1/255. Case report: rupture of a gastric varix in liver cirrhosis associated with glycogen storage disease type iii.glycogen storage disease type iii, or Cori's disease, is caused by a deficiency of amylo-1,6-glucosidase (debranching enzyme), which leads to the storage of an abnormal glycogen in the liver and in skeletal and heart muscle. glycogen storage disease type iii is usually characterized by hepatic symptoms, growth failure and myopathy. Even though liver cirrhosis is reported, portal hypertension is a rare complication of this disease. We describe the case of a glycogen storage disease type III patient who was diagnosed at 3 years of age and developed complications (liver cirrhosis and rupture of a gastric varix) at 31 years of age. We discuss the histological progression to cirrhosis of the liver and describe the liver enzyme profile at 3 and 31 years of age.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
2/255. Disappearance of gastric antral vascular ectasia after percutaneous transhepatic obliteration.We report on a patient with severe iron deficiency anemia due to gastric antral vascular ectasia (GAVE), complicating hepatocellular carcinoma (HCC) and esophageal varices. Esophago-gastro-duodenoscopy (EGDS) 6 months after transarterial embolization (TAE) for the HCC and percutaneous transhepatic obliteration (PTO) for esophageal varices, showed the absence of GAVE. As GAVE did not recur in spite of the recurrence of the tumor thrombus later, lowered antral congestion by PTO might be the main cause of disappearance of GAVE. This case suggests that PTO may be an effective treatment against GAVE with portal hypertension with uncontrollable bleeding.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
3/255. Hepatic sarcoidosis complicated by hepatocellular carcinoma.A case of a 63-year-old man with a long-standing history of portal hypertension secondary to hepatic sarcoidosis who developed hepatocellular carcinoma is reported.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
4/255. Successful pregnancy in a woman with secondary biliary cirrhosis with portal hypertension from recurrent pyogenic cholangitis. A case report.BACKGROUND: pregnancy in women with secondary biliary cirrhosis due to recurrent pyogenic cholangitis is extremely rare. Little information is available on the effect of pregnancy on the disease and vice versa. CASE: A patient who had secondary biliary cirrhosis due to recurrent pyogenic cholangitis complicated by splenomegaly and portal hypertension had a successful pregnancy. Although she had a history of esophageal variceal bleeding before this pregnancy, there was no such bleeding during pregnancy. She had an uneventful antenatal course except that her liver enzyme level fluctuated slightly. The serum bilirubin level increased during the third trimester of pregnancy but returned to the prepregnant level after delivery. CONCLUSION: Termination of pregnancy may not be the only option for management. The management protocol for patients with primary biliary cirrhosis complicating pregnancy, which includes regular fetal surveillance and monitoring of maternal liver function, should be considered for pregnant women with secondary biliary cirrhosis.- - - - - - - - - - ranking = 5keywords = hypertension (Clic here for more details about this article) |
5/255. Endoscopic management of bleeding ectopic varices with histoacryl.Bleeding from antral and duodenal varices is an uncommon feature in patients with portal hypertension. We report a patient with cirrhosis and portal vein thrombosis, who had a massive bleed from antral and duodenal varices. Bleeding was controlled with endoscopic injection of varices using histoacryl. Endoscopic treatment and the relatively uncommon occurrence of antral and duodenal varices are highlighted.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
6/255. Two cases of esophageal varix caused by local hyperdynamic state of gastric arteries.With increasing evidence on the role of endogenous nitric oxide in the splanchnic hyperdynamics, the existence of esophageal varix induced by hepatopetal flow of portal hypertension in a cirrhotic patient has become more convincing. Herein, we report 2 cases of esophageal varix caused by local hyperdynamic states of the right, left and posterior gastric arteries, respectively. Angiographic evidence suggests the existence of forward flows via the submucosal A-V shunt of the gastric arteries as the etiologic causes of the varices. The treatment of the current cases was unsatisfactory due to improper recognition of the local hyperdynamic state before the treatment. Unlike the "backward flow"-type esophageal varix, the treatment strategies of the "forward flow"-type esophageal varix of hyperdynamic state caused by splanchnic A-V shunts should be considered differently.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
