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Rare hereditary metabolic disease with peripheral neuropathy

MedGen UID:
1843366
Concept ID:
C5680814
Disease or Syndrome
Orphanet: ORPHA207018

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRare hereditary metabolic disease with peripheral neuropathy

Professional guidelines

PubMed

Schwartzlow C, Kazamel M
J Clin Neuromuscul Dis 2020 Mar;21(3):144-156. doi: 10.1097/CND.0000000000000270. PMID: 32073460
Maurer MS, Bokhari S, Damy T, Dorbala S, Drachman BM, Fontana M, Grogan M, Kristen AV, Lousada I, Nativi-Nicolau J, Cristina Quarta C, Rapezzi C, Ruberg FL, Witteles R, Merlini G
Circ Heart Fail 2019 Sep;12(9):e006075. Epub 2019 Sep 4 doi: 10.1161/CIRCHEARTFAILURE.119.006075. PMID: 31480867Free PMC Article
Donnelly JP, Hanna M
Cleve Clin J Med 2017 Dec;84(12 Suppl 3):12-26. doi: 10.3949/ccjm.84.s3.02. PMID: 29257735

Recent clinical studies

Etiology

Manganelli F, Fabrizi GM, Luigetti M, Mandich P, Mazzeo A, Pareyson D
Neurol Sci 2022 Dec;43(Suppl 2):595-604. Epub 2020 Nov 14 doi: 10.1007/s10072-020-04889-2. PMID: 33188616Free PMC Article
Bart NK, Thomas L, Korczyk D, Atherton JJ, Stewart GJ, Fatkin D
Heart Lung Circ 2020 Apr;29(4):575-583. Epub 2019 Dec 17 doi: 10.1016/j.hlc.2019.11.019. PMID: 32001152
Ashrafi MR, Amanat M, Garshasbi M, Kameli R, Nilipour Y, Heidari M, Rezaei Z, Tavasoli AR
Expert Rev Neurother 2020 Jan;20(1):65-84. Epub 2019 Dec 12 doi: 10.1080/14737175.2020.1699060. PMID: 31829048
Maurer MS, Bokhari S, Damy T, Dorbala S, Drachman BM, Fontana M, Grogan M, Kristen AV, Lousada I, Nativi-Nicolau J, Cristina Quarta C, Rapezzi C, Ruberg FL, Witteles R, Merlini G
Circ Heart Fail 2019 Sep;12(9):e006075. Epub 2019 Sep 4 doi: 10.1161/CIRCHEARTFAILURE.119.006075. PMID: 31480867Free PMC Article
Ando Y, Coelho T, Berk JL, Cruz MW, Ericzon BG, Ikeda S, Lewis WD, Obici L, Planté-Bordeneuve V, Rapezzi C, Said G, Salvi F
Orphanet J Rare Dis 2013 Feb 20;8:31. doi: 10.1186/1750-1172-8-31. PMID: 23425518Free PMC Article

Diagnosis

Manganelli F, Fabrizi GM, Luigetti M, Mandich P, Mazzeo A, Pareyson D
Neurol Sci 2022 Dec;43(Suppl 2):595-604. Epub 2020 Nov 14 doi: 10.1007/s10072-020-04889-2. PMID: 33188616Free PMC Article
Griffin JM, Rosenblum H, Maurer MS
Circ Res 2021 May 14;128(10):1554-1575. Epub 2021 May 13 doi: 10.1161/CIRCRESAHA.121.318187. PMID: 33983835Free PMC Article
Ashrafi MR, Amanat M, Garshasbi M, Kameli R, Nilipour Y, Heidari M, Rezaei Z, Tavasoli AR
Expert Rev Neurother 2020 Jan;20(1):65-84. Epub 2019 Dec 12 doi: 10.1080/14737175.2020.1699060. PMID: 31829048
Maurer MS, Bokhari S, Damy T, Dorbala S, Drachman BM, Fontana M, Grogan M, Kristen AV, Lousada I, Nativi-Nicolau J, Cristina Quarta C, Rapezzi C, Ruberg FL, Witteles R, Merlini G
Circ Heart Fail 2019 Sep;12(9):e006075. Epub 2019 Sep 4 doi: 10.1161/CIRCHEARTFAILURE.119.006075. PMID: 31480867Free PMC Article
Ando Y, Coelho T, Berk JL, Cruz MW, Ericzon BG, Ikeda S, Lewis WD, Obici L, Planté-Bordeneuve V, Rapezzi C, Said G, Salvi F
Orphanet J Rare Dis 2013 Feb 20;8:31. doi: 10.1186/1750-1172-8-31. PMID: 23425518Free PMC Article

