Back To Search Results

Trimethylaminuria

Editor: Catherine Anastasopoulou Updated: 7/15/2023 11:36:33 AM

Introduction

Trimethylaminuria (TMAU), also known as fish odor syndrome or stale fish syndrome, is a rare metabolic disorder characterized by the abnormal accumulation and excretion of trimethylamine (TMA). The accumulation of TMA results in a strong, offensive odor resembling that of rotting fish.[1] 

The underlying pathogenesis of TMAU is usually a deficient or dysfunctional hepatic enzyme, flavin-containing monooxygenase 3 (FMO3). This enzyme metabolizes TMA into an odorless compound, trimethylamine N-oxide (TMAO).[2] 

TMA has a strong fishy smell and is a pheromone in different organisms.[2] A case report described this condition in 1970, but it is believed that trimethylaminuria had been in existence long before then.[3] 

Etiology

Register For Free And Read The Full Article
Get the answers you need instantly with the StatPearls Clinical Decision Support tool. StatPearls spent the last decade developing the largest and most updated Point-of Care resource ever developed. Earn CME/CE by searching and reading articles.
  • Dropdown arrow Search engine and full access to all medical articles
  • Dropdown arrow 10 free questions in your specialty
  • Dropdown arrow Free CME/CE Activities
  • Dropdown arrow Free daily question in your email
  • Dropdown arrow Save favorite articles to your dashboard
  • Dropdown arrow Emails offering discounts

Learn more about a Subscription to StatPearls Point-of-Care

Etiology

Primary Trimethylaminuria

Primary trimethylaminuria occurs secondary to a genetic mutation of the FMO3 gene, located on chromosome 1q24.3. Various FMO3 mutations result in decreased enzyme activity, impaired substrate binding, or disrupted protein structure.[4] Primary TMAU is predominately inherited in an autosomal recessive manner; both copies of FMO3 need to be mutated for TMAU to manifest. However, some rare cases of TMAU may exhibit partial enzyme deficiency due to heterozygosity, compound heterozygosity, or regulatory mutational variants affecting FMO3 expression.[5]

Failure to metabolize TMA results in its accumulation; it is subsequently excreted via urine, sweat, breath, and other body secretions. The excess excretion is what causes the fishy odor.[6] 

In rare instances, an inborn error of metabolism resulting from a deficiency of the enzyme dimethylglycine dehydrogenase leads to a 100-fold serum and 20-fold urine increase of N,N-dimethylglycine (DMG); these patients also exhibit a fishy odor.[7] 

Secondary Trimethylaminuria

Secondary trimethylaminuria also results in excessive accumulation of TMA. Patients with secondary TMAU have functional enzymes that become overwhelmed due to excess dietary intake of the precursors to the offending chemical. These dietary precursors include choline in eggs, beans, and peas and carnitine in red meats and fish. Some individuals use carnitine as a performance-enhancing supplement.[8][6] Choline is used in the treatment of Alzheimer disease and Huntington disease.

Colonic anaerobic bacteria produce TMA from dietary precursors such as choline, carnitine, and lecithin, which are absorbed into the enterohepatic circulation by simple diffusion.[9] Symptoms of TMAU can develop when the liver enzyme flavin-containing monooxygenase 3 becomes overwhelmed.

Other potential causes of secondary trimethylaminuria are liver failure, portosystemic shunting, menstruation, viral hepatitis, and testosterone therapy.[10]

Epidemiology

The global prevalence of TMAU is estimated at 1 in 200,000 to 1 in 1,000,000 individuals.[11] However, due to the lack of awareness and low diagnosis rates, the true prevalence may be higher, particularly in specific populations. It has been reported that individuals of Ashkenazi Jewish descent may have a higher incidence of TMAU. The carrier rate among individuals of New Guinean origin is 11%.[12] 

TMAU affects both males and females; there may be differences in symptom severity. Some studies have suggested that females may experience more severe symptoms due to hormonal fluctuations during menstruation and pregnancy.[13] While TMAU can present at any age, symptoms often become more noticeable during puberty when hormonal changes occur.[14]

Pathophysiology

Primary TMAU is caused by a dysfunction or deficiency of the flavin-containing monooxygenase 3 (FMO3) enzyme due to mutations in FMO3.[15] The FMO3 enzyme plays a crucial role in the metabolism of TMA, a compound with a fishy odor.

