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Distal trisomy 6p

MedGen UID:
928220
Concept ID:
C4302551
Disease or Syndrome
Synonyms: Distal duplication 6p; distal duplication 6p; distal trisomy 6p; Distal trisomy 6p syndrome; distal trisomy type 6p; Telomeric duplication 6p; telomeric duplication 6p; Trisomy 6pter; trisomy 6pter
SNOMED CT: Distal duplication 6p (722430008); Telomeric duplication 6p (722430008); Distal trisomy 6p syndrome (722430008)
 
Monarch Initiative: MONDO:0015769
Orphanet: ORPHA1745

Definition

Distal trisomy of the short arm of chromosome 6 has characteristics of pre and postnatal growth retardation, a pattern of specific facial features (mostly of the eyes), microcephaly, and developmental delay. The duplicated region almost always includes 6pter, with proximal breakpoints ranging from 6p21 to 6p25. Interstitial duplications of 6p have also been reported with different phenotypes depending on their size and location. Most cases of distal trisomy 6p result from missegregation of a familial balanced translocation, or pericentric inversion, and are accompanied by another chromosomal imbalance. Intrachromosomal duplications or de novo translocations are also observed. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDistal trisomy 6p

Recent clinical studies

Diagnosis

Jankauskienė A, Koczkowska M, Bjerre A, Bernaciak J, Schaefer F, Lipska-Ziętkiewicz BS
BMC Nephrol 2016 Mar 21;17:32. doi: 10.1186/s12882-016-0246-2. PMID: 27000031Free PMC Article
Bonaglia MC, Giorda R, Beri S, Peters GB, Kirk EP, Hung D, Ciccone R, Gottardi G, Zuffardi O
Eur J Med Genet 2008 Mar-Apr;51(2):148-55. Epub 2007 Dec 8 doi: 10.1016/j.ejmg.2007.11.005. PMID: 18226592
Bonaglia MC, Giorda R, Cavallini A, Pramparo T, Rocchi M, Borgatti R, Zuffardi O
J Med Genet 2003 Aug;40(8):e94. doi: 10.1136/jmg.40.8.e94. PMID: 12920086Free PMC Article
Pierpont ME, Hentges AS, Gears LJ, Hirsch B, Sinaiko A
Am J Med Genet 2000 Nov 27;95(3):275-80. PMID: 11102935

Clinical prediction guides

Bonaglia MC, Giorda R, Beri S, Peters GB, Kirk EP, Hung D, Ciccone R, Gottardi G, Zuffardi O
Eur J Med Genet 2008 Mar-Apr;51(2):148-55. Epub 2007 Dec 8 doi: 10.1016/j.ejmg.2007.11.005. PMID: 18226592

Supplemental Content

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    Genetic Testing Registry

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