Ketogenic diet in a patient with congenital hyperinsulinism: a novel approach to prevent brain damage

@inproceedings{Maiorana2015KetogenicDI,
  title={Ketogenic diet in a patient with congenital hyperinsulinism: a novel approach to prevent brain damage},
  author={Arianna Maiorana and Lucilla Manganozzi and Fabrizio Barbetti and Silvia M Bernabei and Giorgia Gallo and R Cusmai and Stefania Caviglia and Carlo Dionisi-Vici},
  booktitle={Orphanet journal of rare diseases},
  year={2015}
}
BACKGROUND Congenital hyperinsulinism (CHI) is the most frequent cause of hypoglycemia in children. In addition to increased peripheral glucose utilization, dysregulated insulin secretion induces profound hypoglycemia and neuroglycopenia by inhibiting glycogenolysis, gluconeogenesis and lipolysis. This results in the shortage of all cerebral energy substrates (glucose, lactate and ketones), and can lead to severe neurological sequelae. Patients with CHI unresponsive to medical treatment can be… CONTINUE READING
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