Abstract
To determine the role of glucagon in idiopathic leucine-sensitive hypoglycemia (LSH) and the effects of oral diazoxide (D), responses to oral L-leucine (L) were studied in 3 LSH children and in 6 controls. In controls mean plasma insulin (IRI), glucose (G) and glucagon (IRG) values were unchanged after L, 150 mg/kg. In LSH children after L, 50 mg/kg, mean±SEM plasma IRI rose from 14±3 to 41±5 μU/ml, mean plasma G fell from 72±4 to 35±3 mg/dl, mean plasma IRG increased 48±8%; each significantly different from beseline (P<0.025). Maximum changes in mean IRI, G and IRG values were at 15, 30 and 45 min.,respectively. In LSH children given L, while on D, 10 mg/kg/day, baseline values were similar, mean peak IRI was significantly lower and mean nadir G was significantly higher (P<0.05 and <0.025 respectively) compared to no D therapy; mean IRG rose less, but not significantly. One LSH child given L while on D, 20 mg/kg/day, showed minimal changes in IRI, G and IRG values. Following arginine infusions in another LSH child, ΔG values were 1 and 12 mg/dl, ΔIRI values were 56 and 21 μU/ml and ΔIRG values were 252 and 254 pg/ml, off and on D therapy, respectively. Peak changes were at 30 min.
Conclusions: 1) In LSH children, given L orally, a rise in IRI precedes the fall in G and the rise in IRG. 2) D therapy blunts all of these changes in proportion to the D dose. 3) D does not directly inhibit IRG release. 4) The rise in IRG following L is secondary to the fall in G.
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Roe, T., Kogut, M. GLUCAGON RESPONSES IN LEUCINE-SENSITIVE HYPOGLYCEMIA. Pediatr Res 11, 521 (1977). https://doi.org/10.1203/00006450-197704000-00905
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DOI: https://doi.org/10.1203/00006450-197704000-00905