Y Kusuyama, R Tsukino, H Oomori, K Kuribayashi, H Katayama, M Koike, K Saito
Oligomeganephronia (OMN) is a rare, renal hypoplasia, consisting of a reduced number of hypertrophied nephrons. This disorder has been considered to be a congenital but not a genetic disease. We describe the first report, to the best of our knowledge, of familial cases of OMN; two male siblings ran rapidly downhill courses and died 11 and 8 days after birth, respectively. In addition, the two patients had similar multiple anomalies; microcephaly, prominent glabella, hypertelorism, antimongoloid slant, epicanthal folds, broad nose, cleft lip and palate, down-turned mouth, short philtrum, micrognathia, low set ears, hypospadias, and cryptorchism...
March 1985: Acta Pathologica Japonica
A Janin-Mercier, J B Palcoux, M C Gubler, M de Latour, H Dalens, Y Fonck
Bilateral renal hypoplasia with oligomeganephronia, associated with bilateral tapetoretinal degeneration was observed in a child; this association has been reported only once before. Light, ultrastructural and immunofluorescent microscopic studies of the renal tissue were performed. The glomeruli were few and hypertrophic, with numerous mesangial cells, mesangial deposits, focal glomerular sclerosis and prominent thickened basement membrane. Two types of tubular changes were observed: focal necrosis of proximal tubules and focal atrophy of tubules surrounded by a thickened basement membrane...
1985: Virchows Archiv. A, Pathological Anatomy and Histopathology
K M Sergeeva, N N Smirnova, L A Klochko, I V Vorontsova
No abstract text is available yet for this article.
1988: Pediatriia
B M Brenner, D L Garcia, S Anderson
A primary role for the kidney in the initiation and maintenance of hypertension has long been recognized, but the pathogenetic interactions among renal hemodynamics, hormonal and hereditary factors, and dietary sodium intake remain enigmatic. Reduction in filtration surface area, whether acquired in the course of intrinsic renal disease or after surgical renal ablation, leads to systemic hypertension as well as to progressive renal insufficiency, sequellae made even more severe by dietary sodium excess. Moreover, hypertension and progressive renal disease eventuate in some individuals born with a solitary kidney, as well as in those with more severe degrees of dysgenesis (ie, oligomeganephronia)...
October 1988: American Journal of Hypertension
M Zeier, G Tariverdian, R Waldherr, K Andrassy, E Ritz
Acrorenal syndrome is characterized by central longitudinal axis defects of the limbs, ie, split hand and/or foot. Associated renal lesions described so far comprise agenesis, bilateral hypoplasia (originally diagnosed as oligomeganephronia), and duplication abnormalities. The case of a 29-year-old patient with split hand resulting from bilateral aplasia of the third phalanges associated with dysplasia of the third and fourth metacarpals is reported. In addition, the following lesions were noted: hypoplasia of the middle phalanx of the fifth toe, arched palate, pectus excavatum, hypoplastic mammilae, scoliosis, and congenital hip dislocation...
September 1989: American Journal of Kidney Diseases
S Nomura, G Osawa
An open renal biopsy specimen from a twelve-year-old boy with a congenital solitary kidney was studied with light, electron, and fluorescent microscopy. Focal glomerular sclerotic lesions were disclosed by these microscopic examinations. Morphometric analysis revealed statistically significant hypertrophy of the glomeruli and significant reduction in the number of glomeruli when compared with controls of similar age. These findings suggested that not only the loss of one kidney, but also congenital reduction of nephrons contributed to the development of focal glomerular sclerotic lesions in this patient...
January 1990: Clinical Nephrology
K Matsubara, K Yoshioka, N Akano, H Miyamoto, T Takemura, S Maki
Three cases of bilateral renal hypoplasia with oligomeganephronia are reported. In all three patients urine abnormalities were pointed out by annual urine screening for school-children and they showed mild or moderate deterioration of renal function. One patient had repeated episodes of urinary tract infection, and bilateral vesico-ureteral reflux (grade III) was found by the voiding cystourethrography. Light microscopic findings of renal biopsy specimens were similar in three cases; significant hypertrophy of the glomerulus and tubulus; mild mesangial expansion, and interstitial changes...
November 1990: Nihon Jinzo Gakkai Shi
M Sieniawska, D Bialasik, L Helczyński
No abstract text is available yet for this article.
December 20, 1976: Polski Tygodnik Lekarski
R D Adelman, S Shapiro
No abstract text is available yet for this article.
May 1977: Urology
F C Berthoux, S Khalil, J C Sabatier, S Colon, D Lyonnet, C Veyret
No abstract text is available yet for this article.
December 1978: Journal D'urologie et de Néphrologie
A Mansour, R D Reye, L P Roy
No abstract text is available yet for this article.
September 1977: Australian Paediatric Journal
L Pallardo, J Egido, A Barat, J L Alió, L Hernando, C Alférez
No abstract text is available yet for this article.
May 1979: Actas Urologicas Españolas
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