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Distal trisomy 8q

MedGen UID:
1637956
Concept ID:
C4706365
Disease or Syndrome
Synonyms: Distal duplication 8q; distal duplication 8q; distal trisomy 8q; distal trisomy type 8q; Telomeric duplication 8q; telomeric duplication 8q; Trisomy 8qter; trisomy 8qter
SNOMED CT: Distal trisomy 8q (763277009); Distal duplication 8q (763277009)
 
Monarch Initiative: MONDO:0019882
Orphanet: ORPHA96100

Definition

A rare chromosomal anomaly syndrome resulting from the partial duplication of the long arm of chromosome 8. The disorder has a highly variable phenotype with typical characteristics of growth and developmental delay, intellectual disability, short stature, craniofacial dysmorphism (microcephaly, prominent forehead, hypertelorism, abnormal palpebral fissures, low-set, large ears, anteverted tip of nose, micro/retrognathia), congenital heart defects, skeletal and limb anomalies. Other reported features include ophthalmologic abnormalities (e.g. megalocornea), cryptorchidism, hypertrichosis, and neurologic manifestations (e.g. hypotonia, hearing loss and seizures). [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDistal trisomy 8q

Recent clinical studies

Etiology

Balci S, Aypar E, Beksaç MS, Bartsch O
Genet Couns 2009;20(2):125-32. PMID: 19650409

Diagnosis

Farcas S, Erdelean D, Anne-Elise Szekely F, Navolan D, Andreescu N, Cioca A
Taiwan J Obstet Gynecol 2019 Jan;58(1):36-39. doi: 10.1016/j.tjog.2018.11.005. PMID: 30638476
Balci S, Aypar E, Beksaç MS, Bartsch O
Genet Couns 2009;20(2):125-32. PMID: 19650409

Prognosis

Bruni V, Roppa K, Scionti F, Apa R, Sestito S, Di Martino MT, Pensabene L, Concolino D
Cytogenet Genome Res 2019;158(2):74-82. Epub 2019 May 28 doi: 10.1159/000500619. PMID: 31141803
Farcas S, Erdelean D, Anne-Elise Szekely F, Navolan D, Andreescu N, Cioca A
Taiwan J Obstet Gynecol 2019 Jan;58(1):36-39. doi: 10.1016/j.tjog.2018.11.005. PMID: 30638476

Clinical prediction guides

Farcas S, Erdelean D, Anne-Elise Szekely F, Navolan D, Andreescu N, Cioca A
Taiwan J Obstet Gynecol 2019 Jan;58(1):36-39. doi: 10.1016/j.tjog.2018.11.005. PMID: 30638476
Puvabanditsin S, Garrow E, Rabi FA, Titapiwatanakun R, Kuniyoshi KM
Ann Genet 2004 Oct-Dec;47(4):399-403. doi: 10.1016/j.anngen.2004.07.004. PMID: 15581839
Townes PL, White MR
Am J Dis Child 1978 May;132(5):498-501. doi: 10.1001/archpedi.1978.02120300058012. PMID: 645677

Supplemental Content

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    Genetic Testing Registry

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