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Ring chromosome 5 syndrome

MedGen UID:
1634446
Concept ID:
C4707450
Disease or Syndrome
Synonym: Ring chromosome 5
SNOMED CT: Ring chromosome 5 syndrome (765487008); Ring chromosome 5 (765487008)

Definition

A rare chromosomal anomaly syndrome with a highly variable phenotype. Principle characteristics are a neonatal mewing cry, severe developmental delay and intellectual disability, short stature, hypotonia, dysmorphic features (including microcephaly, facial asymmetry, hypertelorism, epicanthal folds, abnormal ears, micro/retrognathia), congenital cardiac anomalies (such as atrial and ventricular septal defect, tricuspid insufficiency, hypoplastic aorta) and skeletal abnormalities (for example hypoplastic thumbs, anomalous ulna/radius, dysplastic metacarpals and phalanges). [from SNOMEDCT_US]

Professional guidelines

PubMed

Bartsota M, Jowett V, Manuel D, Mortensen K, Wolfenden J, Marek J, Carvalho JS
Ultrasound Obstet Gynecol 2023 Jul;62(1):69-74. Epub 2023 Jun 9 doi: 10.1002/uog.26186. PMID: 36864493
Rinaldi B, Vaisfeld A, Amarri S, Baldo C, Gobbi G, Magini P, Melli E, Neri G, Novara F, Pippucci T, Rizzi R, Soresina A, Zampini L, Zuffardi O, Crimi M
Orphanet J Rare Dis 2017 Apr 11;12(1):69. doi: 10.1186/s13023-017-0606-4. PMID: 28399932Free PMC Article
Schroeder T, Kuendgen A, Kayser S, Kröger N, Braulke F, Platzbecker U, Klärner V, Zohren F, Haase D, Stadler M, Schlenk R, Czibere AG, Bruns I, Fenk R, Gattermann N, Haas R, Kobbe G, Germing U
Haematologica 2012 Feb;97(2):206-12. Epub 2011 Oct 11 doi: 10.3324/haematol.2011.049114. PMID: 21993688Free PMC Article

Recent clinical studies

Etiology

Ivanoff AE, Ivanoff CS
Folia Med (Plovdiv) 2023 Feb 28;65(1):20-29. doi: 10.3897/folmed.65.e71784. PMID: 36855970
Bois E, Nassar M, Zenaty D, Léger J, Van Den Abbeele T, Teissier N
Eur Ann Otorhinolaryngol Head Neck Dis 2018 Feb;135(1):21-24. Epub 2017 Sep 21 doi: 10.1016/j.anorl.2017.08.006. PMID: 28941966
Vignoli A, Bisulli F, Darra F, Mastrangelo M, Barba C, Giordano L, Turner K, Zambrelli E, Chiesa V, Bova S, Fiocchi I, Peron A, Naldi I, Milito G, Licchetta L, Tinuper P, Guerrini R, Dalla Bernardina B, Canevini MP
Epilepsy Res 2016 Dec;128:83-93. Epub 2016 Oct 24 doi: 10.1016/j.eplepsyres.2016.10.004. PMID: 27816898
D'Antonio F, Khalil A, Zidere V, Carvalho JS
Ultrasound Obstet Gynecol 2016 Apr;47(4):423-32. Epub 2016 Mar 16 doi: 10.1002/uog.15805. PMID: 26643657
Qu Y, Dang S, Hou P
Clin Chim Acta 2013 Sep 23;424:53-65. Epub 2013 May 10 doi: 10.1016/j.cca.2013.05.002. PMID: 23669186

