Innovations in Childhood Interstitial and Diffuse Lung Disease

Abstract

Children's interstitial and diffuse lung diseases (chILDs) are a heterogenous and diverse group of lung disorders presenting during childhood. Infants and children with chILD disorders present with respiratory signs and symptoms as well as diffuse lung imaging abnormalities. ChILD disorders are associated with significant health care resource utilization and high morbidity and mortality. The care of patients with chILD has been improved through multidisciplinary care, multicenter collaboration, and the establishment of patient research networks in the United Stated and abroad. This review details past and current innovations in the diagnosis and clinical care of children with chILD.

Keywords: Childhood interstitial lung disease; Neuroendocrine hyperplasia of infancy; Pediatric pulmonology; Pulmonary fibrosis; Surfactant dysfunction.

Fig. 1.

Classification categories of chILD disorders. There is overlap between conditions presenting most often in infancy, those secondary to a systemic disease process, and conditions due to environmental exposures. Diagnosis and management involve synthesis of the patient’s clinical context, histopathologic classification, and underlying disease processes.

Fig. 2.

A 10 month old female infant presented with 5 months history of tachypnea, recurrent lower respiratory tract infections associated with respiratory exacerbations, and inability to wean from supplemental oxygen for chronic hypoxemia. Chest radiograph (A and B) demonstrates mild central perihilar interstitial opacities, nonspecific findings. Chest CT (C and D) demonstrates diffuse ground glass opacities in a well-demarcated, geographic pattern that is central/perihilar, and most significant in the right middle lobe and lingula. A 5 day old male infant presented with respiratory distress at birth requiring intubation, mechanical ventilation, and high supplemental oxygen (FiO₂ 1.0). Administration of exogenous surfactant resulted in transient improvements in oxygenation and ventilation. Chest radiograph (E) demonstrates diffuse ground glass opacities and air bronchograms. Genetic testing identified biallelic pathogenic variants in SFTPB. Chest CT (F) demonstrates diffuse ground glass opacities.