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4p16.3 microduplication syndrome

MedGen UID:
1387521
Concept ID:
C4512053
Disease or Syndrome
Synonyms: Distal duplication 4p; distal duplication 4p; Distal trisomy 4p; distal trisomy 4p; Telomeric duplication 4p; telomeric duplication 4p; Trisomy 4pter; trisomy 4pter
SNOMED CT: 4p16.3 microduplication syndrome (726706008); Distal trisomy 4p (726706008)
 
Monarch Initiative: MONDO:0019873
Orphanet: ORPHA96072

Definition

A rare genetic syndrome that results from the partial duplication of the short arm of chromosome 4. It has a highly variable phenotype, principally with characteristics of psychomotor and language delay, seizures and dysmorphic features such as high forehead with frontal bossing, hypertelorism, prominent glabella, long narrow palpebral fissures, low set ears and short neck. Eye abnormalities (glaucoma, irregular iris pigmentation, hyperopia) have also been reported. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • 4p16.3 microduplication syndrome

Professional guidelines

PubMed

Li C, Zhang J, Li J, Qiao G, Zhan Y, Xu Y, Yang H
Mol Diagn Ther 2021 May;25(3):339-349. Epub 2021 Apr 7 doi: 10.1007/s40291-021-00522-w. PMID: 33826125

Recent clinical studies

Diagnosis

Bi W, Cheung SW, Breman AM, Bacino CA
Am J Med Genet A 2016 Oct;170(10):2540-50. Epub 2016 Jun 10 doi: 10.1002/ajmg.a.37796. PMID: 27287194

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