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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 63-year-old male with atrial fibrillation and mild mitral valve regurgitation was referred to hospital because of a descending aortic aneurysm. During the evaluation, he developed an encephalopathy because of hyperammoniaemia. Further examination revealed a portal systemic shunt, perhaps caused by the noncirrhotic portal hypertension. The patient underwent successful replacement of the aneurysm after controlling the blood ammonia level by eliminating protein from the diet and removal of nitrogen from the gastrointestinal tract. Cardiovascular surgery in a patient with noncirrhotic portal hypertension and a portal systemic shunt has not been previously reported. Meticulous management of the perioperative blood ammonia concentration is essential.
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PMID:Surgical treatment of a descending aortic aneurysm in a patient with noncirrhotic portal hypertension and a portal systemic shunt. 1249 28

Extracorporeal liver devices have gained great attention as a complementary approach to liver transplantation in patients with acute on chronic liver failure. Among others, Molecular Adsorbent Recycling System (MARS) is a hemodiafiltration against albumin able to remove low molecular weight toxins. We aimed to validate the use of MARS in patients presenting with acute on chronic liver failure with severe cholestasis. We enrolled 7 patients with acute on chronic liver failure, presenting with bilirubin >25 mg/dl and hepatorenal syndrome and/or hepatic encephalopathy grade >II. Liver biochemistry, coagulation, blood cell count, electrolytes, ammonia, lactate, blood urea nitrogen, creatinine, bile acids, Fischer ratio, and encephalopathy grade were assessed before and after each MARS treatment. MARS can represent a safe therapeutic choice to achieve a quick improvement of neurological status, a hemodynamic stability, and a better clinical outcome. In particular, our encouraging results suggest that also, patients with severe cholestasis may represent in the future a good indication for MARS treatment.
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PMID:Successful MARS treatment in severe cholestatic patients with acute on chronic liver failure. 1278 May 11

Spontaneous bacterial peritonitis (SBP) is frequently associated with renal failure. This study assessed if systemic and hepatic hemodynamics are also affected by this condition. Standard laboratory tests, tumor necrosis factor alpha (TNF-alpha) in plasma and ascitic fluid, plasma renin activity (PRA) and norepinephrine (NE), and systemic and hepatic hemodynamics were determined in 23 patients with SBP at diagnosis and after resolution of infection. Eight patients developed renal failure during treatment. At diagnosis of infection, patients developing renal failure showed significantly higher values of TNF-alpha, blood urea nitrogen (BUN), PRA and NE, peripheral vascular resistance, and hepatic venous pressure gradient (HVPG) and lower cardiac output than patients not developing renal failure. During treatment, a significant reduction in cardiac output and arterial pressure and increase in PRA and NE, HVPG, and Child-Pugh score were observed in the first group but not in the second. Peripheral vascular resistance remained unmodified in both groups. Changes in PRA and NE correlated inversely with changes in arterial pressure and directly with changes in BUN, Child-Pugh score, and HVPG. Five patients in the renal failure group developed encephalopathy, and 6 died. In the group without renal failure, none of the patients developed encephalopathy or expired. In conclusion, patients with SBP frequently develop a rapidly progressive impairment in systemic hemodynamics, leading to severe renal and hepatic failure, aggravation of portal hypertension, encephalopathy, and death. This occurs despite rapid resolution of infection and is associated with an extremely poor prognosis.
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PMID:Systemic, renal, and hepatic hemodynamic derangement in cirrhotic patients with spontaneous bacterial peritonitis. 1523 12

Nutritional factors play a major role both in the pathogenesis as well as management of hepatic encephalopathy (HE). Physicians treating patients with chronic liver disease often restrict the intake of dietary protein to prevent a rise in blood ammonia levels. The role of protein restriction in patients with chronic hepatic encephalopathy (CHE) has been questioned recently as the efficacy of protein withdrawal in patients with CHE has never been subjected to a controlled trial. Evidence suggests that protein intake plays only a limited role in precipitating encephalopathy. In fact, measures taken to suppress endogenous protein breakdown are more effective than dietary restrictions in reducing the load of amino acids on the decompensated liver. A protein intake of less than 40 g per day, as has been indicated, contributes to a negative nitrogen balance, which along with increased endogenous protein breakdown, worsens encephalopathy. A positive nitrogen balance may have positive effects on encephalopathy. Rather, depressed plasma branched-chain amino acid (BCAA) levels, implicated in the pathogenesis of HE, also supervene in cirrhosis only when malnutrition is present as well. Therefore, the emphasis in the nutritional management of patients with HE should not be on the reduction of protein intake. Instead, the goal should be to promote synthesis by making available ample amounts of amino acids, while instituting other measures to reverse the ongoing catabolism. Different protein sources have varying effects on HE and efforts should be made to identify the most tolerated protein source to prevent malnutrition and maintain these patients on a long-term basis.
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PMID:Nutrition in the management of hepatic encephalopathy. 1460 21

