Introduction: Congenital hyperinsulinism (CHI) is characterised by abnormally regulated and excessive insulin secretion by pancreatic β cells. First line management includes an oral suspension of Diazoxide but a standardised formulation is not universally employed. Anecdotal evidence suggests that different formulations can alter the management of glucose levels. Lack of glucose control can lead to permanent brain damage and adversely affect neuro development.
Aims: To assess i) the information given to general practitioners (GPs) and carers about the Diazoxide formulation, and ii) effects on home blood glucose levels observed with changes in the Diazoxide formulation.
Methods: The management of 33 CHI patients treated with Diazoxide suspension was evaluated by i) reviewing electronic GP letters or nurses records and ii) a structured telephone interview with carers.
Results: Electronic GP letters and nurses records were available for 29/33 patients. Of these, 14% (4/29) specified the Diazoxide formulation as Proglycem (Gates Pharmaceuticals, USA). The specific Diazoxide formulation was not explicitly stated in the remaining. Telephone interviews with carers were conducted for 24/33 patients. Of these, 29% (7/24) children were on Proglycem and 96% (23/24) reported changes to the formulation when a repeat prescription was obtained from the GP. Among the latter, 48% (11/23) had variations in blood glucose control and hypoglycaemia in association with a change to Diazoxide formulations other than Proglycem.
Conclusion: Recurrence of hypoglycaemia was reported in almost half of the children with CHI when a formulation other than Proglycem was prescribed. Proglycem should be stated as the formulation of choice for Diazoxide suspension in all written communication to GPs and in the verbal information given to carers.
09 - 11 Nov 2011
British Society for Paediatric Endocrinology and Diabetes