ESPEYB17 2. Antenatal and Neonatal Endocrinology Neonatal Diabetes Mellitus (5 abstracts)
2.8. Patterns of post-meal insulin secretion in individuals with sulfonylurea-treated KCNJ11 neonatal diabetes show predominance of non-KATP-channel pathways
Bowman P , McDonald TJ , Knight BA , Flanagan SE , Leveridge M , Spaull SR , Shields BM , Hammersley S , Shepherd MH , Andrews RC , Patel KA & Hattersley AT
To read the full abstract: BMJ Open Diabetes Research and Care2019; 7:e000721. PMID: 31908791
Understanding of the molecular mechanisms underlying neonatal diabetes mellitus (NDM) has helped to transform the clinical management of some patients. Those with NDM due to mutations in the KCNJ11/ABCC8 genes can now be switched to oral sulphonylurea treatment and their daily insulin injections stopped. Despite use of high doses of sulphonylureas, severe hypoglycemia is rare in patients with NDM due to KCNJ11 mutations. However anecdotal reports from patients with NDM due to KCNJ11 mutations have suggested that they have mild to moderate hypoglycemia after meals rich in protein/fat and lacking carbohydrate, or after meals smaller than usual in size (1).
This study of a small number of adult patients with two KCNJ11 mutations provides potential insights into the mechanism of hypoglycaemia. Patients with NDM due to KCNJ11 mutations had higher blood glucose levels after a carbohydrate meal compared to after a protein/fat meal. However, their plasma insulin levels were the same for both groups. This observation suggests that the insulin level might be ‘inappropriately high’ for these patients when they eat a protein/fat meal and this may then lead to post-prandial hypoglycaemia. As amino acids (and fatty acids) are known to trigger insulin secretion via alternative pathways (non KATPchannel), this could be one potential mechanism for the dysregulated insulin secretion in response to a protein/fat meal. These observations have important clinical implications for those patients who are on sulphonylurea therapy. These patients should have sufficient carbohydrates in their diet and should not miss any meal, as this will increase the risk of hypoglycaemia.
Reference:
1. Lanning MS, Carmody D, Szczerbinski L, et al. Hypoglycemia in sulfonylurea-treated KCNJ11-neonatal diabetes: Mild-moderate symptomatic episodes occur infrequently but none involving unconsciousness or seizures. Pediatr Diabetes2018 May;19(3):393–397.
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ESPE Yearbook of Paediatric Endocrinology
ISSN 1662-4009 (Online)
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