By experiments of acute carbon-monoxide intoxication, acute nitrogen hypoxia and histotoxic hypoxia using sodium cyanide in cats, and by hemodynamic studies using plastic branch models, the following was elucidated; (1) severe tissue hypoxia, regardless of the underlying cause, and subsequent slight ischemia of the brain due to mild hypotension induce selective involvement of the cerebral white matter and pallidum, these two conditions being necessary and sufficient and this encephalopathy should be separately categorized as "hypoxic-ischemic encephalopathy" in hypoxic brain injuries, (2) the background of the selective involvement of these structures is an enormous development of the cerebrum in the brain, which induces thick white matter resulting in proper and long medullary artery, and especially small diameter ratio of the pallidal perforators to the middle cerebral artery, (3) the long course of the medullary artery produces the blood pressure drop in the deep white matter according to Hagen-Poiseuille's low, and according to that the smaller the diameter ratio, the larger the branching-loss coefficient (energy-loss co-efficient), smaller diameter ratio of the pallidal perforator, as compared with that of the putaminal perforator, induces more severe loss of the local blood flow selectively to the pallidum. This state seems to be a failure of compromise between the cardiovascular system and the brain parenchyma.
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PMID:Concept and pathogenesis of "hypoxic-ischemic encephalopathy". 1475 93

Bismuth salts are widely used to treat peptic ulcers. Acute toxicity with colloidal bismuth subcitrate overdose causes nephrotoxicity. There have been numerous reports of encephalopathy after long-term consumption of bismuth salts, but only a few cases of nephrotoxicity (adult and pediatric) have been documented to date. This report presents a case of acute renal failure due to colloidal bismuth subcitrate overdose in adolescent. A 16-year-old girl presented with complaints of nausea, vomiting, and facial paresthesia. Ten days earlier she had tried to commit suicide by taking 60 tablets of De-nol (colloidal bismuth subcitrate 18 g). The physical examination findings on admission indicated minimal fluid overload but no signs of encephalopathy. Laboratory tests on admission showed blood urea nitrogen 102 mg/dl, serum creatinine 19.9 mg/dl, and serum bismuth level 495 microg/l. The patient was started on appropriate fluid therapy and penicillamine as a chelating agent and then began hemodialysis on alternate days. The patient's renal function gradually returned to normal over 9 weeks and by 64 days after the overdose her serum bismuth level had fallen to almost half the level detected 2 days after admission. The patient made a complete recovery. The case demonstrates that acute renal failure can develop as a manifestation of acute toxicity from colloidal bismuth ingestion, and that the prognosis may be favorable if the patient receives appropriate supportive treatment and dialysis.
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PMID:Acute renal failure after overdose of colloidal bismuth subcitrate. 1594 79

Reactive nitrogen and oxygen species (O2*-, H2O2, NO* and ONOO-) have been strongly implicated in the pathophysiology of neurodegenerative and mitochondrial diseases. In the present study, we examined the effects of nitrosative and/or nitrative stress generated by DETA-NO {(Z)-1-[2-aminoethyl-N-(2-ammonioethyl)amino]diazen-1-ium-1,2-diolate}, SIN-1 (3-morpholinosydnonimine hydrochloride) and SNP (sodium nitroprusside) on U87MG glioblastoma cybrids carrying wt (wild-type) and mutant [A3243G (Ala3243-->Gly)] mtDNA (mitochondrial genome) from a patient suffering from MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes). The mutant cybrids had reduced activity of cytochrome c oxidase, significantly lower ATP level and decreased mitochondrial membrane potential. However, endogenous levels of reactive oxygen species were very similar in all cybrids regardless of whether they carried the mtDNA defects or not. Furthermore, the cybrids were insensitive to the nitrosative and/or nitrative stress produced by either DETA-NO or SIN-1 alone. Cytotoxicity, however, was observed in response to SNP treatment and a combination of SIN-1 and glucose-deprivation. The mutant cybrids were significantly more sensitive to these insults compared with the wt controls. Ultrastructural examination of dying cells revealed several characteristic features of autophagic cell death. We concluded that nitrosative and/or nitrative stress alone were insufficient to trigger cytotoxicity in these cells, but cell death was observed with a combination of metabolic and nitrative stress. The vulnerability of the cybrids to these types of injury correlated with the cellular energy status, which were compromised by the MELAS mutation.
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PMID:Effects of nitric oxide donors on cybrids harbouring the mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) A3243G mitochondrial DNA mutation. 1596 53

