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dc.contributor.authorAbraham, M. B.en
dc.contributor.authorShetty, V. B.en
dc.contributor.authorPrice, G.en
dc.contributor.authorSmith, N.en
dc.contributor.authorBock, M. deen
dc.contributor.authorSiafarikas, A.en
dc.contributor.authorResnick, S.en
dc.contributor.authorWhan, E.en
dc.contributor.authorEllard, Sianen
dc.contributor.authorFlanagan, Sarahen
dc.contributor.authorDavis, E.A.en
dc.contributor.authorJones, T. W.en
dc.contributor.authorHussain, K.en
dc.contributor.authorChoong, C. S.en
dc.identifier.citationEfficacy and safety of sirolimus in a neonate with persistent hypoglycaemia following near-total pancreatectomy for hyperinsulinaemic hypoglycaemia. 2015, 28 (11-12):1391-8 J. Pediatr. Endocrinol. Metab.en
dc.description.abstractHyperinsulinaemic hypoglycaemia (HH) is characterised by inappropriate insulin secretion and is the most common cause for persistent neonatal hypoglycaemia. The only treatment available for medically unresponsive hypoglycaemia is a near-total pancreatectomy. A neonate with severe HH, due to a homozygous ABCC8 mutation, was not responsive to treatment with maximal doses of diazoxide and subcutaneous daily octreotide, and underwent a near-total pancreatectomy; however, hypoglycaemia persisted. Introduction of sirolimus, an mTOR (mammalian target of rapamycin) inhibitor, obviated the requirement for glucose infusion. Euglycaemia was achieved with no significant adverse events from the drug. Sirolimus therapy was ceased at 13 months of age. No episodes of persistent hypoglycaemia were observed after cessation of sirolimus. This report demonstrates the successful use of sirolimus for persistent hypoglycaemia in the critically ill infant post pancreatectomy. Sirolimus could be considered in patients with severe HH not responsive to conventional medical and surgical therapy. However, the long-term efficacy and safety with this immunosuppressive drug in very young patients are not assured.en
dc.publisherDe Gruyteren
dc.rightsArchived with thanks to Journal of pediatric endocrinology & metabolism : JPEMen
dc.subjectWessex Classification Subject Headings::Endocrinology::Diabetesen
dc.subjectWessex Classification Subject Headings::Paediatricsen
dc.titleEfficacy and safety of sirolimus in a neonate with persistent hypoglycaemia following near-total pancreatectomy for hyperinsulinaemic hypoglycaemia.en
dc.typeJournal Articleen
dc.typeResearch Support, Non-U.S. Gov'ten
dc.identifier.journalJournal of Pediatric Endocrinology & Metabolism : JPEMen

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