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2-hydroxyglutarate aciduria

MedGen UID:
1689600
Concept ID:
C5139239
Finding
HPO: HP:0032278

Definition

The concentration of 2-hydroxyglutaric acid in the urine, normalized for urine concentration, is above the upper limit of normal. [from HPO]

Professional guidelines

PubMed

Phua YL, D'Annibale OM, Karunanidhi A, Mohsen AW, Kirmse B, Dobrowolski SF, Vockley J
Mol Genet Metab 2024 Jul;142(3):108495. Epub 2024 May 15 doi: 10.1016/j.ymgme.2024.108495. PMID: 38772223
Steenweg ME, Jakobs C, Errami A, van Dooren SJ, Adeva Bartolomé MT, Aerssens P, Augoustides-Savvapoulou P, Baric I, Baumann M, Bonafé L, Chabrol B, Clarke JT, Clayton P, Coker M, Cooper S, Falik-Zaccai T, Gorman M, Hahn A, Hasanoglu A, King MD, de Klerk HB, Korman SH, Lee C, Meldgaard Lund A, Mejaski-Bosnjak V, Pascual-Castroviejo I, Raadhyaksha A, Rootwelt T, Roubertie A, Ruiz-Falco ML, Scalais E, Schimmel U, Seijo-Martinez M, Suri M, Sykut-Cegielska J, Trefz FK, Uziel G, Valayannopoulos V, Vianey-Saban C, Vlaho S, Vodopiutz J, Wajner M, Walter J, Walter-Derbort C, Yapici Z, Zafeiriou DI, Spreeuwenberg MD, Celli J, den Dunnen JT, van der Knaap MS, Salomons GS
Hum Mutat 2010 Apr;31(4):380-90. doi: 10.1002/humu.21197. PMID: 20052767
Gibson KM, ten Brink HJ, Schor DS, Kok RM, Bootsma AH, Hoffmann GF, Jakobs C
Pediatr Res 1993 Sep;34(3):277-80. doi: 10.1203/00006450-199309000-00007. PMID: 8134166

Recent clinical studies

Etiology

Cansever MS, Zubarioglu T, Oruc C, Kiykim E, Gezdirici A, Neselioglu S, Erel O, Yalcinkaya C, Aktuglu-Zeybek C
Metab Brain Dis 2019 Feb;34(1):283-288. Epub 2018 Nov 29 doi: 10.1007/s11011-018-0354-8. PMID: 30499066
Patay Z, Orr BA, Shulkin BL, Hwang SN, Ying Y, Broniscer A, Boop FA, Ellison DW
J Inherit Metab Dis 2015 Mar;38(2):273-7. Epub 2014 Oct 23 doi: 10.1007/s10545-014-9782-8. PMID: 25338511Free PMC Article
Nota B, Struys EA, Pop A, Jansen EE, Fernandez Ojeda MR, Kanhai WA, Kranendijk M, van Dooren SJ, Bevova MR, Sistermans EA, Nieuwint AW, Barth M, Ben-Omran T, Hoffmann GF, de Lonlay P, McDonald MT, Meberg A, Muntau AC, Nuoffer JM, Parini R, Read MH, Renneberg A, Santer R, Strahleck T, van Schaftingen E, van der Knaap MS, Jakobs C, Salomons GS
Am J Hum Genet 2013 Apr 4;92(4):627-31. doi: 10.1016/j.ajhg.2013.03.009. PMID: 23561848Free PMC Article
Korman SH, Salomons GS, Gutman A, Brooks R, Jakobs C
Neuropediatrics 2004 Jun;35(3):151-6. doi: 10.1055/s-2004-817905. PMID: 15248096
Kwong KL, Mak T, Fong CM, Poon KH, Wong SN, So KT
Acta Paediatr 2002;91(6):716-8. doi: 10.1080/080352502760069188. PMID: 12162609

Diagnosis

Collins RRJ, Patel K, Putnam WC, Kapur P, Rakheja D
Clin Chem 2017 Dec;63(12):1812-1820. Epub 2017 Oct 16 doi: 10.1373/clinchem.2016.267666. PMID: 29038145
Anghileri E, Bertolino N, Salsano E, Antelmi L, Carpinelli P, Castellotti B, Zucca I, Gellera C, Bisogno R, Caccia C, Cuccarini V
Brain Res 2016 Oct 1;1648(Pt A):506-511. Epub 2016 Aug 16 doi: 10.1016/j.brainres.2016.08.013. PMID: 27543339
Steenweg ME, Jakobs C, Errami A, van Dooren SJ, Adeva Bartolomé MT, Aerssens P, Augoustides-Savvapoulou P, Baric I, Baumann M, Bonafé L, Chabrol B, Clarke JT, Clayton P, Coker M, Cooper S, Falik-Zaccai T, Gorman M, Hahn A, Hasanoglu A, King MD, de Klerk HB, Korman SH, Lee C, Meldgaard Lund A, Mejaski-Bosnjak V, Pascual-Castroviejo I, Raadhyaksha A, Rootwelt T, Roubertie A, Ruiz-Falco ML, Scalais E, Schimmel U, Seijo-Martinez M, Suri M, Sykut-Cegielska J, Trefz FK, Uziel G, Valayannopoulos V, Vianey-Saban C, Vlaho S, Vodopiutz J, Wajner M, Walter J, Walter-Derbort C, Yapici Z, Zafeiriou DI, Spreeuwenberg MD, Celli J, den Dunnen JT, van der Knaap MS, Salomons GS
Hum Mutat 2010 Apr;31(4):380-90. doi: 10.1002/humu.21197. PMID: 20052767
Struys EA
J Inherit Metab Dis 2006 Feb;29(1):21-9. doi: 10.1007/s10545-006-0317-9. PMID: 16601864
Nyhan WL, Shelton GD, Jakobs C, Holmes B, Bowe C, Curry CJ, Vance C, Duran M, Sweetman L
J Child Neurol 1995 Mar;10(2):137-42. doi: 10.1177/088307389501000216. PMID: 7782605

