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Unilateral multicystic dysplastic kidney

MedGen UID:
292613
Concept ID:
C1567426
Disease or Syndrome
Synonym: Unilateral Multicystic Dysplastic Kidney
SNOMED CT: Unilateral multicystic renal dysplasia (1187460003); Unilateral MCKD (multicystic kidney disease) (1187460003)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Monarch Initiative: MONDO:0019981
Orphanet: ORPHA97363

Definition

A rare congenital anomaly in which one kidney is large, distended by multiple cysts and non-functional. Unilateral multicystic kidney disease is typically asymptomatic if the other kidney is fully functional but may occasionally present with abdominal obstructive signs when the cysts become too large. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUnilateral multicystic dysplastic kidney

Professional guidelines

PubMed

Harmer MJ, Stewart DJ, Prasad P, Veligratli F, Pickles C, Kim JS, Raja M
Clin Pediatr (Phila) 2024 May;63(4):482-487. Epub 2023 Jun 6 doi: 10.1177/00099228231177808. PMID: 37278332
Gungor Ugurlucan F, Dural O, Yasa C, Kirpinar G, Akhan SE
Clin Imaging 2020 Feb;59(2):172-178. Epub 2019 Nov 30 doi: 10.1016/j.clinimag.2019.11.013. PMID: 31821975
Hains DS, Bates CM, Ingraham S, Schwaderer AL
Pediatr Nephrol 2009 Feb;24(2):233-41. Epub 2008 May 15 doi: 10.1007/s00467-008-0828-8. PMID: 18481111

Recent clinical studies

Etiology

Harmer MJ, Stewart DJ, Prasad P, Veligratli F, Pickles C, Kim JS, Raja M
Clin Pediatr (Phila) 2024 May;63(4):482-487. Epub 2023 Jun 6 doi: 10.1177/00099228231177808. PMID: 37278332
Faruque A, Narayanan S, Marley I, Lai C, Khosa J, Barker A, Samnakay N
J Pediatr Surg 2020 Nov;55(11):2497-2503. Epub 2020 Jan 10 doi: 10.1016/j.jpedsurg.2019.12.008. PMID: 31987520
Cleper R
Pediatr Endocrinol Rev 2012 Nov;10(1):152-63. PMID: 23469392
Aytaç B, Sehıtoğlu I, Vuruskan H
Turk Patoloji Derg 2011;27(3):210-4. doi: 10.5146/tjpath.2011.01077. PMID: 21935870
Hains DS, Bates CM, Ingraham S, Schwaderer AL
Pediatr Nephrol 2009 Feb;24(2):233-41. Epub 2008 May 15 doi: 10.1007/s00467-008-0828-8. PMID: 18481111

Diagnosis

Harmer MJ, Stewart DJ, Prasad P, Veligratli F, Pickles C, Kim JS, Raja M
Clin Pediatr (Phila) 2024 May;63(4):482-487. Epub 2023 Jun 6 doi: 10.1177/00099228231177808. PMID: 37278332
Blachman-Braun R, Camp MM, Becerra MF, Guevara CG, Velasquez MC, Moscardi PRM, Ballesteros N, Gosalbez R, Labbie AS, Castellan MA, Restrepo R, Alam A
Urology 2020 May;139:156-160. Epub 2020 Feb 18 doi: 10.1016/j.urology.2020.02.005. PMID: 32081673
Kumar B, Upadhyaya VD, Gupta MK, Bharti LK, Rao RN, Kumar S
Eur J Pediatr Surg 2017 Dec;27(6):533-537. Epub 2017 Mar 27 doi: 10.1055/s-0037-1599837. PMID: 28346956
Aytaç B, Sehıtoğlu I, Vuruskan H
Turk Patoloji Derg 2011;27(3):210-4. doi: 10.5146/tjpath.2011.01077. PMID: 21935870
Hains DS, Bates CM, Ingraham S, Schwaderer AL
Pediatr Nephrol 2009 Feb;24(2):233-41. Epub 2008 May 15 doi: 10.1007/s00467-008-0828-8. PMID: 18481111