7/255. Long-term interferon alpha maintenance therapy for chronic hepatitis c infection in a patient with common variable immune deficiency.A 46-year-old woman with common variable immune deficiency acquired acute non-A, non-B hepatitis from contaminated intravenous gamma globulin in 1983. For 6 years she had fluctuating elevations of her serum aminotransferase levels. In 1990 her serum was documented to be hepatitis c virusribonucleic acid positive by polymerase chain reaction, and her liver biopsy revealed chronic hepatitis with early cirrhosis (Knodell score, 15 points). hepatitis c virus genotyping indicated that she had been infected with the type 3 genotype. She subsequently underwent treatment with interferon alpha (IFN-alpha) for 1 year and experienced biochemical, virologic, and histologic (Knodell score, 9) suppression. She was continued on maintenance therapy for an additional 7 years, with sustained biochemical and virologic suppression. During the sixth year of therapy, complications of portal hypertension were noted with mild ascites and eventually bleeding esophageal varices. This case report documents a favorable biochemical, virologic, and histologic response to IFN-alpha therapy in this setting; supports the notion that the natural progression of hepatitis c virus infection may be more aggressive in patients with common variable immune deficiency; and, although complications of portal hypertension eventually occurred, the suppressive maintenance IFN therapy may have delayed their onset. The future establishment of the long-term effects of IFN therapy on important clinical outcomes is necessary to understand better its therapeutic benefit in chronic hepatitis c infection.- - - - - - - - - - ranking = 2keywords = hypertension (Clic here for more details about this article) |
8/255. The experience of endoscopic tissue glue injection in the treatment of hepatic sarcoidosis related gastric variceal bleeding: report of a case.A case of hepatic sarcoidosis complicated with portal hypertension and gastric variceal bleeding is described. A 53 year-old male suffered from persistent fever and massive hematemesis. Acute gastric variceal bleeding was diagnosed. Endoscopic tissue glue injection stopped this acute episode and ablated the varices after another two sessions of endoscopic tissue glue injection treatment. Subsequent administration of corticosteroids improved the symptoms and liver function. This was probably the first case of hepatic sarcoidosis associated with gastric variceal bleeding which was successfully treated by endoscopic tissue glue injection to be reported.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
9/255. Massive esophageal variceal hemorrhage triggered by complicated endotracheal intubation.Esophageal variceal hemorrhage is frequently a catastrophic event. The specific events that trigger variceal rupture are not well understood. Acute elevations in systemic blood pressure and increased splanchnic blood flow, however, may lead to increased intravariceal pressure followed by variceal rupture and hemorrhage. This report describes a strong temporal association between complicated endotracheal intubation and abrupt onset of life-threatening variceal hemorrhage. A 52-year-old man with a history of portal hypertension was intubated emergently for airway protection because of respiratory insufficiency due to sepsis. intubation was complicated by initial inadvertent esophageal intubation and by a peak mean arterial blood pressure of 155 mmHg. At the conclusion of the procedure, the patient sustained large volume hematemesis due to esophageal variceal rupture. This case suggests a risk of triggering variceal hemorrhage as a result of intubation-induced increase in blood pressure. A number of agents, including fentanyl, have been shown to be effective in attenuating the cardiovascular response to intubation. This case report provides strong evidence in support of administering fentanyl, or a suitable alternative adjunctive medication, before intubation of patients with documented portal hypertension and a history of esophageal variceal hemorrhage.- - - - - - - - - - ranking = 2keywords = hypertension (Clic here for more details about this article) |
10/255. Hepatic hyper-vitaminosis A: importance of retinyl ester level determination.We report the case of a 32-year-old man with portal hypertension without cirrhosis due to chronic vitamin a intoxication. Portal hypertension revealed by oesophageal varice rupture progressively worsened and ascites occurred 5 years after the patient stopped vitamin a intake. Initially, serum retinyl palmitate concentration was increased whereas serum retinol concentration was normal. There was no hepatic fibrosis on light microscopic examination of liver biopsy specimens. Five years after the patient stopped excessive vitamin a intake, serum retinol and retinol-binding protein concentrations were below the normal range even though there was an increased hepatic retinyl ester content. This was attributed to the late development of peri-sinusoidal fibrosis. This case mainly shows the importance of retinyl ester level determination: serum retinyl palmitate should be measured immediately after intoxication and hepatic retinyl esters should be measured initially and particularly later. Indeed, later serum and hepatic retinol levels in chronic hyper-vitaminosis A may be normal and lead to under-estimation of liver vitamin a overload.- - - - - - - - - - ranking = 2keywords = hypertension (Clic here for more details about this article) |
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