Abstract
A one year old North American Indian boy who presented with episodes of vomiting, lethargy and periodic hypoglycaemia was shown to have hereditary fructose intolerance. A liver biopsy showed typical histological changes and fructose-l-phosphate aldolase activity was greatly diminished.
Although blood fructose increased only slightly during an oral fructose tolerance test, he developed severe hypoglycaemia which did not respond to glucagon. Insulin levels remained low. Plasma lactic acid, uric acid, magnesium, methionine, valine, leucine, isoleucine and aspartate aminotransferase rose during the test while potassium and phosphate fell markedly. Continuous EEG monitoring showed no significant changes, but an ECG showed a slight cardiac arrhythmia when potassium was at its lowest level. Urinary excretion of uric acid and magnesium increased during the fructose load, but there was no rise in the excretion of potassium, phosphate, or bicarbonate.
Patients with hereditary fructose intolerance may exhibit a Fanconi syndrome and this has been blamed for the falling plasma potassium during a fructose load. The hypokalemic response to acute fructose loading in this patient has been demonstrated not to be due to renal losses. We speculate that potassium probably entered the intracellular space when fructose was administered.
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Wong, L., Davidson, A., Dooley, K. et al. 574 HEREDITARY FRUCTOSE INTOLERANCE: FATE OF PLASMA POTASSIUM DURING FRUCTOSE LOAD. Pediatr Res 12 (Suppl 4), 459 (1978). https://doi.org/10.1203/00006450-197804001-00579
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DOI: https://doi.org/10.1203/00006450-197804001-00579