PEDIATRICS Vol. 36 No. 2 August 1965, pp. 272-275
This Article
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by SCHNECK, L.
Right arrow Articles by VOLK, B. W.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by SCHNECK, L.
Right arrow Articles by VOLK, B. W.

Fructose Tolerance in Tay-Sachs' Disease

LARRY SCHNECK M.D.1, GUTA PERLE B.A.1, and BRUNO W. VOLK M.D.1

1 Isaac Albert Research Institute of the Jewish Chronic Disease Hospital, Brooklyn 3, New York

1. Children with Tay-Sachs' disease and patients with hereditary fructose intolerance both have severely depressed levels of serum fructose-1-phosphate aldolase.

2. Administration of fructose to three patients with Tay-Sachs' disease did not precipitate the drop in serum glucose and inorganic phosphorus and the rise in serum magnesium that is characteristic of the response to exogenous fructose of patients with hereditary fructose intolerance.

3. The normal tolerance to exogenous fructose displayed by patients with Tay-Sachs' disease is consistent with the findings that their hepatic fructose-1-phosphate aldolase is reduced to only about 60% of normal, in contrast to the virtual absence of this enzyme in cases of hereditary fructose intolerance.

4. It is suggested that even though the serum fructose-1-phosphate aldolase is low in Tay-Sachs' disease, the defect in cerebral ganglioside metabolism may involve galactose rather than fructose.