Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035400 (Reye's syndrome)
 879 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Calcium hopantenate (HOPA) has been widely used as an activator of cerebral metabolism in Japan. However, several cases of acute encephalopathy during HOPA administration were recently reported, which were characterized by marked metabolic acidosis and hypoglycemia. The encephalopathy in these patients was named Reye-like syndrome because of the similarity to Reye's syndrome in children. The purposes of this presentation are to report on 5 patients with acute encephalopathy developing during HOPA administration, to summarize their symptoms and clinical courses, and to discuss the pathogenesis of metabolic acidosis and hypoglycemia. Initial characteristics of the clinical course in all patients were loss of appetite, nausea and vomiting, followed by unconsciousness. Laboratory examinations revealed marked metabolic acidosis, severe hypoglycemia, hyperlactacidemia, leukocytosis, ketonuria, and increased Ht and BUN. A few days after development of the initial symptoms, mild renal and liver dysfunction, and elevation of serum amylase were observed in all patients. Hyperlactacidemia was present in 4 in the initial period. Blood concentration of HOPA was 2.131 micrograms/ml in patient 1 (8-10 hours after final administration), and 10.7 micrograms/ml in patient 5 (24 hours after final administration). These values are extremely high, because usually HOPA concentration is almost negligible 7 hours after the drug is taken. As the pathogenesis of acute encephalopathy due to HOPA administration, the failure of fatty acid beta-oxidation has been proposed by some investigators. However, the serum concentrations of CoA, pantothenic acid and carnitine during the initial stage were not reduced in our patients. Furthermore, it is very difficult to explain the severe hypoglycemia in terms of the beta-oxidation theory.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Metabolic acidosis and hypoglycemia during calcium hopantenate administration--report on 5 patients]. 258 87

The aetiology and pathogenesis of Reye's syndrome (RS) are incompletely understood. A number of environmental toxins and biological agents, including viruses, have been postulated to cause RS, either acting alone or synergistically. Most investigations have suggested that the primary insult is in the liver mitochondria, leading to a complex biochemical catastrophe, with death from encephalopathy. Margosa oil (MO), a long-chain fatty acid compound, has been shown to cause a Reye-like syndrome with death from hepatoencephalopathy, in children in Malaysia and India. The present time-course study performed in MO-administered mice showed the development of hepatic lesions with many features of RS. MO acts rapidly, within 30 min, on the nuclei of hepatocytes inducing mitoses and binucleated cells. This is followed by mitochondrial injury, with swelling, rarefaction of matrix, loss of dense bodies, pleomorphism, and loss of ribosomes starting at 60 min. There is loss of liver glycogen, and proliferation and hypertrophy of the endoplasmic reticulum (ER), followed by the presence of lipid droplets in the hyaloplasm, and globules within dilated cisterns of the ER. Additional fatty acids from lipolysis of body adipocytes, and fat globules from intestinal MO ingestion further aggravate the liver fatty change. There is evidence of fat globule ingestion by endocytosis into hepatocytes at the level of the sinusoids. The development of microvesicular liver steatosis and glycogen depletion due to involvement of liver cell organelles occur rapidly as in RS.
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PMID:Animal model of margosa oil ingestion with Reye-like syndrome. Pathogenesis of microvesicular fatty liver. 259 49

High levels of the serum free fatty acids (FFA) are found in Reye's syndrome (RS). While this is attributed to enhanced adipose tissue lipolysis, the possibility that intravascular lipolysis could augment this process was investigated by measuring lipase activity in sera from RS and other subjects. Ordinarily, lipolytic activity is not detectable in serum from unheparinized subjects. Significant lipolytic activities ranging from 1-3 mumol/ml serum per hour were detected in sera from 5 of the 7 RS patients studied. Similar activities were also found in sera from two other subjects one of whom was a long-term survivor of RS and the other who had recurrent bouts of biliary obstruction and encephalopathy. Lipase activity was negligible in the serum from 2 other RS patients, 4 other long-term survivors of RS, 2 siblings, one RS parent and in 20 disease controls including patients with influenza, diabetic ketoacidosis and cerebral edema, meningitis and febrile infections with diarrhea and vomiting. None of these individuals had received heparin. An inverse relationship was found between LPL and hepatic lipase (HL) activities. Glucose levels tended to correlate directly with LPL and inversely with HL activity. The basis for the presence of LPL activity in RS sera is not known but the presence of serum lipase activity in unheparinized patients supports the notion that the TG in the circulating lipoprotein particles probably also serve as another source of FFA in the sera of RS patients.
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PMID:Serum lipolytic activity in Reye's syndrome. 259 64

Recently a number of defects of fatty acid transport and beta-oxidation have been described. Primary carnitine deficiency presents itself as muscle carnitine deficiency, systemic carnitine deficiency or a cardiomyopathic subtype. Another abnormality of fatty acid transport is due to inner carnitine palmitoyl transferase deficiency, usually associated with a myoglobinuric phenotype. Long chain, medium and short chain acyl-CoA-dehydrogenase defects are usually associated to Reye's syndrome, encephalopathy, organic aciduria and secondary carnitine deficiency.
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PMID:[Alterations of mitochondria in metabolic diseases. Carnitine deficiency, carnitine palmitoyltransferase deficiency and beta oxidation]. 260 79

