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22q11 Deletion Syndrome

MedGen UID:
422333
Concept ID:
C2936346
Disease or Syndrome
Synonyms: 22q11 Deletion Syndromes; Deletion Syndrome, 22q11; Deletion Syndromes, 22q11; Syndrome, 22q11 Deletion; Syndromes, 22q11 Deletion

Definition

Condition with a variable constellation of phenotypes due to deletion polymorphisms at chromosome location 22q11. It encompasses several syndromes with overlapping abnormalities including the DIGEORGE SYNDROME, VELOCARDIOFACIAL SYNDROME, and CONOTRUNCAL AMOMALY FACE SYNDROME. In addition, variable developmental problems and schizoid features are also associated with this syndrome. (From BMC Med Genet. 2009 Feb 25;10:16) Not all deletions at 22q11 result in the 22q11deletion syndrome. [from MeSH]

Term Hierarchy

Professional guidelines

PubMed

Biggs SE, Gilchrist B, May KR
Curr Allergy Asthma Rep 2023 Apr;23(4):213-222. Epub 2023 Mar 10 doi: 10.1007/s11882-023-01071-4. PMID: 36897497Free 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
Swillen A, Moss E, Duijff S
Am J Med Genet A 2018 Oct;176(10):2160-2166. Epub 2018 Apr 25 doi: 10.1002/ajmg.a.38709. PMID: 29696780Free PMC Article

Recent clinical studies

Etiology

Duckett KA, Poupore NS, Carroll WW, Pecha PP
Laryngoscope 2024 Jun;134(6):2551-2561. Epub 2023 Dec 5 doi: 10.1002/lary.31181. PMID: 38050953Free PMC Article
Frascarelli M, Padovani G, Buzzanca A, Accinni T, Carlone L, Ghezzi F, Lattanzi GM, Fanella M, Putotto C, Di Bonaventura C, Girardi N, Pasquini M, Biondi M, Di F
Ann Ist Super Sanita 2020 Jan-Mar;56(1):107-113. doi: 10.4415/ANN_20_01_15. PMID: 32242542
Zinkstok JR, Boot E, Bassett AS, Hiroi N, Butcher NJ, Vingerhoets C, Vorstman JAS, van Amelsvoort TAMJ
Lancet Psychiatry 2019 Nov;6(11):951-960. Epub 2019 Aug 5 doi: 10.1016/S2215-0366(19)30076-8. PMID: 31395526Free PMC Article
Trevisan P, Barbosa S, Sperotto G, Costi C, de Omena Filho RL, Silva AP, Varella-Garcia M, Fiegenbaum M, Rosa RF, Zen PR
Am J Med Genet A 2015 Jun;167(6):1406-8. Epub 2015 Apr 21 doi: 10.1002/ajmg.a.36531. PMID: 25899105
Milczuk HA
Curr Opin Otolaryngol Head Neck Surg 2012 Dec;20(6):522-6. doi: 10.1097/MOO.0b013e32835873cc. PMID: 22929115

Diagnosis

Zinkstok JR, Boot E, Bassett AS, Hiroi N, Butcher NJ, Vingerhoets C, Vorstman JAS, van Amelsvoort TAMJ
Lancet Psychiatry 2019 Nov;6(11):951-960. Epub 2019 Aug 5 doi: 10.1016/S2215-0366(19)30076-8. PMID: 31395526Free PMC Article
Solot CB, Sell D, Mayne A, Baylis AL, Persson C, Jackson O, McDonald-McGinn DM
Am J Speech Lang Pathol 2019 Aug 9;28(3):984-999. Epub 2019 Jul 22 doi: 10.1044/2019_AJSLP-16-0147. PMID: 31330115Free PMC Article
Trevisan P, Barbosa S, Sperotto G, Costi C, de Omena Filho RL, Silva AP, Varella-Garcia M, Fiegenbaum M, Rosa RF, Zen PR
Am J Med Genet A 2015 Jun;167(6):1406-8. Epub 2015 Apr 21 doi: 10.1002/ajmg.a.36531. PMID: 25899105
Rosa RFM, Rosa RCM, Krumenauer RCP, Varella-Garcia M, Paskulin GA
Braz J Otorhinolaryngol 2011 Jul-Aug;77(4):540. doi: 10.1590/S1808-86942011000400024. PMID: 21860987Free PMC Article
Baker K, Baldeweg T, Sivagnanasundaram S, Scambler P, Skuse D
Biol Psychiatry 2005 Jul 1;58(1):23-31. doi: 10.1016/j.biopsych.2005.03.020. PMID: 15935994

