in survival over the past few decades. The recent development of disease‐modifying drugs targeting the underlying CFTR mutation has revolutionized treatment for CF. Despite these advances, individuals with CF who are racial and ethnic minorities, from low socioeconomic status, or female sex have worse clinical outcomes. The inequitable access to CFTR modulators from cost and/or genetic eligibility has the potential to further worsen the existing health disparities seen within the CF community.
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