Fish odour syndrome

Li, Min; Al-Sarraf, Ahmad; Sinclair, Graham; Fröhlich, Jiří

doi:10.1503/cmaj.100642

Cited by 13 publications

(24 citation statements)

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“…Further evidence that estrogens have an important role in the control of TMAO metabolism is the metabolic condition Trimethylaminuria or Fish Odour Syndrome which is caused by an inherited defect in the FMO3 enzyme 39 . This condition can be exacerbated around puberty and in females the symptoms worsen before and during menstruation and with the use of oral contraceptives as a result of the hormonal inhibition of the FMO3 enzyme 40 . It is postulated that premenopausal female subjects also have a different choline requirement to males in order to support fetal development 34 particularly at the point where choline turnover links to folate metabolism and with the conversion of homocysteine to methionine (Figure 1).…”

Section: Tmao the Gut Microbiome And Metabolomicsmentioning

confidence: 99%

Does Our Gut Microbiome Predict Cardiovascular Risk?

Griffin

Wang

Stanley

2015

Circ Cardiovasc Genet

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Millions of microbes are found in the human gut, and are collectively referred as the gut microbiota. Recent studies have estimated that the microbiota genome contains 100-fold more genes than the host genome. These microbiota contribute to digestion by processing energy substrates unutilized by the host, with about half of the total genome of the gut microbiota being related to central carbon and amino acid metabolism as well as the biosynthesis of secondary metabolites. Therefore, the gut microbiome and its interaction with the host influences many aspects of health and disease, including influencing the composition of biofluids such as urine and blood plasma. Metabolomics is uniquely suited to capture these functional host-microbe interactions. This review aims at providing an overview of recent metabolomics evidence of gut microbiota-host metabolic interactions with a specific focus on cardiovascular disease and related aspects of the metabolic syndrome. Furthermore, the emphasis is given on the complexities of translating these metabolite signatures as potential clinical biomarkers, as the composition and activity of gut microbiome change with many factors, particularly with diet with special reference to trimethylamine-oxide.

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Section: Tmao the Gut Microbiome And Metabolomicsmentioning

confidence: 99%

Does Our Gut Microbiome Predict Cardiovascular Risk?

Griffin

Wang

Stanley

2015

Circ Cardiovasc Genet

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“…Only one trimethylaminuria patient with hypothyroidism had been reported. 12 In our patient, since clinical features appeared at the same time and both diseases are related to monooxygenase system, we supposed that thyroid hormone synthesis especially in the level of iodine oxidation with thyroid peroxidase might be affected secondary to monooxygenase system. To date, only two trimethylaminuria cases including this case had concomitant hypothyroidism, it is difficult to say that there is a real pathophysiological link.…”

Section: Discussionmentioning

confidence: 67%

Primary trimethylaminuria (fish odor syndrome) and hypothyroidism in an adolescent

Kılıç¹

2017

Turk J Pediatr

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Primary trimethylaminuria (fish-odor syndrome) is a rare autosomal recessive inherited metabolic disorder due to decreased metabolism of trimethylamine by enzyme flavin-containing monooxygenase 3. We report an adolescent boy who was socially distressed with malodor and diagnosed trimethylaminuria by molecular analyses. The patient was previously found to have primary hypothyroidism at another center when he was being investigated for malodor. Our aim is to remind awareness of this rare, socially devastating metabolic disorder to physicians, especially family doctors and specialists such as pediatricians, adolescent doctors, psychiatrists and dermatologists. We also searched for a possible association between trimethylaminuria and hypothyroidism but didn't find out an exact pathophysiological link. Coexistence of primary trimethylaminuria and hypothyroidism might be coincidental. Key words: trimethylaminuria, fish odor syndrome, malodor, FMO3, hypothyroidism.Trimethylaminuria is a rare, socially debilitating metabolic disorder due to decreased metabolism of trimethylamine in liver by enzyme flavincontaining monooxygenase 3 (FMO3) secondary to FMO3 gene defect. 1-4 Affected patients have episodic or persistent unpleasant odor and also their breath, sweat, urine, saliva and other secretions smell characteristic of rotting fish. 5 Hypertension, migraine and other tyramine reactions are also described in these patients. 1,3 Psychiatric counseling is also important as it has strong psychosocial effects. We report one adolescent boy who was socially distressed with malodor and was diagnosed with primary trimethylaminuria. He was previously found to have hypothyroidism at another center when he was being investigated for malodor. We also analyzed if primary trimethylaminuria and primary hypothyroidism is associated or their coexistence is coincidental. Case ReportA twelve-year-old Turkish adolescent boy was admitted to hospital for the malodor of urine and sweat which had been noticed at six years of age. The complaint of patient was increasing by meat consumption. Urine and sweat was in normal color but smelled unpleasant. Parents had distant kinship. He was healthy and had normal diet. There was a history of levothyroxine treatment for hypothyroidism starting at six-years-old and varicocele operation at 11years of age. Hypothyroidism was diagnosed at another center when he was being investigated for malodor. At that time TSH level was 17 µIU/ ml (N: 0.6-5.5), free T4 level was 0.89 ng/dl (0.8-1.9) and levothyroxine 50 µg/day (2 µg/ kg/day) was started. His mother had a goiter and his sister also had hypothyroidism. Since the etiology of malodor was not detected, he was referred to our center. Physical examination findings were normal. Laboratory findings (total blood count, liver and kidney function tests, urine analyses and culture, tandem mass, urine organic acid, blood and urine quantitative amino acid analyses, biotinidase activity, homocysteine, lactate, pyruvate) were unremarkable except for the mild TSH elevati...

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“…Individuals with mutations in this enzyme excrete instead the malodorous trimethylamine, a condition known as fish odour syndrome. 3 The gut bacteria Bacteroides thetaomicron induces production of fucosylated glycoconjugates on the small bowel epithelium, which it can hydrolyse to L-fucose and utilize as an energy source. 4 This organism simultaneously provides benefit to the host by hydrolysing non-digestible plant polysaccharides.…”

Section: The Superorganism: a New Paradigm Of Health And Diseasementioning

confidence: 99%