Cases reported "Kidney Diseases, Cystic"

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1/15. Acquired glomerulocystic kidney disease following hemolytic uremic syndrome.

    Glomerulocystic kidney disease (GCKD) is a rare congenital condition that is usually reported in infants and young children. Only five cases of acquired GCKD after an acquired renal disease have been reported. Among these, two patients have developed cystic glomerular lesions following hemolytic uremic syndrome (HUS). We report a third case in a 2-year-old patient with this association. Common features between these three cases include atypical HUS, development of GCKD after prolonged peritoneal dialysis treatment, severe hypertension, and normal-sized kidneys without development of macroscopic cysts. pathology findings in our patient include heavy expression of epidermal growth factor receptor in proximal tubules and evidence of obstruction of the glomerular outflow. We speculate that cystic dilatation of the Bowman's capsule may be secondary to ischemic lesions leading to proximal tubular obstruction.
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2/15. A spectrum of renal tubular ectasia and hepatic fibrosis.

    Renal tubular cystic disease and hepatic fibrosis exhibit a specific genetic pattern and pathological findings. The renal collecting tubules are dilated and the liver shows fibrosis with proliferation and dilatation of the bile ducts. The findings fall into a spectrum with marked renal disease and mild liver involvement at one end and mild renal involvement with severe liver disease at the other. Between these extremes lies an intermediate form which is genetically and pathologically similar, but exhibits a wide range of clinical and radiological findings due to the variability of renal and liver involvement.
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3/15. Infantile multilocular renal cyst.

    A case of a Japanese 14-month-old male infant with multilocular renal cyst is presented. He was admitted with a chief complaint of an abdominal mass. Although clinically and radiographically multilocular renal cyst in the right kidney was suspected, total nephrectomy was performed because of extreme difficulty of partial nephrectomy and impossibility of complete neglect of malignant cystic tumor. Microscopically, immature glomeruloid tissue, primitive tubules, rhabdomyocytes and nerve fibers were noted. The histological diagnosis of multilocular renal cyst with partially differentiated dysplasia was made. Since the first report by Edmunds in 1892, the etiology of multilocular renal cyst has been discussed for years. In our present case, the pathologic findings suggest that multilocular cystic formation in the right kidney might be the result of developmental disturbances.
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4/15. angiomyolipoma with epithelial cysts (AMLEC): a distinct cystic variant of angiomyolipoma.

    Renal angiomyolipoma (AML) is typically a solid lesion, composed of varying amounts of adipose, vascular, and muscular tissue, lacking an epithelial component. Although it is known that entrapped renal tubules may be observed in AML, presentation as a cystic mass has not been previously reported. We report the clinicopathologic and immunohistochemical features of four cystic renal AML. The lesions were found in 2 male and 2 female patients, ranging in age from 37 to 76 years, none with a history of hormonal therapy. One of the four patients had known tuberous sclerosis, and this patient and 1 other presented with bilateral cystic renal lesions. Grossly, the lesions measured between 1.3 and 4.5 cm in greatest dimension. Histologically, the lesions were each composed of three components: 1) epithelial cysts lined by cuboidal to hobnail cells; 2) a compact subepithelial "cambium-like" layer of cellular, mullerian-like AML stroma with prominent admixed chronic inflammation; and 3) muscle-predominant AML with associated dysmorphic blood vessels exterior to the cellular subepithelial stroma. Immunohistochemically, the stromal components of all four lesions labeled with HMB-45 and Melan-A, most intensely in the cellular subepithelial stroma, whereas smooth muscle actin and desmin demonstrated the opposite pattern, with greatest intensity in the muscle-predominant AML areas. Immunoreactivity for estrogen and progesterone receptors (ER, PR) and CD10 was present in all cases, with strongest and most diffuse staining in the subepithelial AML cells. The cyst lining cells were positive for pancytokeratin and soy bean agglutinin (the latter characteristic of distal nephron epithelium) but did not label for ER, PR, or melanocytic markers. Although these distinctive lesions may be confused with mixed epithelial-stromal tumor, their lack of female predominance or association with hormone therapy, their distinctive dysplastic blood vessels and disorganized exterior muscular layer, along with their immunohistochemical profile, support their classification as AML with epithelial cysts. Whereas the cysts appear to arise primarily from entrapped collecting ducts, the peculiar subepithelial condensation of AML stromal cells, with morphologic and immunohistochemical features suggesting differentiation toward endometrial stroma, may represent epithelial-induced mullerian differentiation not previously reported in AML.
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5/15. Ultrasonographic features of glomerulocystic disease in infancy: similarity to infantile polycystic kidney disease.