While transjugular intrahepatic portosystemic shunt (TIPS) is a common therapy for cirrhotic patients with diuretic-resistant or diuretic-refractory ascites, some patients are unsuitable for the procedure for technical or medical reasons. We report our experience with the use of chronic intravenous albumin infusions to achieve diuresis in this difficult patient population and review the historic experience of chronic albumin infusions as a treatment for ascites. Nineteen patients with cirrhosis and diuretic-resistant or diuretic-refractory ascites who were deemed unsuitable for TIPS received outpatient intravenous albumin infusions (50 g) weekly for at least 4 weeks. The following endpoints were retrospectively recorded: serum sodium, serum creatinine, blood urea nitrogen, hematocrit, bilirubin, albumin, international normalized ratio, body weight, and Model for End-stage Liver Disease (MELD) score. The contraindications for TIPS included the following: portal vein thrombosis, two; advanced age, one; encephalopathy, nine; hyperbilirubinemia, five; and other, two. Compared to pretreatment, posttreatment weight decreased in 17 patients, remained unchanged in 0 patients, and increased in 2 patients. The overall mean change in body weight (before vs. after therapy) was 8 lb (P < 0.05). The only significant change in biochemistry was an increase in serum albumin from 2.5 g/dl before therapy to 3.5 g/dl after therapy (P < 0.05). We conclude that (1) recurrent intravenous weekly albumin infusions resulted in significant loss of edema and ascites as measured by loss of body weight, and (2) clinicians may want to consider chronic albumin infusions for selected patients with refractory ascites who are not candidates for TIPS.
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PMID:Chronic albumin infusions to achieve diuresis in patients with ascites who are not candidates for transjugular intrahepatic portosystemic shunt (TIPS). 1604 87

To clarify the laboratory characteristics and deduce the pathogenesis of acute encephalopathy associated with multiple organ dysfunctions in Japan. We measured cytokine levels [tumor necrosis factor alpha (TNF-alpha), soluble tumor necrosis factor-receptor 1 (sTNF-R1), and interleukin-6 (IL-6)] in serum and cerebrospinal fluid (CSF) as well as general laboratory examinations in 27 patients with acute encephalopathy. Urea nitrogen (UN), creatinine (Cr), aspartate aminotransferase (AST), lactic dehydrogenase (LDH), and C-reactive protein (CRP) levels in blood, and CSF protein levels at the initial stage were significantly higher in patients with an unfavorable outcome. TNF-alpha, sTNF-R1, and IL-6 levels at the initial stage were higher in the serum than in the CSF of patients with acute encephalopathy. Serum cytokine levels correlated well with patient outcome. The high CSF protein level and the high UN, Cr, AST, LDH, and CRP levels in the blood represent the severity of vascular leakage through the blood-brain barrier and multiple organ dysfunctions, respectively, and thus suggest an unfavorable prognosis. The high serum inflammatory cytokine levels at the initial stage and the good correlation of those levels with the outcome suggest that intravascular inflammation has a significant role in vascular leakage and multiple organ dysfunctions in acute encephalopathy.
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PMID:Laboratory characteristics of acute encephalopathy with multiple organ dysfunctions. 1619 4

The urea cycle consists of six consecutive enzymatic reactions that convert waste nitrogen into urea. Urea cycle disorders are a group of inborn errors of hepatic metabolism that often result in life threatening hyperammonemia and hyperglutaminemia. Deficiencies of all of the enzymes of the cycle have been described and although each specific disorder results in the accumulation of different precursors, hyperammonemia and hyperglutaminemia are common biochemical hallmarks of these disorders. Arginase is the enzyme involved in the last step of the urea cycle. It catalyzes the conversion of arginine to urea and ornithine. The latter reenters the mitochondrion to continue the cycle. Hyperargininemia is an autosomal recessive disorder caused by a defect in the arginase I enzyme. Unlike other urea cycle disorders, this condition is not generally associated with a hyperammonemic encephalopathy in the neonatal period. It typically presents later in childhood between 2 and 4 years of age with predominantly neurological features. If untreated, it progresses with gradual developmental regression. A favorable outcome can be achieved if dietary treatment and alternative pathway therapy are instituted early in the disease course. With this approach, further neurological deterioration is prevented and partial recovery of skills ensues. Early diagnosis of this disorder through newborn screening programs may lead to a better outcome. This review article summarizes the clinical characterization of this disorder; as well as its biochemical, enzymatic, and molecular features. Treatment, prenatal diagnosis and diagnosis through newborn screening are also discussed.
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PMID:Clinical, biochemical, and molecular spectrum of hyperargininemia due to arginase I deficiency. 1660 94


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