1/46. Portal hypertension due to extensive hepatic cysts in autosomal dominant polycystic kidney disease.Liver cysts are a well-recognized feature of autosomal dominant polycystic kidney disease (ADPKD) and occur in 77% of patients more than 60 years old. Serious sequelae, however, are rare, the two most common complications being pain and cyst infections. Portal hypertension has been reported in ADPKD due to the rare presence of congenital hepatic fibrosis. We report a case of ADPKD in a patient who had portal hypertension due to distortion of portal vein and venules by extensive hepatic cysts.- - - - - - - - - - ranking = 1keywords = autosomal dominant polycystic kidney, autosomal dominant polycystic, dominant polycystic kidney, dominant polycystic, polycystic kidney, polycystic, autosomal dominant, kidney, dominant (Clic here for more details about this article) |
2/46. Successful treatment of chronic budd-chiari syndrome with a transjugular intrahepatic portosystemic shunt.budd-chiari syndrome is characterized by obstruction of the hepatic venous outflow tract. Therapeutic options for chronic budd-chiari syndrome are limited. We report the case of a 28-year-old woman who presented with recurrence of chronic budd-chiari syndrome with total obstruction of all major hepatic veins. Due to worsening liver function over the course of 1 year, she had to be listed for liver transplantation. Because of therapy-refractory ascites, declining renal function and severe esophageal varices, a transjugular intrahepatic portosystemic shunt (TIPS) was placed, planned as a bridge to transplantation. Following TIPS, a marked recovery of liver function could be observed, accompanied by disappearance of ascites, esophageal varices, and normalization of kidney function. Therefore, the patient could be removed from the waiting list for liver transplantation. This case demonstrates for the first time that the use of TIPS in chronic budd-chiari syndrome may result in marked recovery of liver function.- - - - - - - - - - ranking = 0.00010986463245094keywords = kidney (Clic here for more details about this article) |
3/46. rupture of the left kidney following renosplenic shunt.Kidney rupture following division of the left renal vein during portal decompression is presented. A review of the renal venous anatomy is described and a possible explanation of this ususual occurrence is offered.- - - - - - - - - - ranking = 0.00043945852980378keywords = kidney (Clic here for more details about this article) |
4/46. Chronic renal failure and portal hypertension--is portosystemic shunt indicated?We report two girls with histories of recessive polycystic kidney disease. Both were on maintenance hemodialysis. They had undergone surgical distal portocaval shunt because of portal hypertension. Later, bilateral nephrectomy was performed, and they presented with hepatic encephalopathy (HE) and evolution towards irreversible hepatic coma and death. Portosystemic shunt is the treatment of choice of portal hypertension. The kidney plays a pivotal role in ammonia disposal during portosystemic shunt. Thus, we stress the risk of HE after portosystemic shunt followed by bilateral nephrectomy in patients with end-stage renal failure and suggest that combined liver-kidney transplantation should be considered.- - - - - - - - - - ranking = 0.028730873851178keywords = polycystic kidney, polycystic, kidney (Clic here for more details about this article) |
5/46. Splenic vascular malformations and portal hypertension in hereditary hemorrhagic telangiectasia: sonographic findings.Hereditary hemorrhagic telangiectasia, or Osler-Rendu-Weber disease, is an autosomal dominant disorder in which a variety of vascular dysplasias occur throughout the organ systems. We report the gray-scale and color Doppler sonographic findings in a case of hereditary hemorrhagic telangiectasia. Gray-scale sonographic examination revealed massive splenomegaly, multiple dilated intrasplenic vascular structures (some with adjacent punctate calcifications), an aneurysmal dilatation of the splenic vein, dilated intrahepatic portal branches, and marked atrophy of the right hepatic lobe. color Doppler sonography showed dilatation of the truncus coeliacus and high-velocity flow in the splenic artery. There were significant aliasing in the splenic hilum and an abnormal, arterialized flow in intrasplenic branches of the splenic vein. The splenic vein was massively enlarged with increased flow velocity and contained an isolated aneurysmal dilatation in the hilum. There were multiple serpiginous retroperitoneal collateral vessels, and the left gastric vein was dilated with hepatofugal flow. The patient had portal hypertension that developed secondary to the increased portal flow.- - - - - - - - - - ranking = 0.00025646507705637keywords = autosomal dominant, dominant (Clic here for more details about this article) |
