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Glucose Kinetics in Leprechaunism: Accelerated Fasting due to Insulin Resistance^*

Departments of Medicine and Pediatrics, Washington University School of Medicine St. Louis, Missouri 63110
Department of Pediatrics, the University of Arkansas for Medical Science Little Rock, Arkansas 72201
Department of Medicine, University of Colorado School of Medicine Denver, Colorado 80220
Department of Medicine, University of Chicago School of Medicine Chicago, Illinois 60601
Department of Pharmacology, University of Virginia School of Medicine Charlottesville, Virginia 22904

Address requests for reprints to: M. Joycelyn Elders, M.D., Department of Pediatrics, University of Arkansas for Medical Sciences, 4301 West Markham, Little Rock, Arkansas 72201.

Postprandial and postabsorptive glucose metabolism was studied in a 3-yr-old girl with leprechaunism by substrate and hormonal measurements and by quantifying hepatic glucose output during continuous infusion of D-[6,6-²H2]- glucose. Hepatic glycogen content and the activity of glycogen synthase and phosphorylase were also measured in the postprandial state on a separate occasion.

During the 4-h postprandial state, plasma glucose, alanine, lactate, β-hydroxybutyrate, and glycerol were normal, as were hepatic glycogen, glycogen synthase, phosphorylase, and hepatic glucose output of 7.5 mg kg^-1 min^-1. Intravenous injection of glucagon (30 g kg^-1) caused an immediate almost 3-fold rise in glucose production consistent with brisk glycogenolysis. During the 8- to 12-h postabsorptive state, however, the patient had elevated levels of glycerol (330-508 M) and β-hydroxybutyrate (3291-3801 M) and decreased levels of glucose (24-29 mg/dl) and alanine (121-135 M) consistent with a much longer period of fasting in the normal child. Furthermore, hepatic glucose output was reduced to 3.9 mg kg^-1 min^-1, and iv glucagon injection failed to increase this rate; both of these observations are consistent with a hepatic state generally found only later in fasting in the normal child.

From these observations we conclude that the hypoglycemia reported in the leprechaunism syndrome is due to an accelerated fasting state secondary to insulin resistance. As with long-fasted, glycogen-depleted normal children, gluconeogenesis alone is often not capable of adequately meeting the child’s large noninsulin- dependent cerebral glucose requirements. (J Clin EndocrinolMetab 51: 988, 1980)

^* This work was supported in part by The National Foundation- March of Dimes and Grants HD-10667, HD-11862, AM-14334, AM- 17042, AM-19905, AM-15901, AM-13941, AM-20595, and AM-20993 from the NIH.

Received January 2, 1980.

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