Reye's syndrome is a rare but potentially fatal disease that affects all organs of the body, with an especially devastating attack upon the liver and brain. It is characterized by encephalopathy with severe edema of the brain, increased intracranial pressure, hypoglycemia, and fatty infiltration of the liver. It is a two-phase illness, almost always associated with a previous viral infection. Studies strongly support a link between the use of aspirin and Reye's syndrome. Reported cases are on the decline. Recent epidemiologic studies show the incidence for children 10 to 19 years old to be stable, but reduced for younger children. Special attention needs to be directed toward educating adolescents and parents of older children about Reye's syndrome and cautioning against the use of aspirin to treat influenza, upper respiratory infections, or varicella.
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PMID:Reye's syndrome: review and update. 267 93

This is the case of a 29 year-old woman, admitted because of diarrhea and incoercible vomiting; later in the course of the disease she developed signs and symptoms of encephalopathy. The diagnosis of Reye's syndrome was made after death.
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PMID:[Reye's syndrome in adults. Presentation of a new case and review of the literature]. 269 55

There have been over 3,500 cases of Reye's syndrome reported to the CDC since 1973. The temporal use of aspirin with a prodromal viral illness has been statistically associated with the etiology of the syndrome. Reye's syndrome presents as an acute progressive encephalopathy with varying levels of coma and normal cerebrospinal fluid (CSF). Hepatic transaminases are elevated, and the serum aspartate aminotransaminase (AST) is usually elevated three to 30 times normal. Histological changes on liver biopsy are characterized by microvesicular intrahepatocyte lipid deposition, which is rarely found in other diseases. The syndrome has rarely been reported to occur in adults over 18 years old. We report the 25th case of Reye's syndrome occurring in an adult, which initially presented as acetominophen toxicity but was shown to be Reye's syndrome on histological examination of the liver.
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PMID:Reye's syndrome in an adult. 270 76

Reye's syndrome or encephalopathy with fatty infiltration of the liver occurs mainly in childhood and to a lesser degree in adult age. Salicylate may play a role in the pathogenesis of this syndrome. Still's disease is a form of juvenile rheumatoid arthritis very rare in the adult. A case is reported of a 39-year old woman who developed Reye's syndrome while on aspirin therapy for the treatment of a classical adult Still's disease.
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PMID:[Reye's syndrome in an adult following therapy with salicylates in Still's disease]. 273 36

Reye's syndrome is an acute non-inflammatory encephalopathy with fatty degeneration of inner organs. The disease seems to be the metabolic response to a generalized mitochondrial insult. From 1982 to 1987, we studied 7 patients, age range being 2 months to 12 years, affected by R.S. None of them had taken salicylic acid before; three of them had been treated with acetaminophen. Two out of the seven patients examined had serious neurologic problems; other two recovered completely both under clinical and histological aspects, as it is shown by the hepatic biopsy done before and after the disease, and the remaining three died. We have tried to demonstrate the importance of hepatic biopsy in the diagnosis of R.S. and in the verification of the reversibility of the histological phenomenon. Hepatic biopsy demonstrated that the disease stopped at the initial stage, limited itself and evolved towards a complete recovery.
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PMID:[Reye's syndrome: reports of 7 cases in the period 1982-1987]. 276 98

We examined the oxidation of different chain length fatty acids in the leukocytes and the quantity of lipid peroxides in the plasma of two Reye syndrome patients. We have found that the oxidation of [1-14C] octanoic acid in homogenates of leukocytes from one of the Reye syndrome patients was only 38 percent of the control, whereas oxidation of [1-14C] palmitic and [1-14C] lignoceric acid was slightly increased. The level of lipid peroxides in the serum of both of the Reye Syndrome patients was 4.42 and 3.04 times higher than the control level. These results suggest that impaired oxidation of medium chain fatty acids (octanoic acid) and higher levels of lipid peroxides may contribute to the pathogenesis of cellular toxicity in Reye Syndrome. Reye Syndrome (RS) was first described by Reye et. al. in 1963 and is now recognized as an important cause of morbidity and mortality in infants and children. The clinical course in RS consists of an antecedent viral illness with subsequent encephalopathy and hepatic dysfunction. Laboratory findings in RS include hypoglycemia, hyperammonemia, free fatty acidemia, elevated organic acids and amino aciduria. The ultrastructural findings in RS patients include changes in mitochondria, smooth endoplasmic reticulum morphology, and an increase in the number of peroxisomes. The elevation of serum free fatty acids in RS and their decrease in patients who improve clinically suggests a disturbance in fatty acid metabolism. To understand the role of free fatty acids in the pathogenesis of RS, we examined the levels of lipid peroxides in plasma and catabolism of fatty acids of different chain lengths in leukocytes from RS patients.
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PMID:Reye syndrome: rate of oxidation of fatty acids in leukocytes and serum levels of lipid peroxides. 276 52


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