6/46. A case of successful management of portosystemic shunt with autosomal dominant polycystic kidney disease by balloon-occluded retrograde transvenous obliteration and partial splenic embolization.We describe a patient with autosomal dominant polycystic kidney disease who was successfully managed for severe abdominal distension, impaired liver function and a portosystemic shunt by interventional therapies. The patient's intra-hepatic portal vein was compressed and narrowed by multiple liver cysts, which resulted in a decrease of the portal blood flow and portal hypertension due to a huge gastro-renal shunt These haemodynamic changes were assumed to contribute to insufficient protein synthesis in the liver. Therefore, we first repeatedly performed minocycline hydrochloride instillations to treat the multiple liver cysts. Then, we conducted a partial splenic embolization to prevent elevation of the portal vein pressure prior to balloon-occluded retrograde transvenous obliteration which was performed to increase the portal blood flow. The portal blood flow markedly increased, and protein synthesis in the liver also recovered and the clinical symptoms improved. The patient has been monitored for more than two years up to the present and her liver function parameters have remained within the normal range. renal insufficiency is known to be a major prognostic factor in autosomal dominant polycystic kidney disease. In some cases, however, liver involvement with multiple cysts may result in a fatal outcome. In such cases, interventional therapies, as provided to this patient, should be considered.- - - - - - - - - - ranking = 1.2keywords = autosomal dominant polycystic kidney, autosomal dominant polycystic, dominant polycystic kidney, dominant polycystic, polycystic kidney, polycystic, autosomal dominant, kidney, dominant (Clic here for more details about this article) |
7/46. Interesting RI accumulation in hepatic images with Tc-99m GSA SPECT scintigraphy in idiopathic portal hypertension.The authors report a case of idiopathic portal hypertension (IPH), which showed interesting RI accumulation on Single Photon Emission CT (SPECT) images, with Tc-99m galactosyl human serum albumin (GSA) scintigraphy. Accumulation of Tc-99m GSA was decreased in the periphery of the liver where strong enhancement was revealed only in the arterial phase on dynamic CT. These findings imply that portal flow is decreased in the periphery of the liver where arterial flow is dominant. It was thought that secondary reduced activity of GSA due to a decrease in portal flow results in reduced radioactivity in the periphery of the liver in IPH. This interesting accumulation of Tc-99m GSA may be one of the sign of IPH.- - - - - - - - - - ranking = 2.9437151960474E-5keywords = dominant (Clic here for more details about this article) |
8/46. Transjugular intrahepatic portosystemic shunt prior to renal transplantation in a child with autosomal-recessive polycystic kidney disease and portal hypertension: A case report.Autosomal-recessive polycystic kidney disease (ARPKD) can cause renal failure and portal hypertension in children. Portal hypertension may complicate the course of renal transplantation (Tx). We report the successful outcome of a patient with end-stage renal disease (ESRD) and portal hypertension treated with transjugular intrahepatic portosystemic shunt (TIPS), a minimally invasive endovascular technique of portosystemic shunt, prior to renal Tx.- - - - - - - - - - ranking = 0.14255572293138keywords = polycystic kidney, polycystic, kidney (Clic here for more details about this article) |
9/46. Portal hypertension due to intrahepatic obstruction in non-Hodgkin's lymphoma.Portal hypertension is rare in the setting of non-Hodgkin's lymphoma. We report here the case of a 73-year-old man presenting with diffuse high-grade B-cell lymphoma affecting predominantly the liver with large space occupying lesions. Histological examination of liver specimens showed abnormal large lymphoid cells whereas adjacent non-tumoural liver was normal. Portal hypertension was documented by upper gastrointestinal endoscopy that showed grade II oesophageal varices and measurement of portal pressures via transjugular approach showing increased hepatic venous pressure gradient (24 mmHg). We assume that portal hypertension was mainly related to these space occupying lesions.- - - - - - - - - - ranking = 2.9437151960474E-5keywords = dominant (Clic here for more details about this article) |
10/46. Double selective shunting for esophagogastric and rectal varices in portal hypertension due to congenital hepatic polycystic disease.A 55-year-old woman with ascites, pancytopenia by hypersplenism, recurrent hemorrhagic esophagogastric varices, and large rectal varices due to congenital hepatic polycystic disease underwent splenectomy and simultaneous double selective shunt; a left gastric venacaval direct shunt for esophagogastric varices and a sigmoid venous left ovarian vein shunt for rectal varices. Her preoperative child-Pugh grade was A (score 6). Postoperative course was uneventful. Serum NH3 level decreased from 90 micrograms/dL to 36 micrograms/dL after shunt surgery. She was discharged on the 21st postoperative day. The remarkable improvement of both the esophagogastric varices and the rectal varices was demonstrated by postoperative fiberscope. We strongly consider sigmoid venous left ovarian shunting to be as selective as the Inokuchi shunt preventing encephalopathy and an effective surgical approach to anorectal varices.- - - - - - - - - - ranking = 0.038532113304486keywords = polycystic (Clic here for more details about this article) |
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