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Mucopolysaccharidosis, type vi, severe

MedGen UID:
865690
Concept ID:
C4017253
Finding
Synonym: MUCOPOLYSACCHARIDOSIS, TYPE VI, SEVERE
 
OMIM®: 611542

Professional guidelines

PubMed

Entchev E, Antonelli S, Mauro V, Cimbolini N, Jantzen I, Roussey A, Germain JM, Zhang H, Luccarrini JM, Lacombe O, Young SP, Feraille L, Tallandier M
Mol Genet Metab 2022 Feb;135(2):143-153. Epub 2021 Jul 25 doi: 10.1016/j.ymgme.2021.07.008. PMID: 34417096
Brands MM, Hoogeveen-Westerveld M, Kroos MA, Nobel W, Ruijter GJ, Özkan L, Plug I, Grinberg D, Vilageliu L, Halley DJ, van der Ploeg AT, Reuser AJ
Orphanet J Rare Dis 2013 Apr 4;8:51. doi: 10.1186/1750-1172-8-51. PMID: 23557332Free PMC Article
Braunlin EA, Harmatz PR, Scarpa M, Furlanetto B, Kampmann C, Loehr JP, Ponder KP, Roberts WC, Rosenfeld HM, Giugliani R
J Inherit Metab Dis 2011 Dec;34(6):1183-97. Epub 2011 Jul 9 doi: 10.1007/s10545-011-9359-8. PMID: 21744090Free PMC Article

Recent clinical studies

Etiology

Rintz E, Banacki M, Ziemian M, Kobus B, Wegrzyn G
Mol Genet Metab 2024 Jul;142(3):108507. Epub 2024 May 24 doi: 10.1016/j.ymgme.2024.108507. PMID: 38815294
Brunetti-Pierri N, Ferla R, Ginocchio VM, Rossi A, Fecarotta S, Romano R, Parenti G, Yildiz Y, Zancan S, Pecorella V, Dell'Anno M, Graziano M, Alliegro M, Andria G, Santamaria F, Brunetti-Pierri R, Simonelli F, Nigro V, Vargas M, Servillo G, Borgia F, Soscia E, Gargaro M, Funghini S, Tedesco N, Le Brun PR, Rupar CA, Prasad C, O'Callaghan M, Mitchell JJ, Danos O, Marteau JB, Galimberti S, Valsecchi MG, Veron P, Mingozzi F, Fallarino F, la Marca G, Sivri HS, Auricchio A
NEJM Evid 2022 Jul;1(7):EVIDoa2200052. Epub 2022 Jun 6 doi: 10.1056/EVIDoa2200052. PMID: 38319253
D'Avanzo F, Zanetti A, De Filippis C, Tomanin R
Int J Mol Sci 2021 Dec 15;22(24) doi: 10.3390/ijms222413456. PMID: 34948256Free PMC Article
Brunelli MJ, Atallah ÁN, da Silva EM
Cochrane Database Syst Rev 2021 Sep 17;9(9):CD009806. doi: 10.1002/14651858.CD009806.pub3. PMID: 34533215Free PMC Article
Brunelli MJ, Atallah ÁN, da Silva EM
Cochrane Database Syst Rev 2016 Mar 4;3:CD009806. doi: 10.1002/14651858.CD009806.pub2. PMID: 26943923

Diagnosis

D'Avanzo F, Zanetti A, De Filippis C, Tomanin R
Int J Mol Sci 2021 Dec 15;22(24) doi: 10.3390/ijms222413456. PMID: 34948256Free PMC Article
Concolino D, Deodato F, Parini R
Ital J Pediatr 2018 Nov 16;44(Suppl 2):120. doi: 10.1186/s13052-018-0562-1. PMID: 30442189Free PMC Article
Braunlin EA, Harmatz PR, Scarpa M, Furlanetto B, Kampmann C, Loehr JP, Ponder KP, Roberts WC, Rosenfeld HM, Giugliani R
J Inherit Metab Dis 2011 Dec;34(6):1183-97. Epub 2011 Jul 9 doi: 10.1007/s10545-011-9359-8. PMID: 21744090Free PMC Article
Valayannopoulos V, Nicely H, Harmatz P, Turbeville S
Orphanet J Rare Dis 2010 Apr 12;5:5. doi: 10.1186/1750-1172-5-5. PMID: 20385007Free PMC Article
Vijayalakshmi AM
Indian Pediatr 2002 Jun;39(6):594-5. PMID: 12084959

