2014, Number 4
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Rev Cubana Pediatr 2014; 86 (4)
Interstitial pulmonary diseases seen in the child
Razón BR, Hevia BD
Language: Spanish
References: 29
Page: 479-494
PDF size: 311.66 Kb.
ABSTRACT
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
Revista Cubana de Pediatría. 2014;86(4):479-494
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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.
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