Therapy

Gaynor JW, Moldenhauer JS, Zullo EE, Burnham NB, Gerdes M, Bernbaum JC, D'Agostino JA, Linn RL, Klepczynski B, Randazzo I, Gionet G, Choi GH, Karaj A, Russell WW, Zackai EH, Johnson MP, Gebb JS, Soni S, DeBari SE, Szwast AL, Ahrens-Nicklas RC, Drivas TG, Jacobwitz M, Licht DJ, Vossough A, Nicolson SC, Spray TL, Rychik J, Putt ME
JAMA Netw Open 2024 May 1;7(5):e2412291. doi: 10.1001/jamanetworkopen.2024.12291. PMID: 38805228Free PMC Article
Dufournet B, Nguyen K, Charles P, Grabli D, Jacquette A, Borg M, Danaila T, Mutez E, Drapier S, Colin O, Eusebio A, Philip N, Azulay JP
Rev Neurol (Paris) 2017 Jun;173(6):406-410. Epub 2017 Apr 29 doi: 10.1016/j.neurol.2017.03.021. PMID: 28461026
Bouajila S, Chalard A, Dauphin C
Cardiol Young 2017 Sep;27(7):1406-1409. Epub 2017 Mar 21 doi: 10.1017/S1047951117000403. PMID: 28322179
Astor MC, Løvås K, Debowska A, Eriksen EF, Evang JA, Fossum C, Fougner KJ, Holte SE, Lima K, Moe RB, Myhre AG, Kemp EH, Nedrebø BG, Svartberg J, Husebye ES
J Clin Endocrinol Metab 2016 Aug;101(8):3045-53. Epub 2016 May 17 doi: 10.1210/jc.2016-1477. PMID: 27186861Free PMC Article
Milczuk HA
Curr Opin Otolaryngol Head Neck Surg 2012 Dec;20(6):522-6. doi: 10.1097/MOO.0b013e32835873cc. PMID: 22929115

Prognosis

Framme JL, Lundqvist C, Lundell AC, van Schouwenburg PA, Lemarquis AL, Thörn K, Lindgren S, Gudmundsdottir J, Lundberg V, Degerman S, Zetterström RH, Borte S, Hammarström L, Telemo E, Hultdin M, van der Burg M, Fasth A, Oskarsdóttir S, Ekwall O
J Clin Immunol 2022 Apr;42(3):618-633. Epub 2022 Jan 26 doi: 10.1007/s10875-021-01201-5. PMID: 35080750Free PMC Article
Trevisan P, Barbosa S, Sperotto G, Costi C, de Omena Filho RL, Silva AP, Varella-Garcia M, Fiegenbaum M, Rosa RF, Zen PR
Am J Med Genet A 2015 Jun;167(6):1406-8. Epub 2015 Apr 21 doi: 10.1002/ajmg.a.36531. PMID: 25899105
Gennery AR
Curr Opin Pediatr 2013 Dec;25(6):730-5. doi: 10.1097/MOP.0000000000000027. PMID: 24136300
Duijff SN, Klaassen PW, Swanenburg de Veye HF, Beemer FA, Sinnema G, Vorstman JA
Res Dev Disabil 2013 Sep;34(9):2937-45. Epub 2013 Jun 29 doi: 10.1016/j.ridd.2013.06.001. PMID: 23816629
Baker K, Baldeweg T, Sivagnanasundaram S, Scambler P, Skuse D
Biol Psychiatry 2005 Jul 1;58(1):23-31. doi: 10.1016/j.biopsych.2005.03.020. PMID: 15935994

Clinical prediction guides

Perrot A, Rickert-Sperling S
Adv Exp Med Biol 2024;1441:505-534. doi: 10.1007/978-3-031-44087-8_27. PMID: 38884729
Ge R, Ching CRK, Bassett AS, Kushan L, Antshel KM, van Amelsvoort T, Bakker G, Butcher NJ, Campbell LE, Chow EWC, Craig M, Crossley NA, Cunningham A, Daly E, Doherty JL, Durdle CA, Emanuel BS, Fiksinski A, Forsyth JK, Fremont W, Goodrich-Hunsaker NJ, Gudbrandsen M, Gur RE, Jalbrzikowski M, Kates WR, Lin A, Linden DEJ, McCabe KL, McDonald-McGinn D, Moss H, Murphy DG, Murphy KC, Owen MJ, Villalon-Reina JE, Repetto GM, Roalf DR, Ruparel K, Schmitt JE, Schuite-Koops S, Angkustsiri K, Sun D, Vajdi A, van den Bree M, Vorstman J, Thompson PM, Vila-Rodriguez F, Bearden CE
Hum Brain Mapp 2024 Jan;45(1):e26553. doi: 10.1002/hbm.26553. PMID: 38224541Free 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
Trevisan P, Barbosa S, Sperotto G, Costi C, de Omena Filho RL, Silva AP, Varella-Garcia M, Fiegenbaum M, Rosa RF, Zen PR
Am J Med Genet A 2015 Jun;167(6):1406-8. Epub 2015 Apr 21 doi: 10.1002/ajmg.a.36531. PMID: 25899105
Baker K, Baldeweg T, Sivagnanasundaram S, Scambler P, Skuse D
Biol Psychiatry 2005 Jul 1;58(1):23-31. doi: 10.1016/j.biopsych.2005.03.020. PMID: 15935994

Recent systematic reviews

Duckett KA, Poupore NS, Carroll WW, Pecha PP
Laryngoscope 2024 Jun;134(6):2551-2561. Epub 2023 Dec 5 doi: 10.1002/lary.31181. PMID: 38050953Free 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
Vo OK, McNeill A, Vogt KS
Am J Med Genet A 2018 Oct;176(10):2215-2225. Epub 2018 Mar 25 doi: 10.1002/ajmg.a.38673. PMID: 29575505Free PMC Article
Homans JF, Tromp IN, Colo D, Schlösser TPC, Kruyt MC, Deeney VFX, Crowley TB, McDonald-McGinn DM, Castelein RM
Am J Med Genet A 2018 Oct;176(10):2104-2120. Epub 2017 Nov 21 doi: 10.1002/ajmg.a.38545. PMID: 29159873
Schecklmann M, Schwenck C, Taurines R, Freitag C, Warnke A, Gerlach M, Romanos M
J Neural Transm (Vienna) 2013 Jan;120(1):121-30. Epub 2012 Jul 19 doi: 10.1007/s00702-012-0855-2. PMID: 22806003

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