Abstract
Two hundred consecutive cases of the sudden infant death syndrome were reviewed for the presence of fat in the liver; 14 showed diffuse panlobular microvesicular fatty change indistinguishable from that found in Reye's syndrome. Samples of frozen liver were available in five of the 14 cases; histochemical analysis showed well preserved cytochrome oxidase and succinate dehydrogenase activity in all five, uncharacteristic of Reye's syndrome. Fatty acyl-coenzyme A dehydrogenase activity in the liver was assayed biochemically in two of the same five cases with severe hepatic fatty infiltration; both showed a defect in medium chain acyl-coenzyme A dehydrogenase activity using the substrate octanoyl-coenzyme A. Both cases also showed cerebral oedema in association with fatty infiltration of renal tubules, myocardium, and skeletal muscle, characteristic of Reye's syndrome. It is concluded that diffuse panlobular microvesicular fatty change of the liver in victims of the sudden infant death syndrome, although essentially non-specific, indicates that the state of mitochondrial enzymes should be investigated.
Full text
PDF


Images in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Bellman M. H., Hall S. M. Aetiology of Reye's syndrome. Arch Dis Child. 1983 Sep;58(9):670–672. doi: 10.1136/adc.58.9.670. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Bove K. E., McAdams A. J., Partin J. C., Partin J. S., Hug G., Schubert W. K. The hepatic lesion in Reye's syndrome. Gastroenterology. 1975 Sep;69(3):685–697. [PubMed] [Google Scholar]
- Brandt N. J. Symptoms and signs in organic acidurias. J Inherit Metab Dis. 1984;7 (Suppl 1):23–27. doi: 10.1007/BF03047369. [DOI] [PubMed] [Google Scholar]
- Corey L., Rubin R. J., Bregman D., Gregg M. B. Diagnostic criteria for influenza B-associated Reye's syndrome: clinical vs. pathologic criteria. Pediatrics. 1977 Nov;60(5):702–708. [PubMed] [Google Scholar]
- DeLong G. R., Glick T. H. Encephalopathy of Reye's syndrome: a review of pathogenetic hypotheses. Pediatrics. 1982 Jan;69(1):53–63. [PubMed] [Google Scholar]
- Duran M., De Klerk J. B., Wadman S. K., Bruinvis L., Ketting D. The differential diagnosis of dicarboxylic aciduria. J Inherit Metab Dis. 1984;7 (Suppl 1):48–51. doi: 10.1007/BF03047374. [DOI] [PubMed] [Google Scholar]
- Howat A. J., Bennett M. J., Variend S., Shaw L. Deficiency of medium chain fatty acylcoenzyme A dehydrogenase presenting as the sudden infant death syndrome. Br Med J (Clin Res Ed) 1984 Mar 31;288(6422):976–976. doi: 10.1136/bmj.288.6422.976. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Mitchell R. A., Ram M. L., Arcinue E. L., Chang C. H. Comparison of cytosolic and mitochondrial hepatic enzyme alterations in Reye's syndrome. Pediatr Res. 1980 Nov;14(11):1216–1221. doi: 10.1203/00006450-198011000-00013. [DOI] [PubMed] [Google Scholar]
- Mowat A. P. Reye's syndrome: 20 years on. Br Med J (Clin Res Ed) 1983 Jun 25;286(6383):1999–2001. doi: 10.1136/bmj.286.6383.1999. [DOI] [PMC free article] [PubMed] [Google Scholar]
- REYE R. D., MORGAN G., BARAL J. ENCEPHALOPATHY AND FATTY DEGENERATION OF THE VISCERA. A DISEASE ENTITY IN CHILDHOOD. Lancet. 1963 Oct 12;2(7311):749–752. doi: 10.1016/s0140-6736(63)90554-3. [DOI] [PubMed] [Google Scholar]
- Shaw L., Engel P. C. The purification and properties of ox liver short-chain acyl-CoA dehydrogenase. Biochem J. 1984 Mar 1;218(2):511–520. doi: 10.1042/bj2180511. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Sherlock S. Acute fatty liver of pregnancy and the microvesicular fat diseases. Gut. 1983 Apr;24(4):265–269. doi: 10.1136/gut.24.4.265. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Sherratt H. S., Veitch R. K. Animal models for dicarboxylic aciduria. J Inherit Metab Dis. 1984;7 (Suppl 1):52–56. doi: 10.1007/BF03047375. [DOI] [PubMed] [Google Scholar]
- Sinclair-Smith C., Dinsdale F., Emery J. Evidence of duration and type of illness in children found unexpectedly dead. Arch Dis Child. 1976 Jun;51(6):424–429. doi: 10.1136/adc.51.6.424. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Stanley C. A., Hale D. E., Coates P. M., Hall C. L., Corkey B. E., Yang W., Kelley R. I., Gonzales E. L., Williamson J. R., Baker L. Medium-chain acyl-CoA dehydrogenase deficiency in children with non-ketotic hypoglycemia and low carnitine levels. Pediatr Res. 1983 Nov;17(11):877–884. doi: 10.1203/00006450-198311000-00008. [DOI] [PubMed] [Google Scholar]