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6q terminal deletion syndrome

MedGen UID:
930183
Concept ID:
C4304514
Disease or Syndrome
SNOMED CT: 6q terminal deletion syndrome (719666002)
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:0019164
Orphanet: ORPHA75857

Definition

Syndrome marked by a characteristic facial dysmorphism, short neck and psychomotor retardation, generally associated with a range of non-specific malformations. Isolated terminal 6q deletion syndrome is very rare with less than 20 cases being reported in the literature. The most frequent craniofacial anomalies include microcephaly, broad nose with prominent nasal root and bulbous nasal tip, large ears that may be malformed and low-set and a characteristic downturned mouth. The most commonly described neurological features are psychomotor retardation, hypotonia and seizures. Retinal anomalies are also common. The breakpoints are located between chromosome regions 6q25.3 and 6q26, within the fragile site FRA6E. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • 6q terminal deletion syndrome

Professional guidelines

PubMed

Zou Z, Huang L, Lin S, He Z, Zhu H, Zhang Y, Fang Q, Luo Y
Prenat Diagn 2018 Jan;38(2):91-98. Epub 2018 Jan 10 doi: 10.1002/pd.5190. PMID: 29171036

Recent clinical studies

Etiology

Conti V, Carabalona A, Pallesi-Pocachard E, Parrini E, Leventer RJ, Buhler E, McGillivray G, Michel FJ, Striano P, Mei D, Watrin F, Lise S, Pagnamenta AT, Taylor JC, Kini U, Clayton-Smith J, Novara F, Zuffardi O, Dobyns WB, Scheffer IE, Robertson SP, Berkovic SF, Represa A, Keays DA, Cardoso C, Guerrini R
Brain 2013 Nov;136(Pt 11):3378-94. Epub 2013 Sep 20 doi: 10.1093/brain/awt249. PMID: 24056535

Diagnosis

Iwamoto H, Muroi A, Sekine T, Tsurubuchi T, Ishikawa E, Matsumura A
Pediatr Neurosurg 2019;54(6):419-423. Epub 2019 Oct 9 doi: 10.1159/000503108. PMID: 31597145
Striano P, Malacarne M, Cavani S, Pierluigi M, Rinaldi R, Cavaliere ML, Rinaldi MM, De Bernardo C, Coppola A, Pintaudi M, Gaggero R, Grammatico P, Striano S, Dallapiccola B, Zara F, Faravelli F
Am J Med Genet A 2006 Sep 15;140(18):1944-9. doi: 10.1002/ajmg.a.31435. PMID: 16906558
Elia M, Striano P, Fichera M, Gaggero R, Castiglia L, Galesi O, Malacarne M, Pierluigi M, Amato C, Musumeci SA, Romano C, Majore S, Grammatico P, Zara F, Striano S, Faravelli F
Epilepsia 2006 May;47(5):830-8. doi: 10.1111/j.1528-1167.2006.00522.x. PMID: 16686647
Bertini V, De Vito G, Costa R, Simi P, Valetto A
Am J Med Genet A 2006 Jan 1;140(1):74-81. doi: 10.1002/ajmg.a.31020. PMID: 16329114

Therapy

Su PH, Chen JY, Chen SJ, Yang KC
Pediatr Neonatol 2008 Jun;49(3):88-93. doi: 10.1016/S1875-9572(08)60019-4. PMID: 18947005

Clinical prediction guides

Conti V, Carabalona A, Pallesi-Pocachard E, Parrini E, Leventer RJ, Buhler E, McGillivray G, Michel FJ, Striano P, Mei D, Watrin F, Lise S, Pagnamenta AT, Taylor JC, Kini U, Clayton-Smith J, Novara F, Zuffardi O, Dobyns WB, Scheffer IE, Robertson SP, Berkovic SF, Represa A, Keays DA, Cardoso C, Guerrini R
Brain 2013 Nov;136(Pt 11):3378-94. Epub 2013 Sep 20 doi: 10.1093/brain/awt249. PMID: 24056535
Abu-Amero KK, Hellani A, Salih MA, Al Hussain A, al Obailan M, Zidan G, Alorainy IA, Bosley TM
Ophthalmic Genet 2010 Mar;31(1):1-11. doi: 10.3109/13816810903312535. PMID: 20141352

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