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Congenital disorder of glycosylation with hepatic involvement

MedGen UID:
1843224
Concept ID:
C5679910
Disease or Syndrome
Synonym: CDG with hepatic involvement
 
Orphanet: ORPHA371157

Professional guidelines

PubMed

Albokhari D, Ng BG, Guberinic A, Daniel EJP, Engelhardt NM, Barone R, Fiumara A, Garavelli L, Trimarchi G, Wolfe L, Raymond KM, Morava E, He M, Freeze HH, Lam C, Edmondson AC
J Inherit Metab Dis 2022 Sep;45(5):969-980. Epub 2022 Jun 30 doi: 10.1002/jimd.12527. PMID: 35716054Free PMC Article
Čechová A, Altassan R, Borgel D, Bruneel A, Correia J, Girard M, Harroche A, Kiec-Wilk B, Mohnike K, Pascreau T, Pawliński Ł, Radenkovic S, Vuillaumier-Barrot S, Aldamiz-Echevarria L, Couce ML, Martins EG, Quelhas D, Morava E, de Lonlay P, Witters P, Honzík T
J Inherit Metab Dis 2020 Jul;43(4):671-693. Epub 2020 Apr 21 doi: 10.1002/jimd.12241. PMID: 32266963Free PMC Article

Recent clinical studies

Etiology

Kasapkara CS, Olgac A, Derinkuyu BE, Oztoprak U, Jaeken J
J Coll Physicians Surg Pak 2022 Dec;32(12):SS221-SS226. doi: 10.29271/jcpsp.2022.Supp0.SS221. PMID: 36597345
Albokhari D, Ng BG, Guberinic A, Daniel EJP, Engelhardt NM, Barone R, Fiumara A, Garavelli L, Trimarchi G, Wolfe L, Raymond KM, Morava E, He M, Freeze HH, Lam C, Edmondson AC
J Inherit Metab Dis 2022 Sep;45(5):969-980. Epub 2022 Jun 30 doi: 10.1002/jimd.12527. PMID: 35716054Free PMC Article
Jansen JC, Timal S, van Scherpenzeel M, Michelakakis H, Vicogne D, Ashikov A, Moraitou M, Hoischen A, Huijben K, Steenbergen G, van den Boogert MA, Porta F, Calvo PL, Mavrikou M, Cenacchi G, van den Bogaart G, Salomon J, Holleboom AG, Rodenburg RJ, Drenth JP, Huynen MA, Wevers RA, Morava E, Foulquier F, Veltman JA, Lefeber DJ
Am J Hum Genet 2016 Feb 4;98(2):322-30. Epub 2016 Jan 28 doi: 10.1016/j.ajhg.2015.12.011. PMID: 26833330Free PMC Article
Rohlfing AK, Rust S, Reunert J, Tirre M, Du Chesne I, Wemhoff S, Meinhardt F, Hartmann H, Das AM, Marquardt T
Gene 2014 Jan 25;534(2):345-51. Epub 2013 Oct 21 doi: 10.1016/j.gene.2013.10.013. PMID: 24157261
Vantyghem MC, Dobbelaere D, Mention K, Wemeau JL, Saudubray JM, Douillard C
Orphanet J Rare Dis 2012 Jan 28;7:11. doi: 10.1186/1750-1172-7-11. PMID: 22284844Free PMC Article

Diagnosis

Albokhari D, Ng BG, Guberinic A, Daniel EJP, Engelhardt NM, Barone R, Fiumara A, Garavelli L, Trimarchi G, Wolfe L, Raymond KM, Morava E, He M, Freeze HH, Lam C, Edmondson AC
J Inherit Metab Dis 2022 Sep;45(5):969-980. Epub 2022 Jun 30 doi: 10.1002/jimd.12527. PMID: 35716054Free PMC Article
Čechová A, Altassan R, Borgel D, Bruneel A, Correia J, Girard M, Harroche A, Kiec-Wilk B, Mohnike K, Pascreau T, Pawliński Ł, Radenkovic S, Vuillaumier-Barrot S, Aldamiz-Echevarria L, Couce ML, Martins EG, Quelhas D, Morava E, de Lonlay P, Witters P, Honzík T
J Inherit Metab Dis 2020 Jul;43(4):671-693. Epub 2020 Apr 21 doi: 10.1002/jimd.12241. PMID: 32266963Free PMC Article
Freeze HH, Eklund EA, Ng BG, Patterson MC
Lancet Neurol 2012 May;11(5):453-66. doi: 10.1016/S1474-4422(12)70040-6. PMID: 22516080Free PMC Article
Vantyghem MC, Dobbelaere D, Mention K, Wemeau JL, Saudubray JM, Douillard C
Orphanet J Rare Dis 2012 Jan 28;7:11. doi: 10.1186/1750-1172-7-11. PMID: 22284844Free PMC Article
Gordon N
Postgrad Med J 2000 Mar;76(893):145-9. doi: 10.1136/pmj.76.893.145. PMID: 10684323Free PMC Article

Therapy

Damen G, de Klerk H, Huijmans J, den Hollander J, Sinaasappel M
J Pediatr Gastroenterol Nutr 2004 Mar;38(3):282-7. doi: 10.1097/00005176-200403000-00010. PMID: 15076627
Gordon N
Postgrad Med J 2000 Mar;76(893):145-9. doi: 10.1136/pmj.76.893.145. PMID: 10684323Free PMC Article

Prognosis

Tahata S, Weckwerth J, Ligezka A, He M, Lee HE, Heimbach J, Ibrahim SH, Kozicz T, Furuya K, Morava E
Mol Genet Metab 2023 Apr;138(4):107559. Epub 2023 Mar 17 doi: 10.1016/j.ymgme.2023.107559. PMID: 36965289Free PMC Article
Ondruskova N, Honzik T, Vondrackova A, Stranecky V, Tesarova M, Zeman J, Hansikova H
J Inherit Metab Dis 2020 Jul;43(4):694-700. Epub 2020 Apr 7 doi: 10.1002/jimd.12237. PMID: 32216104Free PMC Article
Girard M, Poujois A, Fabre M, Lacaille F, Debray D, Rio M, Fenaille F, Cholet S, Ruel C, Caussé E, Selves J, Bridoux-Henno L, Woimant F, Dupré T, Vuillaumier-Barrot S, Seta N, Alric L, de Lonlay P, Bruneel A
Mol Genet Metab 2018 Jul;124(3):228-235. Epub 2018 May 9 doi: 10.1016/j.ymgme.2018.05.002. PMID: 29759592
Freeze HH, Eklund EA, Ng BG, Patterson MC
Lancet Neurol 2012 May;11(5):453-66. doi: 10.1016/S1474-4422(12)70040-6. PMID: 22516080Free PMC Article
Gordon N
Postgrad Med J 2000 Mar;76(893):145-9. doi: 10.1136/pmj.76.893.145. PMID: 10684323Free PMC Article

Clinical prediction guides

Rosato BE, Marra R, D'Onofrio V, Del Giudice F, Della Monica S, Iolascon A, Andolfo I, Russo R
Int J Mol Sci 2022 Jan 24;23(3) doi: 10.3390/ijms23031304. PMID: 35163229Free PMC Article
Ondruskova N, Honzik T, Vondrackova A, Stranecky V, Tesarova M, Zeman J, Hansikova H
J Inherit Metab Dis 2020 Jul;43(4):694-700. Epub 2020 Apr 7 doi: 10.1002/jimd.12237. PMID: 32216104Free PMC Article

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