The FMO3 enzyme is primarily expressed in the liver and functions as a monooxygenase enzyme. The principal function of the FMO3 enzyme is to oxidize and convert TMA into trimethylamine N-oxide (TMAO), which is odorless and nontoxic.[16] The dysfunctional or deficient FMO3 enzyme in patients with primary TMAU impairs TMA conversion, resulting in an increased TMA:TMAO ratio.[17][18] The accumulated TMA is passively absorbed into the bloodstream and is released through various excretory routes, including sweat, breath, and urine, giving rise to the distinctive fishy odor associated with TMAU.[19]

The gut microbiota produces TMA by metabolizing certain nitrogen-containing compounds, such as choline and carnitine, in certain foods. In individuals with TMAU, the excessive production of TMA by colonic bacteria further contributes to the accumulation of TMA in the body.[9]

History and Physical

The characteristic presenting features of TMAU are a fishy body odor, halitosis, and malodorous urine. The persistent and often unpredictable nature of the odor associated with TMAU can have significant psychological and social implications.[20] Individuals with TMAU may experience embarrassment, low self-esteem, social isolation, and a negative impact on their overall quality of life. Depression is reported in many individuals with trimethylaminuria.[2]

The hallmark symptom of TMAU is the presence of a persistent and unpleasant body odor similar to rotting fish. This odor may begin at birth or nearer to puberty.[21] Not everyone can smell TMA; some patients have reported being unaware of the smell. The odor arises from the excretion of TMA through sweat, breath, urine, and other bodily secretions.[22] The intensity of the odor can fluctuate and may be influenced by factors such as diet, stress, hormonal changes, and medications. The odor may be described as fishy, musty, or ammonia-like, and it may be particularly pronounced after consuming TMA-rich foods.[23]

In addition to body odor, individuals with TMAU may also experience halitosis or bad breath, resulting from TMA excretion through the breath, leading to an unpleasant smell.[24]

The urine may have a distinct smell reminiscent of rotten fish or ammonia.

Evaluation

Diagnosing TMAU requires clinical evaluation and biochemical and genetic testing. Biochemical laboratory testing may require several steps.

Elevation of urinary TMA levels suggests impaired metabolism and can indicate TMAU.[25] To perform this test, a urine sample is collected and analyzed for TMA using specialized laboratory techniques. Patients with results suggestive of TMAU may undergo a TMA challenge or TMA load test. A TMA challenge test begins with administering a large dose of TMA, which can be administered by eating a 300 g portion of marine fish. A random urine collection within 2 to 12 hours after the TMA load is sampled and the levels of TMA and TMAO are quantified.[16] In patients with deficient or dysfunctional FMO3 activity, urinary TMA levels will be elevated following the challenge.[26]

The enzymatic activity of FMO3 may be quantified directly using blood samples.[27] Reduced or absent FMO3 enzymatic activity supports the diagnosis of TMAU.

Patients with TMAU may undergo genetic testing to identify specific FMO3 mutations. Genetic testing is performed using blood or saliva to detect specific genetic variants associated with TMAU. Genetic testing can confirm the diagnosis, identify the specific genetic mutation, and help determine the inheritance pattern of the disorder.[16]

Treatment / Management

The management of TMAU focuses on minimizing the symptoms and improving the quality of life for affected individuals.[6] While there is currently no cure for TMAU, various approaches can produce symptom management. A specific questionnaire for patients with TMAU has been developed to evaluate treatment efficacy; the tool includes questions about different aspects of health to address all disease consequences.[28] The treatment options for TMAU include dietary modification, antibiotic therapy, activated charcoal administration, modifications to personal hygiene, and psychological support.(B2)

Dietary modifications to reduce the intake of TMA precursors are often recommended. Foods rich in choline, carnitine, and TMAO should be limited or avoided. This may include reducing or eliminating the consumption of certain types of fish, red meats, liver, eggs, legumes, and specific vegetables. Working with a registered dietitian specializing in metabolic disorders can be beneficial in developing an appropriate dietary plan.[29](B3)

In some cases, low-dose antibiotics may be prescribed to reduce the population of TMA-producing bacteria in the gut. Antibiotics like neomycin and metronidazole have been used with varying degrees of success.[30] However, long-term antibiotic use should be carefully monitored due to potential side effects and the risk of antibiotic resistance.

