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Query: UMLS:C0035400 (Reye's syndrome)
 879 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Authors studied nine cases of Reye's syndrome, seen over a period of five years. Their ages ranged from 45 days to 3 6/12 years, eight being less than a year old. Mortality rate was 55% and two of the four surviving children presented serious neurological sequelas. In all cases liver pathology was studied by percutaneous biopsy and/or necropsy. Clinical, analitical and anatomopathological findings were similar to those usually described in this syndrome. Medical literature was revised as to etiology and pathology, noting specially the tendency of this syndrome to present in groups, its possible relationship to viral epidemics and the inconsistency of the results of certain therapeutic measures widely used until now. The possible relationship between Reye's syndrome and the usual infant vaccinations, as they found in two cases, as well as the convenience of hepatic studies in the event of postvaccinal encephalopathy is suggested.
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PMID:[Reye's syndrome. Clinical revision (author's transl)]. 3 83

Evidence of circulating endotoxin was sought in children with Reye's syndrome, on the thesis that severe hepatic failure is likely to result in loss of capacity to detoxify intestinal endotoxins entering the circulation. A modification of the Limulus assay was used to demonstrate high levels of endotoxin-like activity (E.L.A.) in nine comatose patients with Reye's syndrome and in one of the two non-comatose patients. The symptom-free sibling of one patient had raised liver enzymes and a negative Limulus test. Plasma E.L.A. correlated significantly with degree of electroencephalographic disturbance early in the course of the illness. E.L.A. was also found in both of two cerebrospinal fluids evaluated. Preliminary in-vitro characterisation of this substance indicated that it resembled endotoxin derived from anaerobic intestinal bacteria. Intestinally derived endotoxin could be one factor in the pathogenesis of encephalopathy and other features of Reye's syndrome.
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PMID:Possible pathogenic role of endotoxin in Reye's syndrome. 4 99

The EEGs of 7 children with Reye's syndrome (fatty liver and encephalopathy) were continuously recorded on magnetic tape for times ranging from 12 to 80 h. During these times the major therapy consisted of exchange blood transfusions. The tapes were automatically processed on a LINC-8 computer using a peak-detection algorithm. The main parameters investigated were the delta and theta indices, the mean EEG frequency, the mean peak-to-peak amplitude, and the frequency versus amplitude characteristics of the delta waves. In 5 patients who responded to therapy, a marked decrease in the delta activity and an increase in mean frequency were noted subsequent to exchange transfusions. In all 5 cases the delta waves exhibited a similar pattern of decreasing amplitude and increasing frequency following therapy. However, in 2 patients with a fatal outcome, no favorable EEG response was observed during treatment, and a simultaneous decline in both delta frequency and delta amplitude was noted as the clinical condition worsened. The results of this study indicate that continuous EEG monitoring in Reye's syndrome may be an important clinical adjunct in evaluating the effectiveness of exchange transfusion therapy and in signaling the need for further treatment.
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PMID:Computerized evaluation of electroencephalographic changes accompanying exchange transfusion in Reye's syndrome. 6 30

Skeletal or cardiac muscle fibers can be separated by brief (3--5 second) dissociation of formalin-fixed pieces with a Willems Polytron (Brinkmann Instrument Co.). Such separated fibers are useful for demonstration of abnormal accumulations of lipids, carbohydrates, proteins and minerals in metabolic diseases. Staining techniques for demonstration of various stored materials include: 1) toluidine blue at pH 2.8 for acid mucopolysaccharide in skeletal muscle fibers in Pompe's glycogenesis 2, 2) one-step trichrome stain for nemaline myopathy and for abnormal mitochondria in X-linked infantile cardiomyopathy, 3) periodic acid-methenamine silver stain for glycolipid-containing lysosomes in I-cell disease (mucolipidosis 2), 4) Sudan black B stain for lipid in skeletal muscle fibers in Reye's syndrome, infantile lactic acidosis, Leigh's infantile subacute necrotizing encephalopathy and Jansky-Bielschowsky late infantile ceroid lipofuscinosis, 5) iron stain for iron in cardiac and skeletal muscle fibers in thalassemia with advanced hemosiderosis, and 6) autofluorescence for "ceroid" in skeletal muscle fibers in Jansky-Bielschowsky disease.
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PMID:Histochemical methods for dissociated muscle fibers. 9 Apr 4

