PEDIATRICS Vol. 42 No. 6 December 1968, pp. 889-903
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 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 DeJong, B. P.
Right arrow Articles by Schafer, I. A.
Right arrow Search for Related Content
PubMed
Right arrow Articles by DeJong, B. P.
Right arrow Articles by Schafer, I. A.

FAILURE TO INDUCE SCURVY BY ASCORBIC ACID DEPLETION IN A PATIENT WITH HURLER'S SYNDROME

Bernard P. DeJong M.D.1, W. van B. Robertson Ph.D.1, and Irwin A. Schafer M.D.1

1 Department of Pediatrics, Stanford University School of Medicine and the Stanford Childrens Convalescent Hospital, Palo Alto, California

An infant with Hurler's syndrome was placed on an ascorbic acid-deficient diet at age 10 weeks. The clinical, radiological, and chemical changes that occurred during 1 year on this diet are described. Although the disease was progressive in the skeleton, there was little clinical involvement of the liver, spleen, cornea, and brain. Physical growth and psychomotor development were within normal limits during the year. The urinary excretion of glycosaminoglycans was not altered by ascorbic acid depletion. The clinical variability observed in Hurler patients precludes any conclusions as to the effect of this diet on the natural history of this disease.

Ascorbic acid levels became undetectable in the plasma and buffy coat, respectively, at 16 and 32 weeks of the diet. Even after 52 weeks on the scorbutogenic diet, the patient did not demonstrate any clinical or chemical evidence of scurvy, and wounds healed normally. This raises the question whether isolated ascorbic acid deficiency with an otherwise adequate diet will produce infantile scurvy, or whether patients with Hurler's syndrome have a unique requirement for vitamin C.

Submitted on November 24, 1967
Accepted on March 5, 1968