Activated charcoal can absorb and reduce TMA levels in the gut. It may be used as a supplement to help minimize TMA production and absorption. However, its effectiveness can vary among individuals.

Copper chlorophyllin, a chlorophyll derivative, has been used as a supplement to help reduce body odor. Copper chlorophyllin is believed to neutralize odor-causing compounds; its effectiveness in managing TMAU symptoms is still being investigated, and individual responses may vary.

Maintaining good personal hygiene practices, including regular bathing or showering using odor-reducing soaps or washes, can help minimize the odor associated with TMAU.

Trimethylaminuria can have a significant impact on self-esteem and mental well-being. Seeking support from mental health professionals, joining support groups, and engaging in counseling can be valuable in managing the emotional and psychological aspects of living with TMAU.[20] 

A recent study revealed that the endovascular closure treatment resolved TMAU in patients with congenital portosystemic shunts when performed between birth and age 21.[31][32]

Differential Diagnosis

When evaluating a patient with suspected TMAU, it is essential to consider other genetic, systemic, or precise conditions that may present with similar symptoms or overlapping biochemical abnormalities.[33]

Vaginal disorders, such as bacterial vaginosis or trichomoniasis, can cause a fishy odor similar to that of TMAU. These isolated vaginal conditions can be identified through careful examination, including genitourinary examination, pH testing, and microscopic evaluation of vaginal secretions. Nucleic acid amplification testing is also available.

Certain foods, such as seafood, can cause transient body odor that resembles TMAU. However, this type of dietary-related odor is temporary and dissipates once the food is eliminated from the diet. Obtaining a detailed dietary history and evaluating the relationship between food intake and the onset of symptoms can help differentiate between dietary-induced odor and TMAU.

Liver or kidney dysfunction can lead to changes in body odor. Hepatic diseases, such as cirrhosis or hepatic enzyme deficiencies, can result in a fishy or ammonia-like body odor.

Various metabolic disorders can manifest abnormal body odors. For instance, isovaleric acidemia, a rare genetic disorder, can cause a distinctive "sweaty feet" odor. Other metabolic disorders associated with characteristic odors include maple syrup urine disease and phenylketonuria (PKU). Comprehensive metabolic screening tests and targeted investigations can aid in ruling out these conditions.

Hormonal fluctuations, particularly during puberty, menstruation, or pregnancy, can influence body odor. Changes in sweat composition and secretion during these periods can sometimes lead to a fishy odor. Assessing the relationship between hormonal changes and the onset of symptoms can help differentiate hormonal-induced body odor from trimethylaminuria.

Poor personal hygiene practices, such as infrequent bathing or inadequate use of hygiene products, can lead to body odor.

Prognosis

TMAU is a chronic condition, and while there is currently no cure, it is not life-threatening. With proper management and support, many individuals with TMAU can lead fulfilling lives. Regular appointments with healthcare professionals specializing in metabolic disorders, adherence to treatment plans, and maintaining a healthy lifestyle can help individuals manage symptoms and optimize their prognosis. 

Complications

The characteristic fishy odor associated with TMAU can lead to significant social and psychological distress. Individuals with TMAU may experience embarrassment, social isolation, and low self-esteem due to the persistent and often unpredictable nature of the odor.[16] They may also be at risk of anxiety and depression due to social stigma.[2] These psychological and emotional challenges can profoundly impact relationships, employment opportunities, and overall quality of life.

Deterrence and Patient Education

Patient education plays a vital role in TMAU management by empowering individuals with knowledge about their condition and providing them with strategies to cope with its challenges. Several patient education modalities may be employed when caring for patients with TMAU.

Individualized one-on-one counseling sessions with healthcare professionals, such as geneticists, metabolic specialists, or registered dietitians, can provide tailored information about TMAU. These sessions allow for a personalized approach, addressing the specific needs, concerns, and questions of the individual. Individual counseling can cover dietary modifications, hygiene practices, treatment options, and coping strategies.[34]

Group education sessions or support groups can bring together individuals with TMAU to share experiences, learn from one another, and receive information from healthcare professionals. These sessions foster a sense of community, reduce feelings of isolation, and provide a platform for mutual support and encouragement.

The development of mobile applications specifically designed for individuals with TMAU can provide on-the-go access to educational resources, diet trackers, hygiene reminders, and community forums. These interactive applications allow individuals to track their symptoms, monitor their dietary intake, and receive personalized recommendations.

