1/33. 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 = polycystic kidney disease, polycystic kidney, kidney disease, polycystic, kidney (Clic here for more details about this article) |
2/33. 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 = 8.3367117439637E-5keywords = kidney (Clic here for more details about this article) |
3/33. 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 = 0.00033346846975855keywords = kidney (Clic here for more details about this article) |
4/33. 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 = 0.20016673423488keywords = polycystic kidney disease, polycystic kidney, kidney disease, polycystic, kidney (Clic here for more details about this article) |
5/33. 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 = polycystic kidney disease, polycystic kidney, kidney disease, polycystic, kidney (Clic here for more details about this article) |
6/33. 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 = 1keywords = polycystic kidney disease, polycystic kidney, kidney disease, polycystic, kidney (Clic here for more details about this article) |
7/33. 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 = 0.029230039057695keywords = polycystic (Clic here for more details about this article) |
8/33. Multidisciplinary Conferences in gastroenterology. Cardiovascular effects of severe liver disease.This is the definitive review of the cardiovascular effects of liver disease. Physiologic and pathologic studies accomplished primarily in the 1950s and 1960s have been applied to a case of a 16-year-old boy with cirrhosis of the liver who presented with weakness, dyspnea, cyanosis, and clubbing. The probable mechanisms for his signs and symptoms are discussed in detail. The cause for the hyperkinetic circulation is unknown. By exclusion, the cyanosis is attributed to intrapulmonary shunting. Portapulmonary shunts are not quantitatively important. The suitability of the terms micronodular and macronodular cirrhosis is highlighted. Indications for various types of surgical portal shunts are discussed. The value of preoperative hemodynamic measurements of the portal circulation to the individual patient is debated. A liver transplant is considered in this case with no promising medical therapy and a very poor prognosis. The renal disease manifested by red cell casts in the urine is thought to be caused by an immunologic reaction in the kidney somehow related to his liver disease. This discussion is led by Dr. Jack Myers, but his opinions are correlated with those of a pathologist, radiologist, surgeon, and gastroenterologist. It is a clinical tour de force, although not all the predictions are confirmed by laboratory studies such as this patient's hepatic wedge pressure, pulmonary artery pressure, and cardiac fluoroscopy.- - - - - - - - - - ranking = 8.3367117439637E-5keywords = kidney (Clic here for more details about this article) |
9/33. Acute visual loss in a child with autosomal recessive polycystic kidney disease: case report and review of the literature.Acute visual loss secondary to ischemic optic neuropathy in children is extremely rare. The causes are usually hypotension or anemia. We describe the clinical course of a 9-year-old boy with a functional renal transplant who presented to the emergency room hemodynamically stable after waking up with complete bilateral loss of vision (no light perception). Examination showed that he had suffered massive nocturnal blood loss from esophageal varices secondary to portal hypertension. The patient's end-stage renal disease was secondary to autosomal recessive polycystic kidney disease (ARPKD), an entity comprised of renal cysts and hepatic fibrosis. Ophthalmologic findings in ARPKD are rarely cited in the literature. A literature search revealed 3 other cases of sudden visual loss reported in nonophthalmologic journals in patients with ARPKD. Funduscopic examination showed bilateral optic nerve head pallor and swelling with associated flame hemorrhages. The fact that this patient already had mildly pale nerves on presentation, along with hemodynamically compensated blood pressure and pulse, suggested chronic as well as acute ischemia. Based on our findings and other reported cases in the literature, ophthalmologic examinations may be indicated in all patients with ARPKD.- - - - - - - - - - ranking = 1keywords = polycystic kidney disease, polycystic kidney, kidney disease, polycystic, kidney (Clic here for more details about this article) |
10/33. Interstitial nephritis associated with glomerulonephritis in a patient with Hashimoto's disease and idiopathic portal hypertension.A middle-aged women with hypothyroidism, idiopathic portal hypertension and nephrotic syndrome is presented. This unusual clinical appearance could not be explained as SLE by serological examinations. Pathohistological examinations showed "Banti's liver", Hashimoto's thyroiditis and diffuse proliferative glomerulonephritis with severe tubulo-interstitial nephritis. Immunohistochemical studies revealed IgA deposits in glomeruli. Electron microscopic study disclosed peculiar lucent areas of rarefaction with osmiophilic particles in tubular basement membranes. This tubulointerstitial nephritis was considered to be related to the immunological mechanism involving thyroid gland, liver and kidney disorders. This case thus had a clinically rare combination of these three.- - - - - - - - - - ranking = 8.3367117439637E-5keywords = kidney (Clic here for more details about this article) |
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