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Kleefstra syndrome due to 9q34 microdeletion(KLEFS1)

MedGen UID:
1610338
Concept ID:
CN970821
Disease or Syndrome
Synonyms: 9q- SYNDROME; 9q34 subtelomeric deletion syndrome; Chromosome 9q deletion syndrome; CHROMOSOME 9q34.3 DELETION SYNDROME; KLEFS1
 
Monarch Initiative: MONDO:0019896
Orphanet: ORPHA96147

Definition

Submicroscopic subtelomeric deletions of chromosome 9q are associated with a recognizable mental retardation syndrome (Harada et al., 2004; Iwakoshi et al., 2004; Stewart et al., 2004; Neas et al., 2005). Common features in patients with 9q subtelomeric deletion syndrome are severe mental retardation, hypotonia, brachy(micro)cephaly, epileptic seizures, flat face with hypertelorism, synophrys, anteverted nares, everted lower lip, carp mouth with macroglossia, and heart defects. Genetic Heterogeneity of Kleefstra Syndrome KLEFS2 (617768) is caused by mutation in the KMT2C gene (606833) on chromosome 7q36. [from OMIM]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVKleefstra syndrome due to 9q34 microdeletion

Recent clinical studies

Etiology

Stefekova A, Capkova P, Capkova Z, Curtisova V, Srovnal J, Mracka E, Klaskova E, Prochazka M
Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2022 May;166(2):187-194. Epub 2021 Mar 31 doi: 10.5507/bp.2021.019. PMID: 33824538
Wang F, Ren D, Liang X, Ke S, Zhang B, Hu B, Song X, Wang X
Nucleic Acids Res 2019 Jul 9;47(12):6315-6329. doi: 10.1093/nar/gkz444. PMID: 31127312Free PMC Article
Willemsen MH, Beunders G, Callaghan M, de Leeuw N, Nillesen WM, Yntema HG, van Hagen JM, Nieuwint AW, Morrison N, Keijzers-Vloet ST, Hoischen A, Brunner HG, Tolmie J, Kleefstra T
Clin Genet 2011 Jul;80(1):31-8. Epub 2011 Jan 10 doi: 10.1111/j.1399-0004.2010.01607.x. PMID: 21204793

Diagnosis

Lee TN, Rechetello HEL, Lima Júnior JBA, Cornelio JPFF, Pegoraro NB, Raskin S, Mikami LR
Rev Paul Pediatr 2023;42:e2022230. Epub 2023 Sep 15 doi: 10.1590/1984-0462/2024/42/2022230. PMID: 37729241Free PMC Article
Guterman S, Hervé B, Rivière J, Fauvert D, Clement P, Vialard F
J Obstet Gynaecol Res 2018 Mar;44(3):570-575. Epub 2017 Nov 21 doi: 10.1111/jog.13517. PMID: 29160022
Atik T, Karaca E, Ozkinay E, Cogulu O
Genet Couns 2015;26(4):431-5. PMID: 26852514
Campbell CL, Collins RT 2nd, Zarate YA
Birth Defects Res A Clin Mol Teratol 2014 Dec;100(12):985-90. Epub 2014 Nov 7 doi: 10.1002/bdra.23324. PMID: 25380126
Chen CP, Lin CJ, Chen YY, Wang LK, Chern SR, Wu PS, Su JW, Chen LF, Town DD, Pan CW, Wang W
Gene 2013 Dec 10;532(1):80-6. Epub 2013 Sep 18 doi: 10.1016/j.gene.2013.09.025. PMID: 24055486

Prognosis

Okayasu T, Quesnel AM, Reinshagen KL, Nadol JB Jr,
Laryngoscope 2020 Aug;130(8):2028-2033. Epub 2019 Nov 21 doi: 10.1002/lary.28380. PMID: 31750954
Verhoeven WM, Egger JI, Vermeulen K, van de Warrenburg BP, Kleefstra T
Am J Med Genet A 2011 Oct;155A(10):2409-15. Epub 2011 Sep 9 doi: 10.1002/ajmg.a.34186. PMID: 21910222
Nillesen WM, Yntema HG, Moscarda M, Verbeek NE, Wilson LC, Cowan F, Schepens M, Raas-Rothschild A, Gafni-Weinstein O, Zollino M, Vijzelaar R, Neri G, Nelen M, Bokhoven Hv, Giltay J, Kleefstra T
Hum Mutat 2011 Jul;32(7):853-9. doi: 10.1002/humu.21523. PMID: 21538692

Clinical prediction guides

Giacomini T, Cordani R, Bagnasco I, Vercellino F, Giordano L, Milito G, Ferrero GB, Mandrile G, Scala M, Meli M, Falsaperla R, Luria G, De Grandis E, Canale E, Amadori E, Striano P, Nobili L, Siri L
Neuropediatrics 2023 Dec;54(6):433-438. Epub 2023 Oct 6 doi: 10.1055/s-0043-1775977. PMID: 37802085
Ciaccio C, Scuvera G, Tucci A, Gentilin B, Baccarin M, Marchisio P, Avignone S, Milani D
Cytogenet Genome Res 2018;156(3):127-133. Epub 2018 Nov 17 doi: 10.1159/000494532. PMID: 30448833
Yatsenko SA, Hixson P, Roney EK, Scott DA, Schaaf CP, Ng YT, Palmer R, Fisher RB, Patel A, Cheung SW, Lupski JR
Hum Genet 2012 Dec;131(12):1895-910. Epub 2012 Aug 14 doi: 10.1007/s00439-012-1216-9. PMID: 22890305Free PMC Article
Nillesen WM, Yntema HG, Moscarda M, Verbeek NE, Wilson LC, Cowan F, Schepens M, Raas-Rothschild A, Gafni-Weinstein O, Zollino M, Vijzelaar R, Neri G, Nelen M, Bokhoven Hv, Giltay J, Kleefstra T
Hum Mutat 2011 Jul;32(7):853-9. doi: 10.1002/humu.21523. PMID: 21538692

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