oligomeganephronic renal hypolasia

Synopsis

 extremely small kidneys
 oligomeganephronia
 reduced number of lobes (may be only 1 or 2)
 reduced number of layers of glomeruli (premature cessation of glomerulogenesis)
 small number of hypertrophic nephrons
 increased size of glomeruli (volume 12x)
 dilated tubules with hypertrophic epithelium (x17)
 prominent juxtaglomerular apparatuses
 focal interstitial fibrosis

Associations

 autosomal recessive acrorenal syndrome (1344975)
 tapetoretinal degeneration (3931347)
 monosomy 4p (4p- syndrome)

See also

 renal hypoplasia
 renal malformations

References

 Salomon R, Tellier AL, Attie-Bitach T, Amiel J, Vekemans M, Lyonnet S, Dureau P, Niaudet P, Gubler MC, Broyer M. PAX2 mutations in oligomeganephronia. Kidney Int. 2001 Feb;59(2):457-62. PMID: 11168927

 Broyer M, Soto B, Gagnadoux MF, Adi M, Rica C, Gubler MC. Oligomeganephronic renal hypoplasia. Adv Nephrol Necker Hosp. 1997;26:47-63. PMID: 8922124

 Van Acker KJ, Roodhooft AM, Melis K. Monozygotic twins non-concordant for oligomeganephronic renal hypoplasia: artery-vein placental shunting as a possible pathogenetic mechanism.
Clin Nephrol. 1986 Mar;25(3):165-8. PMID: 3955916