Abstract
Studies were performed to evaluate glucose homeostasis in children with intrahepatic biliary hypoplasia (IBH) with chronic cholestatic syndrome and growth retardation. Eight I.V. glucagon tolerance tests (30 μg/kg) on five IBH patients (4-12 yrs of age) were compared to 8 controls. Fasting (14-16 hr) glucose was lower in IBH (67 ± 4 mg% vs 80.8 ± 3.67; p < 0.025), and peak rise was significantly less (107 ± 10.6 vs 147 ± 7.9; p < 0.01). Baseline urinary cyclic AMP levels in IBH vs controls (5.8 ± 2 M ± S.E.M. nmoles/mg creatinine vs 4.5 ± 0.4) and 2 hour excretion response to glucagon (31.1 ± 3 vs 28.6 ± 5.2) were not significantly different. Insulin response to glucagon were similar in IBH vs control. Glucagon has previously been shown to lower plasma gluconeogenic amino acids as a indication of hepatic uptake and gluconeogenesis. Fasting plasma alanine was not significantly different in IBH vs control (210 ± 38 umoles/L vs 245 ± 40). Controls had a significant delta decline at 30 minutes post - glucagon of 100 umoles/L for alanine, 66 for glycine and 39 for glutamine plus asparagine. IBH patients failed to elicit a consistent reduction in gluconeogenic amino acids. These studies suggest that hepatic gluconeogenesis and glycogen storage are impaired in chronic liver disease. The noted cyclic AMP response may reflect adequate adenyl cyclase activity in spite of hepatocellular damage.
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Linarelli, L., Rubin, H., Pai, K. et al. METABOLIC RESPONSE TO I.V. GLUCAGON IN CHILDREN WITH CHRONIC LIVER DISEASE. Pediatr Res 8, 435 (1974). https://doi.org/10.1203/00006450-197404000-00568
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DOI: https://doi.org/10.1203/00006450-197404000-00568