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Five cases of desquamative interstitial pneumonia diagnosed in children aged 2 months to 3 years have been followed over a 4 year period. The variable clinical presentations included cough, tachypnea, cyanosis and failure to thrive associated with apparent preceeding viral respiratory infections developing over a period ranging from less than 24 hours to several weeks. One infant had probable Wiskott-Aldrich syndrome. Another child, becoming symptomatic in 24 hours, required ventilatory assistance and recovered rapidly with therapy. All chest radiographs showed a diffuse ground glass appearance with accentuated perihilar markings and variable patchy infiltrates. Diagnosis was confirmed by open lung biopsy, showing characteristic packing of alveoli with granular pneumocytes in a monotonous pattern. Initial therapy was 2-4 mg/kg of prednisone in 5/5. 4/5 had an initial good response with clinical and radiographic clearing. 2 of these subsequently relapsed and expired; one in 19 months after additional chloroquine therapy, the other with Wiskott-Aldrich syndrome died in 5 months of pseudomonas sepsis. One has remained well on no therapy for 2 years, and the one who had no response initially has had progressive hypoxemia over 4 years. Open lung biopsy should be considered in children with tachypnea, cyanosis and compatible x-ray findings, of obscure etiology, for only with a definitive diagnosis can aggressive therapy with high dose steroids, and possibly inmunosupressants, be pursued.

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Harwood, I., Olmsted, N. & Giamnona, S. DESQUAMATIVE INTERSTITIAL PNEUMONIA IN INFANCY AND CHILDHOOD. Pediatr Res 11, 572 (1977).

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