Therapy

Phua YL, D'Annibale OM, Karunanidhi A, Mohsen AW, Kirmse B, Dobrowolski SF, Vockley J
Mol Genet Metab 2024 Jul;142(3):108495. Epub 2024 May 15 doi: 10.1016/j.ymgme.2024.108495. PMID: 38772223
Geoerger B, Schiff M, Penard-Lacronique V, Darin N, Saad SM, Duchon C, Lamazière A, Desmons A, Pontoizeau C, Berlanga P, Ducassou S, Yen K, Su M, Schenkein D, Ottolenghi C, De Botton S
Nat Med 2023 Jun;29(6):1358-1363. Epub 2023 May 29 doi: 10.1038/s41591-023-02382-9. PMID: 37248298
Mühlhausen C, Salomons GS, Lukacs Z, Struys EA, van der Knaap MS, Ullrich K, Santer R
J Inherit Metab Dis 2014 Sep;37(5):775-81. Epub 2014 Apr 1 doi: 10.1007/s10545-014-9702-y. PMID: 24687295
Seijo-Martínez M, Navarro C, Castro del Río M, Vila O, Puig M, Ribes A, Butron M
Arch Neurol 2005 Apr;62(4):666-70. doi: 10.1001/archneur.62.4.666. PMID: 15824270
Watanabe H, Yamaguchi S, Saiki K, Shimizu N, Fukao T, Kondo N, Orii T
Clin Chim Acta 1995 Jul 14;238(2):115-24. doi: 10.1016/0009-8981(95)06074-n. PMID: 7586571

Prognosis

AlBalawy S, Ul Islam SS, Tasbahji N
Saudi Med J 2024 Jul;45(7):745-748. doi: 10.15537/smj.2024.45.7.20230325. PMID: 38955445Free PMC Article
Mühlhausen C, Salomons GS, Lukacs Z, Struys EA, van der Knaap MS, Ullrich K, Santer R
J Inherit Metab Dis 2014 Sep;37(5):775-81. Epub 2014 Apr 1 doi: 10.1007/s10545-014-9702-y. PMID: 24687295
Rakheja D, Mitui M, Boriack RL, DeBerardinis RJ
Pediatr Blood Cancer 2011 Mar;56(3):379-83. Epub 2010 Nov 22 doi: 10.1002/pbc.22697. PMID: 21225914
Seijo-Martínez M, Navarro C, Castro del Río M, Vila O, Puig M, Ribes A, Butron M
Arch Neurol 2005 Apr;62(4):666-70. doi: 10.1001/archneur.62.4.666. PMID: 15824270
Moroni I, Bugiani M, D'Incerti L, Maccagnano C, Rimoldi M, Bissola L, Pollo B, Finocchiaro G, Uziel G
Neurology 2004 May 25;62(10):1882-4. doi: 10.1212/01.wnl.0000125335.21381.87. PMID: 15159502

Clinical prediction guides

Geoerger B, Schiff M, Penard-Lacronique V, Darin N, Saad SM, Duchon C, Lamazière A, Desmons A, Pontoizeau C, Berlanga P, Ducassou S, Yen K, Su M, Schenkein D, Ottolenghi C, De Botton S
Nat Med 2023 Jun;29(6):1358-1363. Epub 2023 May 29 doi: 10.1038/s41591-023-02382-9. PMID: 37248298
Pop A, Williams M, Struys EA, Monné M, Jansen EEW, De Grassi A, Kanhai WA, Scarcia P, Ojeda MRF, Porcelli V, van Dooren SJM, Lennertz P, Nota B, Abdenur JE, Coman D, Das AM, El-Gharbawy A, Nuoffer JM, Polic B, Santer R, Weinhold N, Zuccarelli B, Palmieri F, Palmieri L, Salomons GS
J Inherit Metab Dis 2018 Mar;41(2):169-180. Epub 2017 Dec 13 doi: 10.1007/s10545-017-0106-7. PMID: 29238895Free PMC Article
Anghileri E, Bertolino N, Salsano E, Antelmi L, Carpinelli P, Castellotti B, Zucca I, Gellera C, Bisogno R, Caccia C, Cuccarini V
Brain Res 2016 Oct 1;1648(Pt A):506-511. Epub 2016 Aug 16 doi: 10.1016/j.brainres.2016.08.013. PMID: 27543339
Mühlhausen C, Salomons GS, Lukacs Z, Struys EA, van der Knaap MS, Ullrich K, Santer R
J Inherit Metab Dis 2014 Sep;37(5):775-81. Epub 2014 Apr 1 doi: 10.1007/s10545-014-9702-y. PMID: 24687295
Akbay EA, Moslehi J, Christensen CL, Saha S, Tchaicha JH, Ramkissoon SH, Stewart KM, Carretero J, Kikuchi E, Zhang H, Cohoon TJ, Murray S, Liu W, Uno K, Fisch S, Jones K, Gurumurthy S, Gliser C, Choe S, Keenan M, Son J, Stanley I, Losman JA, Padera R, Bronson RT, Asara JM, Abdel-Wahab O, Amrein PC, Fathi AT, Danial NN, Kimmelman AC, Kung AL, Ligon KL, Yen KE, Kaelin WG Jr, Bardeesy N, Wong KK
Genes Dev 2014 Mar 1;28(5):479-90. doi: 10.1101/gad.231233.113. PMID: 24589777Free PMC Article

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