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Mucopolysaccharidosis(MPS)

MedGen UID:
7733
Concept ID:
C0026703
Disease or Syndrome
Synonyms: MPS; Mucopolysaccharidoses
SNOMED CT: Mucopolysaccharidosis (11380006); MPS - Mucopolysaccharidosis (11380006)
 
Monarch Initiative: MONDO:0019249
OMIM® Phenotypic series: PS607014
Orphanet: ORPHA79213

Definition

A group of autosomal recessive or X-linked inherited lysosomal storage disorders affecting the metabolism of mucopolysaccharides, resulting in the accumulation of mucopolysaccharides in the body. Signs and symptoms include organomegaly, intellectual disabilities, abnormal skeletal development, heart disorders, hearing loss, and central nervous system deficiencies. [from NCI]

Professional guidelines

PubMed

Muschol N, Giugliani R, Jones SA, Muenzer J, Smith NJC, Whitley CB, Donnell M, Drake E, Elvidge K, Melton L, O'Neill C; MPS III Guideline Development Group
Orphanet J Rare Dis 2022 Oct 27;17(1):391. doi: 10.1186/s13023-022-02484-6. PMID: 36303195Free PMC Article
Spahiu L, Behluli E, Peterlin B, Nefic H, Hadziselimovic R, Liehr T, Temaj G
Pediatr Endocrinol Diabetes Metab 2021;27(3):201-208. doi: 10.5114/pedm.2021.109270. PMID: 34743503Free PMC Article
Sawamoto K, Álvarez González JV, Piechnik M, Otero FJ, Couce ML, Suzuki Y, Tomatsu S
Int J Mol Sci 2020 Feb 23;21(4) doi: 10.3390/ijms21041517. PMID: 32102177Free PMC Article

Recent clinical studies

Etiology

Muschol N, Giugliani R, Jones SA, Muenzer J, Smith NJC, Whitley CB, Donnell M, Drake E, Elvidge K, Melton L, O'Neill C; MPS III Guideline Development Group
Orphanet J Rare Dis 2022 Oct 27;17(1):391. doi: 10.1186/s13023-022-02484-6. PMID: 36303195Free PMC Article
Bay L, Amartino H, Antacle A, Arberas C, Berretta A, Botto H, Cazalas M, Copiz A, De Cunto C, Drelichman G, Espada G, Eiroa H, Fainboim A, Fano V, Guelber R, Maffey A, Parisi C, Pereyra M, Remondino R, Schenone A, Spécola N, Staciuk R, Zuccaro G
Arch Argent Pediatr 2021 Apr;119(2):e121-e128. doi: 10.5546/aap.2021.eng.e121. PMID: 33749201
Gilkes JA, Heldermon CD
Pediatr Endocrinol Rev 2014 Sep;12 Suppl 1:133-40. PMID: 25345095
de Ruijter J, Valstar MJ, Wijburg FA
Curr Pharm Biotechnol 2011 Jun;12(6):923-30. doi: 10.2174/138920111795542651. PMID: 21235449
Cormier-Daire V
Best Pract Res Clin Rheumatol 2008 Mar;22(1):33-44. doi: 10.1016/j.berh.2007.12.009. PMID: 18328979

Diagnosis

Kühn AL, McGillicuddy GT, Singh J
CMAJ 2022 Nov 7;194(43):E1486. doi: 10.1503/cmaj.220607. PMID: 36343955Free PMC Article
Kumar P, Das PC, Das A
JAMA Dermatol 2022 Dec 1;158(12):1438. doi: 10.1001/jamadermatol.2022.4049. PMID: 36260294
Spahiu L, Behluli E, Peterlin B, Nefic H, Hadziselimovic R, Liehr T, Temaj G
Pediatr Endocrinol Diabetes Metab 2021;27(3):201-208. doi: 10.5114/pedm.2021.109270. PMID: 34743503Free PMC Article
Bay L, Amartino H, Antacle A, Arberas C, Berretta A, Botto H, Cazalas M, Copiz A, De Cunto C, Drelichman G, Espada G, Eiroa H, Fainboim A, Fano V, Guelber R, Maffey A, Parisi C, Pereyra M, Remondino R, Schenone A, Spécola N, Staciuk R, Zuccaro G
Arch Argent Pediatr 2021 Apr;119(2):e121-e128. doi: 10.5546/aap.2021.eng.e121. PMID: 33749201
D'Avanzo F, Rigon L, Zanetti A, Tomanin R
Int J Mol Sci 2020 Feb 13;21(4) doi: 10.3390/ijms21041258. PMID: 32070051Free PMC Article

Therapy

Gentner B, Tucci F, Galimberti S, Fumagalli F, De Pellegrin M, Silvani P, Camesasca C, Pontesilli S, Darin S, Ciotti F, Sarzana M, Consiglieri G, Filisetti C, Forni G, Passerini L, Tomasoni D, Cesana D, Calabria A, Spinozzi G, Cicalese MP, Calbi V, Migliavacca M, Barzaghi F, Ferrua F, Gallo V, Miglietta S, Zonari E, Cheruku PS, Forni C, Facchini M, Corti A, Gabaldo M, Zancan S, Gasperini S, Rovelli A, Boelens JJ, Jones SA, Wynn R, Baldoli C, Montini E, Gregori S, Ciceri F, Valsecchi MG, la Marca G, Parini R, Naldini L, Aiuti A, Bernardo ME; MPSI Study Group
N Engl J Med 2021 Nov 18;385(21):1929-1940. doi: 10.1056/NEJMoa2106596. PMID: 34788506
Roberts J, Stewart C, Kearney S
Br J Nurs 2016 Jan 14-27;25(1):22, 24, 26-30. doi: 10.12968/bjon.2016.25.1.22. PMID: 26768041
Drugs R D 2006;7(4):254-8. doi: 10.2165/00126839-200607040-00005. PMID: 16784250
Hopwood JJ, Bate G, Kirkpatrick P
Nat Rev Drug Discov 2006 Feb;5(2):101-2. doi: 10.1038/nrd1962. PMID: 16521329
Danos O, Heard JM
Mol Cell Biol Hum Dis Ser 1995;5:350-67. doi: 10.1007/978-94-011-0547-7_17. PMID: 9532574

