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Fish odor

MedGen UID:
1387060
•Concept ID:
C4477064
•
Sign or Symptom
Synonyms: Fishy body odor; Fishy body odour; Fishy odor; Fishy odour
 
HPO: HP:0410020

Definition

Body odor characterized by an offensive body odor and the smell of rotting fish due to the excessive excretion of trimethylamine (TMA) in the urine, sweat, and breath of affected individuals. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFish odor

Conditions with this feature

Dimethylglycine dehydrogenase deficiency
MedGen UID:
343006
•Concept ID:
C1853892
•
Disease or Syndrome
Dimethylglycine dehydrogenase deficiency (DMGDHD) is an inborn error of metabolism characterized by a fish-like odor, chronic fatigue, and increased level of the muscle form of creatine kinase in serum (Moolenaar et al., 1999).
See: Condition Record
Dimethylglycine dehydrogenase deficiency

Professional guidelines

PubMed

Trimethylaminuria (fish odor syndrome): genotype characterization among Portuguese patients.
Ferreira F, Esteves S, Almeida LS, Gaspar A, da Costa CD, Janeiro P, Bandeira A, Martins E, Teles EL, Garcia P, Azevedo L, Vilarinho L
Gene 2013 Sep 15;527(1):366-70. Epub 2013 Jun 17 doi: 10.1016/j.gene.2013.05.025. PMID: 23791655

Recent clinical studies

Etiology

Choline-related-inherited metabolic diseases-A mini review.
Wortmann SB, Mayr JA
J Inherit Metab Dis 2019 Mar;42(2):237-242. Epub 2019 Jan 25 doi: 10.1002/jimd.12011. PMID: 30681159Free PMC Article
Novel variants and haplotypes of human flavin-containing monooxygenase 3 gene associated with Japanese subjects suffering from trimethylaminuria.
Shimizu M, Yoda H, Igarashi N, Makino M, Tokuyama E, Yamazaki H
Xenobiotica 2019 Oct;49(10):1244-1250. Epub 2018 Nov 29 doi: 10.1080/00498254.2018.1539279. PMID: 30351217
When preschoolers follow their eyes and older children follow their noses: visuo-olfactory social affective matching in childhood.
Cavazzana A, Wesarg C, Parish-Morris J, Lundström JN, Parma V
Dev Sci 2018 Jan;21(1) Epub 2016 Nov 17 doi: 10.1111/desc.12507. PMID: 27859959
Archaebiotics: proposed therapeutic use of archaea to prevent trimethylaminuria and cardiovascular disease.
Brugère JF, Borrel G, Gaci N, Tottey W, O'Toole PW, Malpuech-Brugère C
Gut Microbes 2014 Jan-Feb;5(1):5-10. Epub 2013 Oct 31 doi: 10.4161/gmic.26749. PMID: 24247281Free PMC Article
Low-dose secnidazole in the treatment of bacterial vaginosis.
Núñez JT, Gómez G
Int J Gynaecol Obstet 2005 Mar;88(3):281-5. Epub 2005 Jan 20 doi: 10.1016/j.ijgo.2004.11.028. PMID: 15733882

Diagnosis

Quality consideration for the validation of urine TMA and TMAO measurement by nuclear magnetic resonance spectroscopy in Fish Odor Syndrome.
Veyrat-Durebex C, Benz-de-Bretagne I, Clavier V, Bruno C, Andres CR, Antar C, Hennart B, Maillot F, Nadal-Desbarats L, Blasco H
Anal Biochem 2021 Oct 1;630:114330. Epub 2021 Aug 5 doi: 10.1016/j.ab.2021.114330. PMID: 34364856
Primary trimethylaminuria (fish odor syndrome) and hypothyroidism in an adolescent.
Kılıç M
Turk J Pediatr 2017;59(5):614-616. doi: 10.24953/turkjped.2017.05.020. PMID: 29745129
How Many and Which Odor Identification Items Are Needed to Establish Normal Olfactory Function?
Lötsch J, Ultsch A, Hummel T
Chem Senses 2016 May;41(4):339-44. Epub 2016 Feb 8 doi: 10.1093/chemse/bjw006. PMID: 26857742
Trimethylaminuria: the fish malodor syndrome.
Mitchell SC, Smith RL
Drug Metab Dispos 2001 Apr;29(4 Pt 2):517-21. PMID: 11259343
Fish-odor syndrome: an olfactory diagnosis.
Ruocco V, Florio M
Int J Dermatol 1995 Feb;34(2):92-3. doi: 10.1111/j.1365-4362.1995.tb03585.x. PMID: 7737783

