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Query: UMLS:C0085584 (encephalopathy)
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We report our results with orthotopic liver transplantation in children with fulminant liver failure. Thirty-five children with fulminant liver failure were evaluated for liver transplantation. The main causes of liver failure were viral hepatitis (54.2%), drug-induced liver injury (14.2%) and Wilson's disease (11.4%). Children were considered as candidates for liver transplantation only if hepatic encephalopathy was associated with a decrease in the level of factor V to below 25%. Seven children (20%) did not meet this criterion and recovered spontaneously. Six children (17.1%) had contraindications for liver transplantation and died. In three of these six children, contraindications included irreversible brain damage at the time of admission. Twenty-two children (62.8%) met the criteria for liver transplantation and were placed on the emergency transplant list. Three of them died awaiting grafts. Nineteen children underwent liver transplantation; 13 of them (68.4%) are alive without sequelae, after 6 mo to 4 yr of follow-up, at this writing. Four of the children who died after surgery had severe encephalopathy on admission that did not improve after liver transplantation. In conclusion, emergency liver transplantation appears to be an effective treatment for children with fulminant liver failure. Nevertheless, irreversible brain damage developed in 10 patients, and they died before or after surgery. We postulate that many of these deaths could have been avoided if children had been transferred to a liver transplantation facility and had undergone transplantation earlier. We emphasize that children with acute liver failure should be transferred to a center that performs liver transplantation before the development of hepatic encephalopathy.
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PMID:Emergency liver transplantation for fulminant liver failure in infants and children. 142 54

Symptomatic viral hepatitis A usually only requires supportive therapy and the majority of cases are managed in the community. The prodromal symptoms of nausea, anorexia and lethargy tend to improve with the onset of clinical jaundice. Fulminant hepatic failure is said to be an uncommon complication, occurring in only 0.14-0.35% of hospitalized cases. However, an increasing incidence has been documented in some northern European countries where up to 20% of cases of fulminant viral hepatitis is due to hepatitis A. This trend parallels the increasingly delayed exposure to hepatitis A and the increased severity of the illness when contracted in later life. The risk of developing fulminant hepatic failure is best monitored using coagulation factor assays, with the prothrombin time and factor V levels being the most favoured. The diagnosis is established with the onset of encephalopathy. Patients progressing to grade 4 encephalopathy have a reasonably good prognosis compared to other aetiologies and survival rates of up to 67% have been obtained with medical management, despite the co-existence of such complications as cerebral oedema, renal and respiratory failure and the metabolic sequelae of acute liver failure. Nevertheless, some patients require emergency liver transplantation and 10 such patients have been reported to date. Transplantation is especially required in older patients (> 40 years) and those who are jaundiced for > 7 days before the onset of encephalopathy. The serum bilirubin and the prothrombin time complement these parameters in the decision making process.
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PMID:Management of acute and fulminant hepatitis A. 147

Fipexide belongs to a new class of cognition activators and is noted for its lack of amphetamin-like side effects. We describe three patients who developed fulminant hepatic failure less than 2 months after beginning fipexide administration. The mean interval from the onset of jaundice to the onset of encephalopathy was 8 days. Emergency liver transplantation was undertaken when factor V was 20% of normal or less and coma developed. All patients were transplanted less than 1 week after the onset of encephalopathy. Two survived and one died immediately after transplantation. Histologic examination of the livers revealed massive liver cell necrosis, predominantly centrilobular, and a moderate inflammatory infiltrate within the portal spaces. We conclude that fipexide can induce massive liver cell necrosis and fulminant liver failure. As a result of this life-threatening complication, reconsideration of the indications for this drug is warranted.
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PMID:Fipexide-induced fulminant hepatitis. Report of three cases with emergency liver transplantation. 150 32

