1/464. 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.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
2/464. Intrachromosomal triplication of 2q11.2-q21 in a severely malformed infant: case report and review of triplications and their possible mechanism.A female fetus with brain malformations, multicystic kidneys, absence of the right thumb, and a posterior cleft of palate was delivered at 32 weeks of gestation. Cytogenetic studies including FISH showed a novel intrachromosomal triplication of the proximal long arm of chromosome 2 (q11.2-q21), resulting in tetrasomy for this segment. The middle repeat was inverted. At least 11 patients with intrachromosomal triplications have been reported, mostly involving chromosome 15q. The mechanism involved in formation of these rearrangements is compatible with U-type exchange events among three chromatids.- - - - - - - - - - ranking = 0.14285714285714keywords = kidney (Clic here for more details about this article) |
3/464. 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.- - - - - - - - - - ranking = 1.1428571428571keywords = kidney (Clic here for more details about this article) |
4/464. 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.- - - - - - - - - - ranking = 1.1428571428571keywords = kidney (Clic here for more details about this article) |
5/464. 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.- - - - - - - - - - ranking = 0.85714285714286keywords = kidney (Clic here for more details about this article) |
6/464. Nocardia thyroiditis: unusual location of infection.nocardia asteroides complex is an important opportunistic agent in immunocompromised hosts. Usually, primary pulmonary infection occurs and is followed by dissemination of the pathogen to the central nervous system and soft tissues. As described in the literature, almost every organ can be infected, but to our knowledge, Nocardia has been described as a pathogen responsible for thyroid abscess in only one report, which was published in 1993. The present report is the second case report of Nocardia thyroiditis. The patient was under immunosuppressor treatment following a combined liver-kidney transplant and presented with a preexisting nodular goiter which was probably a predisposing factor to the start and development of the thyroid infection.- - - - - - - - - - ranking = 0.14285714285714keywords = kidney (Clic here for more details about this article) |
7/464. 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.- - - - - - - - - - ranking = 0.85714285714286keywords = kidney (Clic here for more details about this article) |
8/464. 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.- - - - - - - - - - ranking = 0.28571428571429keywords = kidney (Clic here for more details about this article) |
9/464. 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.- - - - - - - - - - ranking = 0.28571428571429keywords = kidney (Clic here for more details about this article) |
10/464. 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.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
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