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

Autor:	van Albada ME; Department of Pediatric Endocrinology, University Medical Center Groningen, University of Groningen, Groningen, Netherlands., Shah P; Department 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 Kingdom., Derks TGJ; Department of Metabolic Diseases, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, Netherlands., Fuchs S; Department of Metabolic Diseases, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, Netherlands., Jans JJM; Department of Genetics, Section Metabolic Diagnostics, University Medical Center Utrecht, Utrecht, Netherlands., McLin V; Swiss Pediatric Liver Center, Department of Pediatrics, Obstetrics, and Gynecology, University of Geneva, Geneva, Switzerland., van der Doef HPJ; Department of Pediatric Gastroenterology and Hepatology, University Medical Center Groningen, University of Groningen, Groningen, Netherlands.
Jazyk:	angličtina
Zdroj:	Frontiers in endocrinology [Front Endocrinol (Lausanne)] 2023 Aug 18; Vol. 14, pp. 1190473. Date of Electronic Publication: 2023 Aug 18 (Print Publication: 2023).
DOI:	10.3389/fendo.2023.1190473
Abstrakt:	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. Competing Interests: VM consults for Mirum Pharmaceuticals, Albireo, and AstraZeneca. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. (Copyright © 2023 van Albada, Shah, Derks, Fuchs, Jans, McLin and van der Doef.)
Databáze:	MEDLINE
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