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9p partial trisomy syndrome

MedGen UID:
120539
Concept ID:
C0265428
Disease or Syndrome
Synonyms: Chromosome 9, partial trisomy 9p; Duplication 9p partial; Partial trisomy of the short arm of chromosome 9; Trisomy 9p; Trisomy 9p partial
SNOMED CT: 9p partial trisomy syndrome (77527000); Réthoré syndrome (77527000); Trisomy 9p syndrome (77527000); Rethore syndrome (77527000)
 
Monarch Initiative: MONDO:0016526
Orphanet: ORPHA236

Definition

Trisomy 9p is a rare chromosomal anomaly syndrome, resulting from a partial or complete trisomy of the short arm of chromosome 9, with a wide phenotypic variablility, typically characterized by intellectual disability, craniofacial dysmorphism (e.g. microcephaly, large anterior fontanel, hypertelorism, strabismus, downslanting palpebral fissures, malformed, low-set, protruding ears, bulbous nose, macrostomia, down-turned corners of mouth, micrognathia), digital anomalies (brachydactyly and clinodactyly), and short stature. Less frequently patients present with cardiopathy and renal, skeletal, and central nervous system malformations. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGV9p partial trisomy syndrome
  • 9p partial trisomy syndrome
    • 9p partial trisomy syndrome
      • 9p partial trisomy syndrome
        • 9p partial trisomy syndrome
          • 9p partial trisomy syndrome
            • 9p partial trisomy syndrome

Professional guidelines

PubMed

Wilsterman EJ Jr, Nellis ME, Panisello J, Al-Subu A, Breuer R, Kimura D, Krawiec C, Mallory PP, Nett S, Owen E, Parsons SJ, Sanders RC Jr, Garcia-Marcinkiewicz A, Napolitano N, Shults J, Nadkarni VM, Nishisaki A; National Emergency Airway Registry for Children (NEAR4KIDS) and Pediatric Acute Lung Injury and Sepsis Investigators (PALISI) Network
Pediatr Crit Care Med 2024 Apr 1;25(4):335-343. Epub 2023 Dec 7 doi: 10.1097/PCC.0000000000003418. PMID: 38059735Free PMC Article
Pan C, Li Z, Cheng G, Luo X, Nie F, Gao J, Yang P
Medicine (Baltimore) 2023 Sep 1;102(35):e34762. doi: 10.1097/MD.0000000000034762. PMID: 37657051Free PMC Article
Sun Y, Zhang N, Tian H, Zhang P, Li Y
J Genet 2021;100 PMID: 34238780

Recent clinical studies

Etiology

Noël L, Guy GP, Jones S, Forenc K, Buck E, Papageorghiou AT, Thilaganathan B
Ultrasound Obstet Gynecol 2021 Oct;58(4):540-545. Epub 2021 Sep 13 doi: 10.1002/uog.23669. PMID: 33998078
Datta AN, Crawford J, Wong PKH
Can J Neurol Sci 2021 Nov;48(6):839-844. Epub 2021 Jan 21 doi: 10.1017/cjn.2021.12. PMID: 33472713
Milligan MCP, Jackson LE, Maurer SH
Am J Hosp Palliat Care 2021 Oct;38(10):1225-1229. Epub 2020 Dec 30 doi: 10.1177/1049909120985210. PMID: 33375814
Chen CP
Taiwan J Obstet Gynecol 2007 Mar;46(1):1-8. doi: 10.1016/S1028-4559(08)60099-6. PMID: 17389182
Littooij AS, Hochstenbach R, Sinke RJ, van Tintelen P, Giltay JC
Am J Med Genet 2002 Apr 22;109(2):125-32. doi: 10.1002/ajmg.10322. PMID: 11977161

