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Abstract
Examination of glucose kinetics, pancreatic alpha and beta cell function, plasma lipids,
urinary acidification and calcium excretion has been undertaken in a patient with
hereditary fructose intolerance. This case was unusual as it was associated with insulin-requiring
diabetes, type IV hyperlipemia, hypercalciuria and renal calculi. He also demonstrated
the previously described fructoseinduced defect of urine acidification. Glucagon and
C-peptide assays showed that the pancreatic alpha cells were stimulated by fructose
and that the beta cells did not respond to fructose. It is not known whether the latter
was due to his diabetes or to the lack of a beta cell response to this sugar. Primed
14C-glucose infusions were used for the first time to study nonsteady state glucose
kinetics in man. They showed that, 24 hours after the last insulin injection and under
basal conditions, the glucose concentrations increased because glucose production
exceeded glucose utilization. However, after the administration of sorbltol the plasma
glucose concentration decreased because glucose production decreased. After the administration
of sorbitol there was no change in the metabolic clearance of glucose. This reflects
the lack of a peripheral insulin effect and is consistent with the lack of any measurable
C-peptide. Glucose utilization also decreased, but this decrease was less than the
decrease in glucose production. Because the metabolic clearance of glucose remained
unchanged, it was concluded that the change in glucose utilization was solely due
to the decrease in glucose concentration. The absence of C-peptide in the plasma indicated
that changes in glucose turnover were not related to any changes in endogenous plasma
insulin. Furthermore, the plasma glucagon concentration increased and, hence, changes
in this hormone could not account for the decrease in glucose production. Therefore,
it was concluded that the sorbitol-induced decline in glucose production was due to
a direct effect on hepatic metabolism.
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Article info
Publication history
Accepted:
February 23,
1976
Footnotes
☆This work was supported by the Medical Research Council of Canada (MT 2834 and MT 2197).
Identification
Copyright
© 1977 Published by Elsevier Inc.
