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22q partial monosomy(VCFS)

MedGen UID:
539297
Concept ID:
C0265489
Disease or Syndrome
Synonyms: Chromosome 22q deletion; Partial deletion of the long arm of chromosome 22; VCFS
SNOMED CT: 22q partial monosomy (19550003)
 
Monarch Initiative: MONDO:0022760
Orphanet: ORPHA262182

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGV22q partial monosomy

Professional guidelines

PubMed

Blagowidow N, Nowakowska B, Schindewolf E, Grati FR, Putotto C, Breckpot J, Swillen A, Crowley TB, Loo JCY, Lairson LA, Óskarsdóttir S, Boot E, Garcia-Minaur S, Cristina Digilio M, Marino B, Coleman B, Moldenhauer JS, Bassett AS, McDonald-McGinn DM
Genes (Basel) 2023 Jan 6;14(1) doi: 10.3390/genes14010160. PMID: 36672900Free PMC Article
Barry JC, Crowley TB, Jyonouchi S, Heimall J, Zackai EH, Sullivan KE, McDonald-McGinn DM
J Clin Immunol 2017 Jul;37(5):476-485. Epub 2017 May 24 doi: 10.1007/s10875-017-0403-9. PMID: 28540525
Yatsenko SA, Peters DG, Saller DN, Chu T, Clemens M, Rajkovic A
Genet Med 2015 Oct;17(10):836-8. Epub 2015 Jan 8 doi: 10.1038/gim.2014.197. PMID: 25569438Free PMC Article

Recent clinical studies

Etiology

Vong KI, Lee S, Au KS, Crowley TB, Capra V, Martino J, Haller M, Araújo C, Machado HR, George R, Gerding B, James KN, Stanley V, Jiang N, Alu K, Meave N, Nidhiry AS, Jiwani F, Tang I, Nisal A, Jhamb I, Patel A, Patel A, McEvoy-Venneri J, Barrows C, Shen C, Ha YJ, Howarth R, Strain M, Ashley-Koch AE, Azam M, Mumtaz S, Bot GM, Finnell RH, Kibar Z, Marwan AI, Melikishvili G, Meltzer HS, Mutchinick OM, Stevenson DA, Mroczkowski HJ, Ostrander B, Schindewolf E, Moldenhauer J, Zackai EH, Emanuel BS, Garcia-Minaur S, Nowakowska BA, Stevenson RE, Zaki MS, Northrup H, McNamara HK, Aldinger KA, Phelps IG, Deng M, Glass IA; Spina Bifida Sequencing Consortium‡, Morrow B, McDonald-McGinn DM, Sanna-Cherchi S, Lamb DJ, Gleeson JG
Science 2024 May 3;384(6695):584-590. Epub 2024 May 2 doi: 10.1126/science.adl1624. PMID: 38696583
Linders CC, van Eeghen AM, Zinkstok JR, van den Boogaard MJ, Boot E
Genes (Basel) 2023 Jul 25;14(8) doi: 10.3390/genes14081514. PMID: 37628566Free PMC Article
Blagowidow N, Nowakowska B, Schindewolf E, Grati FR, Putotto C, Breckpot J, Swillen A, Crowley TB, Loo JCY, Lairson LA, Óskarsdóttir S, Boot E, Garcia-Minaur S, Cristina Digilio M, Marino B, Coleman B, Moldenhauer JS, Bassett AS, McDonald-McGinn DM
Genes (Basel) 2023 Jan 6;14(1) doi: 10.3390/genes14010160. PMID: 36672900Free PMC Article
Ng HK, Li KK, Chung NY, Chan JY, Poon MF, Wong QH, Kwan JS, Poon WS, Chen H, Chan DT, Shi ZF, Mao Y
Brain Pathol 2023 May;33(3):e13120. Epub 2022 Sep 27 doi: 10.1111/bpa.13120. PMID: 36167400Free PMC Article
Namavar Y, Duineveld DJ, Both GIA, Fiksinski AM, Vorstman JAS, Verhoeven-Duif NM, Zinkstok JR
Am J Med Genet B Neuropsychiatr Genet 2021 Jul;186(5):289-317. Epub 2021 Jul 24 doi: 10.1002/ajmg.b.32869. PMID: 34302426

