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49,XYYYY syndrome

MedGen UID:
1384259
Concept ID:
C4518342
Disease or Syndrome
SNOMED CT: 49,XYYYY syndrome (734028007)
 
Monarch Initiative: MONDO:0020470
Orphanet: ORPHA99330

Definition

A rare Y chromosome number anomaly with a variable phenotype. The main characteristics of this disorder are moderate to severe intellectual disability, speech delay, hypotonia and mild dysmorphic features, including facial asymmetry, hypertelorism, bilateral low set ''lop'' ears, and micrognathia. Skeletal abnormalities (such as skull deformities, radioulnar synostosis, elbow flexion, clinodactyly, brachydactyly) have also been associated with this condition. Genitalia are normal at birth, although hypogonadism and azoospermia has been reported in adults. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGV49,XYYYY syndrome

Recent clinical studies

Etiology

Demily C, Poisson A, Peyroux E, Gatellier V, Nicolas A, Rigard C, Schluth-Bolard C, Sanlaville D, Rossi M
BMC Med Genet 2017 Jan 31;18(1):9. doi: 10.1186/s12881-017-0371-1. PMID: 28137251Free PMC Article

Clinical prediction guides

Demily C, Poisson A, Peyroux E, Gatellier V, Nicolas A, Rigard C, Schluth-Bolard C, Sanlaville D, Rossi M
BMC Med Genet 2017 Jan 31;18(1):9. doi: 10.1186/s12881-017-0371-1. PMID: 28137251Free PMC Article

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