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Isovaleric acidemia, type I

MedGen UID:
865493
Concept ID:
C4017056
Finding
Synonym: ISOVALERIC ACIDEMIA, TYPE I
 
OMIM®: 607036

Professional guidelines

PubMed

Tuncel AT, Boy N, Morath MA, Hörster F, Mütze U, Kölker S
J Inherit Metab Dis 2018 Sep;41(5):765-776. Epub 2018 Jan 15 doi: 10.1007/s10545-017-0135-2. PMID: 29335813
Heringer J, Valayannopoulos V, Lund AM, Wijburg FA, Freisinger P, Barić I, Baumgartner MR, Burgard P, Burlina AB, Chapman KA, I Saladelafont EC, Karall D, Mühlhausen C, Riches V, Schiff M, Sykut-Cegielska J, Walter JH, Zeman J, Chabrol B, Kölker S; additional individual contributors of the E-IMD consortium
J Inherit Metab Dis 2016 May;39(3):341-353. Epub 2015 Dec 21 doi: 10.1007/s10545-015-9907-8. PMID: 26689403
Scolamiero E, Cozzolino C, Albano L, Ansalone A, Caterino M, Corbo G, di Girolamo MG, Di Stefano C, Durante A, Franzese G, Franzese I, Gallo G, Giliberti P, Ingenito L, Ippolito G, Malamisura B, Mazzeo P, Norma A, Ombrone D, Parenti G, Pellecchia S, Pecce R, Pierucci I, Romanelli R, Rossi A, Siano M, Stoduto T, Villani GR, Andria G, Salvatore F, Frisso G, Ruoppolo M
Mol Biosyst 2015 Jun;11(6):1525-35. doi: 10.1039/c4mb00729h. PMID: 25689098

Curated

American College of Medical Genetics and Genomics, Newborn Screening ACT Sheet, Elevated C5 Acylcarnitine, Isovaleric Acidemia, 2022

American College of Medical Genetics and Genomics, Algorithm, Isovaleric Acidemia: Increased C5 (isolated), 2022

Recent clinical studies

Etiology

Magdy RM, Abd-Elkhalek HS, Bakheet MA, Mohamed MM
Arch Pediatr 2022 Jan;29(1):36-43. Epub 2021 Nov 27 doi: 10.1016/j.arcped.2021.11.002. PMID: 34848132
Yang N, Gong LF, Zhao JQ, Yang HH, Ma ZJ, Liu W, Wan ZH, Kong YY
J Pediatr Endocrinol Metab 2020 May 26;33(5):639-645. doi: 10.1515/jpem-2019-0420. PMID: 32304307
Tuncel AT, Boy N, Morath MA, Hörster F, Mütze U, Kölker S
J Inherit Metab Dis 2018 Sep;41(5):765-776. Epub 2018 Jan 15 doi: 10.1007/s10545-017-0135-2. PMID: 29335813
Ogier de Baulny H, Saudubray JM
Semin Neonatol 2002 Feb;7(1):65-74. doi: 10.1053/siny.2001.0087. PMID: 12069539
Volchenboum SL, Vockley J
J Biol Chem 2000 Mar 17;275(11):7958-63. doi: 10.1074/jbc.275.11.7958. PMID: 10713113

Diagnosis

Tuncel AT, Boy N, Morath MA, Hörster F, Mütze U, Kölker S
J Inherit Metab Dis 2018 Sep;41(5):765-776. Epub 2018 Jan 15 doi: 10.1007/s10545-017-0135-2. PMID: 29335813
Kölker S, Valayannopoulos V, Burlina AB, Sykut-Cegielska J, Wijburg FA, Teles EL, Zeman J, Dionisi-Vici C, Barić I, Karall D, Arnoux JB, Avram P, Baumgartner MR, Blasco-Alonso J, Boy SP, Rasmussen MB, Burgard P, Chabrol B, Chakrapani A, Chapman K, Cortès I Saladelafont E, Couce ML, de Meirleir L, Dobbelaere D, Furlan F, Gleich F, González MJ, Gradowska W, Grünewald S, Honzik T, Hörster F, Ioannou H, Jalan A, Häberle J, Haege G, Langereis E, de Lonlay P, Martinelli D, Matsumoto S, Mühlhausen C, Murphy E, de Baulny HO, Ortez C, Pedrón CC, Pintos-Morell G, Pena-Quintana L, Ramadža DP, Rodrigues E, Scholl-Bürgi S, Sokal E, Summar ML, Thompson N, Vara R, Pinera IV, Walter JH, Williams M, Lund AM, Garcia-Cazorla A
J Inherit Metab Dis 2015 Nov;38(6):1059-74. Epub 2015 Apr 15 doi: 10.1007/s10545-015-9840-x. PMID: 25875216
Moorthie S, Cameron L, Sagoo GS, Bonham JR, Burton H
J Inherit Metab Dis 2014 Nov;37(6):889-98. Epub 2014 Jul 15 doi: 10.1007/s10545-014-9729-0. PMID: 25022222
Ogier de Baulny H, Saudubray JM
Semin Neonatol 2002 Feb;7(1):65-74. doi: 10.1053/siny.2001.0087. PMID: 12069539
Saudubray JM, Nassogne MC, de Lonlay P, Touati G
Semin Neonatol 2002 Feb;7(1):3-15. doi: 10.1053/siny.2001.0083. PMID: 12069534