Therapy

D'Avanzo F, Zanetti A, De Filippis C, Tomanin R
Int J Mol Sci 2021 Dec 15;22(24) doi: 10.3390/ijms222413456. PMID: 34948256Free PMC Article
Brunelli MJ, Atallah ÁN, da Silva EM
Cochrane Database Syst Rev 2021 Sep 17;9(9):CD009806. doi: 10.1002/14651858.CD009806.pub3. PMID: 34533215Free PMC Article
Brunelli MJ, Atallah ÁN, da Silva EM
Cochrane Database Syst Rev 2016 Mar 4;3:CD009806. doi: 10.1002/14651858.CD009806.pub2. PMID: 26943923
Hum Gene Ther Clin Dev 2015 Jun;26(2):86-8. doi: 10.1089/humc.2015.2525. PMID: 26086756
Valayannopoulos V, Wijburg FA
Rheumatology (Oxford) 2011 Dec;50 Suppl 5:v49-59. doi: 10.1093/rheumatology/ker396. PMID: 22210671

Prognosis

Rintz E, Banacki M, Ziemian M, Kobus B, Wegrzyn G
Mol Genet Metab 2024 Jul;142(3):108507. Epub 2024 May 24 doi: 10.1016/j.ymgme.2024.108507. PMID: 38815294
Concolino D, Deodato F, Parini R
Ital J Pediatr 2018 Nov 16;44(Suppl 2):120. doi: 10.1186/s13052-018-0562-1. PMID: 30442189Free PMC Article
Valayannopoulos V, Nicely H, Harmatz P, Turbeville S
Orphanet J Rare Dis 2010 Apr 12;5:5. doi: 10.1186/1750-1172-5-5. PMID: 20385007Free PMC Article
Ashworth JL, Biswas S, Wraith E, Lloyd IC
Surv Ophthalmol 2006 Jan-Feb;51(1):1-17. doi: 10.1016/j.survophthal.2005.11.007. PMID: 16414358
Vijayalakshmi AM
Indian Pediatr 2002 Jun;39(6):594-5. PMID: 12084959

Clinical prediction guides

Brunelli MJ, Atallah ÁN, da Silva EM
Cochrane Database Syst Rev 2021 Sep 17;9(9):CD009806. doi: 10.1002/14651858.CD009806.pub3. PMID: 34533215Free PMC Article
Parini R, Deodato F
Int J Mol Sci 2020 Apr 23;21(8) doi: 10.3390/ijms21082975. PMID: 32340185Free PMC Article
Brunelli MJ, Atallah ÁN, da Silva EM
Cochrane Database Syst Rev 2016 Mar 4;3:CD009806. doi: 10.1002/14651858.CD009806.pub2. PMID: 26943923
Jurecka A, Zakharova E, Cimbalistiene L, Gusina N, Malinova V, Różdżyńska-Świątkowska A, Golda A, Kulpanovich A, Kaldenovna Abdilova G, Voskoboeva E, Tylki-Szymańska A
Pediatr Int 2014 Aug;56(4):520-5. Epub 2014 Apr 1 doi: 10.1111/ped.12281. PMID: 24373060
Debette S, Germain DP
Handb Clin Neurol 2014;119:565-76. doi: 10.1016/B978-0-7020-4086-3.00037-0. PMID: 24365320

Recent systematic reviews

Brunelli MJ, Atallah ÁN, da Silva EM
Cochrane Database Syst Rev 2021 Sep 17;9(9):CD009806. doi: 10.1002/14651858.CD009806.pub3. PMID: 34533215Free PMC Article
Brunelli MJ, Atallah ÁN, da Silva EM
Cochrane Database Syst Rev 2016 Mar 4;3:CD009806. doi: 10.1002/14651858.CD009806.pub2. PMID: 26943923
Noh H, Lee JI
J Clin Pharm Ther 2014 Jun;39(3):215-24. Epub 2014 Feb 25 doi: 10.1111/jcpt.12136. PMID: 24612142

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