Studies of glucose turnover and renal function in an unusual case of hereditary fructose intolerance.

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 fructose-induced 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 sorbitol 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.

• Authors: G Steiner, D Wilson, M Vranic
• Journal: The American journal of medicine (Am J Med) Vol. 62 Issue 1 Pg. 150-8 (Jan 1977) ISSN: 0002-9343 [Print] United States
• PMID: 13654 (Publication Type: Case Reports, Journal Article)

Chemical References

• Triglycerides
• Fructose
• Sorbitol
• Glucose

Topics

• Administration, Oral
• Carbohydrate Metabolism, Inborn Errors (metabolism)
• Diabetes Complications
• Fructose (administration & dosage, metabolism)
• Fructose Intolerance (genetics, metabolism, physiopathology)
• Glucose (biosynthesis, metabolism)
• Humans
• Hydrogen-Ion Concentration
• Hyperlipidemias (complications)
• Infusions, Parenteral
• Islets of Langerhans (physiopathology)
• Kidney (physiopathology)
• Kidney Calculi (complications)
• Kinetics
• Male
• Middle Aged
• Pancreas (physiopathology)
• Sorbitol (metabolism)
• Triglycerides (biosynthesis)
• Urine