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Elevated urinary 5-hydroxyindoleacetic acid level

MedGen UID:
1054471
Concept ID:
CN377905
Finding
HPO: HP:6000756

Definition

The amount of 5-hydroxyindoleacetic acid (5-HIAA) in the urine, normalized for urine concentration, is above the upper limit of normal. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVElevated urinary 5-hydroxyindoleacetic acid level

Conditions with this feature

Brain dopamine-serotonin vesicular transport disease
MedGen UID:
929215
Concept ID:
C4303546
Disease or Syndrome
An infantile-onset neurometabolic disease with characteristics of dystonia, parkinsonism, nonambulation, autonomic dysfunction, developmental delay and mood disturbances. The prevalence is unknown. It has been described in 8 patients from one Saudi Arabian family to date. Caused by a mutation in the SLC18A2 gene (10q25), encoding the vesicular monoamine transporter 2 (VMAT2) which is responsible for the transport of dopamine and serotonin into synaptic vesicles. Mutations in this gene lead to the impairment of VMAT2 and consequently to problems with motor control, autonomic functioning and mood regulation. It is inherited in an autosomal recessive manner.

Professional guidelines

PubMed

Sebeková K, Revúsová V, Polakovicová D, Drahosová J, Zverková D, Dzúrik R
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Recent clinical studies

Etiology

Pretorius C, Luies L
Metabolomics 2024 Aug 3;20(5):92. doi: 10.1007/s11306-024-02161-8. PMID: 39096437Free PMC Article
Bosak Versic A, Glavan N, Bukvic N, Tomasic Z, Nikolic H
Emerg Med J 2016 Dec;33(12):848-852. Epub 2016 Jul 27 doi: 10.1136/emermed-2015-205559. PMID: 27466348
Mulder EJ, Anderson GM, Kemperman RF, Oosterloo-Duinkerken A, Minderaa RB, Kema IP
Neuropsychobiology 2010;61(1):27-32. Epub 2009 Nov 13 doi: 10.1159/000258640. PMID: 19923863
Beck O, Helander A
Addiction 2003 Dec;98 Suppl 2:63-72. doi: 10.1046/j.1359-6357.2003.00583.x. PMID: 14984243
Minderaa RB, Anderson GM, Volkmar FR, Akkerhuis GW, Cohen DJ
Biol Psychiatry 1987 Aug;22(8):933-40. doi: 10.1016/0006-3223(87)90002-3. PMID: 2440483

Diagnosis

Pretorius C, Luies L
Metabolomics 2024 Aug 3;20(5):92. doi: 10.1007/s11306-024-02161-8. PMID: 39096437Free PMC Article
Bosak Versic A, Glavan N, Bukvic N, Tomasic Z, Nikolic H
Emerg Med J 2016 Dec;33(12):848-852. Epub 2016 Jul 27 doi: 10.1136/emermed-2015-205559. PMID: 27466348
Beck O, Helander A
Addiction 2003 Dec;98 Suppl 2:63-72. doi: 10.1046/j.1359-6357.2003.00583.x. PMID: 14984243
Eriksson B, Oberg K, Stridsberg M
Digestion 2000;62 Suppl 1:33-8. doi: 10.1159/000051853. PMID: 10940685
Minderaa RB, Anderson GM, Volkmar FR, Akkerhuis GW, Cohen DJ
Biol Psychiatry 1987 Aug;22(8):933-40. doi: 10.1016/0006-3223(87)90002-3. PMID: 2440483

Therapy

Gregersen T, Grønbæk H, Worsøe J, Schlageter V, Laurberg S, Krogh K
Scand J Gastroenterol 2011 Jul;46(7-8):895-902. Epub 2011 May 30 doi: 10.3109/00365521.2011.579157. PMID: 21623673
Hernandez R, Jain A, Rosiere L, Henderson SO
Am J Emerg Med 2008 Mar;26(3):282-6. doi: 10.1016/j.ajem.2007.05.012. PMID: 18358937
Joy T, Walsh G, Tokmakejian S, Van Uum SH
Can J Gastroenterol 2008 Jan;22(1):49-53. doi: 10.1155/2008/472159. PMID: 18209781Free PMC Article
Spies CD, Herpell J, Beck O, Müller C, Pragst F, Borg S, Helander A
Alcohol 1999 Jan;17(1):19-27. doi: 10.1016/s0741-8329(98)00028-7. PMID: 9895033
Feldman JM
J Clin Pharmacol 1987 Apr;27(4):288-92. doi: 10.1002/j.1552-4604.1987.tb03015.x. PMID: 3680586

Prognosis

Tsoukalas N, Chatzellis E, Rontogianni D, Alexandraki KI, Boutzios G, Angelousi A, Kaltsas G
Medicine (Baltimore) 2017 Apr;96(16):e6201. doi: 10.1097/MD.0000000000006201. PMID: 28422824Free PMC Article
Bosak Versic A, Glavan N, Bukvic N, Tomasic Z, Nikolic H
Emerg Med J 2016 Dec;33(12):848-852. Epub 2016 Jul 27 doi: 10.1136/emermed-2015-205559. PMID: 27466348
Sen Gupta P, Grozinsky-Glasberg S, Drake WM, Akker SA, Perry L, Grossman AB, Druce MR
Clin Endocrinol (Oxf) 2014 Feb;80(2):246-52. Epub 2013 Jul 29 doi: 10.1111/cen.12270. PMID: 23790044
Hernandez R, Jain A, Rosiere L, Henderson SO
Am J Emerg Med 2008 Mar;26(3):282-6. doi: 10.1016/j.ajem.2007.05.012. PMID: 18358937
Eriksson B, Oberg K, Stridsberg M
Digestion 2000;62 Suppl 1:33-8. doi: 10.1159/000051853. PMID: 10940685

Clinical prediction guides

Bosak Versic A, Glavan N, Bukvic N, Tomasic Z, Nikolic H
Emerg Med J 2016 Dec;33(12):848-852. Epub 2016 Jul 27 doi: 10.1136/emermed-2015-205559. PMID: 27466348
Sen Gupta P, Grozinsky-Glasberg S, Drake WM, Akker SA, Perry L, Grossman AB, Druce MR
Clin Endocrinol (Oxf) 2014 Feb;80(2):246-52. Epub 2013 Jul 29 doi: 10.1111/cen.12270. PMID: 23790044
Chojnacki C, Walecka-Kapica E, Klupińska G, Wachowska-Kelly P, Żylińska K, Winczyk K, Chojnacki J
Pol Arch Med Wewn 2012;122(9):392-7. Epub 2012 Jul 19 PMID: 22814406
Wedekind D, Herchenhein T, Kirchhainer J, Bandelow B, Falkai P, Engel K, Malchow B, Havemann-Reinecke U
J Psychiatr Res 2010 Dec;44(16):1163-9. Epub 2010 Jun 25 doi: 10.1016/j.jpsychires.2010.04.002. PMID: 20579662
Hernandez R, Jain A, Rosiere L, Henderson SO
Am J Emerg Med 2008 Mar;26(3):282-6. doi: 10.1016/j.ajem.2007.05.012. PMID: 18358937

Recent systematic reviews

Joish VN, Shah S, Tierce JC, Patel D, McKee C, Lapuerta P, Zacks J
Future Oncol 2019 Apr;15(12):1397-1406. Epub 2019 Feb 8 doi: 10.2217/fon-2018-0960. PMID: 30734573

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