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
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