Congenital hypernephronic nephromegaly with tubular dysgenesis: a distinctive inherited renal anomaly

• Published in: Pediatric pathology Vol. 4; no. 3-4; p. 231
• Main Authors: Voland, J R, Hawkins, E P, Wells, T R, Saunders, B, Jones, M, Benirschke, K
• Format: Journal Article
• Language: English
• Published: United States 1985
• Subjects: Amniotic Fluid; Consanguinity; Female; Genes, Recessive; Humans; Infant, Newborn; Kidney - abnormalities; Kidney - blood supply; Kidney - pathology; Kidney Tubules - abnormalities; Kidney Tubules - pathology; Male; Microscopy, Electron; Nephrons - abnormalities; Nephrons - pathology; Pedigree; Phenotype; Sex Factors; X Chromosome

This paper describes a distinct, apparently inherited renal disorder we call congenital hypernephronic nephromegaly with tubular dysgenesis. The disorder is characterized by oligohydramnios, the Potter phenotype, and enlarged nonfunctional kidneys. Light microscopy demonstrates increased numbers of...