Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cruveilhier-Baumgarten Disease is a rare clinical entity. The distinction between C-B Disease and C-B syndrome rests solely on the liver morphology. In C-B Disease, the liver is morphologically normal. A case of a 27-year-old juvenile diabetic female with C-B Disease is presented. She manifested many of the metabolic and hemodynamic changes of cirrhosis including portal hypertension, encephalopathy, and a hyperdynamic cardiovascular state. There was also associated splenomegaly, hypersplenism, and splenic artery aneurysms. Splenectomy and splenic artery ligation were performed. Liver biopsies had normal morphology. The patient has had no recurrence of her symptoms. The pathogenesis of C-B Disease is unknown.
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PMID:Cruveilhier-Baumgarten disease with associated splenic artery aneurysms. 86 36

The article reports on a patient with Cruveilhier-Baumgarten disease. The anamnesis of the 59-year-old housewife revealed previous anemia, splenomegaly and abnormal liver function 21 years ago. Three years ago, esophageal varices were found. A tortuously distended vein was seen in the falciform ligament at the time of laparoscopy, and this finding was confirmed by angiography and ultrasonography. Esophageal varices and hypersplenism were also noted. Despite these findings, liver biopsy specimens of both lobes showed only slight fibrosis with minimal lymphocyte infiltration in some portal areas, and no evidence of cirrhosis. Patency of the umbilical vein and portal hypertension without significant histologic change of the liver, are both in keeping with the features of this disease. Dilatation of the umbilical vein seemed to be congenital and did not contribute to active blood flow of portal hypertension in this patient. In Japan, development of "caput medusae" in portal hypertension is rather rare, whereas esophageal varices and splenomegaly are more frequent. Venous hum is also seldom found. 14 cases of Cruveilhier-Baumgarten disease and 28 cases of Cruveilhier-Baumgarten syndrome have been reported from Japan in the literature since 1911.
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PMID:Cruveilhier-Baumgarten disease in Japan--on the basis of our own case. 645 Jul 20