PEDIATRICS Vol. 34 No. 3 September 1964, pp. 346-356
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 Israels, S.
Right arrow Articles by Ford, J. D.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Israels, S.
Right arrow Articles by Ford, J. D.

CHRONIC ACIDOSIS DUE TO AN ERROR IN LACTATE AND PYRUVATE METABOLISM

Report of Two Cases

S. Israels M.D., F.R.C.P.(C)1, J. C. Haworth M.D., M.R.C.P.1, Beth Gourley M.Sc.1, and J. D. Ford M.Sc.1

1 Department of Clinical Investigation and Research, Winnipeg Children's Hospital, and the Department of Paediatrics, University of Manitoba

A description is given of two unrelated North American Indian children who, at 11 and 9 months of age respectively, were found to have metabolic acidosis, associated with a high concentration of lactate and pyruvate in the blood. Both children were mentally retarded, suffered from convulsions, and showed obesity and muscular hypotonia.

Liver glycogen disease, diabetes mellitus, and chronic hypoxia were excluded in both cases. Decreased removal of pyruvate and lactate from the blood due to some, as yet, undetermined metabolic block appeared to be the most likely causative mechanism. As well as a defect in the metabolism of lactate and pyruvate, evidence of an abnormality of agr-keto glutarate metabolism was found in one case.

Submitted on September 26, 1963
Accepted on May 10, 1964