Abnormal glucose homeostasis and fasting intolerance in patients with congenital porto-systemic shunts
Publication date
2023-08-18
Authors
van Albada, Mirjam E.
Shah, Pratik
Derks, Terry G.J.
Fuchs, Sabine A
Jans, Judith J
McLin, Valérie
van der Doef, Hubert P.J.
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Advisors
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Document Type
Article
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Abstract
In physiological glucose homeostasis, the liver plays a crucial role in the extraction of glucose from the portal circulation and storage as glycogen to enable release through glycogenolysis upon fasting. In addition, insulin secreted by the pancreas is partly eliminated from the systemic circulation by hepatic first-pass. Therefore, patients with a congenital porto-systemic shunt present a unique combination of (a) postabsorptive hyperinsulinemic hypoglycaemia (HH) because of decreased insulin elimination and (b) fasting (ketotic) hypoglycaemia because of decreased glycogenolysis. Patients with porto-systemic shunts therefore provide important insight into the role of the portal circulation and hepatic function in different phases of glucose homeostasis.
Keywords
congenital hyperinsulinism, congenital porto-systemic shunt, glucose metabolism, hyperinsulinism, hypoglycaemia, insulin, portal circulation, Endocrinology, Diabetes and Metabolism
Citation
van Albada, M E, Shah, P, Derks, T G J, Fuchs, S, Jans, J J M, McLin, V & van der Doef, H P J 2023, 'Abnormal glucose homeostasis and fasting intolerance in patients with congenital porto-systemic shunts', Frontiers in Endocrinology, vol. 14, 1190473. https://doi.org/10.3389/fendo.2023.1190473