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[Fructose and sorbitol as energy-supplying substrates for parenteral nutrition].

The glucose substitutes (fructose, sorbitol, xylitol) have significant metabolic advantages in stress situations as compared to glucose. Side-effects are usually not to be expected if indications and dosage are carefully determined. The only contraindication for fructose and for sorbitol is hereditary fructose intolerance. However, this metabolic disease should be known to physicians who are involved in parenteral nutrition. Fructose intolerance might also be diagnosed by the metabolic alterations caused by fructose or by sorbitol administration in such patients. It was shown repeatedly during the last few decades that glucose formed in the liver during metabolism of glucose substitutes and delivered to the circulation is obviously metabolized without an increase in blood glucose concentration and without additional requirement of insulin. No explanation for this amazing, long-observed metabolic effect is known to date. These reproducible results should stimulate new investigations. Such effects should not be rejected or questioned because they do not fit into the hypotheses.

Blood Glucose↗

[Fructose metabolism disorders and infusions].

The authors give an account of views regarding the use of non-glucose energy sources in parenteral nutrition during the immediate post-load period/serious operations, severe injuries). Attention is devoted to the metabolic pathway of fructose and its disorders. In hereditary fructose intolerance an infusion of D-fructose or D-glucitol (= sorbitol) can induce life threatening hypoglycaemia (unless glucose is administered concurrently). According to some views, in subjects with this intolerance the organism is threatened also by hepatic and renal failure; their development may be independent on hypoglycaemia. Fructose and D-glucitol (sorbitol) therefore should not be administered by the parenteral route. This view is supported by cases where hereditary fructose intolerance could not be revealed from the case-history and clinical manifestations. Some countries have already eliminated fructose and D-glucitol (sorbitol) from their pharmacopoeias.

Food, Formulated↗

Changes of liver metabolite concentrations in adults with disorders of fructose metabolism after intravenous fructose by 31P magnetic resonance spectroscopy.

A novel 31P magnetic resonance spectroscopy procedure allows the estimation of absolute concentrations of certain phosphorus-containing compounds in liver. We have validated this approach by measuring ATP, phosphomonesters, and inorganic phosphate (Pi) during fasting and after an i.v. fructose bolus in healthy adults and in three adults with disorders of fructose metabolism and by comparing results with known metabolic concentrations measured chemically. During fasting, the ATP concentration averaged 2.7 +/- 0.3 (SD, n = 9) mmol/L, which, after due correction for other nucleoside triphosphates, was 2.1 mmol/L and corresponded well with known concentrations. Fructose-1-phosphate (F-1-P) could not be measured during fasting; its concentration after fructose was calculated from the difference of the phosphomonester signals before (2.9 +/- 0.2 mmol/L) and after fructose. Pi was 1.4 +/- 0.3 mmol/L and represented the one fourth of Pi visible in magnetic resonance spectra. In the three healthy controls after fructose (200 mg/kg, 20% solution, 2.5 min), the fructokinase-mediated increase of F-1-P was rapid, reaching 4.9 mmol/L within 3 min, whereas the uncorrected ATP decreased from 2.7 to 1.8 mmol/L and the Pi from 1.4 to 0.3 mmol/L. The subsequent decrease of F-1-P, mediated by fructaldolase, was accompanied by an overshooting rise of Pi to 2.7 mmol/L. In the patient with essential fructosuria, the concentrations of F-1-P, ATP, and Pi remained unchanged, confirming that fructokinase was indeed inactive. In the patient with hereditary fructose intolerance, initial metabolic changes were the same as in the controls, but baseline concentrations were not yet reestablished after 7 h, indicating weak fructaldolase activity.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenosine Triphosphate↗