1/227. Ileal varices associated with recurrent bleeding in a patient with liver cirrhosis.We report a rare case of massive and recurrent bleeding from ileal varices in a patient with hepatitis c virus-positive liver cirrhosis. A 66-year old woman, who had undergone laparotomy and blood transfusion 36 years before (because of an extrauterine pregnancy) and endoscopic sclerotherapy for esophageal varices 1 year previously, was admitted to our hospital with loss of bright red blood per rectum. The bleeding was massive and recurrent, and frequent blood transfusions were required. Endoscopic studies failed to find the bleeding site. In the venous phase of selective superior mesenteric angiography, mesenteric varices in the lower part of the abdominal cavity were observed. laparotomy was performed to control the repeated bleeding which had lasted for more than 1 month. Varices communicating with the right ovarian vein were found on the ileal wall and segmental resection of the ileum was performed. Histological examination demonstrated a massive varicose vein and several dilated veins in the submucosa. The patient's postoperative course was favorable, with no hemorrhagic events during a follow-up of more than 6 months after surgery. Ileal varices should be considered in the diagnosis of a patient who presents with lower gastrointestinal bleeding and portal hypertension.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
2/227. 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) |
3/227. 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) |
4/227. Improvement of pulmonary hypertension after liver transplantation.Pulmonary hypertension at the end stage of chronic liver disease is not an uncommon situation. This association termed portopulmonary hypertension raises the question of the feasibility of performing orthotopic liver transplantation (OLT). In the case reported herein, there was a favorable outcome after OLT, even though the mean pulmonary artery pressure (MPAP) before transplantation was increased to 45 mm Hg. Before OLT, the cardiac index (CI) was considerably elevated (7.69 L/min/m2), giving evidence of a marked hyperdynamic circulatory state. The CI decreased significantly after OLT (3.38 L/min/m2), and this produced a significant decrease in the MPAP. Our observation suggests that portopulmonary hypertension due to a marked increase in the CI can be managed successfully by OLT.- - - - - - - - - - ranking = 7keywords = hypertension (Clic here for more details about this article) |
5/227. 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) |
6/227. Pulmonary hypertension following hepatopulmonary syndrome in a patient with cirrhosis.We report the case of a patient with liver cirrhosis who successively developed hepatopulmonary syndrome and portopulmonary hypertension. Initially, the patient presented with severe dyspnea and hypoxemia at rest. technetium-99 macroaggregated albumin lung perfusion scan demonstrated right-to-left shunt, and hemodynamic study revealed a hyperdynamic state with low pulmonary vascular resistance, thus confirming the diagnosis of hepatopulmonary syndrome. More than 2 years after the onset of pulmonary symptoms, a marked improvement in dyspnea and gas exchange was observed. lung perfusion scan did not disclose any right-to-left shunt and right-sided heart catheterization showed evidence of severe pulmonary hypertension. We conclude that hepatopulmonary syndrome and portopulmonary hypertension are not mutually exclusive. We hypothesize that, by reversing the phenomenon of intrapulmonary vasodilatation, the development of portopulmonary hypertension interfered with each of the potential causes of hypoxemia in hepatopulmonary syndrome (ventilation-perfusion inequalities, intrapulmonary shunting, oxygen diffusion limitation) and, as a result, led to a correction of hypoxemia.- - - - - - - - - - ranking = 8keywords = hypertension (Clic here for more details about this article) |
7/227. Pulmonary hypertension in a child with juvenile-type autosomal recessive polycystic kidney disease.An 11 year-old girl, whose condition was diagnosed as juvenile-type autosomal recessive polycystic kidney disease (ARPKD) at five years of age, presented with chest pain and dyspnea that had developed suddenly two months previously. Two-dimensional echocardiography, doppler study and cardiac catheterization confirmed pulmonary hypertension. The underlying mechanism of the diagnosis was not defined. Two and a half months after the onset of symptoms, the patient died of pulmonary hypertensive crisis. Careful regular checks of cardiopulmonary status using two-dimensional echocardiography and Doppler should be considered for the early detection of pulmonary hypertension even in an asymptomatic patient with juvenile-type ARPKD.- - - - - - - - - - ranking = 6keywords = hypertension (Clic here for more details about this article) |
8/227. A large spontaneous splenorenal shunt in a patient with liver cirrhosis and uncomplicated portal hypertension.The aim of this paper is to describe and discuss, on the basis of a thorough review of the literature, the case of a 70-year-old woman with probable cirrhosis secondary to chronic hepatitis b and C, uncomplicated portal hypertension (without ascites, encephalopathy or bleeding varices), splenomegaly and hypersplenism, and an unusual, spontaneous, large splenorenal shunt and recanalization of the umbilical vein. The tortuous and varicose splenorenal shunt was diagnosed by abdominal ultrasound and CT investigations. A duplex Doppler ultrasonography evaluation was performed to study shunt flow direction and velocity. No gastroesophageal varices were identified on endoscopic examination. The clinical relevance of spontaneous splenorenal shunt, often associated with fundic gastric varices, is discussed.- - - - - - - - - - ranking = 5keywords = hypertension (Clic here for more details about this article) |
9/227. Caval inflow to the graft: a successful way to overcome diffuse portal system thrombosis in liver transplantation.portal vein thrombosis was considered to be a major contraindication to liver transplantation before the introduction of vessel grafts from the recipient's area of confluence of the splenic and superior mesenteric veins, behind the neck of the pancreas, to the graft's portal vein. Refinement in surgical technique has given rise to a large number of possibilities to overcome portal vein thrombosis in OLT recipients, ranging from portal vein thrombectomy to several different venous graft jump reconstructions. All these reconstructions require the presence of a patent vein of the portal system. When neither splanchnic veins nor sufficiently large venous collaterals are available, liver transplantation has been considered impossible. Salvage solutions include arterialization of the portal vein with the associated risk of liver damage in the longterm, a combined liver and bowel transplantation has been proposed but not yet reported (and in any case the results of combined liver and bowel transplants are not as good as those of liver transplantation alone) and finally the use of blood inflow from the inferior vena cava as first reported by Tzakis and coworkers. Portal flow from the inferior vena cava may be performed as a last resort. Although the consequences of severe pretransplantation portal hypertension remain and should be treated before, during, and after transplantation, liver function is normal in the short and midterm. With this new procedure, diffuse portal vein thrombosis is no longer an absolute contraindication to liver transplantation. But this needs to be confirmed in light of further experience and longterm followup.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
10/227. 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 = 2keywords = hypertension (Clic here for more details about this article) |
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