Hereditary Fructose Intolerance

Abstract

Abstract. The clinical and metabolic aspects of hereditary fructose intolerance (HFI) are reviewed and some new observations on children with HFI are reported. The acute rise in plasma FFA level which was earlier shown to follow an acute exposure to fructose in these patients, was found to be associated with an increase in epinephrine excretion and a decrease in plasma TG level. Abolition of the fructose induced hypoglycemia by an infusion of glucose after injection of fructose prevented the rise in plasma FFA, but did not modify the increase in plasma lactate or urate concentration. Maintaining normal or high plasma inorganic phosphate levels after fructose injection by infusion of phosphate buffer did not alter the fructose‐induced changes in blood glucose or lactate, or in plasma FFA or urate concentration. The hyperuricemia and hyperuricosuria after fructose were larger in HFI patients than in normal children. Administration of fructose in the small doses of 0.33‐1.3 g/kg daily for 3–5 days brought about a gradual fall in plasma cholesterol level, and a rise in the excretion of urate and 17‐ketogenic steroids, but no increase in proteinuria.

Medical Management of Chronic Liver Diseases in Children (Part I)

“…High levels of fructose-1-phosphate inhibit both gluconeogenesis and glycogenolysis. [48][49][50][51] Tissue toxicity results in hepatic enlargement, hepatic failure, and renal dysfunction.…”