Prognosis

Gentner B, Tucci F, Galimberti S, Fumagalli F, De Pellegrin M, Silvani P, Camesasca C, Pontesilli S, Darin S, Ciotti F, Sarzana M, Consiglieri G, Filisetti C, Forni G, Passerini L, Tomasoni D, Cesana D, Calabria A, Spinozzi G, Cicalese MP, Calbi V, Migliavacca M, Barzaghi F, Ferrua F, Gallo V, Miglietta S, Zonari E, Cheruku PS, Forni C, Facchini M, Corti A, Gabaldo M, Zancan S, Gasperini S, Rovelli A, Boelens JJ, Jones SA, Wynn R, Baldoli C, Montini E, Gregori S, Ciceri F, Valsecchi MG, la Marca G, Parini R, Naldini L, Aiuti A, Bernardo ME; MPSI Study Group
N Engl J Med 2021 Nov 18;385(21):1929-1940. doi: 10.1056/NEJMoa2106596. PMID: 34788506
Khan SA, Peracha H, Ballhausen D, Wiesbauer A, Rohrbach M, Gautschi M, Mason RW, Giugliani R, Suzuki Y, Orii KE, Orii T, Tomatsu S
Mol Genet Metab 2017 Jul;121(3):227-240. Epub 2017 May 26 doi: 10.1016/j.ymgme.2017.05.016. PMID: 28595941Free PMC Article
James RA, Singh-Grewal D, Lee SJ, McGill J, Adib N; Australian Paediatric Rheumatology Group
J Paediatr Child Health 2016 Mar;52(3):262-71. doi: 10.1111/jpc.13122. PMID: 27124840
Cormier-Daire V
Best Pract Res Clin Rheumatol 2008 Mar;22(1):33-44. doi: 10.1016/j.berh.2007.12.009. PMID: 18328979
Wraith EJ, Hopwood JJ, Fuller M, Meikle PJ, Brooks DA
BioDrugs 2005;19(1):1-7. doi: 10.2165/00063030-200519010-00001. PMID: 15691212

Clinical prediction guides

Harmatz P, Giugliani R, Martins AM, Hamazaki T, Kubo T, Kira R, Minami K, Ikeda T, Moriuchi H, Kawashima S, Takasao N, So S, Sonoda H, Hirato T, Tanizawa K, Schmidt M, Sato Y
Mol Ther 2024 Mar 6;32(3):609-618. Epub 2024 Jan 10 doi: 10.1016/j.ymthe.2024.01.009. PMID: 38204164Free PMC Article
Pontesilli S, Baldoli C, Rosa PAD, Cattoni A, Bernardo ME, Meregalli P, Gasperini S, Motta S, Fumagalli F, Tucci F, Baciga F, Consiglieri G, Canonico F, De Lorenzo P, Chiapparini L, Gentner B, Aiuti A, Biondi A, Rovelli A, Parini R
J Pediatr 2022 Jan;240:297-301.e5. Epub 2021 Sep 20 doi: 10.1016/j.jpeds.2021.09.020. PMID: 34547335
Donati MA, Pasquini E, Spada M, Polo G, Burlina A
Ital J Pediatr 2018 Nov 16;44(Suppl 2):126. doi: 10.1186/s13052-018-0552-3. PMID: 30442156Free PMC Article
Zechner G, Moser M
Acta Otolaryngol 1987 May-Jun;103(5-6):384-6. PMID: 3113169
Young ID, Harper PS, Newcombe RG, Archer IM
J Med Genet 1982 Dec;19(6):408-11. doi: 10.1136/jmg.19.6.408. PMID: 6818348Free PMC Article

Recent systematic reviews

Chen C, Methley A, Naicker R, Rust S, Stepien KM
Mol Genet Metab 2023 Feb;138(2):106980. Epub 2022 Dec 22 doi: 10.1016/j.ymgme.2022.106980. PMID: 36709537
Kong W, Wu S, Zhang J, Lu C, Ding Y, Meng Y
J Pediatr Endocrinol Metab 2021 Oct 26;34(10):1225-1235. Epub 2021 Jul 19 doi: 10.1515/jpem-2020-0742. PMID: 34271605
Ysselstein D, Shulman JM, Krainc D
Mov Disord 2019 May;34(5):614-624. Epub 2019 Feb 6 doi: 10.1002/mds.27631. PMID: 30726573Free PMC Article
Pantel JT, Zhao M, Mensah MA, Hajjir N, Hsieh TC, Hanani Y, Fleischer N, Kamphans T, Mundlos S, Gurovich Y, Krawitz PM
J Inherit Metab Dis 2018 May;41(3):533-539. Epub 2018 Apr 5 doi: 10.1007/s10545-018-0174-3. PMID: 29623569Free PMC Article
Bradley LA, Haddow HRM, Palomaki GE
Genet Med 2017 Nov;19(11):1187-1201. Epub 2017 May 18 doi: 10.1038/gim.2017.30. PMID: 28640238

Supplemental Content

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    Clinical resources

    Practice guidelines

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