Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 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

Two cases of Cruveilhier-Baumgarten syndrome not clinically evident and without esophageal varices in patients with liver cirrhosis and portal hypertension are presented. The diagnosis was made by real-time ultrasonography, which showed echographic caput medusae with large afferent umbilical veins and efferent inferior superficial epigastric veins. Doppler flowmetry documented high blood flow rates in these collateral portal-systemic circulations, and this explained the absence of large varices at endoscopy. The role of massive spontaneous portal-systemic shunts in preventing the formation of other shunts and particularly esophageal variceal bleeding is discussed.
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PMID:Cruveilhier-Baumgarten syndrome without esophageal varices: ultrasonographic diagnosis and echo-Doppler study. 266

A patient with post-necrotic liver cirrhosis, presenting with recurrent haematemesis and melena due to oesophageal varices, received repeated endoscopic injection sclerotherapy. The Cruveilhier-Baumgarten syndrome developed 11 months after variceal obliteration. Neither rebleeding nor recurrence of varices was observed during a follow up period of 12 months after obliteration of varices.
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PMID:Development of the Cruveilhier-Baumgarten syndrome after endoscopic obliteration of oesophageal varices. Report of a case. 277 3

Within the framework of a prospective study, we were able to establish that in 50 per cent of 220 patients submitted to laparoscopy, the ligamentum teres hepatis did not run from the porta hepatis directly to the umbilicus, but to a point of insertion craniad to the umbilicus in the median line of the anterior abdominal wall. As a rule, this topographic variant has no clinical relevance. It is, however, of importance in patients with cirrhosis of the liver and portocaval collateral channels. In our group, 25 per cent of the patients revealed complete cirrhotic transformation of the liver; in a quarter of these, the Cruveilhier-Baumgarten syndrome presented, and thus also the possibility that large-caliber porto-femoral "umbilical" vessels might be running a course to the left of the umbilicus. The danger of injuring such a vessel during laparoscopy can, however, be prevented: in seven out of eight patients with portofemoral collaterals, this situation was established by ultrasonic examination, and in three cases, the periumbilical course of these vessels was accurately determined prior to carrying out the procedure. In our opinion, the definition of the Cruveilhier-Baumgarten syndrome needs to be extended. The results of our investigation show that, within the framework of portal neo-vascularisation, direct umbilical vein recanalisation is a relatively rare occurrence (approximately 15 per cent), while porto-femoral collateral formation is considerably more common (about 70 per cent).
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PMID:The importance of variant insertions of the ligamentum teres hepatis in the Cruveilhier-Baumgarten syndrome. 297 27

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

We report a rare case of acute liver failure due to embolization of the liver after an umbilical hernia repair in a patient with Child B liver cirrhosis and status posttransjugular intrahepatic portosystemic shunt (TIPSS). This patient initially presented with a symptomatic umbilical hernia. His umbilical vein was open (Cruveilhier-Baumgarten syndrome). After hernia repair the patient developed thrombosis of the umbilical vein with consequent partial embolization to, and acute failure of, the liver. The patient underwent successful emergency liver transplantation. This disease needs close collaboration among surgeons, gastroenterologists, hepatologists, radiologists, nutritionists, and transplant teams to establish an effective treatment plan.
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PMID:Emergency liver transplantation after umbilical hernia repair: a case report. 2083 97

Cruveilhier-Baumgarten syndrome (CBS) is a rare complication of cirrhosis. We reported a case of CBS who came to hospital with progressive ascites, jaundice, fever and upper gastrointestinal bleeding. The cause of cirrhosis and hepatitis might be due to alcohol and possibly local Thai herb [Boesenbergia pundurata (Roxb.) Schitr or Krachaidum].
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PMID:Cruveilhier-Baumgarten syndrome in a cirrhotic patient taking alcohol with Boesenbergia pundurata. 2107 49