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Elevated urinary homovanillic acid

MedGen UID:
866486
Concept ID:
C4020736
Finding
Synonym: Increased urinary homovanillic acid
 
HPO: HP:0011977

Definition

An increased concentration of homovanillic acid in the urine. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVElevated urinary homovanillic acid

Conditions with this feature

Neuroblastoma, susceptibility to, 1
MedGen UID:
412713
Concept ID:
C2749485
Finding
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

Alvarado CS, Faraj BA, Kim TH, Camp VM, Bain RP, Ragab AH
Am J Pediatr Hematol Oncol 1985 Fall;7(3):221-7. doi: 10.1097/00043426-198507030-00002. PMID: 3934994

Recent clinical studies

Etiology

Peitzsch M, Butch ER, Lovorn E, Mangelis A, Furman WL, Santana VM, Hero B, Berthold F, Shulkin BL, Huebner A, Eisenhofer G
Pediatr Blood Cancer 2020 Feb;67(2):e28081. Epub 2019 Nov 14 doi: 10.1002/pbc.28081. PMID: 31724812
Brun L, Ngu LH, Keng WT, Ch'ng GS, Choy YS, Hwu WL, Lee WT, Willemsen MA, Verbeek MM, Wassenberg T, Régal L, Orcesi S, Tonduti D, Accorsi P, Testard H, Abdenur JE, Tay S, Allen GF, Heales S, Kern I, Kato M, Burlina A, Manegold C, Hoffmann GF, Blau N
Neurology 2010 Jul 6;75(1):64-71. Epub 2010 May 26 doi: 10.1212/WNL.0b013e3181e620ae. PMID: 20505134
Davis JM, Koslow SH, Gibbons RD, Maas JW, Bowden CL, Casper R, Hanin I, Javaid JI, Chang SS, Stokes PE
Arch Gen Psychiatry 1988 Aug;45(8):705-17. doi: 10.1001/archpsyc.1988.01800320015002. PMID: 2456047
Couch MW, Greer DM, Williams CM
Clin Chim Acta 1986 Jul 15;158(1):109-14. doi: 10.1016/0009-8981(86)90121-x. PMID: 3091293
Davis KL, Hollister LE, Mathé AA, Davis BM, Rothpearl AB, Faull KF, Hsieh JY, Barchas JD, Berger PA
Am J Psychiatry 1981 Dec;138(12):1555-62. doi: 10.1176/ajp.138.12.1555. PMID: 6171169

Diagnosis

Fauler G, Leis HJ, Huber E, Schellauf C, Kerbl R, Urban C, Gleispach H
J Mass Spectrom 1997 May;32(5):507-14. doi: 10.1002/(SICI)1096-9888(199705)32:5<507::AID-JMS503>3.0.CO;2-9. PMID: 9180052
Croitoru DP, Sinsky AB, Laberge JM
J Pediatr Surg 1992 Oct;27(10):1320-1. doi: 10.1016/0022-3468(92)90286-g. PMID: 1403512
Tuchman M, Auray-Blais C, Ramnaraine ML, Neglia J, Krivit W, Lemieux B
Clin Biochem 1987 Jun;20(3):173-7. doi: 10.1016/s0009-9120(87)80116-9. PMID: 3652438
Rajnherc JR, van Gennip AH, Abeling NG, van der Zee JM, Voûte PA
Med Pediatr Oncol 1984;12(2):81-4. doi: 10.1002/mpo.2950120203. PMID: 6422219
LaBrosse EH, Comoy E, Bohuon C, Zucker JM, Schweisguth O
J Natl Cancer Inst 1976 Sep;57(3):633-8. doi: 10.1093/jnci/57.3.633. PMID: 10450

Therapy

Tuchman M, Ramnaraine ML, Woods WG, Krivit W
Pediatrics 1987 Feb;79(2):203-5. PMID: 3808793
Feldman JM
J Clin Pharmacol 1987 Apr;27(4):288-92. doi: 10.1002/j.1552-4604.1987.tb03015.x. PMID: 3680586
Tuchman M, Auray-Blais C, Ramnaraine ML, Neglia J, Krivit W, Lemieux B
Clin Biochem 1987 Jun;20(3):173-7. doi: 10.1016/s0009-9120(87)80116-9. PMID: 3652438
Martin PR, Ebert MH, Gordon EK, Weingartner H, Kopin IJ
Psychopharmacology (Berl) 1984;84(1):58-63. doi: 10.1007/BF00432025. PMID: 6436892
Davis KL, Hollister LE, Mathé AA, Davis BM, Rothpearl AB, Faull KF, Hsieh JY, Barchas JD, Berger PA
Am J Psychiatry 1981 Dec;138(12):1555-62. doi: 10.1176/ajp.138.12.1555. PMID: 6171169

Prognosis

Peitzsch M, Butch ER, Lovorn E, Mangelis A, Furman WL, Santana VM, Hero B, Berthold F, Shulkin BL, Huebner A, Eisenhofer G
Pediatr Blood Cancer 2020 Feb;67(2):e28081. Epub 2019 Nov 14 doi: 10.1002/pbc.28081. PMID: 31724812
Brun L, Ngu LH, Keng WT, Ch'ng GS, Choy YS, Hwu WL, Lee WT, Willemsen MA, Verbeek MM, Wassenberg T, Régal L, Orcesi S, Tonduti D, Accorsi P, Testard H, Abdenur JE, Tay S, Allen GF, Heales S, Kern I, Kato M, Burlina A, Manegold C, Hoffmann GF, Blau N
Neurology 2010 Jul 6;75(1):64-71. Epub 2010 May 26 doi: 10.1212/WNL.0b013e3181e620ae. PMID: 20505134
Rajnherc JR, van Gennip AH, Abeling NG, van der Zee JM, Voûte PA
Med Pediatr Oncol 1984;12(2):81-4. doi: 10.1002/mpo.2950120203. PMID: 6422219
Domino EF
Schizophr Bull 1980;6(2):238-44. doi: 10.1093/schbul/6.2.238. PMID: 6154967
LaBrosse EH, Comoy E, Bohuon C, Zucker JM, Schweisguth O
J Natl Cancer Inst 1976 Sep;57(3):633-8. doi: 10.1093/jnci/57.3.633. PMID: 10450

Clinical prediction guides

Liu R, Hao J, Zhao X, Lai Y
Drug Metab Dispos 2023 Jul;51(7):844-850. Epub 2023 Apr 14 doi: 10.1124/dmd.123.001277. PMID: 37059471
McKenna KF, Baker GB, Coutts RT
Neurochem Res 1993 Sep;18(9):1023-7. doi: 10.1007/BF00966763. PMID: 8232718
Alvarado CS, Faraj BA, Kim TH, Camp VM, Bain RP, Ragab AH
Am J Pediatr Hematol Oncol 1985 Fall;7(3):221-7. doi: 10.1097/00043426-198507030-00002. PMID: 3934994
Davis KL, Hollister LE, Mathé AA, Davis BM, Rothpearl AB, Faull KF, Hsieh JY, Barchas JD, Berger PA
Am J Psychiatry 1981 Dec;138(12):1555-62. doi: 10.1176/ajp.138.12.1555. PMID: 6171169
Domino EF
Schizophr Bull 1980;6(2):238-44. doi: 10.1093/schbul/6.2.238. PMID: 6154967

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