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

We report a 3-year-old Japanese girl who developed acute encephalopathy while receiving calcium hopantenate (Calcium D-(+)-4-(2, 4-dihydroxy-3,3-dimethylbutyramido) butyrate hemihydrate). She had hyperammonemia, elevated CPK, lactic acidemia and pyruvic acidemia, however, she did not show elevated SGOT or SGPT. Calcium hopantenate has been used in Japan for the treatment of mental retardation with behavior abnormalities. Recently there have been three reports on the occurrence of Reye-like syndrome in patients receiving this drug. Clinical signs and laboratory data of these patients are similar to those of Reye syndrome. Calcium hopantenate causes pantothenic acid deficiency in the young rat, which may reduce the content of coenzyme A. If this drug decreases coenzyme A biosynthesis, it may reduce beta-oxidation of fatty acids and levels of dicarboxylic acids would increase because of increasing omega-oxidation. We suspect that there is a possible relationship between the occurrence of acute encephalopathy and calcium hopantenate therapy.
 ...
PMID:Acute encephalopathy with hyperammonemia and dicarboxylic aciduria during calcium hopantenate therapy: a patient report. 382 51

Unique CT findings were obtained in four cases of acute toxic encephalopathy, including two cases of clinical Reye's syndrome in the acute stage. Common findings in these cases were symmetrical low density in the thalami and the cerebellum (mainly the dentate nuclei) and similar changes in the surroundings of the lateral ventricles.
 ...
PMID:Acute toxic encephalopathy with symmetrical low density areas in the thalami and the cerebellum. 387 16

Between 1980 and 1982 in the Campania region of Italy, several cases of encephalopathy in children who had only a week previously been given diphtheria-tetanus immunizations were reported to the Italian Ministry of Health in Rome. A case-control study was therefore set up to test the association between the syndrome and immunization. For this purpose, cases were defined as children between 3 and 48 months who had been admitted to an intensive care unit with symptoms such as convulsions of unknown origin, Reye's syndrome, or death from unknown causes. The fatality rate from encephalopathy among the immunized children in the study was 25 out of 29 (86%). In Campania (population 5.4 million), the annual incidence of encephalopathy associated with diphtheria-tetanus immunization was estimated to be 2.9 per 100 000 doses of the vaccine.
 ...
PMID:Case-control study on encephalopathy associated with diphtheria-tetanus immunization in Campania, Italy. 387 3

Serum concentrations of short and medium chain fatty acids, including octanoate, are elevated in hepatic encephalopathy and Reye syndrome. Injection of octanoate into animals produces features reminiscent of Reye syndrome, but the mechanisms are unknown. To evaluate the effect of octanoate on blood-brain barrier permeability, three techniques were used. Entry of horseradish peroxidase and trypan blue into brain was not observed after octanoate injection. Brain uptake of tryptamine, tyrosine and methionine was increased significantly by octanoate, while uptake of insulin was unchanged. This study suggests that octanoate may produce central nervous system alterations by facilitating entry of certain low molecular weight compounds into brain. This may represent one mechanism for the development of encephalopathy in liver disease and Reye syndrome.
 ...
PMID:Effect of octanoate injection on rat blood-brain barrier. 388 Mar 86

Reye syndrome has emerged as the quintessential example of an acute metabolic encephalopathy. The clinical presentation is quite stereotyped in most instances permitting rapid, accurate diagnosis and early therapeutic intervention. Intoxications and certain inborn metabolic errors may mimic Reye syndrome. All patients with a recurrent episode should be investigated thoroughly for evidence of a metabolic disorder associated with an enzyme deficiency. Notable in this regard are inborn errors affecting organic acid, ammonia, and carbohydrate metabolism. The mitochondrial disturbance in Reye syndrome is well documented but the pathophysiologic sequence linking the antecedent viral illness to the mitochondrial injury remains obscure. Recent identification of a spontaneous Reye-like illness in mice that is associated with a coronavirus infection may provide an opportunity to investigate this initial phase of the pathophysiologic sequence. The primary cerebral insult presumably derives from insufficient substrate availability and results in massive cytotoxic cerebral edema. Treatment revolves around the continuous infusion of hypertonic glucose and intermittent infusion of hypertonic mannitol. Management is designed to attenuate or avoid the various compounding metabolic insults during this critical period when the child is metabolically crippled. In 1963, the disorder was considered to be rare and almost irreversibly fatal. Today, the disorder is recognized to be more common, and the outcome is very satisfactory in 85 to 90 per cent of the cases. The role of aspirin remains very controversial. A number of studies suggest an association between this potential mitochondrial toxin and Reye syndrome, but a causal relationship has not been established. Until better understood, it seems advisable to avoid use of aspirin in children exhibiting symptoms suggestive of Reye syndrome.
 ...
PMID:Reye syndrome. 388 30

