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Authors describe a case of desquamative interstitial pneumonia in a seven month old boy. Initial manifestations were anorexia, feeding difficulties, retarded ponderal and progressive respiratory distress. Pathologic diagnosis was performed by open pulmonary biopsy. He was treated with steroids and followed a severe clinical course dying at 9 months of age. Etiopathogenesis, diagnosis and treatment as well as cases published in pediatric literature are reviewed.
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