The adjectives fulminant and subfulminant apply to those forms of acute hepatitis where the prothrombin level falls below 50 p. 100 of its normal value and which is complicated by clinical hepatic encephalopathy. The most frequent cause is an acute viral hepatitis, notably that due to the B virus. Beside jaundice and encephalopathy, the most striking manifestations consist of low factor V level, cardiocirculatory hyperkinesia, acute renal failure due to hypovolaemia, respiratory alkalosis and sometimes hypoglycaemia or hypophosphataemia. The mortality rate, which is 75 p. 100 overall, varies with the causative virus. The prognosis is based on the degree of factor V decrease rather than on the severity of the encephalopathy. Coagulant fractions and nervous sedatives may obscure the prognosis and should not be administered. With emergency liver transplantation, 60 p. 100 of the patients who would have died survive. Early hospitalization of patients with severe acute hepatitis (prothrombin level below 50 p. 100 of its normal value, but no hepatic encephalopathy) is associated with much lower mortality rates and could be used to prevent transformation into fulminant or subfulminant hepatitis.
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PMID:[Fulminant and subfulminant viral hepatitis]. 237 17

Twenty-nine patients with fulminant hepatic failure and at least grade III encephalopathy were treated by haemodialysis with a polyacrylonitrile membrane. Aetiology was toxic in five patients, viral in eleven (2 due to hepatitis A virus and 9 presumed due to hepatitis B virus), not found in thirteen. Each patient was dialysed for 4 h every day, until he regained consciousness or died. Conscious level was improved after dialysis in 59% of patients. Thirteen patients survived (44.8%) :4 toxic hepatitis, 4 viral hepatitis B, 1 viral hepatitis A, 4 hepatitis of unknown aetiology. A comparison of plasma concentrations of amino acids measured by chromatography before and after 113 periods of haemodialysis in 23 patients showed significant decrease in aromatic amino acids (p less than 0.001), a significant increase in two branched-chain amino acids :leucine (p less than 0.001) and isoleucine (p less than 0.001), and a significant increase in Fischer's ratio (p less than 0.001). In survivors, factor V concentration on admission and Fischer's ratio on admission were significantly higher than in those who died (p less than 0.02 for both), but there was no significant difference in the difference between Fischer's ratio before and after haemodialysis. Haemodialysis was well tolerated, except for short periods of hypotension and a small but significant fall in platelet counts. Improvement in cerebral function during haemodialysis was previously demonstrated by various authors, but the effect on survival rate remained controversial. The survival rate obtained in this controlled study is clearly higher than those obtained by conservative management alone.
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PMID:[Hemodialysis with a high permeability membrane in the treatment of encephalopathy of fulminant hepatitis]. 343 87

One hundred and thirty-one patients underwent clinical and biological investigation with the following determinations performed on the same day; presence or absence of ascites, icterus and/or encephalopathy, coagulation study, biochemical determinations including albumin, transferrin and immunoglobulins immunoassays. The principal component analysis of biological data showed two sets of highly representative and inversely correlated data; one included coagulation tests, albumin and transferrin, and the other included immunoglobulin A/transferrin ratio, immunoglobulin A and total bilirubin. Clinical and biological data were computed using discriminant analysis between dead and survivors. Six parameters were then selected (total bilirubin, encephalopathy, factor V, AST, antithrombin III and transferrin) giving a correct prognosis in 81.6% (31/38) of cases in a test sample. Neither ascites nor immunoglobulins were useful for the estimation of the prognosis.
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PMID:Multivariate analysis of clinical and biological data in cirrhotic patients: application to prognosis. 679 41

Isoniazid and pyrazinamide are well-known hepatotoxic drugs, often used in combination. The aim of this study was to assess the prognostic influence of pyrazinamide on the outcome of fulminant or subfulminant liver failure caused by antituberculous therapy. Eighteen patients with fulminant or subfulminant liver failure due to antituberculous therapy were studied. Nine patients received isoniazid and rifampicin without pyrazinamide (group 1), and nine patients received isoniazid and rifampicin together with pyrazinamide (group 2). The severity of fulminant and subfulminant liver failure, as judged by the prevalence of coma and the lowest level of factor V, was similar in the two groups. Spontaneous survival was greater in group 1 (eight of nine) than in group 2 (two of nine) (P < .02). The authors conclude that pyrazinamide co-administration was associated with an increased mortality in patients with fulminant or subfulminant hepatitis occurring during antituberculous therapy. In these patients, pyrazinamide administration and an interval of more than 15 days between the onset of antituberculous treatment and jaundice, combined with grade III encephalopathy and factor V below 20%, predicted death without liver transplantation.
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PMID:Deleterious influence of pyrazinamide on the outcome of patients with fulminant or subfulminant liver failure during antituberculous treatment including isoniazid. 770 2