Therapy

Kose E, Kuyum P, Aksoy B, Häberle J, Arslan N, Ozturk Y
J Clin Pharm Ther 2018 Feb;43(1):124-128. Epub 2017 Jul 25 doi: 10.1111/jcpt.12593. PMID: 28741715
Gokcay G, Baykal T, Gokdemir Y, Demirkol M
J Inherit Metab Dis 2006 Apr-Jun;29(2-3):304-10. doi: 10.1007/s10545-005-0255-y. PMID: 16763892
MacDonald A, Depondt E, Evans S, Daly A, Hendriksz C, Chakrapani A A, Saudubray JM
J Inherit Metab Dis 2006 Apr-Jun;29(2-3):299-303. doi: 10.1007/s10545-006-0332-x. PMID: 16763891
Worthen HG, al Ashwal A, Ozand PT, Garawi S, Rahbeeni Z, al Odaib A, Subramanyam SB, Rashed M
Brain Dev 1994 Nov;16 Suppl:81-5. doi: 10.1016/0387-7604(94)90100-7. PMID: 7726385

Prognosis

Tuncel AT, Boy N, Morath MA, Hörster F, Mütze U, Kölker S
J Inherit Metab Dis 2018 Sep;41(5):765-776. Epub 2018 Jan 15 doi: 10.1007/s10545-017-0135-2. PMID: 29335813
Heringer J, Valayannopoulos V, Lund AM, Wijburg FA, Freisinger P, Barić I, Baumgartner MR, Burgard P, Burlina AB, Chapman KA, I Saladelafont EC, Karall D, Mühlhausen C, Riches V, Schiff M, Sykut-Cegielska J, Walter JH, Zeman J, Chabrol B, Kölker S; additional individual contributors of the E-IMD consortium
J Inherit Metab Dis 2016 May;39(3):341-353. Epub 2015 Dec 21 doi: 10.1007/s10545-015-9907-8. PMID: 26689403
Kölker S, Valayannopoulos V, Burlina AB, Sykut-Cegielska J, Wijburg FA, Teles EL, Zeman J, Dionisi-Vici C, Barić I, Karall D, Arnoux JB, Avram P, Baumgartner MR, Blasco-Alonso J, Boy SP, Rasmussen MB, Burgard P, Chabrol B, Chakrapani A, Chapman K, Cortès I Saladelafont E, Couce ML, de Meirleir L, Dobbelaere D, Furlan F, Gleich F, González MJ, Gradowska W, Grünewald S, Honzik T, Hörster F, Ioannou H, Jalan A, Häberle J, Haege G, Langereis E, de Lonlay P, Martinelli D, Matsumoto S, Mühlhausen C, Murphy E, de Baulny HO, Ortez C, Pedrón CC, Pintos-Morell G, Pena-Quintana L, Ramadža DP, Rodrigues E, Scholl-Bürgi S, Sokal E, Summar ML, Thompson N, Vara R, Pinera IV, Walter JH, Williams M, Lund AM, Garcia-Cazorla A
J Inherit Metab Dis 2015 Nov;38(6):1059-74. Epub 2015 Apr 15 doi: 10.1007/s10545-015-9840-x. PMID: 25875216
Ogier de Baulny H, Saudubray JM
Semin Neonatol 2002 Feb;7(1):65-74. doi: 10.1053/siny.2001.0087. PMID: 12069539
Saudubray JM, Nassogne MC, de Lonlay P, Touati G
Semin Neonatol 2002 Feb;7(1):3-15. doi: 10.1053/siny.2001.0083. PMID: 12069534

Clinical prediction guides

Yang N, Gong LF, Zhao JQ, Yang HH, Ma ZJ, Liu W, Wan ZH, Kong YY
J Pediatr Endocrinol Metab 2020 May 26;33(5):639-645. doi: 10.1515/jpem-2019-0420. PMID: 32304307
Tuncel AT, Boy N, Morath MA, Hörster F, Mütze U, Kölker S
J Inherit Metab Dis 2018 Sep;41(5):765-776. Epub 2018 Jan 15 doi: 10.1007/s10545-017-0135-2. PMID: 29335813
Saudubray JM, Nassogne MC, de Lonlay P, Touati G
Semin Neonatol 2002 Feb;7(1):3-15. doi: 10.1053/siny.2001.0083. PMID: 12069534
Colevas AD, Edwards JL, Hruban RH, Mitchell GA, Valle D, Hutchins GM
Arch Pathol Lab Med 1988 Nov;112(11):1133-9. PMID: 3178428
Dubiel B, Dabrowski C, Wetts R, Tanaka K
J Clin Invest 1983 Nov;72(5):1543-52. doi: 10.1172/JCI111113. PMID: 6630517Free PMC Article

Recent systematic reviews

Moorthie S, Cameron L, Sagoo GS, Bonham JR, Burton H
J Inherit Metab Dis 2014 Nov;37(6):889-98. Epub 2014 Jul 15 doi: 10.1007/s10545-014-9729-0. PMID: 25022222

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • ACMG ACT, 2022
      American College of Medical Genetics and Genomics, Newborn Screening ACT Sheet, Elevated C5 Acylcarnitine, Isovaleric Acidemia, 2022
    • ACMG Algorithm, 2022
      American College of Medical Genetics and Genomics, Algorithm, Isovaleric Acidemia: Increased C5 (isolated), 2022

    Consumer resources