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INTRODUCTION

Childhood interstitial lung disease (chILD) comprises a heterogeneous group of chronic pulmonary disorders, characterized by diffuse parenchymal infiltrates and impaired gas exchange, often leading to hypoxemia. In pediatrics, the term diffuse lung disease is interchangeable with interstitial lung disease and more accurately describes the collection of disorders in which airways and airspaces are also involved, not just the interstitium. The spectrum of chILD differs in infancy compared to older children and adults, and this difference has been the basis for the current classification schemes. The term chILD syndrome refers to an infant (< 2 years old) with diffuse lung disease who has at least 3 of the following 4 criteria: (1) respiratory symptoms (cough, labored breathing, or exercise limitation); (2) respiratory signs (tachypnea, retractions, digital clubbing); (3) hypoxemia; or (4) diffuse abnormalities on chest x-ray or computed tomography (CT) scan. A diagnosis of chILD syndrome should be made only after the common causes of diffuse lung disease are excluded. They include cystic fibrosis, congenital heart disease, bronchopulmonary dysplasia, pulmonary infection, primary ciliary dyskinesia, and recurrent aspiration. There are specific chILD diagnoses that are unique to infancy, whereas the majority of chILD diagnoses in older children (age 2 to 18 years old) are related to disorders of the immunocompromised host and overlap more with adult disorders. This chapter provides an overview of chILD with a focus on the most recent developments. Although no classification scheme is ideal, a list of chILD disorders is given in Table 508-1. It is not possible to discuss each entity in detail, but some of these disorders deserve emphasis.

TABLE 508-1SPECTRUM OF INTERSTITIAL LUNG DISEASE IN CHILDREN

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