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

doi:10.3389/fendo.2023.1190473

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 journal › Short survey › peer-review

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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.

Original language	English
Article number	1190473
Number of pages	7
Journal	Frontiers in Endocrinology
Volume	14
DOIs	https://doi.org/10.3389/fendo.2023.1190473
Publication status	Published - 18 Aug 2023

Keywords

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

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title = "Abnormal glucose homeostasis and fasting intolerance in patients with congenital porto-systemic shunts",

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",

author = "{van Albada}, {Mirjam E.} and Pratik Shah and Derks, {Terry G.J.} and Sabine Fuchs and Jans, {Judith J.M.} and Val{\'e}rie McLin and {van der Doef}, {Hubert P.J.}",

note = "Publisher Copyright: Copyright {\textcopyright} 2023 van Albada, Shah, Derks, Fuchs, Jans, McLin and van der Doef.",

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doi = "10.3389/fendo.2023.1190473",

language = "English",

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T1 - Abnormal glucose homeostasis and fasting intolerance in patients with congenital porto-systemic shunts

AU - van Albada, Mirjam E.

AU - Shah, Pratik

AU - Derks, Terry G.J.

AU - Fuchs, Sabine

AU - Jans, Judith J.M.

AU - McLin, Valérie

AU - van der Doef, Hubert P.J.

PY - 2023/8/18

Y1 - 2023/8/18

N2 - 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.

AB - 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.

KW - congenital hyperinsulinism

KW - congenital porto-systemic shunt

KW - glucose metabolism

KW - hyperinsulinism

KW - hypoglycaemia

KW - insulin

KW - portal circulation

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U2 - 10.3389/fendo.2023.1190473

DO - 10.3389/fendo.2023.1190473

M3 - Short survey

C2 - 37664849

SN - 1664-2392

VL - 14

JO - Frontiers in Endocrinology

JF - Frontiers in Endocrinology

M1 - 1190473

ER -

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

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