Protein-sensitive and fasting hypoglycemia in children with the hyperinsulinism/hyperammonemia syndrome.

@article{Hsu2001ProteinsensitiveAF,
  title={Protein-sensitive and fasting hypoglycemia in children with the hyperinsulinism/hyperammonemia syndrome.},
  author={Betty Y. L. Hsu and Andrea True Kelly and Paul S Thornton and Cheryl Rochman Greenberg and Louise A Dilling and Charles A. Stanley},
  journal={The Journal of pediatrics},
  year={2001},
  volume={138 3},
  pages={383-9}
}
OBJECTIVE Because the hyperinsulinism/hyperammonemia (HI/HA) syndrome is associated with gain of function mutations in the leucine-stimulated insulin secretion pathway, we examined whether protein feeding or fasting was responsible for hypoglycemia in affected patients. STUDY DESIGN Patients with HI/HA (8 children and 6 adults) were studied. All had dominantly expressed mutations of glutamate dehydrogenase and plasma concentrations of ammonium that were 2 to 5 times normal. The responses to a… CONTINUE READING
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