Cases reported "Kidney Diseases, Cystic"

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1/535. Solitary renal cyst, hypertension and renin.

    Solitary renal cysts may cause renin hypersecretion with associated hypertension by compressing surrounding tissue and by distortion of renal vessels. Selective measurements of plasma renin activity in the renal veins can predict the antihypertensive effect of decompression. An illustrative case is presented and its significance is discussed. ( info)

2/535. Pathogenic role of glomerulo-tubular junction stenosis in glomerulocystic disease.

    Glomerulocystic disease is an uncommon cystic renal condition characterized by cystic dilatation forming a glomerular cyst. The pathogenesis of this familial disease is unknown. We performed a serial section study using a biopsy specimen of a 16-year old female patient with glomerular cystic disease who had a family history of end stage renal failure. A total of 14 different glomeruli were analyzed, four of which exhibited a cystic appearance. Five glomerulotubular junctions were observed by serial sections, two of which had a stenotic appearance where glomerular cystic changes and periglomerular fibrosis were observed concomitantly. There were no such cystic glomerular changes in the other three glomeruli with non-stenotic glomerulo-tubular junctions. These findings suggest that the glomerular cystic lesion develops as a consequence of glomerulo-tubular junctional stenosis probably caused by periglomerular fibrosis. ( info)

3/535. Hereditary nephritis (with unusual renal histology): report of a first case from the west indies.

    A 21-year-old Grenadian girl undergoing investigation in Trinidad for anaemia was diagnosed as a case of hereditary nephritis. She had the clinical features of a nephropathy, nerve deafness and an ocular defect. Renal histology was exceptional in that in addition to the typical findings of a hereditary nephritis, cystic areas generally associated with medullary cystic disease were noted. Several members of the patient's maternal family were afflicted with either deafness visual distrubances or renal disease. ( info)

4/535. Transitional cell carcinoma arising in a pyelocaliceal cyst. An unusual cystic renal lesion with cytologic and imaging findings.

    The differentiation between benign and malignant cystic lesions of the kidney is a diagnostic challenge. Medical imaging aids in this task, but many cystic renal lesions require further work-up, frequently by computed tomography-guided fine needle aspiration. We report on the pathological findings in a case of moderately differentiated papillary transitional carcinoma, which arose in a pre-existing pyelocaliceal cyst in a 53-year-old man. In the case of this lesion, the distinction between a benign and a malignant renal cyst is blurred. To our knowledge, this is the third such occurrence to be reported and the first to be diagnosed by fine needle aspiration biopsy. ( info)

5/535. Prenatal collapse of cysts in a dysplastic kidney.

    Postnatal regression of prenatally or neonatally detected multicystic dysplastic kidney disease has been widely documented. However, renal cysts can regress during gestation, although they usually become larger in utero. We present a case of prenatally detected multicystic dysplastic kidney with an atypical course. During the third trimester, unilateral multicystic renal lesions in the fetus first enlarged and later involuted; by the second postpartum year, the kidney had become hypoplastic and nonfunctional. Our case also shows that before birth, blood flow in the affected kidney, measured with Doppler imaging, was normal until the cysts involuted. ( info)

6/535. dialysis-associated renal cystic disease resembling autosomal dominant polycystic kidney disease: a report of two cases.

    Acquired renal cystic disease is common in patients receiving dialysis. Characteristically, the kidneys are small or, less often, normal in size, and the cysts are usually less than 0.6 cm in diameter. We present here 2 patients who, after 5 and 7 years on hemodialysis, developed marked renal enlargement, with large cysts in the kidneys and, in 1 patient, in the liver as well; the appearance on ultrasonography and computed tomography was indistinguishable from autosomal dominant polycystic kidney disease. Before starting dialysis the first patient was a 19-year-old man who developed renal shutdown from crescentic glomerulonephritis, and the second patient was a 33-year-old man who developed end-stage renal failure from malignant hypertension. Neither patient had renal cysts at the onset of end-stage renal failure. ( info)

7/535. A novel syndrome of diabetes mellitus, renal dysfunction and genital malformation associated with a partial deletion of the pseudo-POU domain of hepatocyte nuclear factor-1beta.

    Mutations in the homeodomain-containing transcription factor hepatocyte nuclear factor (HNF)-1beta are the cause of one form of maturity-onset diabetes of the young (MODY), type 5 (MODY5). We have studied a Norwegian family, N5, with a syndrome of mild diabetes, progressive non-diabetic renal disease and severe genital malformations. The sequence of the HNF-1beta gene ( TCF2 ) revealed a 75 bp deletion in exon 2 (409-483del) which would result in the synthesis of a protein lacking amino acids Arg137 to Lys161 (R137-K161del). This deletion is located in the pseudo-POU region of HNF-1beta, a region implicated in the specificity of dna binding. Functional studies of R137-K161del HNF-1beta revealed that it could not bind an HNF-1 target sequence or stimulate transcription of a reporter gene indicating that this is a loss-of-function mutation. The R137-K161del allele co-segregated with diabetes and renal disease in pedigree N5. In addition, two of four female carriers with this mutation had vaginal aplasia and rudimentary uterus (Mullerian aplasia). These studies strongly suggest that heterozygous mutations in the HNF-1beta gene are associated with a syndrome characterized by MODY and severe, non-diabetic renal disease. Moreover, the presence of internal genital malformations in two females suggests that additional clinical features may be associated with HNF-1beta mutations. ( info)

8/535. Renal cortical outline evaluation in excretory urography.

    Capillary phase nephrogram films and tomography were used to evaluate renal cortical outlines and renal parenchymal masses. The method of examination and the results are presented, showing the capillary phase film to be beneficial and to play a significant role in excretory urography. No increase in the incidence of side effects was noted with the rapid injection technique. ( info)

9/535. A case of totally cystic fibroepithelial polyp of the renal pelvis.

    Fibroepithelial polyps are the most frequently observed mesenchymal tumors of the renal pelvis. We report on one case of fibroepithelial polyp of the renal pelvis with unusual CT findings of totally cystic structure with septations. ( info)

10/535. Somatic mosaicism in von hippel-lindau disease.

    von Hippel-Lindau (VHL) disease is an autosomal dominant familial cancer syndrome predisposing to the development of retinal and central nervous system haemangioblastomas, pheochromocytomas, renal and pancreatic cancer. In the course of a molecular analysis conducted to detect germline mutations of this gene in von Hippel-Lindau patients and individuals affected by sporadic tumors, we have identified a case of somatic mosaicism in the asymptomatic mother of a VHL patient who was subsequently diagnosed with pheochromocytoma. This is the first report providing molecular evidence of somatic mosaicism in von hippel-lindau disease. mosaicism could provide some genetic explanation for the clinical heterogeneity and variable severity of the VHL phenotype, and should be considered, as a possible event when evaluating sporadic cases of VHL or patients with isolated VHL-related tumors. Hum Mutat 15:114, 2000. ( info)
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