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renal hypoplasias

Definition: Congenitally small kidney (below two standard deviations below the expected mean) that lacks evidence of parenchymal maldifferentiation (renal dysplasia) or of acquired disease sufficient to explain the reduced size.

Types

- segmental renal hypoplasia (SRH)
- unilateral renal hypoplasia (URH)
- bilateral renal hypoplasia (BRH)

  • bilateral dysplastic renal hypoplasia (bilateral renal hypodysplasia)
  • bilateral non-dysplastic renal hypoplasia
    • bilateral normonephronic renal hypoplasia (bilateral simple renal hypoplasia)
    • bilateral oligonephronic renal hypoplasia
      • oligomeganephronic renal hypoplasia (OMNRH)

Etiological types

- obstructive renal hypoplasia (ORH)
- non-obstructive renal hypoplasia (NORH)

  • isolated renal hypoplasia (non-syndromal renal hypoplasia) (IRH)
    • oligomeganephronic renal hypoplasia (OMNRH)
    • normonephronic renal hypoplasia (NNRH)

Association types

- isolated renal hypoplasia (IRH)

  • nondysplastic renal hypoplasia (NDRH) (#8597836#)

- associated renal hypoplasia (ARH)

  • malformative syndromes (syndromal renal hypoplasia) (SRH)
  • associated renal developmental anomalies
    • renal dysplasia (renal hypodysplasia)
  • cardiac malformations
    • left ventricle hypoplasia
    • bicuspid aortic valve and aortic stenosis (#15731545#)

Etiology

- chromosomal anomalies

  • trisomy 21 (15%)

- RET germline mutations

See also

- renal malformations
- streak kidney