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Mutation-Related Oligomeganephronia in a Young Adult Patient.
Case Rep Nephrol Dial
November 2020
Department of Pathology, Albert Szent-Györgyi Health Center, University of Szeged, Szeged, Hungary.
Oligomeganephronic hypoplasia, commonly referred to as oligomeganephronia (OMN), is a rare pediatric disorder characterized by small kidneys. Histologically a paucity of nephrons is observed which show compensatory enlargement. Hyperfiltration injury leads to end-stage kidney disease.
View Article and Find Full Text PDFRenal Hypoplasia, From Grossly Insufficient to Not Quite Enough: Consideration for Expanded Concepts Based Upon the Author's Perspective With Historical Review.
Adv Anat Pathol
September 2020
Arkana Laboratories, Little Rock, AR.
Hypoplasia is defined in the Merriman-Webster dictionary as "a condition of arrested development in which an organ, or part, remains below the normal size, or in an immature state." The degree of reduced size is not definitional. Renal hypoplasia, however, has historically been defined as a more marked reduction in renal mass such that presentation in childhood is the norm.
View Article and Find Full Text PDFPAX2 mutations in fetal renal hypodysplasia.
Am J Med Genet A
April 2010
AP-HP, Unit of Embryo-Fetal Pathology, Department of Histo-Embryology and Cytogenetics, Necker Hospital, Paris, France.
Papillorenal syndrome also known as renal-coloboma syndrome (OMIM 120330) is an autosomal dominant condition comprising optic nerve anomaly and renal oligomeganephronic hypoplasia. This reduced number of nephron generations with compensatory glomerular hypertrophy leads towards chronic insufficiency with renal failure. We report on two fetuses with PAX2 mutations presenting at 24 and 18 weeks' gestation, respectively, born into two different sibships.
View Article and Find Full Text PDFAge-related pathophysiological changes in rat oligomeganephronic hypoplastic kidney.
Pediatr Nephrol
May 2006
Department of Veterinary Physiology, Nippon Veterinary and Life Science University, 1-7-1 Kyonano-cho, Musashino-shi, Tokyo, 180-8602, Japan.
Rat male hypogonadism (hgn/hgn) is accompanied by oligomeganephronic hypoplastic kidney (HPK), in which each kidney contains one quarter the number of nephrons present in a normal kidney. The nephrons of the HPK are extremely hypertrophied. These characters were apparently common to human oligomeganephronia (OMN).
View Article and Find Full Text PDFGlomerular hyperfiltration and hypertrophy in the rat hypoplastic kidney as a model of oligomeganephronic disease.
Nephrol Dial Transplant
July 2005
Department of Veterinary Physiology, Nippon Veterinary and Animal Science University, 1-7-1 Kyonano-cho, Musashino-shi, Tokyo 180-8602, Japan.
Background: Rat male hypogonadisim (hgn/hgn) is accompanied by bilateral hypoplastic kidney (HPK). The HPK contains a reduced number of nephrons that progress to chronic renal failure. In this study, we describe the renal pathophysiology in adult HPK rats as a potential model of oligomeganephronic disease.
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