Oral glutamine challenge and magnetic resonance spectroscopy in three patients with congenital portosystemic shunts

Ortiz, M.; Córdoba, Juan; Alonso, Juli; Quiroga, Sergi; Jacas, Carlos; Esteban, Rafael; Guardia, J.

doi:10.1016/j.jhep.2004.01.013

Cited by 37 publications

(22 citation statements)

References 28 publications

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“…25 The latter patients may have neurological abnormalities as well as high ammonia levels and MR spectroscopy findings (high glutamate/glutamine pick and low myoinositol pick) similar to those occurring in patients with cirrhosis with HE. 2,26 A large shunt therefore may be the cause of HE even in the absence of significant liver damage. This type of encephalopathy is currently classified as type B (from the word "bypass") HE.…”

Section: Discussionmentioning

confidence: 99%

High prevalence of spontaneous portal-systemic shunts in persistent hepatic encephalopathy: A case-control study

et al. 2005

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Large spontaneous portal-systemic shunts have been occasionally described in patients with cirrhosis. This study was undertaken to assess the prevalence of portal-systemic shunts in patients with cirrhosis with recurrent or persistent hepatic encephalopathy (HE) as compared with patients with cirrhosis without HE. Fourteen patients with cirrhosis with recurrent or persistent HE (cases) and 14 patients with cirrhosis without previous or present signs of overt HE matching for age and degree of liver failure (controls) were studied. Each patient underwent neurological assessment and cerebral magnetic resonance (MR) imaging to exclude organic neurological pathological conditions. HE evaluation included psychometric performance (Trail-Making Test A), electroencephalogram (EEG), mental status examination and grading, arterial, venous, and partial pressure of ammonia determination. The presence of portal-systemic shunts was assessed by portal venous phase multidetector-row spiral computed tomography (CT). Large spontaneous portal-systemic shunts were detected in 10 patients with HE and in only 2 patients without HE (71% vs. 14%; chi square ‫؍‬ 9.16; df ‫؍‬ 1.0; P ‫؍‬ .002). The patients with HE presented ascites (P ‫؍‬ .002) and medium/large esophageal varices (P ‫؍‬ .02) less frequently than the control group. In conclusion, our study suggests that large spontaneous shunts may often sustain the chronicity of HE; the presence of large shunts should be sought in patients with cirrhosis with recurrent or persistent HE.

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Section: Discussionmentioning

confidence: 99%

High prevalence of spontaneous portal-systemic shunts in persistent hepatic encephalopathy: A case-control study

et al. 2005

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“…43,44 This manganese-related MR imaging signal-intensity abnormality has also been described in patients without cirrhosis, such as those receiving total parenteral nutrition, [45][46][47][48] those with occupational exposure to manganese from welding, 49 and those with noncirrhotic portal vein thrombosis or congenital portal-systemic bypass and no intrinsic hepatocellular disease. [50][51][52] In all these situations, the MR imaging signal-intensity changes resolve after discontinuation of manganese intake. [45][46][47] Similar findings were observed in a patient with Alagille syndrome, 53 an autosomal dominant disorder characterized by cholestasis, intrahepatic bile duct paucity, end stage liver disease, and elevated blood manganese.…”