Therapy

Dracopoulos C, Gembicki M, Scharf JL, Welp A, Berg N, Weichert J
Fetal Pediatr Pathol 2022 Oct;41(5):871-880. Epub 2021 Oct 23 doi: 10.1080/15513815.2021.1994068. PMID: 34689682
Ozkan H, Cetinkaya M, Köksal N, Yapici S
J Matern Fetal Neonatal Med 2011 Mar;24(3):521-4. doi: 10.3109/14767058.2010.501120. PMID: 21291342
Carta M, Cimador M, Giuffrè M, Sergio M, Di Pace MR, De Grazia E, Corsello G
Pediatr Nephrol 2007 Jul;22(7):1054-7. Epub 2007 Feb 20 doi: 10.1007/s00467-007-0446-x. PMID: 17310358
Sukthankar S, Watson AR
Acta Paediatr 2000 Jul;89(7):811-3. doi: 10.1080/080352500750043701. PMID: 10943963
Rosenstein BJ, Wheeler JS, Heid PL
J Urol 1990 Jul;144(1):110-2. doi: 10.1016/s0022-5347(17)39384-9. PMID: 2193169

Prognosis

Blachman-Braun R, Camp MM, Becerra MF, Guevara CG, Velasquez MC, Moscardi PRM, Ballesteros N, Gosalbez R, Labbie AS, Castellan MA, Restrepo R, Alam A
Urology 2020 May;139:156-160. Epub 2020 Feb 18 doi: 10.1016/j.urology.2020.02.005. PMID: 32081673
Faruque A, Narayanan S, Marley I, Lai C, Khosa J, Barker A, Samnakay N
J Pediatr Surg 2020 Nov;55(11):2497-2503. Epub 2020 Jan 10 doi: 10.1016/j.jpedsurg.2019.12.008. PMID: 31987520
Kumar B, Upadhyaya VD, Gupta MK, Bharti LK, Rao RN, Kumar S
Eur J Pediatr Surg 2017 Dec;27(6):533-537. Epub 2017 Mar 27 doi: 10.1055/s-0037-1599837. PMID: 28346956
Cleper R
Pediatr Endocrinol Rev 2012 Nov;10(1):152-63. PMID: 23469392
Aytaç B, Sehıtoğlu I, Vuruskan H
Turk Patoloji Derg 2011;27(3):210-4. doi: 10.5146/tjpath.2011.01077. PMID: 21935870

Clinical prediction guides

Faruque A, Narayanan S, Marley I, Lai C, Khosa J, Barker A, Samnakay N
J Pediatr Surg 2020 Nov;55(11):2497-2503. Epub 2020 Jan 10 doi: 10.1016/j.jpedsurg.2019.12.008. PMID: 31987520
Yamamoto K, Kamei K, Sato M, Ogura M, Suzuki M, Hasegawa Y, Ueoka K, Ito S, Ishikura K
Pediatr Nephrol 2019 Feb;34(2):295-299. Epub 2018 Sep 25 doi: 10.1007/s00467-018-4079-z. PMID: 30255447
Kumar B, Upadhyaya VD, Gupta MK, Bharti LK, Rao RN, Kumar S
Eur J Pediatr Surg 2017 Dec;27(6):533-537. Epub 2017 Mar 27 doi: 10.1055/s-0037-1599837. PMID: 28346956
Aytaç B, Sehıtoğlu I, Vuruskan H
Turk Patoloji Derg 2011;27(3):210-4. doi: 10.5146/tjpath.2011.01077. PMID: 21935870
John U, Rudnik-Schöneborn S, Zerres K, Misselwitz J
Pediatr Nephrol 1998 Sep;12(7):567-71. doi: 10.1007/s004670050506. PMID: 9761356

Recent systematic reviews

Schreuder MF, Westland R, van Wijk JA
Nephrol Dial Transplant 2009 Jun;24(6):1810-8. Epub 2009 Jan 26 doi: 10.1093/ndt/gfn777. PMID: 19171687

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