Enhancing Healthcare Team Outcomes

Interprofessional management of TMAU involves a collaborative approach among various healthcare professionals to address the complex challenges associated with this condition. The interprofessional team typically includes primary care practitioners, geneticists, dietitians, psychologists or counselors, and nurses. Together, they aim to provide comprehensive care, education, and support to the patient.[20] 

Geneticists play a crucial role in confirming the diagnosis of TMAU through genetic testing. They can identify specific mutations in FMO3 and provide genetic counseling to affected individuals and their families. 

Primary care practitioners and specialists, such as metabolic disorder specialists, collaborate to monitor symptoms, evaluate treatment effectiveness, address concerns, and make necessary adjustments to the management plan. 

Dietitians or nutritionists are vital in guiding individuals with TMAU to adopt a low-choline diet. They provide personalized dietary recommendations to minimize the intake of choline-rich foods, which are precursors to TMA production. 

Psychologists, counselors, and social workers provide invaluable psychosocial support to individuals with TMAU by addressing emotional challenges, social stigma, and self-esteem and body image issues. Counseling sessions may focus on coping strategies, stress management techniques, and building resilience. Support groups or online communities can also facilitate connections with others facing similar challenges. 

By leveraging the expertise and collaboration of multiple healthcare professionals, the interprofessional management of TMAU aims to provide comprehensive care that addresses the medical, dietary, psychological, and social aspects of the condition. This approach ensures a holistic and patient-centered approach, improving outcomes and quality of life for individuals living with TMAU. 

References


[1]

Loo RL, Chan Q, Nicholson JK, Holmes E. Balancing the Equation: A Natural History of Trimethylamine and Trimethylamine-N-oxide. Journal of proteome research. 2022 Mar 4:21(3):560-589. doi: 10.1021/acs.jproteome.1c00851. Epub 2022 Feb 10     [PubMed PMID: 35142516]


[2]

Messenger J, Clark S, Massick S, Bechtel M. A review of trimethylaminuria: (fish odor syndrome). The Journal of clinical and aesthetic dermatology. 2013 Nov:6(11):45-8     [PubMed PMID: 24307925]


[3]

Humbert JA, Hammond KB, Hathaway WE. Trimethylaminuria: the fish-odour syndrome. Lancet (London, England). 1970 Oct 10:2(7676):770-1     [PubMed PMID: 4195988]


[4]

Antoñanzas J, Querol-Cisneros E, Alkorta-Aranburu G, Patiño-García A, España A. Primary trimethylaminuria syndrome: more than an unpleasant odor. International journal of dermatology. 2023 Mar:62(3):e176-e178. doi: 10.1111/ijd.16401. Epub 2022 Aug 17     [PubMed PMID: 35975805]


[5]

Makiguchi M, Shimizu M, Yokota Y, Shimamura E, Hishinuma E, Saito S, Hiratsuka M, Yamazaki H. Variants of Flavin-Containing Monooxygenase 3 Found in Subjects in an Updated Database of Genome Resources. Drug metabolism and disposition: the biological fate of chemicals. 2023 Jul:51(7):884-891. doi: 10.1124/dmd.123.001310. Epub 2023 Apr 11     [PubMed PMID: 37041084]


[6]

Schmidt AC, Leroux JC. Treatments of trimethylaminuria: where we are and where we might be heading. Drug discovery today. 2020 Sep:25(9):1710-1717. doi: 10.1016/j.drudis.2020.06.026. Epub 2020 Jun 29     [PubMed PMID: 32615074]


[7]

Binzak BA, Vockley JG, Jenkins RB, Vockley J. Structure and analysis of the human dimethylglycine dehydrogenase gene. Molecular genetics and metabolism. 2000 Mar:69(3):181-7     [PubMed PMID: 10767172]


[8]

Fraser-Andrews EA, Manning NJ, Ashton GH, Eldridge P, McGrath J, Menagé Hdu P. Fish odour syndrome with features of both primary and secondary trimethylaminuria. Clinical and experimental dermatology. 2003 Mar:28(2):203-5     [PubMed PMID: 12653714]

Level 3 (low-level) evidence

[9]

Fennema D, Phillips IR, Shephard EA. Trimethylamine and Trimethylamine N-Oxide, a Flavin-Containing Monooxygenase 3 (FMO3)-Mediated Host-Microbiome Metabolic Axis Implicated in Health and Disease. Drug metabolism and disposition: the biological fate of chemicals. 2016 Nov:44(11):1839-1850     [PubMed PMID: 27190056]


