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non-syndromic BNOMRD

Wednesday 21 March 2007

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#

Portfolio

  • non-syndromal bilateral non-obstructive renal dysplasia
  • non-syndromal bilateral non-obstructive renal dysplasia
  • non-syndromal bilateral non-obstructive renal dysplasia
  • non-syndromal bilateral non-obstructive renal dysplasia
  • Non-syndromic non-obstructive multicystic renal dysplasia (BNOMRD)
  • Non-syndromic non-obstructive multicystic renal dysplasia (BNOMRD)
  • Non-syndromic non-obstructive multicystic renal dysplasia (BNOMRD)
  • Non-syndromic non-obstructive multicystic renal dysplasia (BNOMRD)
  • Non-syndromic non-obstructive multicystic renal dysplasia (BNOMRD)