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- W1548249605 abstract "The clinical, pathophysiologic and laboratory features of 4 cases with the chronic cyanotic syndrome of portal hypertension caused by visceral bilharziasis, 2 with complicating portal cirrhosis, are presented. In the presence of hepatosplenomegaly, chronic severe cyanosis is suggestive of pulmonary arteriovenous fistulas. On examination, the degree of cyanosis and clubbing with no significant clinical, laboratory or physiologic pulmonary and cardiac abnormalities is striking. The pathologic lesions responsible for the clinical picture consist chiefly of evidence of portal hypertension and widespread abnormal ramifications of both pulmonary arteries and veins, the great increase in the vascular bed of pulmonary parenchyma, and the presence of arteriovenous fistulas. There was no roentgenologic nor electrocardiographic evidence of pulmonary or cardiac disease. The liver function tests may show moderate impairment as in portal cirrhosis but may show no significant changes as happens in visceral schistosomiasis. Right heart catheterization and pulmonary function tests reveal normal pressure in the right side of the heart and associated great vessels, essentially normal pulmonary function studies and persistence of arterial oxygen unsaturation after administration of 100 per cent oxygen for 15 minutes. The differentiation of the cyanotic syndrome with clubbing of the fingers due to portal hypertension from congenital and acquired cyanotic diseases, pulmonary and cardiovascular, is of paramount importance. Further observations will enable us to learn more about the factors involved in the genesis of the pulmonary vascular findings, but portal hypertension, when present, seems to be an important factor. A similar syndrome in the absence of portal hypertension may be best explained by a vascular proliferative factor." @default.
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- W1548249605 date "1962-10-01" @default.
- W1548249605 modified "2023-09-26" @default.
- W1548249605 title "Cyanotic syndrome of portal hypertension in hepatosplenic schistosomiasis and portal cirrhosis" @default.
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- W1548249605 doi "https://doi.org/10.1016/0002-9149(62)90374-0" @default.
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