Interstitial lung diseases in children

Abstract

Interstitial lung disease (ILD) in infants and children comprises a large spectrum of rare respiratory disorders that are mostly chronic and associated with high morbidity and mortality. These disorders are characterized by inflammatory and fibrotic changes that affect alveolar walls. Typical features of ILD include dyspnea, diffuse infiltrates on chest radiographs, and abnormal pulmonary function tests with restrictive ventilatory defect and/or impaired gas exchange. Many pathological situations can impair gas exchange and, therefore, may contribute to progressive lung damage and ILD. Consequently, diagnosis approach needs to be structured with a clinical evaluation requiring a careful history paying attention to exposures and systemic diseases. Several classifications for ILD have been proposed but none is entirely satisfactory especially in children. The present article reviews current concepts of pathophysiological mechanisms, etiology and diagnostic approaches, as well as therapeutic strategies. The following diagnostic grouping is used to discuss the various causes of pediatric ILD: 1) exposure-related ILD; 2) systemic disease-associated ILD; 3) alveolar structure disorder-associated ILD; and 4) ILD specific to infancy. Therapeutic options include mainly anti-inflammatory, immunosuppressive, and/or anti-fibrotic drugs. The outcome is highly variable with a mortality rate around 15%. An overall favorable response to corticosteroid therapy is observed in around 50% of cases, often associated with sequelae such as limited exercise tolerance or the need for long-term oxygen therapy.

Integrin α3Mutations with Kidney, Lung, and Skin Disease

“…18 The most frequent mutations in CFTR and ABCA3 were ruled out as a cause of the interstitial lung disease. 19 …”

16q24.1 microdeletion in a premature newborn: Usefulness of array-based comparative genomic hybridization in persistent pulmonary hypertension of the newborn

“…PPHN can be primary or secondary to a variety of disorders including fetal or perinatal hypoxia, sepsis, pneumonia, meconium aspiration, congenital diaphragmatic hernia, pulmonary hypoplasia, premature closure of the ductus arteriosus, veno-occlusive disease, capillary hemangiomatosis and congenital heart disease, including pulmonary vein stenosis [1]. Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) is a rare interstitial lung disease affecting primarily the alveolar components [3]. It is recognized as a cause of PPHN [4].…”

Childhood interstitial lung disease: A systematic review

“…These disorders have variable lung pathology associated with poor gas exchange . chILD is clinically complex and associated with high morbidity and mortality . The typical features of chILD are breathlessness and hypoxemia in the presence of diffuse lung infiltrates on radiography or computed tomography.…”