Section: Mr Imaging Marker Of Manganese Accumulation Within the Cnsmentioning

confidence: 99%

MR Imaging Findings in Hepatic Encephalopathy

Rovira¹,

Alonso²,

Córdoba³

2008

AJNR Am J Neuroradiol

299

218

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SUMMARY:The term hepatic encephalopathy (HE) includes a spectrum of neuropsychiatric abnormalities occurring in patients with liver dysfunction. Most cases are associated with cirrhosis and portal hypertension or portal-systemic shunts, but the condition can also be seen in patients with acute liver failure and, rarely, with portal-systemic bypass and no associated intrinsic hepatocellular disease. Although HE is a clinical condition, several neuroimaging techniques, particularly MR imaging, may eventually be useful for the diagnosis because they can identify and measure the consequences of central nervous system (CNS) increase in substances that under normal circumstances, are efficiently metabolized by the liver. Classic MR imaging abnormalities include high signal intensity in the globus pallidum on T1-weighted images, likely a reflection of increased tissue concentrations of manganese, and an elevated glutamine/glutamate peak coupled with decreased myo-inositol and choline signals on proton MR spectroscopy, representing disturbances in cell-volume homeostasis secondary to brain hyperammonemia. Recent data have shown that white matter abnormalities, also related to increased CNS ammonia concentration, can also be detected with several MR imaging techniques such as magnetization transfer ratio measurements, fast fluid-attenuated inversion recovery sequences, and diffusion-weighted images. All these MR imaging abnormalities, which return to normal with restoration of liver function, probably reflect the presence of mild diffuse brain edema, which seems to play an essential role in the pathogenesis of HE. It is likely that MR imaging will be increasingly used to evaluate the mechanisms involved in the pathogenesis of HE and to assess the effects of therapeutic measures focused on correcting brain edema in these patients. Hepatic encephalopathy (HE) reflects a spectrum of neuropsychiatric abnormalities occurring in patients with liver dysfunction. Most cases are associated with cirrhosis and portal hypertension or portal-systemic shunts, but the condition can also be seen in patients with acute liver failure and, rarely, with portal-systemic bypass and no associated intrinsic hepatocellular disease.1,2 The most common clinical pattern in these patients is the development of confusion or coma precipitated by gastrointestinal bleeding, acute superimposed hepatitis, or concomitant infection in a previously asymptomatic patient with cirrhosis. The neurologic manifestations are mainly due to shunt of blood arising from the portal venous bed into the systemic circulation and are reversible once the liver function abnormality or precipitating factor has been corrected. HE can be classified according to the underlying liver disease and the evolution of the neurologic manifestations (Table 1). Clinical FeaturesClinically, HE manifests as a neuropsychiatric syndrome encompassing a wide spectrum of mental and motor disorders.2,3 The changes in mental status range from subtle cognitive dysfunction to severe coma, whereas the...

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“…4 Hyperammonemia and hepatic encephalopathy secondary to portosystemic shunt surgery, despite normal hepatocellular functions, have been demonstrated in patients with congenital portosystemic shunt surgery. 10 Brain MR imaging, 1 H-MR spectroscopy, diffusion and DTI, magnetization transfer, and functional MR imaging have been used to understand the pathophysiological alterations in patients with cirrhosis-induced hepatic encephalopathy. 11,12 A classical triad of increased resonance of Glx along with decreased myo-inositol and choline are considered to be the hall-mark of hepatic dysfunction with hepatic encephalopathy in patients with liver cirrhosis on 1 H-MR spectroscopy.…”

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confidence: 99%

Brain MR Imaging and¹H-MR Spectroscopy Changes in Patients with Extrahepatic Portal Vein Obstruction from Early Childhood to Adulthood

Yadav¹,

Saksena²,

Srivastava³

et al. 2010

AJNR Am J Neuroradiol

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Oral glutamine challenge and magnetic resonance spectroscopy in three patients with congenital portosystemic shunts

Cited by 37 publications

References 28 publications

High prevalence of spontaneous portal-systemic shunts in persistent hepatic encephalopathy: A case-control study

High prevalence of spontaneous portal-systemic shunts in persistent hepatic encephalopathy: A case-control study

MR Imaging Findings in Hepatic Encephalopathy

Brain MR Imaging and¹H-MR Spectroscopy Changes in Patients with Extrahepatic Portal Vein Obstruction from Early Childhood to Adulthood

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Oral glutamine challenge and magnetic resonance spectroscopy in three patients with congenital portosystemic shunts

Cited by 37 publications

References 28 publications

High prevalence of spontaneous portal-systemic shunts in persistent hepatic encephalopathy: A case-control study

High prevalence of spontaneous portal-systemic shunts in persistent hepatic encephalopathy: A case-control study

MR Imaging Findings in Hepatic Encephalopathy

Brain MR Imaging and1H-MR Spectroscopy Changes in Patients with Extrahepatic Portal Vein Obstruction from Early Childhood to Adulthood

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Brain MR Imaging and¹H-MR Spectroscopy Changes in Patients with Extrahepatic Portal Vein Obstruction from Early Childhood to Adulthood