Diagnosis

Sun Y, Zhang N, Tian H, Zhang P, Li Y
J Genet 2021;100 PMID: 34238780
Canton AP, Nishi MY, Furuya TK, Roela RA, Jorge AA
Am J Med Genet A 2016 Apr;170A(4):1046-9. Epub 2015 Dec 22 doi: 10.1002/ajmg.a.37521. PMID: 26689153
Recalcati MP, Bellini M, Norsa L, Ballarati L, Caselli R, Russo S, Larizza L, Giardino D
Gene 2012 Jul 1;502(1):40-5. Epub 2012 Apr 17 doi: 10.1016/j.gene.2012.04.030. PMID: 22537675
Chen CP
Taiwan J Obstet Gynecol 2007 Mar;46(1):1-8. doi: 10.1016/S1028-4559(08)60099-6. PMID: 17389182
Wilson WA, Alfi OS, Donnell GN
Ophthalmology 1979 Jun;86(6):1184-90. doi: 10.1016/s0161-6420(79)35427-6. PMID: 160518

Therapy

Datta AN, Crawford J, Wong PKH
Can J Neurol Sci 2021 Nov;48(6):839-844. Epub 2021 Jan 21 doi: 10.1017/cjn.2021.12. PMID: 33472713
Mocquard C, Aillet S, Riffaud L
Neurochirurgie 2019 Nov;65(5):246-251. Epub 2019 Sep 27 doi: 10.1016/j.neuchi.2019.09.014. PMID: 31568780
Canton AP, Nishi MY, Furuya TK, Roela RA, Jorge AA
Am J Med Genet A 2016 Apr;170A(4):1046-9. Epub 2015 Dec 22 doi: 10.1002/ajmg.a.37521. PMID: 26689153
Wilson GN, Raj A, Baker D
Am J Med Genet 1985 Feb;20(2):277-82. doi: 10.1002/ajmg.1320200211. PMID: 3976721
Pfeiffer RA, Santelmann R
Birth Defects Orig Artic Ser 1977;13(1):319-37. PMID: 322750

Prognosis

Milligan MCP, Jackson LE, Maurer SH
Am J Hosp Palliat Care 2021 Oct;38(10):1225-1229. Epub 2020 Dec 30 doi: 10.1177/1049909120985210. PMID: 33375814
Beaulieu-Boire I, Aquino CC, Fasano A, Poon YY, Fallis M, Lang AE, Hodaie M, Kalia SK, Lozano A, Moro E
Brain Stimul 2016 Nov-Dec;9(6):905-910. Epub 2016 Oct 4 doi: 10.1016/j.brs.2016.07.009. PMID: 27743838
Lyons MJ, Fuller JD, Montoya Mdel C, DuPont BR, Holden KR
J Child Neurol 2013 Apr;28(4):524-6. Epub 2012 Jun 29 doi: 10.1177/0883073812446309. PMID: 22752474
Formigari R, Di Donato RM, Gargiulo G, Di Carlo D, Feltri C, Picchio FM, Marino B
Ann Thorac Surg 2004 Aug;78(2):666-72; discussion 672. doi: 10.1016/j.athoracsur.2003.12.021. PMID: 15276542
Littooij AS, Hochstenbach R, Sinke RJ, van Tintelen P, Giltay JC
Am J Med Genet 2002 Apr 22;109(2):125-32. doi: 10.1002/ajmg.10322. PMID: 11977161

Clinical prediction guides

Mocquard C, Aillet S, Riffaud L
Neurochirurgie 2019 Nov;65(5):246-251. Epub 2019 Sep 27 doi: 10.1016/j.neuchi.2019.09.014. PMID: 31568780
Singh AD, Wang MX, Donoso LA, Shields CL, De Potter P, Shields JA
Semin Oncol 1996 Dec;23(6):768-72. PMID: 8970601
Wilson GN, Raj A, Baker D
Am J Med Genet 1985 Feb;20(2):277-82. doi: 10.1002/ajmg.1320200211. PMID: 3976721
Pfeiffer RA, Santelmann R
Birth Defects Orig Artic Ser 1977;13(1):319-37. PMID: 322750
Centerwall WR, Beatty-DeSana JW
Pediatrics 1975 Nov;56(5):748-55. PMID: 1196731

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