Resumen

RAZON BEHAR, Roberto  y  HEVIA BERNAL, Daisy. Interstitial pulmonary diseases seen in the child. Rev Cubana Pediatr [online]. 2014, vol.86, n.4, pp. 479-494. ISSN 0034-7531.

The interstitial pulmonary diseases seen in the child comprise a large heterogeneous group of rare diffuse pulmonary diseases of varied morbidity. They are characterized by inflammatory and fibrotic changes causing remodeling of alveolar walls and of the distal respiratory pathways, leading to restrictive functional defect and altered gas exchange with progressive hypoxemia. Many diseases can affect the pulmonary insterstice and the etiological forms caused by a variety of pathological processes are multiple including genetic factors, association to systemic diseases and inflammatory or fibrotic responses to different stimuli. However, the origin is sometimes unknown, so they are classified as idiopathic diseases. The pediatric pneumologists have tried to classify the interstitial pulmonary disease cases into the originally described categories for adults; however, there are adult forms that do not occur in childhood and unique pediatric presentations that are not seen in adulthood. The importance of knowledge about these diseases, particularly those of unknown or idiopathic origin was stressed in this article.

Palabras clave : interstitial pulmonary diseases; idiopathic interstitial pneumonias; pulmonary fibrosis.

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