Idiopathic pulmonary hemosiderosis is a rare disease of children and young adults with a well-defined clinical course of hypochromic anemia, intermittent pulmonary bleeding, progressive pulmonary fibrosis, and eventual terminal cardiorespiratory failure. The disease may run a less malignant course in the older child and young adult with prolonged remissions and perhaps eventual recovery. The cause of the malady has yet to be defined other than by speculation based on observation of the clinical course and pathological findings at autopsy.
The disease was first recognized by Ceelen1 in 1931, when he described the autopsy examination of two children with pulmonary fibrosis and massive hemosiderin deposition limited to the Lung. Wyllie2 presented the most comprehensive description of the disease in 1948 and added seven cases to the literature. There have been about 40 cases described in English literature, and 9 cases have been recognized and reported in this country.* Our
IRVIN JM, SNOWDEN PW. Idiopathic Pulmonary Hemosiderosis: Report of a Case with Apparent Remission from Cortisone. AMA Am J Dis Child. 1957;93(2):182–187. doi:10.1001/archpedi.1957.02060040184011
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