Diagnosis

Blagowidow N, Nowakowska B, Schindewolf E, Grati FR, Putotto C, Breckpot J, Swillen A, Crowley TB, Loo JCY, Lairson LA, Óskarsdóttir S, Boot E, Garcia-Minaur S, Cristina Digilio M, Marino B, Coleman B, Moldenhauer JS, Bassett AS, McDonald-McGinn DM
Genes (Basel) 2023 Jan 6;14(1) doi: 10.3390/genes14010160. PMID: 36672900Free PMC Article
Jesus-Ribeiro J, Ribeiro IP, Pires LM, Paiva P, Simões S, Pereira C, Robalo C, Pereira R, Sales F, Rebelo O, Santana I, Freire A, Barbosa Melo J
Cytogenet Genome Res 2022;162(1-2):28-33. Epub 2022 Apr 27 doi: 10.1159/000524130. PMID: 35477180
Shih AR, Chebib I, Deshpande V, Dickson BC, Iafrate AJ, Nielsen GP
Genes Chromosomes Cancer 2019 Nov;58(11):804-808. Epub 2019 Jun 13 doi: 10.1002/gcc.22782. PMID: 31135077
Fung WL, Butcher NJ, Costain G, Andrade DM, Boot E, Chow EW, Chung B, Cytrynbaum C, Faghfoury H, Fishman L, García-Miñaúr S, George S, Lang AE, Repetto G, Shugar A, Silversides C, Swillen A, van Amelsvoort T, McDonald-McGinn DM, Bassett AS
Genet Med 2015 Aug;17(8):599-609. Epub 2015 Jan 8 doi: 10.1038/gim.2014.175. PMID: 25569435Free PMC Article
Emanuel BS
Dev Disabil Res Rev 2008;14(1):11-8. doi: 10.1002/ddrr.3. PMID: 18636632Free PMC Article

Therapy

Vong KI, Lee S, Au KS, Crowley TB, Capra V, Martino J, Haller M, Araújo C, Machado HR, George R, Gerding B, James KN, Stanley V, Jiang N, Alu K, Meave N, Nidhiry AS, Jiwani F, Tang I, Nisal A, Jhamb I, Patel A, Patel A, McEvoy-Venneri J, Barrows C, Shen C, Ha YJ, Howarth R, Strain M, Ashley-Koch AE, Azam M, Mumtaz S, Bot GM, Finnell RH, Kibar Z, Marwan AI, Melikishvili G, Meltzer HS, Mutchinick OM, Stevenson DA, Mroczkowski HJ, Ostrander B, Schindewolf E, Moldenhauer J, Zackai EH, Emanuel BS, Garcia-Minaur S, Nowakowska BA, Stevenson RE, Zaki MS, Northrup H, McNamara HK, Aldinger KA, Phelps IG, Deng M, Glass IA; Spina Bifida Sequencing Consortium‡, Morrow B, McDonald-McGinn DM, Sanna-Cherchi S, Lamb DJ, Gleeson JG
Science 2024 May 3;384(6695):584-590. Epub 2024 May 2 doi: 10.1126/science.adl1624. PMID: 38696583
Bender BU, Gutsche M, Gläsker S, Müller B, Kirste G, Eng C, Neumann HP
J Clin Endocrinol Metab 2000 Dec;85(12):4568-74. doi: 10.1210/jcem.85.12.7015. PMID: 11134110
Murthy SS, Testa JR
J Cell Physiol 1999 Aug;180(2):150-7. doi: 10.1002/(SICI)1097-4652(199908)180:2<150::AID-JCP2>3.0.CO;2-H. PMID: 10395284
Parry DM, MacCollin MM, Kaiser-Kupfer MI, Pulaski K, Nicholson HS, Bolesta M, Eldridge R, Gusella JF
Am J Hum Genet 1996 Sep;59(3):529-39. PMID: 8751853Free PMC Article
Khosla S, Patel VM, Hay ID, Schaid DJ, Grant CS, van Heerden JA, Thibodeau SN
J Clin Invest 1991 May;87(5):1691-9. doi: 10.1172/JCI115186. PMID: 2022740Free PMC Article