Diagnosis

Ivanoff AE, Ivanoff CS
Folia Med (Plovdiv) 2023 Feb 28;65(1):20-29. doi: 10.3897/folmed.65.e71784. PMID: 36855970
Patnaik MM, Lasho TL
Hematology Am Soc Hematol Educ Program 2020 Dec 4;2020(1):450-459. doi: 10.1182/hematology.2020000130. PMID: 33275756Free PMC Article
Vignoli A, Bisulli F, Darra F, Mastrangelo M, Barba C, Giordano L, Turner K, Zambrelli E, Chiesa V, Bova S, Fiocchi I, Peron A, Naldi I, Milito G, Licchetta L, Tinuper P, Guerrini R, Dalla Bernardina B, Canevini MP
Epilepsy Res 2016 Dec;128:83-93. Epub 2016 Oct 24 doi: 10.1016/j.eplepsyres.2016.10.004. PMID: 27816898
Qu Y, Dang S, Hou P
Clin Chim Acta 2013 Sep 23;424:53-65. Epub 2013 May 10 doi: 10.1016/j.cca.2013.05.002. PMID: 23669186
Daber RD, Conlin LK, Leonard LD, Canevini MP, Vignoli A, Hosain S, Brown LW, Spinner NB
Eur J Med Genet 2012 May;55(5):381-7. Epub 2012 Feb 22 doi: 10.1016/j.ejmg.2012.02.004. PMID: 22406087

Therapy

van de Loosdrecht AA, Cremers EMP, Alhan C, Duetz C, In 't Hout FEM, Visser-Wisselaar HA, Chitu DA, Verbrugge A, Cunha SM, Ossenkoppele GJ, Janssen JJWM, Klein SK, Vellenga E, Huls GA, Muus P, Langemeijer SMC, de Greef GE, Te Boekhorst PAW, Raaijmakers MHG, van Marwijk Kooy M, Legdeur MC, Wegman JJ, Deenik W, de Weerdt O, van Maanen-Lamme TM, Jobse P, van Kampen RJW, Beeker A, Wijermans PW, Biemond BJ, Tanis BC, van Esser JWJ, Schaar CG, Noordzij-Nooteboom HS, Jacobs EMG, de Graaf AO, Jongen-Lavrencic M, Stevens-Kroef MJPL, Westers TM, Jansen JH
Leukemia 2024 Apr;38(4):840-850. Epub 2024 Jan 31 doi: 10.1038/s41375-024-02161-6. PMID: 38297135Free PMC Article
Wannes S, El Ahmer I, Rjiba K, Jemmali N, Abdallah HH, Haj RB, Achour A, Bouzidi H, Saad A, Mougou S, Mahjoub B
Growth Horm IGF Res 2023 Aug;71:101550. Epub 2023 Jul 25 doi: 10.1016/j.ghir.2023.101550. PMID: 37531800
Chen M, Ke X, Liang H, Gong F, Yang H, Wang L, Duan L, Pan H, Cao D, Zhu H
Mol Genet Genomic Med 2021 Dec;9(12):e1842. Epub 2021 Nov 8 doi: 10.1002/mgg3.1842. PMID: 34747577Free PMC Article
Vignoli A, Bisulli F, Darra F, Mastrangelo M, Barba C, Giordano L, Turner K, Zambrelli E, Chiesa V, Bova S, Fiocchi I, Peron A, Naldi I, Milito G, Licchetta L, Tinuper P, Guerrini R, Dalla Bernardina B, Canevini MP
Epilepsy Res 2016 Dec;128:83-93. Epub 2016 Oct 24 doi: 10.1016/j.eplepsyres.2016.10.004. PMID: 27816898
Schroeder T, Kuendgen A, Kayser S, Kröger N, Braulke F, Platzbecker U, Klärner V, Zohren F, Haase D, Stadler M, Schlenk R, Czibere AG, Bruns I, Fenk R, Gattermann N, Haas R, Kobbe G, Germing U
Haematologica 2012 Feb;97(2):206-12. Epub 2011 Oct 11 doi: 10.3324/haematol.2011.049114. PMID: 21993688Free PMC Article

