Glucose production rates in type 1 glycogen storage disease

Journal of Inherited Metabolic Disease
J E CollinsA Aynsley-Green

Abstract

Glucose production rates were measured in six patients with glycogen storage disease type 1 (five type 1A, one type 1B) using a primed continuous infusion of either [3-3H]glucose or [6,6-2H2]glucose. In four patients exogenous glucose was needed to maintain normoglycaemia. At blood glucose concentrations of 2.3-4.7 mmol/L, the endogenous glucose production rates were between 34 and 100% of that predicted for healthy subjects. No relationship was found between the blood glucose concentration and glucose production rates but there was a positive correlation between that of blood lactate and glucose production rate. The initial steady state was perturbed either by reducing the exogenous glucose infusion rate or by giving intravenous glucagon (20 micrograms/kg) or alanine (0.1-0.2 g/kg). Reducing the exogenous glucose infusion rate had little short term effect on glucose production rate. Intravenous glucagon increased the glucose production rate as well as blood glucose and lactate concentrations. A bolus of alanine (0.2 g/kg) given intravenously increased the glucose production rate and blood glucose concentrations but blood lactate concentrations fell. In four of the patients the studies were repeated under similar conditions and...Continue Reading

References

Feb 19, 1976·The New England Journal of Medicine·H L GreeneI M Burr
Jun 1, 1989·Proceedings of the National Academy of Sciences of the United States of America·B KalderonA Lapidot
Mar 13, 1986·The New England Journal of Medicine·W F Schwenk, M W Haymond
Jul 1, 1985·American Journal of Diseases of Children·D R AmbrusoJ M Falletta
Apr 1, 1970·The Journal of Pediatrics·A Sadeghi-NejadB Senior
Jul 1, 1971·Clinica Chimica Acta; International Journal of Clinical Chemistry·C Olsen
Jul 1, 1974·The Journal of Pediatrics·A Sadeghi-NejadB Senior
Jun 1, 1983·Archives of Pathology & Laboratory Medicine·J J BuchinoD M Volk
Sep 1, 1983·European Journal of Pediatrics·J Schaub, K Heyne
Nov 1, 1983·The American Journal of Physiology·Y Shimoni, R Rahamimoff
May 1, 1982·European Journal of Pediatrics·D B DungerM A Preece
Jul 1, 1982·The Journal of Pediatrics·S C KalhanS M Savin
May 1, 1982·Archives of Disease in Childhood·D B Dunger, J V Leonard

❮ Previous
Next ❯

Citations

Jan 1, 1991·Journal of Inherited Metabolic Disease·J V Leonard, G N Thompson
Apr 15, 2011·Journal of Inherited Metabolic Disease·Kaustuv Bhattacharya
Apr 17, 2004·The Journal of Biological Chemistry·Jeng-Jer ShiehJanice Yang Chou
Feb 7, 2019·Journal of Inherited Metabolic Disease·Ronald J A WandersRiekelt H Houtkooper
Aug 28, 2020·Frontiers in Physiology·Anna Prats-PuigAbel López-Bermejo
Jan 22, 2005·The Journal of Biological Chemistry·Abhijit GhoshJanice Yang Chou

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

© 2021 Meta ULC. All rights reserved