Prognosis

Ng HK, Li KK, Chung NY, Chan JY, Poon MF, Wong QH, Kwan JS, Poon WS, Chen H, Chan DT, Shi ZF, Mao Y
Brain Pathol 2023 May;33(3):e13120. Epub 2022 Sep 27 doi: 10.1111/bpa.13120. PMID: 36167400Free PMC Article
Janovitz T, Williamson DFK, Wong KS, Dong F, Barletta JA
Histopathology 2021 Oct;79(4):491-498. Epub 2021 Jun 15 doi: 10.1111/his.14374. PMID: 33783022
Shih AR, Chebib I, Deshpande V, Dickson BC, Iafrate AJ, Nielsen GP
Genes Chromosomes Cancer 2019 Nov;58(11):804-808. Epub 2019 Jun 13 doi: 10.1002/gcc.22782. PMID: 31135077
Lin A, Ching CRK, Vajdi A, Sun D, Jonas RK, Jalbrzikowski M, Kushan-Wells L, Pacheco Hansen L, Krikorian E, Gutman B, Dokoru D, Helleman G, Thompson PM, Bearden CE
J Neurosci 2017 Jun 28;37(26):6183-6199. Epub 2017 May 23 doi: 10.1523/JNEUROSCI.3759-16.2017. PMID: 28536274Free PMC Article
Tomlinson GE
Front Biosci (Elite Ed) 2012 Jan 1;4(4):1287-92. doi: 10.2741/459. PMID: 22201954

Clinical prediction guides

Linders CC, van Eeghen AM, Zinkstok JR, van den Boogaard MJ, Boot E
Genes (Basel) 2023 Jul 25;14(8) doi: 10.3390/genes14081514. PMID: 37628566Free PMC Article
Singh J, Sharma R, Shukla N, Narwal P, Katiyar A, Mahajan S, Sahu S, Garg A, Sharma MC, Suri A, Sarkar C, Suri V
J Neurooncol 2023 Jan;161(2):339-356. Epub 2022 Dec 24 doi: 10.1007/s11060-022-04220-3. PMID: 36564673
Namavar Y, Duineveld DJ, Both GIA, Fiksinski AM, Vorstman JAS, Verhoeven-Duif NM, Zinkstok JR
Am J Med Genet B Neuropsychiatr Genet 2021 Jul;186(5):289-317. Epub 2021 Jul 24 doi: 10.1002/ajmg.b.32869. PMID: 34302426
Och W, Szmuda T, Kulbacki K, Witek K, Sikorska B, Zakrzewska M, Springer J, Reszeć J, Parda A, Liberski PP
Neurol Neurochir Pol 2016 Nov-Dec;50(6):395-402. Epub 2016 Jul 26 doi: 10.1016/j.pjnns.2016.07.001. PMID: 27480481
Tomlinson GE
Front Biosci (Elite Ed) 2012 Jan 1;4(4):1287-92. doi: 10.2741/459. PMID: 22201954

Recent systematic reviews

Namavar Y, Duineveld DJ, Both GIA, Fiksinski AM, Vorstman JAS, Verhoeven-Duif NM, Zinkstok JR
Am J Med Genet B Neuropsychiatr Genet 2021 Jul;186(5):289-317. Epub 2021 Jul 24 doi: 10.1002/ajmg.b.32869. PMID: 34302426
Glaeser AB, Santos AS, Diniz BL, Deconte D, Rosa RFM, Zen PRG
Am J Med Genet A 2020 Nov;182(11):2624-2631. Epub 2020 Sep 7 doi: 10.1002/ajmg.a.61841. PMID: 32893956
Mitz AR, Philyaw TJ, Boccuto L, Shcheglovitov A, Sarasua SM, Kaufmann WE, Thurm A
Eur J Hum Genet 2018 Mar;26(3):293-302. Epub 2018 Jan 22 doi: 10.1038/s41431-017-0042-x. PMID: 29358616Free PMC Article

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