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The term interstitial lung disease (ILD) encompasses a group of rare, heterogeneous disorders characterized by diffuse pulmonary disease and disordered gas exchange.1  Although a strict definition implies an abnormality of the interstitium, there are some diseases that have minimal interstitial defect but have pathological abnormalities in the airspaces and distal airway that are considered under the broad term of ILD.2  For this reason, some authors prefer the term diffuse lung disease (DLD). In this chapter, children’s ILD will be used to refer to children’s diffuse and interstitial lung disease. Although individual children’s ILD entities are rare, group estimates...

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