non-syndromic BNOMRD
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Definition: non-syndromic bilateral non-obstructive multicystic renal dysplasia (MRD).
Types
sporadic non-syndromic cystic renal dysplasia
familial non-syndromic cystic renal dysplasia
Synopsis
renal anomalies
- numerous variable size renal cysts
- renal cysts lined by columnar or cuboidal epithelium
- connective tissue without foci of cartilage
- immature conjonctive tissue
- few glomeruli and some nephrogenic tissue scattered among various sized cysts
oligohydramnios sequence (OHAS) (Potter sequence)
Etiology
partial trisomy resulting from parental unbalanced translocation
Associations
situs inversus totalis (#10746410#)
ductal plate malformation (DPM) (congenital hepatic fibrosis) (#11155779#)
See also
renal dysplasias
- bilateral non-obstructive renal dysplasia
- non-syndromic bilateral non-obstructive renal dysplasia
renal developmental anomalies
- renal malformations
References
Sase M, Tsukahara M, Oga A, Kaneko N, Nakata M, Saito T, Kato H. Diffuse cystic renal dysplasia: nonsyndromal familial case. Am J Med Genet. 1996 May 17;63(2):332-4. PMID: #8725780#
Cole BR, Kaufman RL, McAlister WH, Kissane JM. Bilateral renal dysplasia in three siblings: report of a survivor. Clin Nephrol. 1976 Feb;5(2):83-7. PMID: #1253459#