Therapy

Primary trimethylaminuria (fish odor syndrome) and hypothyroidism in an adolescent.
Kılıç M
Turk J Pediatr 2017;59(5):614-616. doi: 10.24953/turkjped.2017.05.020. PMID: 29745129
Archaebiotics: proposed therapeutic use of archaea to prevent trimethylaminuria and cardiovascular disease.
Brugère JF, Borrel G, Gaci N, Tottey W, O'Toole PW, Malpuech-Brugère C
Gut Microbes 2014 Jan-Feb;5(1):5-10. Epub 2013 Oct 31 doi: 10.4161/gmic.26749. PMID: 24247281Free PMC Article
Diet in dermatology: revisited.
Kaimal S, Thappa DM
Indian J Dermatol Venereol Leprol 2010 Mar-Apr;76(2):103-15. doi: 10.4103/0378-6323.60540. PMID: 20228538
Complications of chronic use of skin lightening cosmetics.
Olumide YM, Akinkugbe AO, Altraide D, Mohammed T, Ahamefule N, Ayanlowo S, Onyekonwu C, Essen N
Int J Dermatol 2008 Apr;47(4):344-53. doi: 10.1111/j.1365-4632.2008.02719.x. PMID: 18377596
The fish-odor syndrome.
Mitchell SC
Perspect Biol Med 1996 Summer;39(4):514-26. doi: 10.1353/pbm.1996.0003. PMID: 8753757

Prognosis

Assessment of odor perception related to stimulation modes in a mock MRI scanner.
Zang Y, Chen B, Hummel T
J Neurosci Methods 2020 Jul 15;341:108754. Epub 2020 May 4 doi: 10.1016/j.jneumeth.2020.108754. PMID: 32380225
When preschoolers follow their eyes and older children follow their noses: visuo-olfactory social affective matching in childhood.
Cavazzana A, Wesarg C, Parish-Morris J, Lundström JN, Parma V
Dev Sci 2018 Jan;21(1) Epub 2016 Nov 17 doi: 10.1111/desc.12507. PMID: 27859959
How Many and Which Odor Identification Items Are Needed to Establish Normal Olfactory Function?
Lötsch J, Ultsch A, Hummel T
Chem Senses 2016 May;41(4):339-44. Epub 2016 Feb 8 doi: 10.1093/chemse/bjw006. PMID: 26857742
What's that smell? An ecological approach to understanding preferences for familiar odors.
Schloss KB, Goldberger CS, Palmer SE, Levitan CA
Perception 2015;44(1):23-38. doi: 10.1068/p7807. PMID: 26489214
Fish odor syndrome.
Rehman HU
Postgrad Med J 1999 Aug;75(886):451-2. doi: 10.1136/pgmj.75.886.451. PMID: 10646019Free PMC Article

Clinical prediction guides

Investigation of sensory profiles and hedonic drivers of emerging aquaculture fish species.
Alexi N, Byrne DV, Nanou E, Grigorakis K
J Sci Food Agric 2018 Feb;98(3):1179-1187. Epub 2017 Sep 14 doi: 10.1002/jsfa.8571. PMID: 28737277
How Many and Which Odor Identification Items Are Needed to Establish Normal Olfactory Function?
Lötsch J, Ultsch A, Hummel T
Chem Senses 2016 May;41(4):339-44. Epub 2016 Feb 8 doi: 10.1093/chemse/bjw006. PMID: 26857742
What's that smell? An ecological approach to understanding preferences for familiar odors.
Schloss KB, Goldberger CS, Palmer SE, Levitan CA
Perception 2015;44(1):23-38. doi: 10.1068/p7807. PMID: 26489214
Trimethylaminuria (fish odor syndrome): genotype characterization among Portuguese patients.
Ferreira F, Esteves S, Almeida LS, Gaspar A, da Costa CD, Janeiro P, Bandeira A, Martins E, Teles EL, Garcia P, Azevedo L, Vilarinho L
Gene 2013 Sep 15;527(1):366-70. Epub 2013 Jun 17 doi: 10.1016/j.gene.2013.05.025. PMID: 23791655
Transient trimethylaminuria related to menstruation.
Shimizu M, Cashman JR, Yamazaki H
BMC Med Genet 2007 Jan 27;8:2. doi: 10.1186/1471-2350-8-2. PMID: 17257434Free PMC Article

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