[10]

Donato L, Alibrandi S, Scimone C, Castagnetti A, Rao G, Sidoti A, D'Angelo R. Gut-Brain Axis Cross-Talk and Limbic Disorders as Biological Basis of Secondary TMAU. Journal of personalized medicine. 2021 Jan 31:11(2):. doi: 10.3390/jpm11020087. Epub 2021 Jan 31     [PubMed PMID: 33572540]


[11]

Wise PM, Eades J, Tjoa S, Fennessey PV, Preti G. Individuals reporting idiopathic malodor production: demographics and incidence of trimethylaminuria. The American journal of medicine. 2011 Nov:124(11):1058-63. doi: 10.1016/j.amjmed.2011.05.030. Epub 2011 Aug 16     [PubMed PMID: 21851918]

Level 2 (mid-level) evidence

[12]

Mitchell SC, Zhang AQ, Barrett T, Ayesh R, Smith RL. Studies on the discontinuous N-oxidation of trimethylamine among Jordanian, Ecuadorian and New Guinean populations. Pharmacogenetics. 1997 Feb:7(1):45-50     [PubMed PMID: 9110361]


[13]

Shimizu M, Cashman JR, Yamazaki H. Transient trimethylaminuria related to menstruation. BMC medical genetics. 2007 Jan 27:8():2     [PubMed PMID: 17257434]

Level 3 (low-level) evidence

[14]

Doyle S, O'Byrne JJ, Nesbitt M, Murphy DN, Abidin Z, Byrne N, Pastores G, Kirk R, Treacy EP. The genetic and biochemical basis of trimethylaminuria in an Irish cohort. JIMD reports. 2019 May:47(1):35-40. doi: 10.1002/jmd2.12028. Epub 2019 Mar 25     [PubMed PMID: 31240165]


[15]

Shimizu M, Yamamoto A, Makiguchi M, Shimamura E, Yokota Y, Harano M, Yamazaki H. A family study of compound variants of flavin-containing monooxygenase 3 (FMO3) in Japanese subjects found by urinary phenotyping for trimethylaminuria. Drug metabolism and pharmacokinetics. 2023 Jun:50():100490. doi: 10.1016/j.dmpk.2023.100490. Epub 2023 Jan 9     [PubMed PMID: 36889044]


[16]

Mackay RJ, McEntyre CJ, Henderson C, Lever M, George PM. Trimethylaminuria: causes and diagnosis of a socially distressing condition. The Clinical biochemist. Reviews. 2011 Feb:32(1):33-43     [PubMed PMID: 21451776]


[17]

Murphy HC, Dolphin CT, Janmohamed A, Holmes HC, Michelakakis H, Shephard EA, Chalmers RA, Phillips IR, Iles RA. A novel mutation in the flavin-containing monooxygenase 3 gene, FM03, that causes fish-odour syndrome: activity of the mutant enzyme assessed by proton NMR spectroscopy. Pharmacogenetics. 2000 Jul:10(5):439-51     [PubMed PMID: 10898113]

Level 3 (low-level) evidence

[18]

Hernangómez Vázquez S, González González C, Lancho Monreal EM, Alonso Cristobo ME, Mallol Poyato MJ, García-Vao Bel CM. [Trimethylaminuria: three different mutations in a single family]. Nutricion hospitalaria. 2019 Apr 10:36(2):492-495. doi: 10.20960/nh.2342. Epub     [PubMed PMID: 30864455]


[19]

Krueger ES, Lloyd TS, Tessem JS. The Accumulation and Molecular Effects of Trimethylamine N-Oxide on Metabolic Tissues: It's Not All Bad. Nutrients. 2021 Aug 21:13(8):. doi: 10.3390/nu13082873. Epub 2021 Aug 21     [PubMed PMID: 34445033]


[20]

Roddy D, McCarthy P, Nerney D, Mulligan-Rabbitt J, Smith E, Treacy EP. Impact of trimethylaminuria on daily psychosocial functioning. JIMD reports. 2021 Jan:57(1):67-75. doi: 10.1002/jmd2.12170. Epub 2020 Oct 6     [PubMed PMID: 33473342]


[21]

