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Case Reports
May 1939


Author Affiliations

From the pathologic and cardiac departments of the Children's Memorial Hospital and the Otho S. A. Sprague Memorial Institute.

Am J Dis Child. 1939;57(5):1099-1109. doi:10.1001/archpedi.1939.01990050105011

Primary proliferative pulmonary vascular sclerosis is rare. Brenner,1 in an exhaustive study of the pathology of the vessels of the pulmonary circulation and with an excellent review of the literature, could find but 16 well described cases, including 1 of his own, that could be accepted without reserve. In all other cases appeared some factor, such as chronic cardiac or pulmonary disease, that was thought capable of causing secondary sclerosis, either severe or in mild form. It seems obvious that the latter cases fall into that group reported in the literature as cases of Ayerza's disease2 or syndrome, as in all of these the condition was obviously secondary vascular sclerosis of the pulmonary artery. Without exception it was associated with chronic bronchopulmonary or cardiac disease.3 This is true also of the cases recently described by Kaump and Dry.4 MacCallum5 found but 1 case of primary

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