HEREDITARY HEMORRHAGIC TELANGIECTASES ASSOCIATED WITH PULMONARY ARTERIOVENOUS FISTULA IN TWO MEMBERS OF A FAMILY
- 31 October 1948
- journal article
- research article
- Published by American College of Physicians in Annals of Internal Medicine
- Vol. 29 (5) , 775-802
- https://doi.org/10.7326/0003-4819-29-5-775
Abstract
A family with Kenf-orrhagic telangiectases is reported. Two members had a pulmonary arterio-venous fistula associated with this hereditary defect. One was free of symptoms and showed only a slight polycythemia. The arterio-venous fistula was small. The other exhibited the classical triad of cyanosis, polycythemia and clubbing of the fingers and toes, characteristically seen in this disease. The discussion of these cases emphasizes the abnormal physiology caused by the arterio-venous shunt, which eliminates the capillary bed, and calls attention to pertinent pathological and roentgenological findings observed in such cases.Keywords
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