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20p12.3 microdeletion syndrome

MedGen UID:
930208
Concept ID:
C4304539
Disease or Syndrome
Synonyms: Del(20)(p12.3); Monosomy 20p12.3; monosomy 20p12.3
SNOMED CT: 20p12.3 microdeletion syndrome (719650004); Monosomy 20p12.3 (719650004)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
Concept ID:
CN307042
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0016841
Orphanet: ORPHA261295

Definition

A recently described syndrome with characteristics of Wolff-Parkinson-White syndrome, variable developmental delay and facial dysmorphism. Dysmorphic features include macrocephaly, hypertelorism, down-slanting palpebral fissures and microstomia. This syndrome is caused by an interstitial deletion encompassing 20p12.3. All these deletions except one occurred de novo and have a variable size with the smallest region of overlap including only one gene, BMP2, which is a good candidate gene for explaining the phenotype of Wolff-Parkinson-White syndrome. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • 20p12.3 microdeletion syndrome

Recent clinical studies

Prognosis

Yogi A, Iemura R, Nakatani H, Takasawa K, Gau M, Yamauchi T, Yoshida M, Moriyama K, Ishii T, Hosokawa S, Yamada M, Suzuki H, Kosaki K, Kashimada K, Morio T
Eur J Med Genet 2023 Sep;66(9):104820. Epub 2023 Aug 11 doi: 10.1016/j.ejmg.2023.104820. PMID: 37572998

Clinical prediction guides

Yogi A, Iemura R, Nakatani H, Takasawa K, Gau M, Yamauchi T, Yoshida M, Moriyama K, Ishii T, Hosokawa S, Yamada M, Suzuki H, Kosaki K, Kashimada K, Morio T
Eur J Med Genet 2023 Sep;66(9):104820. Epub 2023 Aug 11 doi: 10.1016/j.ejmg.2023.104820. PMID: 37572998

Supplemental Content

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