Abstract
A 3 yr old boy had numerous episodes of fatigue, irritability, pallor and sweating dating from 11 mo. of age when he had an episode of symptomatic hypoglycemia with ketonuria. His height and weight were below the 3rd percentile. No hepatomegaly or cataracts were found. Intellectual development, liver and renal functions, electrolytes, T4, hGH and cortisol values were normal. He manifested euphoria, mental confusion, drowsiness, nausea and vomiting 1-5 hrs after oral glycerol in doses of 0.5-1 gm/kg. Oral medium chain triglycerides (1 gm/kg) had similar effects. Once, oral glycerol (1 gm/kg) also provoked hypoglycemia with a decline in blood lactate. A 16 1/2 hr fast also induced hypoglycemia, unresponsive to glucagon. IV glycerol (0.09 gm/kg) induced an immediate loss of consciousness with no change in blood glucose. He recovered spontaneously after 30 min. IV fructose (0.25 gm/kg) was tolerated normally. Leukocytes showed normal activity for fructose-1, 6-diphosphatase, glycerol kinase and glycerol phosphate dehydrogenase. However, plasma dopamine β hydroxylase activities were low and fell significantly following IV or oral glycerol. The restriction of dietary fat intake improved his physical and mental activities. These observations suggest a unique, yet undefined metabolic intolerance to glycerol. Supported by grants from the John A. Hartford Foundation and NIH #HD 03959-06 and HD 06291-03.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Maclaren, N., Cowles, C., Ozand, P. et al. GLYCEROL INTOLERANCE: A NEW SYNDROME. Pediatr Res 8, 435 (1974). https://doi.org/10.1203/00006450-197404000-00573
Issue Date:
DOI: https://doi.org/10.1203/00006450-197404000-00573