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July 1976, Vol 130, No. 7 >
Article | July 1976

Reye Syndrome-Reply FREE

M. MICHAEL THALER, MD
Am J Dis Child. 1976;130(7):782. doi:10.1001/archpedi.1976.02120080103023.
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In Reply.—The role of ammonia metabolism in the pathogenesis of Reye syndrome and the exact nature of the implicated defect or defects in the urea cycle remain hypothetical, as indicated by the question in the title of my commentary. The fragmentary information available leaves open most of the possibilities suggested by the hypothesis described in that article, including the speculation that orotic acid may be responsible for the characteristic fatty changes observed in the liver of patients with Reye syndrome. Therefore, although the arguments put forward by Sinatra and co-workers may eventually be proved correct, the inadequate state of present knowledge precludes fixed positions on the subject.

The finding of normal urinary orotic acid excretion in a few patients with Reye syndrome does not exclude the possibility that orotic acid levels may become elevated very early in the course of the disease, then return rapidly to near-normal levels during

REFERENCES

1 +
Thaler MM, Hoogenraad NJ, Boswell M:  Reye syndrome due to a novel protein-tolerant variation of ornithine-transcarbamylase deficiency . Lancet 2:438-440, 1974;.
2 +
Thaler MM:  Pathogenesis of Reye syndrome: A working hypothesis . Pediatrics 56:1081-1084, 1975;.
3 +
Statter M, Russell A, Abzug-Horowitz S, et al:  Abnormal orotic acid metabolism associated with acute hyperammonemia in the rat . Biochem Med 9:1-18, 1974;.
4 +
Arashima S, Matsuda I:  A case of carbamyl phosphate synthetase deficiency . Tohoku J Exp Med 107:143-147, 1972;.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

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References

1 +
Thaler MM, Hoogenraad NJ, Boswell M:  Reye syndrome due to a novel protein-tolerant variation of ornithine-transcarbamylase deficiency . Lancet 2:438-440, 1974;.
2 +
Thaler MM:  Pathogenesis of Reye syndrome: A working hypothesis . Pediatrics 56:1081-1084, 1975;.
3 +
Statter M, Russell A, Abzug-Horowitz S, et al:  Abnormal orotic acid metabolism associated with acute hyperammonemia in the rat . Biochem Med 9:1-18, 1974;.
4 +
Arashima S, Matsuda I:  A case of carbamyl phosphate synthetase deficiency . Tohoku J Exp Med 107:143-147, 1972;.

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