Abnormal glucose homeostasis and fasting intolerance in patients with congenital porto-systemic shunts

Mirjam E. van Albada*, Pratik Shah, Terry G.J. Derks, Sabine Fuchs, Judith J.M. Jans, Valérie McLin, Hubert P.J. van der Doef

*Corresponding author for this work

Research output: Contribution to journalShort surveypeer-review

6 Downloads (Pure)

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.

Original languageEnglish
Article number1190473
Number of pages7
JournalFrontiers in Endocrinology
Volume14
DOIs
Publication statusPublished - 18 Aug 2023

Keywords

  • congenital hyperinsulinism
  • congenital porto-systemic shunt
  • glucose metabolism
  • hyperinsulinism
  • hypoglycaemia
  • insulin
  • portal circulation

Fingerprint

Dive into the research topics of 'Abnormal glucose homeostasis and fasting intolerance in patients with congenital porto-systemic shunts'. Together they form a unique fingerprint.

Cite this