Diabetes Genes

Effects Of Sulphonylurea On The Brain

KCNJ11 and ABCC8 Neonatal Diabetes: Effects on the brain

Around 20% of patients with KCNJ11 or ABCC8 Neonatal Diabetes will have delayed development as well as diabetes. In these people the KCNJ11 and ABCC8 genetic change affects the potassium channels in the brain in addition to the channels in the pancreas which control insulin release. This is known as DEND syndrome as the key features are : Developmental delay, Epilepsy and Neonatal Diabetes. When it is less marked epilepsy is rare and it is called intermediate DEND syndrome (iDEND)


Developmental delay affects approximately 20% of children with KCNJ11 or ABCC8 neonatal diabetes. It is important to identify those patients whose genetic change means they are at high risk of developmental delay since early treatment with high dose sulphonylureas (around 1mg/kg/day) is likely to achieve the best outcome.

For further information or support please contact Professor Andrew Hattersley a.t.hattersley@exeter.ac.uk or Dr Maggie Shepherd on +44 (0) 1392 40 8261 or m.shepherd1@nhs.net

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