Idiopathic pulmonary hemosiderosis: ultrastructural studies and responses to azathioprine.
Two boys are presented who fulfilled criteria for a diagnosis of idiopathic pulmonary hemosiderosis. A lung biopsy specimen from the first patient showed alveolar-capillary basement membrane abnormalities, together with abnormalities of capillary endothelial cells and hemosiderin-laden macrophages. A lung biopsy specimen from the second patient showed mainly capillary endothelial abnnormalities and interestitial fibrosis. Both patients had a noticeable improvement in symptoms and relative stabilization of their roentgenographic and pulmonary function abnormalities following azathioprine therapy.
D000747:Anemia, Hypochromic / Q000473:pathology; D001379:Azathioprine / Q000008:administration & dosage / Q000627:therapeutic use*; D002648:Child; D004305:Dose-Response Relationship, Drug; D006486:Hemosiderosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D008168:Lung / Q000648:ultrastructure*; D008176:Lung Volume Measurements; D008264:Macrophages / Q000648:ultrastructure; D008297:Male; D011650:Pulmonary Alveoli / Q000648:ultrastructure; D011859:Radiography
D001379:Azathioprine