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August 1956

Primary Pulmonary Vascular Disease with Cor Pulmonale: Report of Three Cases in Children, One with Congenital Hypertension and Two Siblings with Allergic Vasculitis and Disorders of Skeletal Epiphyses

Author Affiliations

Pretoria, Union of South Africa
From the Departments of Pathology and Paediatrics, University of Pretoria.

AMA Am J Dis Child. 1956;92(2):115-125. doi:10.1001/archpedi.1956.02060030109002

In the first case here described there was holdover of fetal pulmonary arterial structure with hypertension and cor pulmonale. There were also distinctive segmental arterial lesions and foci of interstitial pneumonia. Arterial lesions outside the lungs were not found. The other two cases were brothers who died of cor pulmonale due to allergic vasculitis confined to the lungs. In addition, one had Perthes' disease and the other dysplasia epiphysalis multiplex.

The first case is presented because cor pulmonale was primarily due to congenital hypertension and because there were distinctive lesions of intrapulmonary arteries. In such lesions capillaries had developed segmentally in arterial walls without apparent other damage, since smooth muscle of the media between capillaries was largely intact. Additional features of this first case were livedo reticularis, syndactyly, fibrosis around hair follicles, and congenital deformity of intramyocardial arteries. The other two cases are recorded because allergic vasculitis limited to pulmonary

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