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bilateral non-obstructive multicystic renal dysplasia

Wednesday 24 November 2004

Synopsis

- immature conjonctive tissue

Types

- non-syndromic bilateral cystic renal dysplasia (non-syndromic BNORD)

  • sporadic non-syndromic cystic renal dysplasia
  • familial non-syndromic cystic renal dysplasia
  • isolated renal Meckel syndrome
  • partial trisomy resulting from parental unbalanced translocation

- syndromal bilateral cystic renal dysplasia (malformative syndromes - multisystem maldevelopment)

  • osteochondrodysplasias
    • short-rib polydactyly syndromes (SRPSs)
      • short-rib polydactyly syndrome type 1 (Saldino-Noonan syndrome) (SRPS1) (MIM.263530)
      • short-rib polydactyly syndrome type 2 (Majewski syndrome) (SRPS2) (MIM.263520)
      • short-rib polydactyly syndrome type 4 (Beemer-Langer syndrome) (SRPS4) (9610623)
  • chromosomal anomalies
    • trisomy 9
    • trisomy 13
    • trisomy 18
    • trisomy 21
    • t(1;2)(q32;p25) (1q32 and 2p25) (12694239)
    • 17q12 deletion including TCF2 (17924346)
  • embryopathies/fetopathies
    • fetal alcohol syndrome
    • diabetic embryopathy
  • miscellaneous
    • TCF2-associated renal dysplasia
    • hypoparathyroidism, deafness, and renal dysplasia syndrome (GATA3-associated renal dysplasia) (15705923)
    • tuberous sclerosis complex
    • Beckwith-Wiedemann syndrome (BWS)

Associations

- other renal anomalies

- BNORD and polydactyly

  • Meckel syndrome (MKS)
  • Bardet-Bield syndrome (BBS)
  • short rib-polydactyly syndromes

- BNORD and heterotaxia (12605452)

  • situs inversus totalis (10746410)
  • renal dysplasia, situs inversus totalis, and multisystem fibrosis (1570238)
  • renal dysplasia, situs inversus totalis, pancreatic dysplasia (9934983)
  • bilateral renal dysplasia with pancreatic fibrosis, meconium ileus and situs inversus totalis (7304173)
  • bilateral renal dysplasia, pancreatic fibrosis, intrahepatic biliary dysgenesis, and situs inversus totalis (3402978)
  • BNORD and asplenia or hyposplenia

- associated malformations

Differential diagnosis

- oligocystic kidneys
- polycystic kidney diseases (PKDs)

  • ADPKD (autosomal dominant polycystic kidney disease)
  • ARPKD (autosomic recessive polycystic kidney disease or hepato-pancreato-renal dysplasia)

Case records

- Case 12763: Non-syndromic non-obstructive multicystic renal dysplasia

See also

- reno-hepato-pancreatic dysplastic syndromes
- bilateral obstructive renal dysplasia (BORD)

References

- Dursun A, Ermis B, Numanoglu V, Bahadir B, Seckiner I. Bilateral multicystic renal dysplasia with potter sequence. A case with penile agenesis. Saudi Med J. 2006 Nov;27(11):1745-7. PMID: 17106555

Reviews

- Bisceglia M, Galliani CA, Senger C, Stallone C, Sessa A. Renal cystic diseases: a review. Adv Anat Pathol. 2006 Jan;13(1):26-56. PMID: 16462154

- Woolf AS, Price KL, Scambler PJ, Winyard PJ. Evolving concepts in human renal dysplasia. J Am Soc Nephrol. 2004 Apr;15(4):998-1007. PMID: 15034102

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