Urea cycle function was evaluated in liver obtained from six patients with Reye's syndrome and from five control subjects. Reye's syndrome patients demonstrated normal activities for the extramitochondrial portion of the urea cycle, but showed marked abnormalities of the mitochondrial enzymes, i.e., carbamyl phosphate synthetase (CPS) and ornithine transcarbamylase (OTC) (Tables 2,3). CPS activity was reduced to less than 15% of control values in all four patients from whom tissues was obtained during the first 72 hr after the onset of encephalopathy. Two patents from whom tissue was not obtained until after 9 days of symptoms showed no reduction in CPS activity. The OTC activity was also reduced (3-67% of control values) in the four patients from whom tissue was obtained early in the illness. In addition, greater than 60% reduction in Vmax and Km for carbamyl phosphate was noted in all four patients in whom sample size permitted kinetic analysis, including both patients in whom CPS and OTC activity were not markedly reduced. The same kinetic abnormality as well as decreased CPS activity were experimentally produced in normal rate liver incubated in the presence of 1.0 mM 4-pentenoic acid, a short chain fatty acid and known hepatic mitochondrial toxin (Table 4).
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PMID:Abnormalities of carbamyl phosphate synthetase and ornithine transcarbamylase in liver of patients with Reye's syndrome. 17 18

During the period of February 1972 until February 1976, we documented six cases of influenza A-associated acute encephalopathy. The illnesses occurred during periods of influenza A activity in our community. The encephalopathy was invariably preceded by an upper respiratory tract infection, and, thereafter, patients soon became confused, vomited, and showed noticeable restlessness. Patients then followed either of two courses, some becoming comatose within 24 hours and others improving rapidly within three days or less. None of these patients exhibited the hepatic and biochemical abnormalities associated with Reye's syndrome. All patients survived but some had sequelae. The syndrome of influenza-associated acute encephalopathy deserves wider recognition.
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PMID:Influenza A virus associated with acute encephalopathy. 22 36

Three patients with encephalopathy clinically indistinguishable from Reye syndrome but associated with elevated cold-agglutinin titers and antiglobulin-I autoimmune hemolytic anemia are reported. The patients were treated with exchange transfusions, dexamethasone, controlled hyperventilation, and intracranial pressure monitoring. Liver biopsy specimens in two of the three patients showed fatty infiltration of the hepatocytes, but the light microscopic and electron microscopic appearance of the liver was not typical for Reye syndrome. These patients are reported to alert physicians to a metabolic encephalopathy in children which mimics Reye syndrome, but is distinctly different in clinical course and complicated by cold agglutinins and an autoimmune hemolytic anemia.
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PMID:Encephalopathy and fatty metamorphosis of the liver associated with cold-agglutinin autoimmune hemolytic anemia. 31 25

Reye's syndrome is characterized by severe encephalopathy and fatty infiltration of the liver. Probably this is a polyetiological syndrome. In most cases the disease is preceded by influenza B or varicella infection. As known to date, damage to mitochondria is the essential feature. Therapy is symptomatic. A review is given of the clinical symptoms and of the many unsolved problems of pathogenesis.
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PMID:[Reye syndrome (author's transl)]. 32 46

Dopamine, norepinephrine, and octopamine levels were estimated in regions of brains obtained postmortem from children who died with Reye syndrome and from age-matched controls. Hypothalamic norepinephrine levels were greatly decreased (to 30 percent of control, p less than 0.02) and octopamine levels were increased (to 700 percent of control, p less than 0.01). Levodopa had little effect on the physiologic condition of the patients. However, CNS dopamine and homovanillic concentrations were not elevated by levodopa, indicating that in the present cases levodopa was not metabolized to its catecholamine products. The findings indicate that the encephalopathy of Reye syndrome (as in other types of hepatic coma) may be linked to the presence of false transmitters in the brain and that levodopa is a rational therapy if administered before irreversible CNS changes occur.
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PMID:Catecholamine and octopamine concentrations in brains of patients with Reye syndrome. 33 8

Utilizing a specific and sensitive radioimmunoassay, palsma and urine tyramine were measured in 14 consecutive patients with liver biopsy-proven Reye's syndrome. Plasma tyrosine was measured in 11 of these patients. The results revealed significant (P less than .003) elevation in plasma (3.4 +/- .52 ng/ml) (mean +/- SEM) and urine (1.00 +/- .26 mg/24 hr) tyramine as well as plasma tyrosine (204 +/- 52.5 mumole/liter) at the onset of the disease when compared to the levels of tyramine and tyrosine in a group of hospitalized patients without hepatic disorders. Furthermore, there was a positive correlation between plasma tyramine and days in coma (r = .86; P less than .001), and between plasma tyramine and tyrosine (r = 0.80; P less than .001). These data suggest that there is s substantial disturbance of tyrosine metabolism in Reye's syndrome and that the accumulation of this amino acid and its metabolite, tyramine, may contribute to the encephalopathy of this disease.
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PMID:Evidence for hypertyraminemia in Reye's syndrome. 45 May 66


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