The risk for developing acute liver failure after halothane exposition was calculated between 1:8,000 and 1:36,000. The case report given on a 22 year old man with halothane-induced hepatic failure is unusual, because the typical risk factors as age over 40, female sex, obesity, and previous exposure to halothane were not present. Two days after exposure to halothane the patient suffered acute liver failure with severe coagulopathy (factor V = 5% activity), and encephalopathy grade IV complicated by renal failure and respiratory insufficiency. Maximal increases of enzymes in blood were AST 3900 U/L, ALT 2570 U/L, LDH 10600 U/L. After six days the patient underwent liver transplantation with complete anuria and instable circulation. Explanted liver showed massive necrosis (70% of parenchyma) and fatty changes. The liver transplant had immediately a good function and renal failure resolved within three days. In the follow-up of 3 1/2 years the patient suffered no further complications. Culturing the patient's lymphocytes in the lymphocyte transformation test a strong reaction could be detected with a stimulatory index of 20. Maximal proliferation was observed when lymphocytes were incubated with plasma metabolites of a volunteer drawn 120 minutes after anesthesia with halothane was started.
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PMID:[Liver transplantation in halothane-induced liver necrosis]. 802 96

We reported a family with early onset cerebrovascular disease. Patient 1 (a 36-year-old man) demonstrated a combination of livedo reticularis and cerebral infarction as previously described as Sneddon syndrome. He also showed transient focal neurologic symptoms and mild dementia. Patient 2 (an elder sister of Patient 1) was suffering from migraine. Their father and paternal uncle died of cerebral infarction, which had developed in their thirties or forties. Patients 1 and 2 showed MRI findings compatible with encephalopathy with Binswanger-type. Contrary to the previous reports on Binswanger-type encephalopathy, both of these patients demonstrated decreased levels of fibrinogen as well as those of factor V, together with negative antiphospholipid antibody. Thus, juvenile onset, autosomal dominant inheritance, the diversity of clinical findings and the coagulopathy in this family were characteristic features. The level of thrombin-antithrombin III complex (TAT) was markedly increased in Patient 1. Treatment with antithrombin (argatroban 20mg i.v. everyday for 28 days) not only reduced the level of TAT but also improved the livedo reticularis and neurological findings. Although gene analysis has not been performed yet on this family, this condition is similar to cerebral autosomal dominant arteriopathy with subcortical infarct and leukoencephalopathy (CADASIL), which involve juvenile cerebral infarction and dementia as well as migraine.
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PMID:[Familial Binswanger-type encephalopathy with Sneddon syndrome]. 875 90

The clinical course of 12 patients with mushroom poisoning was evaluated in order to define the parameters considered to be relevant to the indication for liver transplantation. Eight patients recovered under conservative therapy; one patient died due to pre-existing, concomitant cardiopulmonary disease. In three patients transplantations had to be performed because of severe liver failure. On admission, the transplanted patients had a decreased Quick's test score and factor V value (< 10%). The peak of liver enzymes, serum bilirubin, serum creatinine, partial thromboplastin time and azotemia were not of any prognostic value. Main indications for liver transplantation were a very low initial Quick's test score and factor V value (both < 10%) and their inadequate response under substitution therapy. The development of encephalopathy and renal failure were further parameters indicating poor prognosis.
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PMID:[Indications for liver transplantation in severe amanita phalloides mushroom poisoning]. 901 31


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