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1/323. Polycystic horseshoe kidney.

    It is not uncommon for urologists to be involved in the care of patients with either adult polycystic kidney disease or horseshoe kidney. Personal clinical experience indicates that it is most unusual to have these 2 congenital disorders simultaneously in the same patient. The first known report of such a situation describing removal of a kidney so involved in preparation of renal transplantation is presented.
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keywords = polycystic
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2/323. Recurrent fetal polycystic kidneys associated with glutaric aciduria type II.

    A woman had two pregnancies terminated in the 20th and 21st weeks of gestation after ultrasonographic detection of enlarged hyperechoic kidneys in both fetuses. The combination of polycystic kidneys and steatotic liver found at autopsy suggested glutaric aciduria type II (GA II), which was confirmed by biochemical investigation. GA II or multiple acyl-coa dehydrogenase deficiency is an autosomal recessively inherited defect of mitochondrial energy metabolism, which usually results in neonatal death. When pregnancy is terminated because of enlarged hyperechoic kidneys in the fetus, autopsy is crucial for establishing the correct diagnosis. The combination of polycystic kidneys and steatotic liver should bring GA II to mind, and prompt appropriate biochemical investigations so that genetic counselling and first trimester diagnosis can be offered in future pregnancies.
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ranking = 6
keywords = polycystic
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3/323. Isolated polycystic liver disease not linked to polycystic kidney disease 1 and 2.

    Autosomal dominant polycystic liver disease occurs commonly in association with autosomal dominant polycystic kidney disease, types 1 and 2. It may also exist as a separate entity, genetically distinct from autosomal dominant polycystic kidney disease types 1 and 2, as has been recently established to exist in a Belgian family. We report here a large Argentinian family of Spanish-Belgian ancestry with autosomal dominant polycystic liver disease, where proximal and distal markers for both polycystic kidney disease 1 and 2 failed to demonstrate genetic linkage. The data support the notion that polycystic liver disease and autosomal dominant polycystic kidney disease may have separate chromosomal loci.
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ranking = 15
keywords = polycystic
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4/323. Hemihypertrophy in a boy with renal polycystic disease: varied patterns of presentation of renal polycystic disease in his family.

    Hemihypertrophy is a condition that has been described in association with a variety of other malformations and diseases; quite often these have had a renal origin. -It is the purpose of this paper to record and discuss the occurrence of polycystic disease in a family, to note that the disease was manifest as either the "adult" or the "infantile" form, and detail the fact that one member of the family who had infantile type polycystic disease also had hemihypertrophy. -The pattern of inheritance of renal polycystic disease, its age at presentation and the variation in the boy of its manifestation with the passage of time are discussed. The need for caution in offering a prognosis and genetic counselling is stressed.
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ranking = 11
keywords = polycystic
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5/323. Fibropolycystic disease of the hepatobiliary system and kidneys.

    This complicated case of fibropolycystic disease of the hepatobiliary system and kidneys was ably and incisively analyzed by Professor Sheila Sherlock. Her clinical acumen was revealed by her ability to differentiate congenital hepatic fibrosis, Caroli's disease, and adult polycystic disease of the liver and kidney. Interesting histologic features of this case included hepatic fibrosis with intact limiting plates anc central veins and the presence of bile plugs in the ducts, but the absence of bile statsis in the parenchyma. A percutaneous transhepatic cholangiogram demonstrated the dilated intrahepatic and extrahepatic ducts. Washing out the "gunk" from the biliary tract by T-tube drainage has great limitations in this type of case. Therefore, Dr. Adson suggested irrigation of the biliary ductal system using tubed placed transhepatically, plus a wide choledojejunostomy. Dr. Sherlock questioned this surgical approach. The use of chenodeoxycholic acid for this "gunk" was suggested. In spite of the dilated ducts and pathologic changes in the liver, the patient was not jandiced and did not have stones in her biliary tract. The genetics of this patient's problems was discussed.
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ranking = 6
keywords = polycystic
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6/323. choledochal cyst associated with polycystic kidney disease: report of a case.

    We report a very rare case of type I choledochal cyst associated with a polycystic kidney disease. A 48-year-old female had been dependent on hemodialysis for chronic renal failure due to polycystic kidney disease and was incidentally diagnosed to have a dilated common bile duct by an ultrasonography. An endoscopic retrograde cholangiopancreatography showed a spindle-shaped, dilated common bile duct (type I choledochal cyst) without visualization of the pancreatic duct. She underwent a resection of the choledochal cyst. Intraoperative cholangiography showed no reflux of contrast medium into the pancreatic duct. Amylase level of the aspirated bile from the bile duct was not elevated. In the case of choledochal cyst combined with renal fibropolycystic disease, pancreaticobiliary maljunction may not contribute to the etiology of choledochal cyst. In such cases, management of choledochal cyst is still controversial and requires further discussion.
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ranking = 7
keywords = polycystic
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7/323. adenoma of the ampulla of vater: a genetic condition?

    The etiology of adenoma of the ampulla of vater is not well understood. Previous authors reported the association of this neoplasm with polycystic kidney disease of two fraternal sisters. They concluded that these two conditions were somehow related. We describe a case of ampullary adenoma associated with polycystic kidney disease. This presentation raises again the question of a possible link between these two diseases.
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ranking = 2
keywords = polycystic
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8/323. Neonatal detection and evaluation of infantile polycystic disease by gray scale echography.

    Infantile polycystic disease (IPCD) is an uncommon pathologic entity involving the kidneys and liver. Gray scale echography can detect this pathologic process within the kidneys, despite the presence of renal failure. In addition, the sonic study may detect associated hepatic abnormalities even though isotopic liver scan is normal. These capabilities make the ultrasonic examination uniquely suited for evaluating patients with IPCD.
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ranking = 5
keywords = polycystic
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9/323. prenatal diagnosis of autosomal recessive polycystic kidney disease. A case report.

    We present a case of autosomal recessive polycystic kidney disease diagnosed at 28 weeks' gestation by ultrasonographic examination and magnetic resonance imaging (MRI). The fetal kidneys were symmetrically enlarged and highly echogenic by ultrasonographic examination and showed high-signal intensity on T2-weighted images by MRI. Cystic lesions were recognized by neither examination. In addition, the pulsatility index of the fetal renal artery was normal. These findings suggest a high water content in the renal parenchyma with tiny cysts and normal blood flow in autosomal recessive polycystic kidney disease.
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ranking = 6
keywords = polycystic
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10/323. Retroperitoneoscopic management of infected cysts in adult polycystic kidney disease.

    Conservative measures are the mainstay of therapy in adult polycystic kidney disease (APKD). Pain, infection and obstructive uropathy are the major indications for intervention. chronic pain has been treated with narcotic analgesics, needle aspiration of dominant cysts, and open renal cyst decortication. Laparoscopic cyst decortication, by either transperitoneal or retroperitoneal access, is a new emerging option with similar efficacy to open surgery and less morbidity. Cyst infection in these patients responds poorly to commonly used antibiotics. patients with refractory cyst infection may even require nephrectomy. Herein, we present 2 cases with APKD that were treated by retroperitoneoscopic decortication for painful and infected cysts. Both patients showed prompt and sustained improvement in symptoms, with minimal morbidity and short convalescence. This approach has not hitherto been described for infected cysts in APKD. The retroperitoneoscopic route should be preferred in the presence of infected cysts so as to prevent intraperitoneal contamination.
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ranking = 5
keywords = polycystic
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