Published in:
Open Access
01-12-2015 | Poster presentation
Sirolimus therapy following subtotal pancreatectomy in neonatal hyperinsulinemic hypoglycaemia: a case report
Authors:
Mary Abraham, Sarah Flanagan, Vinutha Shetty, Glynis Price, Martin deBock, Aris Siafarikas, Sian Ellard, Steven Resnick, Elizabeth Whan, Elizabeth Davis, Timothy Jones, Khalid Hussain, Catherine Choong
Published in:
International Journal of Pediatric Endocrinology
|
Special Issue 1/2015
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Excerpt
Hyperinsulinemic hypoglycaemia (HH) occurs due to an unregulated insulin production from the pancreatic β-cells in the presence of low blood glucose. Mutations in ABCC8 and KCNJ11 are associated with severe HH that is unresponsive to conventional medical treatment. The only treatment for patients with medically unresponsive diffuse HH is a subtotal pancreatectomy. However, following surgery, hypoglycaemia may persist and some patients develop diabetes and malabsorption. Overexpression of the mTOR pathway is contributory to HH. Sirolimus, an mTOR inhibitor, is currently used in the treatment of congenital hemangiomas and in post renal transplant. Senniappan et al [
1] recently reported efficacy of sirolimus in four surgically naïve patients with diffuse HH unresponsive to diazoxide and octreotide. …
