PMC4501473_01
Panel A: Chest radiograph upon transfer to our institution at day 7 of hospitalization shows diffuse patchy airspace infiltrates. Panel B: Chest radiograph at 6 months of life shows resolution of pathology.
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A chest X-ray upon arrival showed diffuse patchy airspace infiltrates (Fig. 1A). A bedside flexible bronchoscopy with During her outpatient follow up visits at 3 months and 6 months, she had no cough or respiratory symptoms and her oxygen saturations were 100% on room air with a negative respiratory examination, and normal CXR (Fig. 1B).
PMC4501473_02
Family Pedigree illustrates the autosomal dominant inheritance pattern of this disease. All deceased patients died of pneumonia and pulmonary complications.
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Three young adults have died in the extended family from pulmonary complications and pneumonia (Fig. 2).