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Peroxisomal disorder

MedGen UID:
129185
Concept ID:
C0282528
Disease or Syndrome
Synonyms: Peroxisomal Disorder; Peroxisomal Disorders
SNOMED CT: Disorder of peroxisomal function (238059005)
 
Monarch Initiative: MONDO:0019053
Orphanet: ORPHA68373

Definition

A group of congenital disorders of lipid metabolism, caused by loss of the normal peroxisomes. Signs and symptoms include developmental delays, mental retardation, facial abnormalities, hepatomegaly, and hypotonia. [from NCI]

Professional guidelines

PubMed

Gupta AO, Raymond G, Pierpont EI, Kemp S, McIvor RS, Rayannavar A, Miller B, Lund TC, Orchard PJ
Expert Opin Biol Ther 2022 Sep;22(9):1151-1162. Epub 2022 Sep 19 doi: 10.1080/14712598.2022.2124857. PMID: 36107226
Eng L, Regelmann MO
Curr Opin Endocrinol Diabetes Obes 2020 Feb;27(1):47-55. doi: 10.1097/MED.0000000000000515. PMID: 31789721
Engelen M, Kemp S, de Visser M, van Geel BM, Wanders RJ, Aubourg P, Poll-The BT
Orphanet J Rare Dis 2012 Aug 13;7:51. doi: 10.1186/1750-1172-7-51. PMID: 22889154Free PMC Article

Recent clinical studies

Etiology

Gupta AO, Raymond G, Pierpont EI, Kemp S, McIvor RS, Rayannavar A, Miller B, Lund TC, Orchard PJ
Expert Opin Biol Ther 2022 Sep;22(9):1151-1162. Epub 2022 Sep 19 doi: 10.1080/14712598.2022.2124857. PMID: 36107226
Acharya SV, Gopal RA, Bandgar TR, Joshi SR, Menon PS, Shah NS
Indian J Pediatr 2009 Oct;76(10):1045-7. Epub 2009 Nov 12 doi: 10.1007/s12098-009-0220-0. PMID: 19907937
Wanders RJ, Schutgens RB, Barth PG
J Neuropathol Exp Neurol 1995 Sep;54(5):726-39. doi: 10.1097/00005072-199509000-00016. PMID: 7545216
Aubourg P
Curr Opin Genet Dev 1994 Jun;4(3):407-11. doi: 10.1016/0959-437x(94)90029-9. PMID: 7919919
Sadeghi-Nejad A, Senior B
N Engl J Med 1990 Jan 4;322(1):13-6. doi: 10.1056/NEJM199001043220103. PMID: 2294415

Diagnosis

Engelen M, Kemp S, de Visser M, van Geel BM, Wanders RJ, Aubourg P, Poll-The BT
Orphanet J Rare Dis 2012 Aug 13;7:51. doi: 10.1186/1750-1172-7-51. PMID: 22889154Free PMC Article
Poll-The BT, Engelen M
Semin Neurol 2012 Feb;32(1):42-50. Epub 2012 Mar 15 doi: 10.1055/s-0032-1306385. PMID: 22422205
Acharya SV, Gopal RA, Bandgar TR, Joshi SR, Menon PS, Shah NS
Indian J Pediatr 2009 Oct;76(10):1045-7. Epub 2009 Nov 12 doi: 10.1007/s12098-009-0220-0. PMID: 19907937
Kallijärvi J, Lehesjoki AE, Lipsanen-Nyman M
Adv Exp Med Biol 2003;544:31-7. doi: 10.1007/978-1-4419-9072-3_3. PMID: 14713209
Wanders RJ, Schutgens RB, Barth PG
J Neuropathol Exp Neurol 1995 Sep;54(5):726-39. doi: 10.1097/00005072-199509000-00016. PMID: 7545216

