11/255. Massive hepatic fibrosis in Gaucher's disease: clinico-pathological and radiological features.hepatomegaly is frequent in patients with type 1 Gaucher's disease and is associated with infiltration of the liver with pathological macrophages. Most patients suffer no significant clinical consequences, but a few develop portal hypertension which may progress to parenchymal liver failure. We describe four patients with Gaucher's disease who have developed portal hypertension. We have reviewed their clinical histories and all available histological and radiological material. All had severe Gaucher's disease with multi-organ involvement, and had undergone splenectomy in childhood. Histologically, this advanced liver disease was characterized by a picture of extreme and advanced confluent fibrosis occupying the central region of the liver. This massive fibrosis is associated with characteristic radiological appearances. The liver histology in these cases is highly unusual and virtually unknown in other conditions. Our studies indicate that without specific treatment the liver disease is progressive and rapidly fatal. However, institution of enzyme replacement therapy with imiglucerase may have beneficial effects even when the condition is far advanced.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
12/255. Emergency and elective surgical treatment of portal hypertension. A review of 23 years' experience.A retrospective review of surgical treatment for portal hypertension during a 23-year period in a regional unit is reported and the immediate and subsequent management of patients with bleeding oesophageal varices is discussed. Fifty-four patients with recurrent varix haemorrhage uncontrolled by conservative methods have been treated by oesophageal transection with a mortality of 22.2% (26.6% for cirrhotic patients). Thirty-two per cent of the cirrhotics were alive after 2 years. Only a minority (12%) of the survivors were considered suitable for a subsequent shunt procedure. Therapeutic portacaval anastomosis has been performed on 65 patients with a 51.2% 5-year survival (43-5% for cirrhotic patients). Further haemorrhage due to shunt thrombosis occurred in 5-3% of cases. The frequent occurrence of portal-systemic encephalopathy, increasing with duration of time following a shunt, is emphasized. The high morbidity and mortality in the poor-risk cirrhotic indicated that this type of patient is unsuitable for a portal-systemic shunt and is better treated by medical means.- - - - - - - - - - ranking = 2.5keywords = hypertension (Clic here for more details about this article) |
13/255. Portosystemic shunting in patients with primary biliary cirrhosis: a good risk disease.Five patients with primary biliary cirrhosis underwent portosystemic shunting for the control of variceal bleeding. Three procedures were emergencies and two were elective. There was no operative mortality; all patients were followed until the present or until death. One patient is alive 4 years and another, 2 years postoperatively. One patient died 4 years after operation and another died 16 months postoperatively. Another patient survived for 8 years following her shunt and eventually died as a result of a cerebrovascular accident. This group of patients is compared to a larger group undergoing portosystemic shunting because of portal hypertension secondary to other forms of liver disease. The absence of operative mortality and the fact that several of these patients had moderately long postoperative survival despite apparently poor liver function suggest that the usual criteria for the assessment of operative risk are not valid in primary biliary cirrhosis.- - - - - - - - - - ranking = 0.5keywords = hypertension (Clic here for more details about this article) |
14/255. Immunological studies in familial primary biliary cirrhosis.Tests for cell-mediated immunity and presence of autoantibodies were performed in a mother and daughter with primary biliary cirrhosis. lymphocytes transformation to phytohemagglutinin, delayed cutaneous response to one or more skin test antigens, and percentage of peripheral T and B lymphocytes were normal in both patients. Although successful in the mother, dinitrochlorbenzene sensitization was not achieved in the daughter. histocompatibility antigens 1 and 8, elevated levels of IgM, and antibodies to mitochondria, smooth muscle, and skeletal muscle were present in both patients. However, the clinical course was more severe in the daughter who developed portal hypertension with bleeding esophageal varices requiring portacaval anastomosis. Except for intermittent pruritus, the mother has remained asymptomatic.- - - - - - - - - - ranking = 0.5keywords = hypertension (Clic here for more details about this article) |
