Congenital intrahepatic portocaval shunt associated with trimethylaminuria

Fern�ndez, M. S.; Guti�rrez, C.; Vila, Juan J.; L�pez, A.; Ib��ez, V.; Sang�esa, C.; Lluna, J; Barrios, J. E.

doi:10.1007/bf01350000

Cited by 20 publications

(12 citation statements)

References 9 publications

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“…The sensitivity of sonography screening for the second type of PSVS should be higher with color or power Doppler sonography than with B-mode sonography, because the former 2 modalities are able to directly visualize the shunting blood flow. 4,5,7,[10][11][12]14 CT 3,11-13,15,16 and MRI 5,16 can also be used to detect PSVS. The main advantage of these two modalities over sonography in the detection of PSVS is the absence of interference caused by pulmonary air, bowel gas, and subcutaneous fat; however, these examinations are much more expensive than sonog- raphy and cannot be applied as a screening test.…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, patients with PSVS should receive adequate dietary management until the shunts spontaneously close or are corrected through surgical intervention. 2,7,11,13 Patients should avoid ingesting extralarge amounts of protein because of the high concentration of ammonia produced from proteins by intestinal bacteria and diverted by the shunts from the portal blood directly to the systemic circulation. The patient with PSVS may be unable to tolerate this extra burden of ammonia and may develop hepatic encephalopathy.…”

Section: Discussionmentioning

confidence: 99%

“…Color Doppler sonography is an effective method of detecting intrahepatic PSVS. 4,5,7,[10][11][12]14 The aim of this prospective study was to use B-mode and color Doppler sonography to assess the incidence and clinical significance of spontaneous intrahepatic PSVS without known potential cause in adults.…”

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confidence: 99%

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Incidence and clinical significance of spontaneous intrahepatic portosystemic venous shunts detected by sonography in adults without potential cause

Lin¹,

Chen²,

Hsieh³

et al. 2005

J of Clinical Ultrasound

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Incidence and clinical significance of spontaneous intrahepatic portosystemic venous shunts detected by sonography in adults without potential cause

Lin¹,

Chen²,

Hsieh³

et al. 2005

J of Clinical Ultrasound

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“…A lthough portosystemic shunts are common in patients with portal hypertension, few reports are available concerning congenital portosystemic shunts in the pediatric population. [1][2][3][4][5][6][7][8] We present the cases of 3 neonates in whom color Doppler sonography in the first month of life showed direct communication between the portal circulation and the hepatic veins. Two of these infants underwent transjugular hepatic venography.…”

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confidence: 99%

Color Doppler sonography in the diagnosis of neonatal intrahepatic portosystemic shunts

Valls

Ceres

Urbaneja

et al. 2000

J. Clin. Ultrasound

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Intrahepatic portosystemic shunts are infrequent in children. We report 3 cases of neonates who presented with jaundice during the first month of life. Color Doppler sonography in the first 2 cases showed direct communication between the right portal and hepatic veins. Both infants were asymptomatic, and the shunts disappeared spontaneously. The third case involved several shunts and an aberrant medial portal vein. This patient developed heart failure and died after surgery. Color Doppler sonography was useful in the diagnosis and follow‐up of the shunts in all 3 cases. © 2000 John Wiley & Sons, Inc. J Clin Ultrasound 28:42–46, 2000.

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“…A case of congenital intrahepatic portal–systemic shunt associated with trimethylaminuria has been reported 12. The abnormal overgrowth of small intestinal bacteria in uraemic patients greatly increases trimethylamine liberation from the precursors in the diet and in association with reduced renal clearance, the trimethylamine levels increase in the circulation, to escape via the breath and sweat 13…”

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confidence: 99%

Fish odour syndrome

Rehman

1999

Postgraduate Medical Journal

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SummaryFish odour syndrome (trimethylaminuria) is a metabolic syndrome caused by abnormal excretion of trimethylamine in the breath, urine, sweat, saliva and vaginal secretions. Trimethylamine is derived from the intestinal bacterial degradation of foods rich in choline and carnitine and is normally oxidised by the liver to odourless trimethylamine N-oxide which is then excreted in the urine. Impaired oxidation of trimethylamine is thought to be the cause of the fish odour syndrome and is responsible for the smell of rotting fish. Certain foods rich in choline exacerbate the condition and the patients have a variety of psychological problems. Recognition of the condition is important as dietary adjustments reduce the excretion of trimethylamine and may reduce the odour. Occasionally, a short course of metronidazole, neomycin and lactulose may suppress production of trimethylamine by reducing the activity of gut microflora.Keywords: fish odour syndrome; trimethylaminuria Humbert and colleagues 1 first described fish odour syndrome (trimethylaminuria) in 1970 in a 6-year-old girl who had the clinical stigmata of Noonan's syndrome (short stature, hypertelorism, ptosis, pulmonary stenosis, skeletal abnormalities and mental retardation) and splenomegaly, with intermittent body odour characteristic of rotting fish. Since then, other cases have been described, showing that trimethylaminuria is not necessarily associated with Noonan's syndrome, as observed in the initial case. It is caused by abnormal excretion of a tertiary aliphatic amine (trimethylamine) in the breath, urine, sweat, saliva and vaginal secretions.2 This amine smells of rotting fish, and is readily detected by human nose at very low concentrations (<1 ppm).3 Trimethylamine has a 100-fold greater olfactory potency than the oxide.Trimethylaminuria was thought to be a rare condition, but evidence suggests that its prevalence is much higher than initially thought. In a study involving 82 Jordanian subjects, eight subjects (9.7%) excreted less than 80% of their total trimethylamine as trimethylamine oxide.4 A similar study showed 1.7% of the Jordanian population, 3.8% of the Ecuadorian population and 11% of the New Guinean population excreted less than 80% of their total trimethylamine as the N-oxide. 5 In another study involving 421 British white volunteers, 16 subjects (3.8%) excreted less than 90% of their total trimethylamine output as N-oxide, of which six subjects (1.4%) excreted less than 80% as N-oxide.

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Congenital intrahepatic portocaval shunt associated with trimethylaminuria

Cited by 20 publications

References 9 publications

Incidence and clinical significance of spontaneous intrahepatic portosystemic venous shunts detected by sonography in adults without potential cause

Incidence and clinical significance of spontaneous intrahepatic portosystemic venous shunts detected by sonography in adults without potential cause

Color Doppler sonography in the diagnosis of neonatal intrahepatic portosystemic shunts

Fish odour syndrome

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