Therapy

Terluk MR, Tieu J, Sahasrabudhe SA, Moser A, Watkins PA, Raymond GV, Kartha RV
Neurotherapeutics 2022 Apr;19(3):1007-1017. Epub 2022 Apr 4 doi: 10.1007/s13311-022-01226-7. PMID: 35378685Free PMC Article
Jiang MY, Cai YN, Liang CL, Peng MZ, Sheng HY, Fan LP, Lin RZ, Jiang H, Huang Y, Liu L
Metab Brain Dis 2015 Dec;30(6):1439-44. Epub 2015 Aug 12 doi: 10.1007/s11011-015-9717-6. PMID: 26260157
Deschler EK, Sun JK, Silva PS
Semin Ophthalmol 2014 Sep-Nov;29(5-6):290-300. doi: 10.3109/08820538.2014.959198. PMID: 25325854
Noguer MT, Martinez M
Invest Ophthalmol Vis Sci 2010 Apr;51(4):2277-85. Epub 2009 Nov 20 doi: 10.1167/iovs.09-4020. PMID: 19933185
Acharya SV, Gopal RA, Bandgar TR, Joshi SR, Menon PS, Shah NS
Indian J Pediatr 2009 Oct;76(10):1045-7. Epub 2009 Nov 12 doi: 10.1007/s12098-009-0220-0. PMID: 19907937

Prognosis

Engelen M, Kemp S, de Visser M, van Geel BM, Wanders RJ, Aubourg P, Poll-The BT
Orphanet J Rare Dis 2012 Aug 13;7:51. doi: 10.1186/1750-1172-7-51. PMID: 22889154Free PMC Article
Poll-The BT, Engelen M
Semin Neurol 2012 Feb;32(1):42-50. Epub 2012 Mar 15 doi: 10.1055/s-0032-1306385. PMID: 22422205
Orchard PJ, Tolar J
Semin Hematol 2010 Jan;47(1):70-8. doi: 10.1053/j.seminhematol.2009.10.006. PMID: 20109614
Acharya SV, Gopal RA, Bandgar TR, Joshi SR, Menon PS, Shah NS
Indian J Pediatr 2009 Oct;76(10):1045-7. Epub 2009 Nov 12 doi: 10.1007/s12098-009-0220-0. PMID: 19907937
Aubourg P, Mosser J, Douar AM, Sarde CO, Lopez J, Mandel JL
Biochimie 1993;75(3-4):293-302. doi: 10.1016/0300-9084(93)90089-b. PMID: 8507690

Clinical prediction guides

Terluk MR, Tieu J, Sahasrabudhe SA, Moser A, Watkins PA, Raymond GV, Kartha RV
Neurotherapeutics 2022 Apr;19(3):1007-1017. Epub 2022 Apr 4 doi: 10.1007/s13311-022-01226-7. PMID: 35378685Free PMC Article
Deon M, Marchetti DP, Donida B, Wajner M, Vargas C
Cell Mol Neurobiol 2016 May;36(4):497-512. Epub 2015 Jul 14 doi: 10.1007/s10571-015-0234-2. PMID: 26169524Free PMC Article
Kassmann CM
Biochimie 2014 Mar;98:111-8. Epub 2013 Oct 9 doi: 10.1016/j.biochi.2013.09.020. PMID: 24120688
Jagiello P, Hammans C, Wieczorek S, Arning L, Stefanski A, Strehl H, Epplen JT, Gencik M
Hum Mutat 2003 Jun;21(6):630-5. doi: 10.1002/humu.10220. PMID: 12754710
Biermann J, Gootjes J, Wanders RJ, van den Bosch H
IUBMB Life 1999 Dec;48(6):635-9. doi: 10.1080/713803571. PMID: 10683770

Recent systematic reviews

Regelmann MO, Kamboj MK, Miller BS, Nakamoto JM, Sarafoglou K, Shah S, Stanley TL, Marino R; Pediatric Endocrine Society Drug and Therapeutics/Rare Diseases Committee
J Clin Endocrinol Metab 2018 Nov 1;103(11):4324-4331. doi: 10.1210/jc.2018-00920. PMID: 30289543

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