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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

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
Duetz C, Westers TM, In 't Hout FEM, Cremers EMP, Alhan C, Venniker-Punt B, Visser-Wisselaar HA, Chitu DA, de Graaf AO, Smit L, Jansen JH, van de Loosdrecht AA
Br J Haematol 2021 May;193(4):798-803. Epub 2021 Mar 25 doi: 10.1111/bjh.17414. PMID: 33765355Free PMC Article
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
Al-Achkar W, Wafa A, Assaad M, Ehlers C, Liehr T
Mol Med Rep 2013 May;7(5):1545-8. Epub 2013 Feb 28 doi: 10.3892/mmr.2013.1349. PMID: 23450434
Wong KF, Wong WS, Siu LL, Lau TC, Chan NP
Leuk Lymphoma 2009 Aug;50(8):1333-5. doi: 10.1080/10428190903060103. PMID: 19562618

Diagnosis

Duetz C, Westers TM, In 't Hout FEM, Cremers EMP, Alhan C, Venniker-Punt B, Visser-Wisselaar HA, Chitu DA, de Graaf AO, Smit L, Jansen JH, van de Loosdrecht AA
Br J Haematol 2021 May;193(4):798-803. Epub 2021 Mar 25 doi: 10.1111/bjh.17414. PMID: 33765355Free 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
Lefèvre C, Bondu S, Le Goff S, Kosmider O, Fontenay M
Curr Opin Hematol 2017 May;24(3):191-197. doi: 10.1097/MOH.0000000000000325. PMID: 28072603
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
Wilson WA, Alfi OS, Donnell GN
Ophthalmology 1979 Jun;86(6):1184-90. doi: 10.1016/s0161-6420(79)35427-6. PMID: 160518

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
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
Duetz C, Westers TM, In 't Hout FEM, Cremers EMP, Alhan C, Venniker-Punt B, Visser-Wisselaar HA, Chitu DA, de Graaf AO, Smit L, Jansen JH, van de Loosdrecht AA
Br J Haematol 2021 May;193(4):798-803. Epub 2021 Mar 25 doi: 10.1111/bjh.17414. PMID: 33765355Free 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
Slyper AH, Shadley JD, van Tuinen P, Richton SM, Hoffmann RG, Wyatt DT
Pediatr Res 2000 May;47(5):634-9. doi: 10.1203/00006450-200005000-00013. PMID: 10813589

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
Duetz C, Westers TM, In 't Hout FEM, Cremers EMP, Alhan C, Venniker-Punt B, Visser-Wisselaar HA, Chitu DA, de Graaf AO, Smit L, Jansen JH, van de Loosdrecht AA
Br J Haematol 2021 May;193(4):798-803. Epub 2021 Mar 25 doi: 10.1111/bjh.17414. PMID: 33765355Free 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
Abreu AP, Dauber A, Macedo DB, Noel SD, Brito VN, Gill JC, Cukier P, Thompson IR, Navarro VM, Gagliardi PC, Rodrigues T, Kochi C, Longui CA, Beckers D, de Zegher F, Montenegro LR, Mendonca BB, Carroll RS, Hirschhorn JN, Latronico AC, Kaiser UB
N Engl J Med 2013 Jun 27;368(26):2467-75. Epub 2013 Jun 5 doi: 10.1056/NEJMoa1302160. PMID: 23738509Free PMC Article
Cox TC, Allen LR, Cox LL, Hopwood B, Goodwin B, Haan E, Suthers GK
Hum Mol Genet 2000 Oct 12;9(17):2553-62. doi: 10.1093/hmg/9.17.2553. PMID: 11030761

Clinical prediction guides

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
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
Tocilj A, On KF, Yuan Z, Sun J, Elkayam E, Li H, Stillman B, Joshua-Tor L
Elife 2017 Jan 23;6 doi: 10.7554/eLife.20818. PMID: 28112645Free PMC Article
Abreu AP, Dauber A, Macedo DB, Noel SD, Brito VN, Gill JC, Cukier P, Thompson IR, Navarro VM, Gagliardi PC, Rodrigues T, Kochi C, Longui CA, Beckers D, de Zegher F, Montenegro LR, Mendonca BB, Carroll RS, Hirschhorn JN, Latronico AC, Kaiser UB
N Engl J Med 2013 Jun 27;368(26):2467-75. Epub 2013 Jun 5 doi: 10.1056/NEJMoa1302160. PMID: 23738509Free 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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