We compared the levels of hormones and metabolites in the plasma of 37 survivors of Reye's syndrome with the levels in 8 fatal cases, at four time periods within 72 hours of admission. The most prominent differences were found for norepinephrine (NE), which was significantly elevated in fatal cases compared with survivors at all periods. Lactate and dopamine were elevated in the earlier periods. Epinephrine and alpha-amino acid nitrogen were also elevated in fatal cases, but the differences usually were not significant. NE elevation may reflect an increased sympathoadrenal medullary output associated with brain edema, compounded by impaired hepatic clearance of monoamines. Skeletal muscle ischemia from NE-induced vasoconstriction may explain the association between lactic acidemia and the severity of encephalopathy.
 ...
PMID:The metabolic course of Reye's syndrome: distinction between survivors and nonsurvivors. 395 18

The administration of sublethal doses of Escherichia coli O111:B4 endotoxin to starved rats results in significant increases in plasma ammonia, free fatty acids, and serum lactate compared with starved controls. These metabolic alterations are associated with Reye syndrome-like histological findings of hepatic microvesicular fatty accumulation and hepatic ultrastructural evidence of mitochondrial pleomorphism with matrix disruption. This sublethal endotoxin model may help elucidate the relationship between the hepatic mitochondrial injury, characteristic metabolic impairment, and encephalopathy seen in patients with Reye syndrome.
 ...
PMID:Metabolic and mitochondrial morphological changes that mimic Reye syndrome after endotoxin administration to rats. 396 6

Reye's syndrome, an uncommon childhood encephalopathy of uncertain aetiology, is an important cause of mortality and permanent handicap. Since its recognition treatment has changed, current management focusing upon prevention or control of raised intracranial pressure. Prognosis is radically improved by early recognition and prompt transfer to a specialist centre.
 ...
PMID:Reye's syndrome. 401 9

Factor analysis of admission data from 209 Reye's syndrome patients yielded three factors. Factor 1 was associated with encephalopathy, blood ammonia, creatinine kinase (CK), uric acid and, to a lesser extent, bilirubin. This factor was linked to the encephalopathy and hypermetabolic changes in muscle, possibly prostaglandin-mediated proteolysis. Factor 2 was associated with serum alanine aminotransferase (AlaAT) and aspartate aminotransferase (AspAT), and was identified as a hepatic lesion component. These factors correspond to two etiologic components of Reye's syndrome. Salicylate was only weakly associated with neuropathic and hypercatabolic indicators and not at all associated with the hepatic damage indicators.
 ...
PMID:Hepatic and encephalopathic components of Reye's syndrome: factor analysis of admission data from 209 patients. 402 64

Dengue hemorrhagic fever/dengue shock syndrome is a serious manifestation of dengue fever, which is observed predominantly in the tropical regions of the West Pacific and in Southeast Asia and is associated with secondary infections, mainly in children under age 15. A concomitant microangiopathic coagulopathy has been described; moreover, encephalopathy and even Reye's syndrome have been rarely reported. This report describes a 51-year-old man with secondary dengue infection who presented with clinical evidence of severe hepatitis, encephalopathy, cranial nerve palsy, and microangiopathic coagulopathy and who had a favorable outcome. A careful surveillance for the occurrence of secondary dengue in the Western Hemisphere is proposed, and dengue is suggested as a diagnostic possibility in obscure febrile illnesses presenting as either hepatitis, encephalopathy, or coagulopathy in places in which the mosquito vector, Aedes aegypti, is present.
 ...
PMID:Dengue and hepatic failure. 406 82


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>