Encephalopathy and Fatty Degeneration of the Viscera: A Clinicopathologic Analysis of 40 Cases

Bourgeois, Curtis H.; Olson, Lloyd C.; Comer, D. S.; Evans, Hilary; Keschamras, N.; Cotton, Robert B.; Grossman, Richard A.; Smith, Thomas W.

doi:10.1093/ajcp/56.5.558

Cited by 60 publications

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“…Pollack et al (4) found a correlation between the increased initial values of FFA (>0.85 mEq/liter) and poor patient outcome. The increased FFA may result from hepatic dysfunction in RS and may also be the toxin responsible for the hepatic dysfunction (5)(6)(7)(8)(9).…”

mentioning

confidence: 99%

Abnormal polyunsaturated fatty acid patterns of serum lipids in Reye's syndrome.

Ogburn¹,

Sharp²,

Lloyd-Still³

et al. 1982

Proc. Natl. Acad. Sci. U.S.A.

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Fatty acid patterns ofthe serum lipids were measured in 17 children with Reye's syndrome (RS). Serial measurements oftotal serum free fatty acids (FFA) showed that levels were increased during RS and, after recovery, were significantly lower in the patients who survived. Fatty acid patterns of serum FFA, triglycerides, and phospholipids in patients with RS were significantly different from those in controls. In RS the polyunsaturated fatty acid content of phospholipids was less than control values; in the FFA, it was higher. This was consistent with the possible involvement of increased phospholipase activity. These experiences, together with the fact that RS is a metabolic disturbance, suggested the possible importance of evaluation of essential fatty acid status in RS. Therefore, a comprehensive analysis offatty acid composition ofFFA, phospholipids (PL), triglycerides (TG), and cholesteryl esters (CE) in serum was undertaken during RS and after recovery. METHODSSerum samples were collected from 17 patients with RS. All patients had acute encephalopathy and laboratory evidence of hepatic dysfunction. Liver biopsies were performed on six of the patients; they confirmed the diagnosis and revealed diffuse cellular swelling and lipid accumulation. All patients received glucose intravenously and supportive care. Patients 5-7, 11, and 13, whose subdural pressure was monitored, were given mannitol, dexamethasone, and artificial hyperventilation to decrease intracranial pressure. Eight patients received exchange transfusions. Levels of individual and total FFA were also determined in seven patients who recovered from RS.The methods of lipid separation and fatty acid analyses were those used in our studies of effect of age and sex upon fatty acid patterns in serum lipids (17) and in recent studies on polyunsaturated fatty acids (PUFA) in disease (11-16). Samples of serum drawn from patients undergoing treatment for RS were collected, frozen, and sent to the Hormel Institute for analysis.The samples were thawed and a known quantity of heptadecanoic acid was added to a known volume of serum as an internal standard for quantification ofFFA. Lipids were extracted from the samples with methanol/chloroform, 1:1:1, (vol/vol).The extract was dried under a gentle stream ofnitrogen at room temperature to minimize loss of the volatile shorter chain FFA (<12:0), and the lipids were redissolved in 0.1 ml ofchloroform and spotted onto a 20 x 20 cm sheet of silicic acid-impregnated ITLC paper (Gelman Sciences, Ann Arbor, MI). Chromatograms were developed in petroleum ether (30-600C)/diethyl ether/acetic acid, 90:10:1 (vol/vol), sprayed with 0.1% dichlorofluorescein, and illuminated with UV light. FFA, PL, TG, and CE appeared as distinct bands which were cut apart and put into glass tubes with Teflon-lined caps. These were transesterified with 2 ml of 14% BF3 in methanol (wt/vol) at 759C for 1 hr. After esterification, the methyl esters were extracted with petroleum ether (30-60°C); the samples were taken to dryness under a ...

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confidence: 99%

Abnormal polyunsaturated fatty acid patterns of serum lipids in Reye's syndrome.

Ogburn¹,

Sharp²,

Lloyd-Still³

et al. 1982

Proc. Natl. Acad. Sci. U.S.A.

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“…The strong association between ingestion of salicylate during the antecedent viral illness and the onset of RS, and the accumulation of fatty acids in RS (15)(16)(17)(18), prompted us to examine the effect of salicylic acid on fatty acid metabolism. The salicylate concentrations used herein are similar to the serum levels observed in RS patients (1 1-14).…”

Section: Discussionmentioning

confidence: 99%

“…Because of the high levels of salicylic acid in the plasma of many RS patients, the role of aspirin in the RS disease process has received particular attention (1 1 -14). Mitochondrial defects and an accumulation of free fatty acids (15)(16)(17)(18) and dicarboxylic acids (19,20) in RS suggest an abnormality in fatty acid metabolism. Herein we report the effect of salicylic acid on mitochondrial-peroxisomal fatty acid catabolism.…”

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confidence: 99%

Effect of Salicylic Acid on Mitochondrial-Peroxisomal Fatty Acid Catabolism

et al. 1988

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“…We found that: ( I ) Ti is more prolonged with resistive than with elastic loads; (2) T tends to increase with loading, more so with resistive loads; and ( 3 ) peak nasal pressure is greater with resistive than with elastic loads. These findings suggest that: ( I ) control of Ti is flow dependent; (2) instantaneous frequency tends to decrease with loading; and ( 3 ) if peak nasal pressure reflects tension developed by the respiratory muscles, the latter does not represent the inhibitory information needed to terminate inspiration. REFERENCES …”

mentioning

confidence: 96%

Deficient Activity of Hepatic Pyruvate Dehydrogenase and Pyruvate Carboxylase in Reye's Syndrome

Robinson

Cutz

1977

Pediatr Res

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Encephalopathy and Fatty Degeneration of the Viscera: A Clinicopathologic Analysis of 40 Cases

Cited by 60 publications

References 0 publications

Abnormal polyunsaturated fatty acid patterns of serum lipids in Reye's syndrome.

Abnormal polyunsaturated fatty acid patterns of serum lipids in Reye's syndrome.

Effect of Salicylic Acid on Mitochondrial-Peroxisomal Fatty Acid Catabolism

Deficient Activity of Hepatic Pyruvate Dehydrogenase and Pyruvate Carboxylase in Reye's Syndrome

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