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Distal 22q11.2 microduplication syndrome

MedGen UID:
1637660
Concept ID:
C4706942
Disease or Syndrome
Synonyms: distal 22q11.2 microduplication syndrome; Distal dup(22)(q11.2); distal dup(22)(q11.2); Distal trisomy 22q11.2; distal trisomy 22q11.2
SNOMED CT: Distal trisomy 22q11.2 (764524005); Distal 22q11.2 microduplication syndrome (764524005)
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:0016846
Orphanet: ORPHA261337

Definition

A rare chromosomal anomaly syndrome resulting from the partial duplication of the long arm of chromosome 22 with a highly variable phenotype. Principle characteristics are developmental delay, intellectual disability, hypotonia, growth retardation, velopharyngeal insufficiency, mild craniofacial dysmorphism (microcephaly, tall/broad forehead, small downslanting palpebral fissures, hooded eyelids, flat nasal bridge, low posterior hairline) and digital anomalies. Congenital heart malformations, visual and hearing impairment, urogenital abnormalities and seizures have also been reported. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDistal 22q11.2 microduplication syndrome

Professional guidelines

PubMed

Jiang X, Liang B, He S, Wu X, Zhao W, Xue H, Wang Y, Lin N, Huang H, Xu L
Mol Genet Genomic Med 2024 Jul;12(7):e2498. doi: 10.1002/mgg3.2498. PMID: 39031005Free PMC Article

Recent clinical studies

Etiology

Jiang X, Liang B, He S, Wu X, Zhao W, Xue H, Wang Y, Lin N, Huang H, Xu L
Mol Genet Genomic Med 2024 Jul;12(7):e2498. doi: 10.1002/mgg3.2498. PMID: 39031005Free PMC Article

Diagnosis

Jiang X, Liang B, He S, Wu X, Zhao W, Xue H, Wang Y, Lin N, Huang H, Xu L
Mol Genet Genomic Med 2024 Jul;12(7):e2498. doi: 10.1002/mgg3.2498. PMID: 39031005Free PMC Article
Lafay-Cousin L, Payne E, Strother D, Chernos J, Chan M, Bernier FP
Am J Med Genet A 2009 Dec;149A(12):2855-9. doi: 10.1002/ajmg.a.33119. PMID: 19938088
Descartes M, Franklin J, Diaz de Ståhl T, Piotrowski A, Bruder CE, Dumanski JP, Carroll AJ, Mikhail FM
Am J Med Genet A 2008 Dec 1;146A(23):3075-81. doi: 10.1002/ajmg.a.32572. PMID: 19006218
Stachon AC, Baskin B, Smith AC, Shugar A, Cytrynbaum C, Fishman L, Mendoza-Londono R, Klatt R, Teebi A, Ray PN, Weksberg R
Am J Med Genet A 2007 Dec 15;143A(24):2924-30. doi: 10.1002/ajmg.a.32101. PMID: 18000985

Prognosis

Molck MC, Vieira TP, Simioni M, Sgardioli IC, dos Santos AP, Xavier AC, Gil-da-Silva-Lopes VL
Am J Med Genet A 2015 Jan;167A(1):215-20. Epub 2014 Oct 30 doi: 10.1002/ajmg.a.36809. PMID: 25358462
Coppinger J, McDonald-McGinn D, Zackai E, Shane K, Atkin JF, Asamoah A, Leland R, Weaver DD, Lansky-Shafer S, Schmidt K, Feldman H, Cohen W, Phalin J, Powell B, Ballif BC, Theisen A, Geiger E, Haldeman-Englert C, Shaikh TH, Saitta S, Bejjani BA, Shaffer LG
Hum Mol Genet 2009 Apr 15;18(8):1377-83. Epub 2009 Feb 3 doi: 10.1093/hmg/ddp042. PMID: 19193630Free PMC Article
Ou Z, Berg JS, Yonath H, Enciso VB, Miller DT, Picker J, Lenzi T, Keegan CE, Sutton VR, Belmont J, Chinault AC, Lupski JR, Cheung SW, Roeder E, Patel A
Genet Med 2008 Apr;10(4):267-77. doi: 10.1097/GIM.0b013e31816b64c2. PMID: 18414210

Clinical prediction guides

Molck MC, Vieira TP, Simioni M, Sgardioli IC, dos Santos AP, Xavier AC, Gil-da-Silva-Lopes VL
Am J Med Genet A 2015 Jan;167A(1):215-20. Epub 2014 Oct 30 doi: 10.1002/ajmg.a.36809. PMID: 25358462
Coppinger J, McDonald-McGinn D, Zackai E, Shane K, Atkin JF, Asamoah A, Leland R, Weaver DD, Lansky-Shafer S, Schmidt K, Feldman H, Cohen W, Phalin J, Powell B, Ballif BC, Theisen A, Geiger E, Haldeman-Englert C, Shaikh TH, Saitta S, Bejjani BA, Shaffer LG
Hum Mol Genet 2009 Apr 15;18(8):1377-83. Epub 2009 Feb 3 doi: 10.1093/hmg/ddp042. PMID: 19193630Free PMC Article
Ou Z, Berg JS, Yonath H, Enciso VB, Miller DT, Picker J, Lenzi T, Keegan CE, Sutton VR, Belmont J, Chinault AC, Lupski JR, Cheung SW, Roeder E, Patel A
Genet Med 2008 Apr;10(4):267-77. doi: 10.1097/GIM.0b013e31816b64c2. PMID: 18414210

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