Abstract
We have postulated (APS-SPR, Apr. '72) that short and/or medium chain fatty acid accumulation is important in the pathogenesis of RS, causing mitochondrial swelling, cerebral edema, hypoglycemia and fatty viscera. This study reports gas chromatographic quantitation of serum free fatty acids (C8-C18) from 7 patients with RS, 8 controls (C) and 3 patients with acute and chronic liver failure (LF). Mean total free fatty acids (FAs) were much higher in all RS patients (9.5-185 mg%) vs C (0.35-2.75 mg%) and LF (0.69-3.0 mg%). Of greater significance was the 13-1500 fold increase in caprylic acid (C8) in 5 RS patients (vs C or LF). Because the sera were obtained at different stages of the disease the normal C8 levels in 2 patients may have been due to the very mobile nature of serum FAs. One 10 mo. old with coma had a C8 of 170 mg% (95.5% of total FA) on admission. Four hrs. after treatment with I.V. glucose and insulin the C8=0.15 mg% (normal range=0-0.12 mg%), but her status had not changed, necessitating 2 exchange transfusions 12 hrs. apart. C8 before the 2nd transfusion was 12 mg%. She recovered without sequelae and 12 days later C8= 0.08 mg%. All RS cases had appreciable elevations of other medium chain FAs (as high as 1.9 mg% C10). Long chain FAs were also elevated but only in proportion to the total FA. LF patients had no significant FA elevations. These findings implicate C8 (and possibly other medium chain FAs) as an important toxin in RS.
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Mamunes, P., Devries, G., Miller, C. et al. FATTY ACIDS IN REYE“S SYNDROME. Pediatr Res 8, 436 (1974). https://doi.org/10.1203/00006450-197404000-00576
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DOI: https://doi.org/10.1203/00006450-197404000-00576
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