Pardini RS, Sapien RE. Trimethylaminuria (fish odor syndrome) related to the choline concentration of infant formula. Pediatric emergency care. 2003 Apr:19(2):101-3     [PubMed PMID: 12698036]


[22]

Kloster I, Erichsen MM. Trimethylaminuria. Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke. 2021 Sep 28:141():. doi: 10.4045/tidsskr.21.0142. Epub 2021 Sep 10     [PubMed PMID: 34597008]


[23]

Haugaard LK, Lund AM, Patursson P, Christensen E. [Fish odour--could be a sign of trimethylaminuria]. Ugeskrift for laeger. 2010 Nov 22:172(47):3268-9     [PubMed PMID: 21092725]

Level 3 (low-level) evidence

[24]

Mitchell SC. Trimethylaminuria (fish-odour syndrome) and oral malodour. Oral diseases. 2005:11 Suppl 1():10-3     [PubMed PMID: 15752091]


[25]

Bouchemal N, Ouss L, Brassier A, Barbier V, Gobin S, Hubert L, de Lonlay P, Le Moyec L. Diagnosis and phenotypic assessment of trimethylaminuria, and its treatment with riboflavin: (1)H NMR spectroscopy and genetic testing. Orphanet journal of rare diseases. 2019 Sep 18:14(1):222. doi: 10.1186/s13023-019-1174-6. Epub 2019 Sep 18     [PubMed PMID: 31533761]


[26]

Chalmers RA, Bain MD, Michelakakis H, Zschocke J, Iles RA. Diagnosis and management of trimethylaminuria (FMO3 deficiency) in children. Journal of inherited metabolic disease. 2006 Feb:29(1):162-72     [PubMed PMID: 16601883]

Level 2 (mid-level) evidence

[27]

Dolphin CT, Janmohamed A, Smith RL, Shephard EA, Phillips IR. Missense mutation in flavin-containing mono-oxygenase 3 gene, FMO3, underlies fish-odour syndrome. Nature genetics. 1997 Dec:17(4):491-4     [PubMed PMID: 9398858]

Level 3 (low-level) evidence

[28]

Rutkowski K, Rahman Y, Halter M. Development and feasibility of the use of an assessment tool measuring treatment efficacy in patients with trimethylaminuria: A mixed methods study. Journal of inherited metabolic disease. 2019 Mar:42(2):362-370. doi: 10.1002/jimd.12023. Epub 2019 Feb 7     [PubMed PMID: 30734325]

Level 2 (mid-level) evidence

[29]

Li M, Al-Sarraf A, Sinclair G, Frohlich J. Fish odour syndrome. CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne. 2011 May 17:183(8):929-31. doi: 10.1503/cmaj.100642. Epub 2011 Mar 21     [PubMed PMID: 21422137]

Level 3 (low-level) evidence

[30]

Rehman HU. Fish odor syndrome. Postgraduate medical journal. 1999 Aug:75(886):451-2     [PubMed PMID: 10646019]


[31]

Ponce-Dorrego MD, Hernández-Cabrero T, Garzón-Moll G. Endovascular Treatment of Congenital Portosystemic Shunt: A Single-Center Prospective Study. Pediatric gastroenterology, hepatology & nutrition. 2022 Mar:25(2):147-162. doi: 10.5223/pghn.2022.25.2.147. Epub 2022 Mar 10     [PubMed PMID: 35360378]


[32]

Ponce-Dorrego MD, Garzón-Moll G. Endovascular Closure Resolves Trimethylaminuria Caused by Congenital Portosystemic Shunts. Pediatric gastroenterology, hepatology & nutrition. 2019 Nov:22(6):588-593. doi: 10.5223/pghn.2019.22.6.588. Epub 2019 Nov 7     [PubMed PMID: 31777726]


[33]

Hur E, Gungor O, Bozkurt D, Bozgul S, Dusunur F, Caliskan H, Berdeli A, Akcicek F, Basci A, Duman S. Trimethylaminuria (fish malodour syndrome) in chronic renal failure. Hippokratia. 2012 Jan:16(1):83-5     [PubMed PMID: 23930066]


[34]

Khan SA, Shagufta K. A rare case of fish odor syndrome presenting as depression. Indian journal of psychiatry. 2014 Apr:56(2):185-7. doi: 10.4103/0019-5545.130505. Epub     [PubMed PMID: 24891709]

Level 3 (low-level) evidence