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

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 circulatio...

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Main Authors: Mirjam E. van Albada, Pratik Shah, Terry G. J. Derks, Sabine Fuchs, Judith J. M. Jans, Valérie McLin, Hubert P. J. van der Doef
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-08-01
Series:Frontiers in Endocrinology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fendo.2023.1190473/full
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author Mirjam E. van Albada
Pratik Shah
Terry G. J. Derks
Sabine Fuchs
Judith J. M. Jans
Valérie McLin
Hubert P. J. van der Doef
author_facet Mirjam E. van Albada
Pratik Shah
Terry G. J. Derks
Sabine Fuchs
Judith J. M. Jans
Valérie McLin
Hubert P. J. van der Doef
author_sort Mirjam E. van Albada
collection DOAJ
description 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.
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spelling doaj.art-2890f2219f7e446abc07dfb33d25ebfc2023-08-18T11:23:58ZengFrontiers Media S.A.Frontiers in Endocrinology1664-23922023-08-011410.3389/fendo.2023.11904731190473Abnormal glucose homeostasis and fasting intolerance in patients with congenital porto-systemic shuntsMirjam E. van Albada0Pratik Shah1Terry G. J. Derks2Sabine Fuchs3Judith J. M. Jans4Valérie McLin5Hubert P. J. van der Doef6Department of Pediatric Endocrinology, University Medical Center Groningen, University of Groningen, Groningen, NetherlandsDepartment of Pediatric Endocrinology, The Royal London Childrens Hospital, Barts Health National Health Service (NHS) Trust and William Harvey Research Institute, Queen Mary University London, London, United KingdomDepartment of Metabolic Diseases, Beatrix Children’s Hospital, University Medical Center Groningen, University of Groningen, Groningen, NetherlandsDepartment of Metabolic Diseases, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, NetherlandsDepartment of Genetics, Section Metabolic Diagnostics, University Medical Center Utrecht, Utrecht, NetherlandsSwiss Pediatric Liver Center, Department of Pediatrics, Obstetrics, and Gynecology, University of Geneva, Geneva, SwitzerlandDepartment of Pediatric Gastroenterology and Hepatology, University Medical Center Groningen, University of Groningen, Groningen, NetherlandsIn 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.https://www.frontiersin.org/articles/10.3389/fendo.2023.1190473/fullhypoglycaemiahyperinsulinismcongenital porto-systemic shuntportal circulationglucose metabolisminsulin
spellingShingle Mirjam E. van Albada
Pratik Shah
Terry G. J. Derks
Sabine Fuchs
Judith J. M. Jans
Valérie McLin
Hubert P. J. van der Doef
Abnormal glucose homeostasis and fasting intolerance in patients with congenital porto-systemic shunts
Frontiers in Endocrinology
hypoglycaemia
hyperinsulinism
congenital porto-systemic shunt
portal circulation
glucose metabolism
insulin
title Abnormal glucose homeostasis and fasting intolerance in patients with congenital porto-systemic shunts
title_full Abnormal glucose homeostasis and fasting intolerance in patients with congenital porto-systemic shunts
title_fullStr Abnormal glucose homeostasis and fasting intolerance in patients with congenital porto-systemic shunts
title_full_unstemmed Abnormal glucose homeostasis and fasting intolerance in patients with congenital porto-systemic shunts
title_short Abnormal glucose homeostasis and fasting intolerance in patients with congenital porto-systemic shunts
title_sort abnormal glucose homeostasis and fasting intolerance in patients with congenital porto systemic shunts
topic hypoglycaemia
hyperinsulinism
congenital porto-systemic shunt
portal circulation
glucose metabolism
insulin
url https://www.frontiersin.org/articles/10.3389/fendo.2023.1190473/full
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