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Endocrine Abstracts (2015) 39 EP86 | DOI: 10.1530/endoabs.39.EP86

BSPED2015 e-Posters Miscellaneous/other (12 abstracts)

The use of glucagon in the treatment of hypoglycaemia due to congenital hyperinsulinism

Chandni Jadawji 2 , Maria Estebanez 1 , Raja Padidela 1 , Louise Bowden 1 , Lindsey Rigby 1 , John Kinzell 3 , Karen Cosgrove 2 , Mark Dunne 2 & Indraneel Banerjee 1


1Central Manchester University Hospitals, Manchester, UK; 2University of Manchester, Manchester, UK, 3Xeris Pharmaceuticals, Inc., Texas, USA.


Background: Congenital hyperinsulinism (CHI) can cause severe hypoglycaemia with consequent adverse neurodevelopment. Continuous glucagon infusion (CGI) through intravenous and subcutaneous routes has been utilised to achieve glycaemic stability, but the efficacy has not been reported systematically in a CHI cohort.

Aim: We aimed to investigate the efficacy and safety profile of CGI in the management of hypoglycaemia due to CHI.

Methods: The efficacy of CGI was reviewed in a cohort of 31 children with CHI over a 5 year period by assessing the impact on glucose infusion rate (GIR), a marker of the severity of hypoglycaemia, within 48 h of treatment. Factors influencing the severity of CHI, such as K-ATP channel gene mutations, diazoxide unresponsiveness, requirement for second-line treatment with octreotide and sub-total pancreatectomy were also assessed in relation to CGI.

Results: In patients with CHI, CGI in a dose of 5 μg/kg per h administered either intravenously (n=29) or subcutaneously (n=2) reduced GIR from a mean (interquartile range) of 15.9 (8.1) to 11.5 (4.9) mg/kg per min (P=0.001 for difference). This reduction was independent of gender, K-ATP channel gene mutation status, resolution of hyperinsulinism, focal CHI and therapeutic response to diazoxide. The maximum dose of glucagon required to achieve euglycaemia (12.4 (15) μg/kg per min) was directly correlated with the pre-glucagon GIR (R2=0.7, P<0.001), as expected, but not to other factors determining the severity of hypoglycaemia. In 16 children where the duration of glucagon therapy was recorded, 33 (30) days of CGI helped maintaining euglycaemia along with other medical therapies. Only one child receiving subcutaneous glucagon developed a necrolytic migratory erythema (NME) skin rash.

Conclusions: CGI is effective in reducing GIR in patients with CHI in the short and long term management of children with CHI. Although generally safe, the possibility of NME rash should be considered as an adverse event with CGI treatment.

Volume 39

43rd Meeting of the British Society for Paediatric Endocrinology and Diabetes

British Society for Paediatric Endocrinology and Diabetes 

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