Abstract:The progression of neurological abnormalities through four or five clinically distinguishable levels of deepening coma and the development of a fatty liver are the hallmarks of Reye syndrome. A number of animal models have been described that result in fatty liver formation with minimal, static, or catastrophic neurological changes. In this study, we attempted to produce neurological features in rabbits that reflected a rostral-caudal progression of abnormalities that could be categorized into clinically disti… Show more
“…There were several notable differences in RBC phospholipid fatty acids between RS patients (nos. [8][9][10][11][12][13][14] and ICU controls during the first 48 h of hospital admission. In …”
Section: Resultsmentioning
confidence: 99%
“…In vitro and in vivo experiments in animals have demonstrated that polyunsaturated fatty acids can cause cerebral edema ( 6 4 , a cardinal clinical feature of RS. In a rabbit model of RS, Kang et al (9) have shown that intracisternal injection of free PUFA leads to a reproducible dose-titratable encephalopathy; they and others have proposed that PUFA may be acting as toxins in RS. There have been no attempts to measure the lipid profile of any tissue during acute RS. Since the majority of PUFA in the cells is in phospholipids, we believe tissue lipid analysis could add to the understanding of the role of PUFA in this syndrome.…”
ABSTRACT. Previous studies have demonstrated alterations in plasma free fatty acid content in Reye's syndrome (RS). We have therefore studied erythrocyte membrane lipids to determine if there are concomitant structural and functional modifications attributable to RS. Erythrocyte lipids were measured in children with RS and in critically ill children also requiring intensive care (ICU). In comatose RS patients erythrocyte phospholipid arachidonate was increased 2-fold relative to control ICU patients: 20.46 f 2.14 versus 10.41 f 2.32% of total erythrocyte phospholipid, p < 0.05. RS coma patients also demonstrated an increased ratio of erythrocyte phospholipid polyunsaturatedlsaturated fatty acids (0.76 f 0.10) compared to ICU controls (0.48 f 0.08, p < 0.05). Erythrocyte cholesterol was higher in RS patients (79.00 f 6.61 pg/mg protein) than in ICU controls (59.74 f 6.09, p < 0.05). Erythrocyte malondialdehyde generation was decreased in comatose RS patients (404 rl: 28 nmol malondialdehyde/g hemoglobin) versus ICU (517 f 29, p < 0.05). Although plasma vitamin E was depressed in RS patients, the erythrocyte vitamin E concentrations were no different in RS patients than in ICU patients. All RS patients had a typical viral prodrome and either a history of aspirin intake and/or measurable serum salicylate on admission. All of the biochemical abnormalities in RS patients listed above returned to values comparable to those of healthy RS siblings on recovery. The transient nature of these phenomena suggests that they were related to viral infection and/or aspirin rather than to intrinsic differences in lipid metabolism between RS patients and controls. (Pediatr Res 21: 352-356,1987)
“…There were several notable differences in RBC phospholipid fatty acids between RS patients (nos. [8][9][10][11][12][13][14] and ICU controls during the first 48 h of hospital admission. In …”
Section: Resultsmentioning
confidence: 99%
“…In vitro and in vivo experiments in animals have demonstrated that polyunsaturated fatty acids can cause cerebral edema ( 6 4 , a cardinal clinical feature of RS. In a rabbit model of RS, Kang et al (9) have shown that intracisternal injection of free PUFA leads to a reproducible dose-titratable encephalopathy; they and others have proposed that PUFA may be acting as toxins in RS. There have been no attempts to measure the lipid profile of any tissue during acute RS. Since the majority of PUFA in the cells is in phospholipids, we believe tissue lipid analysis could add to the understanding of the role of PUFA in this syndrome.…”
ABSTRACT. Previous studies have demonstrated alterations in plasma free fatty acid content in Reye's syndrome (RS). We have therefore studied erythrocyte membrane lipids to determine if there are concomitant structural and functional modifications attributable to RS. Erythrocyte lipids were measured in children with RS and in critically ill children also requiring intensive care (ICU). In comatose RS patients erythrocyte phospholipid arachidonate was increased 2-fold relative to control ICU patients: 20.46 f 2.14 versus 10.41 f 2.32% of total erythrocyte phospholipid, p < 0.05. RS coma patients also demonstrated an increased ratio of erythrocyte phospholipid polyunsaturatedlsaturated fatty acids (0.76 f 0.10) compared to ICU controls (0.48 f 0.08, p < 0.05). Erythrocyte cholesterol was higher in RS patients (79.00 f 6.61 pg/mg protein) than in ICU controls (59.74 f 6.09, p < 0.05). Erythrocyte malondialdehyde generation was decreased in comatose RS patients (404 rl: 28 nmol malondialdehyde/g hemoglobin) versus ICU (517 f 29, p < 0.05). Although plasma vitamin E was depressed in RS patients, the erythrocyte vitamin E concentrations were no different in RS patients than in ICU patients. All RS patients had a typical viral prodrome and either a history of aspirin intake and/or measurable serum salicylate on admission. All of the biochemical abnormalities in RS patients listed above returned to values comparable to those of healthy RS siblings on recovery. The transient nature of these phenomena suggests that they were related to viral infection and/or aspirin rather than to intrinsic differences in lipid metabolism between RS patients and controls. (Pediatr Res 21: 352-356,1987)
“…Pathological changes in the brain and liver and a poor neurological outcome are correlated with elevations in levels of serum fatty acids and ammonia (DeLong and Glick, 1982;Heubi et al, 1984;Dezateux et al, 1986). In animal models, administration of octanoic acid, other short-to medium-chain-length fatty acids, or polyunsaturated fatty acids can mimic the neuropathological changes of Reye's syndrome (Glasgow and Chase, 1975;Trauner, 1982;Kang et al, 1984).…”
Octanoic acid has been implicated in the pathogenesis of cytotoxic cerebral edema in Reye's syndrome. Using astrocytes from primary culture, we studied the dose-dependent effects of octanoate on cellular volume regulation and metabolism. Astrocyte volume recovery following hypoosmotic swelling was stimulated by 1.0 mM octanoate and inhibited by 3.0 mM octanoate. Parallel effects were obtained at these concentrations on the activity of the Na+,K+-dependent ATPase. Cellular ATP concentrations also were reduced 36% with the higher octanoate concentration. These effects of octanoate may contribute to the severe astrocyte swelling observed in the brains of Reye's syndrome patients.
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