Therapy

Porcari A, Cappelli F, Nitsche C, Tomasoni D, Sinigiani G, Longhi S, Bordignon L, Masri A, Serenelli M, Urey M, Musumeci B, Cipriani A, Canepa M, Badr-Eslam R, Kronberger C, Chimenti C, Zampieri M, Allegro V, Razvi Y, Patel R, Ioannou A, Rauf MU, Petrie A, Whelan C, Emdin M, Metra M, Merlo M, Sinagra G, Hawkins PN, Solomon SD, Gillmore JD, Fontana M
J Am Coll Cardiol 2024 Jun 18;83(24):2411-2422. doi: 10.1016/j.jacc.2024.03.429. PMID: 38866445
Garcia-Pavia P, Aus dem Siepen F, Donal E, Lairez O, van der Meer P, Kristen AV, Mercuri MF, Michalon A, Frost RJA, Grimm J, Nitsch RM, Hock C, Kahr PC, Damy T
N Engl J Med 2023 Jul 20;389(3):239-250. Epub 2023 May 20 doi: 10.1056/NEJMoa2303765. PMID: 37212440
Griffin JM, Rosenblum H, Maurer MS
Circ Res 2021 May 14;128(10):1554-1575. Epub 2021 May 13 doi: 10.1161/CIRCRESAHA.121.318187. PMID: 33983835Free PMC Article
Adams D, Polydefkis M, González-Duarte A, Wixner J, Kristen AV, Schmidt HH, Berk JL, Losada López IA, Dispenzieri A, Quan D, Conceição IM, Slama MS, Gillmore JD, Kyriakides T, Ajroud-Driss S, Waddington-Cruz M, Mezei MM, Planté-Bordeneuve V, Attarian S, Mauricio E, Brannagan TH 3rd, Ueda M, Aldinc E, Wang JJ, White MT, Vest J, Berber E, Sweetser MT, Coelho T; patisiran Global OLE study group
Lancet Neurol 2021 Jan;20(1):49-59. Epub 2020 Nov 16 doi: 10.1016/S1474-4422(20)30368-9. PMID: 33212063
Coelho T, Maurer MS, Suhr OB
Curr Med Res Opin 2013 Jan;29(1):63-76. Epub 2012 Dec 13 doi: 10.1185/03007995.2012.754348. PMID: 23193944

Prognosis

Porcari A, Razvi Y, Masi A, Patel R, Ioannou A, Rauf MU, Hutt DF, Rowczenio D, Gilbertson J, Martinez-Naharro A, Venneri L, Whelan C, Lachmann H, Wechalekar A, Quarta CC, Merlo M, Sinagra G, Hawkins PN, Fontana M, Gillmore JD
Eur J Heart Fail 2023 Apr;25(4):515-524. Epub 2023 Jan 30 doi: 10.1002/ejhf.2776. PMID: 36644836
Deneubourg C, Ramm M, Smith LJ, Baron O, Singh K, Byrne SC, Duchen MR, Gautel M, Eskelinen EL, Fanto M, Jungbluth H
Autophagy 2022 Mar;18(3):496-517. Epub 2021 Aug 19 doi: 10.1080/15548627.2021.1943177. PMID: 34130600Free PMC Article
Bart NK, Thomas L, Korczyk D, Atherton JJ, Stewart GJ, Fatkin D
Heart Lung Circ 2020 Apr;29(4):575-583. Epub 2019 Dec 17 doi: 10.1016/j.hlc.2019.11.019. PMID: 32001152
Maurer MS, Bokhari S, Damy T, Dorbala S, Drachman BM, Fontana M, Grogan M, Kristen AV, Lousada I, Nativi-Nicolau J, Cristina Quarta C, Rapezzi C, Ruberg FL, Witteles R, Merlini G
Circ Heart Fail 2019 Sep;12(9):e006075. Epub 2019 Sep 4 doi: 10.1161/CIRCHEARTFAILURE.119.006075. PMID: 31480867Free PMC Article
Dyck PJB, González-Duarte A, Obici L, Polydefkis M, Wiesman JF, Antonino I, Litchy WJ, Dyck PJ
J Neurol Sci 2019 Oct 15;405:116424. Epub 2019 Aug 8 doi: 10.1016/j.jns.2019.116424. PMID: 31445300

Clinical prediction guides

Porcari A, Razvi Y, Masi A, Patel R, Ioannou A, Rauf MU, Hutt DF, Rowczenio D, Gilbertson J, Martinez-Naharro A, Venneri L, Whelan C, Lachmann H, Wechalekar A, Quarta CC, Merlo M, Sinagra G, Hawkins PN, Fontana M, Gillmore JD
Eur J Heart Fail 2023 Apr;25(4):515-524. Epub 2023 Jan 30 doi: 10.1002/ejhf.2776. PMID: 36644836
Adams D, Polydefkis M, González-Duarte A, Wixner J, Kristen AV, Schmidt HH, Berk JL, Losada López IA, Dispenzieri A, Quan D, Conceição IM, Slama MS, Gillmore JD, Kyriakides T, Ajroud-Driss S, Waddington-Cruz M, Mezei MM, Planté-Bordeneuve V, Attarian S, Mauricio E, Brannagan TH 3rd, Ueda M, Aldinc E, Wang JJ, White MT, Vest J, Berber E, Sweetser MT, Coelho T; patisiran Global OLE study group
Lancet Neurol 2021 Jan;20(1):49-59. Epub 2020 Nov 16 doi: 10.1016/S1474-4422(20)30368-9. PMID: 33212063
Bart NK, Thomas L, Korczyk D, Atherton JJ, Stewart GJ, Fatkin D
Heart Lung Circ 2020 Apr;29(4):575-583. Epub 2019 Dec 17 doi: 10.1016/j.hlc.2019.11.019. PMID: 32001152
Dyck PJB, González-Duarte A, Obici L, Polydefkis M, Wiesman JF, Antonino I, Litchy WJ, Dyck PJ
J Neurol Sci 2019 Oct 15;405:116424. Epub 2019 Aug 8 doi: 10.1016/j.jns.2019.116424. PMID: 31445300
Conceição I, González-Duarte A, Obici L, Schmidt HH, Simoneau D, Ong ML, Amass L
J Peripher Nerv Syst 2016 Mar;21(1):5-9. doi: 10.1111/jns.12153. PMID: 26663427Free PMC Article

Recent systematic reviews

He X, Tian Z, Guan H, Zhang S
Orphanet J Rare Dis 2022 Sep 2;17(1):337. doi: 10.1186/s13023-022-02481-9. PMID: 36056432Free PMC Article
Finsterer J, Iglseder S, Wanschitz J, Topakian R, Löscher WN, Grisold W
Acta Neurol Scand 2019 Feb;139(2):92-105. Epub 2018 Oct 23 doi: 10.1111/ane.13035. PMID: 30295933

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