Prognosis

van de Loosdrecht AA, Cremers EMP, Alhan C, Duetz C, In 't Hout FEM, Visser-Wisselaar HA, Chitu DA, Verbrugge A, Cunha SM, Ossenkoppele GJ, Janssen JJWM, Klein SK, Vellenga E, Huls GA, Muus P, Langemeijer SMC, de Greef GE, Te Boekhorst PAW, Raaijmakers MHG, van Marwijk Kooy M, Legdeur MC, Wegman JJ, Deenik W, de Weerdt O, van Maanen-Lamme TM, Jobse P, van Kampen RJW, Beeker A, Wijermans PW, Biemond BJ, Tanis BC, van Esser JWJ, Schaar CG, Noordzij-Nooteboom HS, Jacobs EMG, de Graaf AO, Jongen-Lavrencic M, Stevens-Kroef MJPL, Westers TM, Jansen JH
Leukemia 2024 Apr;38(4):840-850. Epub 2024 Jan 31 doi: 10.1038/s41375-024-02161-6. PMID: 38297135Free PMC Article
Patnaik MM, Lasho TL
Hematology Am Soc Hematol Educ Program 2020 Dec 4;2020(1):450-459. doi: 10.1182/hematology.2020000130. PMID: 33275756Free PMC Article
Vignoli A, Bisulli F, Darra F, Mastrangelo M, Barba C, Giordano L, Turner K, Zambrelli E, Chiesa V, Bova S, Fiocchi I, Peron A, Naldi I, Milito G, Licchetta L, Tinuper P, Guerrini R, Dalla Bernardina B, Canevini MP
Epilepsy Res 2016 Dec;128:83-93. Epub 2016 Oct 24 doi: 10.1016/j.eplepsyres.2016.10.004. PMID: 27816898
Grossi A, Crinò A, Luciano R, Lombardo A, Cappa M, Fierabracci A
Ital J Pediatr 2013 Dec 20;39:79. doi: 10.1186/1824-7288-39-79. PMID: 24355069Free PMC Article
Kaminska A, Oguni H
Handb Clin Neurol 2013;111:641-52. doi: 10.1016/B978-0-444-52891-9.00067-1. PMID: 23622212

Clinical prediction guides

Piché J, Van Vliet PP, Pucéat M, Andelfinger G
Cell Cycle 2019 Nov;18(21):2828-2848. Epub 2019 Sep 13 doi: 10.1080/15384101.2019.1658476. PMID: 31516082Free PMC Article
Paz-Y-Miño C, Guevara-Aguirre J, Paz-Y-Miño A, Velarde F, Armendáriz-Castillo I, Yumiceba V, Hernández JM, García JL, Leone PE
J Med Case Rep 2018 Nov 16;12(1):340. doi: 10.1186/s13256-018-1879-5. PMID: 30442194Free PMC Article
Kim T, Bershteyn M, Wynshaw-Boris A
Nucleus 2014 Sep-Oct;5(5):391-5. doi: 10.4161/nucl.36300. PMID: 25482192Free PMC Article
Grossi A, Crinò A, Luciano R, Lombardo A, Cappa M, Fierabracci A
Ital J Pediatr 2013 Dec 20;39:79. doi: 10.1186/1824-7288-39-79. PMID: 24355069Free PMC Article
Daber RD, Conlin LK, Leonard LD, Canevini MP, Vignoli A, Hosain S, Brown LW, Spinner NB
Eur J Med Genet 2012 May;55(5):381-7. Epub 2012 Feb 22 doi: 10.1016/j.ejmg.2012.02.004. PMID: 22406087

Recent systematic reviews

Wannes S, El Ahmer I, Rjiba K, Jemmali N, Abdallah HH, Haj RB, Achour A, Bouzidi H, Saad A, Mougou S, Mahjoub B
Growth Horm IGF Res 2023 Aug;71:101550. Epub 2023 Jul 25 doi: 10.1016/j.ghir.2023.101550. PMID: 37531800
Yamashita H, Arakawa Y, Terada Y, Takeuchi Y, Mineharu Y, Sumiyoshi S, Tokunaga S, Nakajima K, Kawabata N, Tanaka K, Tanji M, Umeda K, Minamiguchi S, Ogawa S, Haga H, Takita J, Miyamoto S
Brain Tumor Pathol 2022 Oct;39(4):232-239. Epub 2022 Jun 24 doi: 10.1007/s10014-022-00440-7. PMID: 35750988
D'Antonio F, Khalil A, Zidere V, Carvalho JS
Ultrasound Obstet Gynecol 2016 Apr;47(4):423-32. Epub 2016 Mar 16 doi: 10.1002/uog.15805. PMID: 26643657

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