renal hypoplasias
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hypoplastic kidneys, renal hypoplasia
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