Abstract
A 13-year-old girl had Cruveilhier-Baumgarten disease. The differential diagnosis between Cruveilhier-Baumgarten disease and Cruveilhier-Baumgarten syndrome is dependent upon the absence of morphologic liver disease in the former. Although portal hypertension and hypersplenism are present in both conditions, the pathogenesis is different. Portal hypertension in Cruveilhier-Baumgarten disease seems to be primary, but develops secondarily due to liver disease in the Cruveilhier-Baumgarten syndrome.
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