    Glomerulocystic kidney disease (GCD) is an unusual condition characterized pathologically by cystic dilatation of Bowman's space and the first portion of the proximal convoluted tubule. We report the serial ultrasound findings in an asymptomatic infant with GCD which were initially confused with infantile polycystic kidney disease. We emphasize the nonspecificity of ultrasonographic findings in cystic renal disease in early infancy and suggest a protocol for the diagnostic work-up of bilateral renal cystic disease in children.
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6/15. Renal retinal dysplasia with diffuse glomerular cysts.

    A 32-year-old male with renal retinal dysplasia is presented. He also showed hearing loss and growth retardation. Laboratory data showed mild proteinuria, renal dysfunction and type-1 renal tubular acidosis. Computed tomography showed multiple cysts at the corticomedullary junction of both kidneys. Ocular examinations disclosed retinitis pigmentosa. On light microscopy of renal biopsy specimens, diffuse cystic dilatation of Bowman's space as well as dilated tubules with interstitial fibrosis and cellular infiltration were noted. Electron microscopy revealed a peculiar chromatin condensation of epithelial cell nuclei in Bowman's capsules, glomeruli and tubules. The association of renal retinal dysplasia with diffuse glomerular cysts has not, to our knowledge, been previously reported.
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7/15. Diverticula on distal tubule, simple renal cysts, and ureteral obstruction. Causal relationship.

    A case of obstruction on a localized kidney zone is reported. Obstruction was considered a precipitating factor in the morphogenesis of diverticula on the distal tubules and the simple cyst, found exclusively in the adjacent renal parenchyma to the obstruction.
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8/15. Multilocular renal cyst. Case report, ultrastructure and review of the literature.

    Multilocular renal cyst is an uncommon focal, unilateral, cystic, epithelial lesion of uncertain pathogenesis. Because nephroblastomatous foci have been found on microscopic examination of several of the reported cases, some authors have proposed that multilocular cysts are differentiated and cystic forms of Wilms' tumour. This proposition is analysed and it is concluded that three possibly interrelated lesions may be defined: (1) Wilms' tumours showing cystic differentiation. (2) Lesions macroscopically indistinguishable from multilocular cyst containing variably differentiated nephroblastomatous foci. All such cases have been described in infants. (3) Typical multilocular renal cysts, which have described in children and adults. review of the literature shows that several cases have been included in the second category solely because of the presence of small intraseptal tubules. Consequent anomalies of interpretation are highlighted and a further case of multilocular cyst in an adult female is reported in which ultrastructural examination, recorded for the first time, confirms the epithelial nature of the cyst lining cells which resemble simplified renal tubular epithelium.
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9/15. Multilocular renal cyst: electron microscopic evidence of pathogenesis.

    Two cases of multilocular renal cyst (MRC) are presented with electron microscopic findings. The epithelial cells of the cysts resembled renal collecting tubule cells and not convoluted tubule or Henle's loop cells. Intercystic areas contained only connective tissue elements. Simple MRC appears to be a congenital malformation that results from segmental maldevelopment of the ureteric bud. In some cases of MRC, the intercystic septa contain immature renal elements. The lesion is then called cystic poorly differentiated nephroblastoma (CPDN). However, in both MRC and CPDN, local recurrence or metastases have not been described. Treatment of these lesions should consist of nephrectomy, careful histologic evaluation to rule out obvious foci of Wilms' tumor, and conservative follow-up.
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10/15. Hyperreninemia, lysozymuria, and erythrocytosis in fanconi syndrome with medullary cystic kidney.

    adult onset fanconi syndrome with medullary cystic kidney was diagnosed in a 30-year-old male with muscular weakness, hypokalemia, normal BP, hyperreninemia, and secondary aldosteronism. He also had non-specific aminoaciduria, lysozymuria, and beta 2-microglobulinuria. Urinary concentrating and acidifying capacity was impaired, and both sodium and potassium were lost into the urine. I.v. pyelography revealed medullary cystic kidney. Renal biopsy showed juxtaglomerular hyperplasia, heavy subintimal deposits and C3 and IgG in preglomerular arteriolar walls, and degenerative changes in the tubules, including loss of brush border and "macula densa-like" lesions. polycythemia with elevated serum erythropoietin levels, and raised blood ACTH values with features of cortisolism were also present. indomethacin therapy decreased plasma renin activity (PRA), plasma aldosterone, and urinary loss of potassium and sodium, while serum potassium approached normal levels. metoprolol, a beta-adrenergic blocking agent, caused similar effects. Insensitivity to the pressor effect of angiotensin ii was reversed by indomethacin treatment. somatostatin infusion lowered PRA and aldosterone without affecting BP. Several biochemical aberrations of this patient resemble Bartter's syndrome, including the effect of indomethacin.
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