15/255. Laparoscopic splenectomy for the treatment of gastric varices secondary to sinistral portal hypertension.Portal hypertension presents significant challenges to the laparoscopic surgeon. Here we review the case of a successful laparoscopic splenectomy in a patient with sinistral portal hypertension. The value of preoperative splenic artery embolization is highlighted.- - - - - - - - - - ranking = 3keywords = hypertension (Clic here for more details about this article) |
16/255. Pancreatic adenocarcinoma presenting as sinistral portal hypertension: an unusual presentation of pancreatic cancer.A rare syndrome, sinistral (left-sided) portal hypertension resulting from splenic vein thrombosis secondary to pancreatic adenocarcinoma of the tail is presented here. Pancreatic cancer is notorious for presenting with vague and nonspecific symptoms, including but not exclusively weight loss, abdominal pain, and anorexia with or without jaundice. However, physicians should be aware that in the presence of splenic vein thrombosis, this finding alone puts pancreatic cancer high on the differential diagnosis.- - - - - - - - - - ranking = 2.5keywords = hypertension (Clic here for more details about this article) |
17/255. The treatment of portal hypertension by videolaparoscopy in situs inversus totalis.A rare case of a 32-year-old male with situs inversus totalis viscerum admitted to hospital for hematemesis owing to portal hypertension of Laennec cirrhosis was treated for the first time by videolaparoscopic surgery. situs inversus diagnosis was confirmed by thoracic radiography, electrocardiogram, echocardiogram, abdominal echography and computed tomography. Upper gastrointestinal endoscopy showed esophageal varices and large varices in the fundus of the stomach. A successful operation (azygo-portal disconnection, splenic artery ligation without splenectomy; transesophageal suturing of esophageal varices without opening the esophagus and cholecystectomy), was performed by videolaparoscopy. The uneventful postoperative evolution (4-day hospitalization) reinforces the viability of the videolaparoscopic approach and the possibility of the application of this procedure even to situs inversus totalis organorum.- - - - - - - - - - ranking = 2.5keywords = hypertension (Clic here for more details about this article) |
18/255. Stent recanalization of chronic portal vein occlusion in a child.An 8-year-old boy with a 21/2 year history of portal hypertension and repeated bleedings from esophageal varices, was referred for treatment. The 3.5-cm-long occlusion of the portal vein was passed and the channel created was stabilized with a balloon-expandable stent; a portosystemic stent-shunt was also created. The portosystemic shunt closed spontaneously within 1 month, while the recanalized segment of the portal vein remained open. The pressure gradient between the intrahepatic and extrahepatic portal vein branches dropped from 17 mmHg to 0 mmHg. The pressure in the portal vein dropped from 30 mmHg to 17 mmHg and the bleedings stopped. The next dilation of the stent was performed 12 months later due to an increased pressure gradient; the gastroesophageal varices disappeared completely. Further dilation of the stent was planned after 2, 4, and 6 years.- - - - - - - - - - ranking = 0.5keywords = hypertension (Clic here for more details about this article) |
19/255. Gastroesophageal variceal bleeding caused by hepatoportal arteriovenous fistula.Hepatoportal arteriovenous fistulas are usually traumatic in origin and may result in portal hypertension and serious complications. We report a 34-year-old female with a history of abdominal trauma, who developed symptoms of tarry stools and hematemesis 5 years later. esophageal and gastric varices with bleeding were diagnosed by upper gastrointestinal endoscopy. Abdominal ultrasonography and computerized tomography favored noncirrhotic portal hypertension. An extrahepatic hepatoportal arteriovenous fistula was demonstrated by angiography. The patient underwent surgery to correct the condition. The liver had a smooth surface and both the common hepatic and gastroduodenal arteries were ligated during surgery. The postoperative course was uneventful. The varices later disappeared.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
20/255. Portal hypertension secondary to spontaneous arterio-portal venous fistulas: transcatheter arterial embolization with n-butyl cyanoacrylate and microcoils.We report a 73-year-old man with recurrent variceal bleeding due to portal hypertension caused by multiple intrahepatic arterio-portal venous fistulas, which were successfully occluded by embolization with n-butyl cyanoacrylate and micro-coils.- - - - - - - - - - ranking = 2.5keywords = hypertension (Clic here for more details about this article) |
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