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Elevated Plasma Vitamin B(12) in Patients with Hepatic Glycogen Storage Diseases

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Elevated Plasma Vitamin B(12) in Patients with Hepatic Glycogen Storage Diseases. / Hinkel, Julia; Schmitt, Johannes; Wurm, Michael; Rosenbaum-Fabian, Stefanie; Schwab, Karl Otfried; Jacobsen, Donald W.; Spiekerkoetter, Ute; Fedosov, Sergey N.; Hannibal, Luciana; Gruenert, Sarah C.

I: Journal of Clinical Medicine, Bind 9, Nr. 8, 2326, 08.2020.

Publikation: Bidrag til tidsskrift/Konferencebidrag i tidsskrift /Bidrag til avisTidsskriftartikelForskningpeer review

Harvard

Hinkel, J, Schmitt, J, Wurm, M, Rosenbaum-Fabian, S, Schwab, KO, Jacobsen, DW, Spiekerkoetter, U, Fedosov, SN, Hannibal, L & Gruenert, SC 2020, 'Elevated Plasma Vitamin B(12) in Patients with Hepatic Glycogen Storage Diseases', Journal of Clinical Medicine, bind 9, nr. 8, 2326. https://doi.org/10.3390/jcm9082326

APA

Hinkel, J., Schmitt, J., Wurm, M., Rosenbaum-Fabian, S., Schwab, K. O., Jacobsen, D. W., Spiekerkoetter, U., Fedosov, S. N., Hannibal, L., & Gruenert, S. C. (2020). Elevated Plasma Vitamin B(12) in Patients with Hepatic Glycogen Storage Diseases. Journal of Clinical Medicine, 9(8), [2326]. https://doi.org/10.3390/jcm9082326

CBE

Hinkel J, Schmitt J, Wurm M, Rosenbaum-Fabian S, Schwab KO, Jacobsen DW, Spiekerkoetter U, Fedosov SN, Hannibal L, Gruenert SC. 2020. Elevated Plasma Vitamin B(12) in Patients with Hepatic Glycogen Storage Diseases. Journal of Clinical Medicine. 9(8):Article 2326. https://doi.org/10.3390/jcm9082326

MLA

Vancouver

Hinkel J, Schmitt J, Wurm M, Rosenbaum-Fabian S, Schwab KO, Jacobsen DW o.a. Elevated Plasma Vitamin B(12) in Patients with Hepatic Glycogen Storage Diseases. Journal of Clinical Medicine. 2020 aug;9(8). 2326. https://doi.org/10.3390/jcm9082326

Author

Hinkel, Julia ; Schmitt, Johannes ; Wurm, Michael ; Rosenbaum-Fabian, Stefanie ; Schwab, Karl Otfried ; Jacobsen, Donald W. ; Spiekerkoetter, Ute ; Fedosov, Sergey N. ; Hannibal, Luciana ; Gruenert, Sarah C. / Elevated Plasma Vitamin B(12) in Patients with Hepatic Glycogen Storage Diseases. I: Journal of Clinical Medicine. 2020 ; Bind 9, Nr. 8.

Bibtex

@article{98b198ca851846ef905a7ed9218d652c,
title = "Elevated Plasma Vitamin B(12) in Patients with Hepatic Glycogen Storage Diseases",
abstract = "Background: Hepatic glycogen storage diseases (GSDs) are inborn errors of metabolism affecting the synthesis or breakdown of glycogen in the liver. This study, for the first time, systematically assessed vitamin B(12)status in a large cohort of hepatic GSD patients.Methods: Plasma vitamin B-12, total plasma homocysteine (tHcy) and methylmalonic acid concentrations were measured in 44 patients with hepatic GSDs and compared to 42 healthy age- and gender-matched controls. Correlations of vitamin B(12)status with different disease markers of GSDs (including liver transaminase activities and triglycerides) as well as the vitamin B(12)intake were studied.Results: GSD patients had significantly higher plasma vitamin B(12)concentrations than healthy controls (p= 0.0002). Plasma vitamin B(12)concentration remained elevated in GSD patients irrespective of vitamin B(12)intake. Plasma vitamin B(12)concentrations correlated negatively with triglyceride levels, whereas no correlations were detected with liver transaminase activities (GOT and GPT) in GSD patients. Merging biomarker data of healthy controls and GSD patients showed a positive correlation between vitamin B(12)status and liver function, which suggests complex biomarker associations. A combined analysis of biomarkers permitted a reliable clustering of healthy controls versus GSD patients.Conclusions: Elevated plasma concentration of vitamin B-12(irrespective of B(12)intake) is a common finding in patients with hepatic GSD. The negative correlation of plasma vitamin B(12)with triglyceride levels suggests an influence of metabolic control on the vitamin B(12)status of GSD patients. Elevated vitamin B(12)was not correlated with GOT and GPT in our cohort of GSD patients. Merging of data from healthy controls and GSD patients yielded positive correlations between these biomarkers. This apparent dichotomy highlights the intrinsic complexity of biomarker associations and argues against generalizations of liver disease and elevated vitamin B(12)in blood. Further studies are needed to determine whether the identified associations are causal or coincidental, and the possible impact of chronically elevated vitamin B(12)on GSD.",
keywords = "glycogen storage disease, glycogen, vitamin B-12, cobalamin, liver transaminases, vitamin B(12)intake, BOVINE SERUM-ALBUMIN, METHYLMALONIC ACID, INTRINSIC-FACTOR, HYPERVITAMINEMIA B12, LIPID-METABOLISM, COBALAMIN, DEFICIENCY, FOLATE, AGE, HOMOCYSTEINE",
author = "Julia Hinkel and Johannes Schmitt and Michael Wurm and Stefanie Rosenbaum-Fabian and Schwab, {Karl Otfried} and Jacobsen, {Donald W.} and Ute Spiekerkoetter and Fedosov, {Sergey N.} and Luciana Hannibal and Gruenert, {Sarah C.}",
year = "2020",
month = aug,
doi = "10.3390/jcm9082326",
language = "English",
volume = "9",
journal = "Journal of Clinical Medicine",
issn = "2077-0383",
publisher = "MDPI AG",
number = "8",

}

RIS

TY - JOUR

T1 - Elevated Plasma Vitamin B(12) in Patients with Hepatic Glycogen Storage Diseases

AU - Hinkel, Julia

AU - Schmitt, Johannes

AU - Wurm, Michael

AU - Rosenbaum-Fabian, Stefanie

AU - Schwab, Karl Otfried

AU - Jacobsen, Donald W.

AU - Spiekerkoetter, Ute

AU - Fedosov, Sergey N.

AU - Hannibal, Luciana

AU - Gruenert, Sarah C.

PY - 2020/8

Y1 - 2020/8

N2 - Background: Hepatic glycogen storage diseases (GSDs) are inborn errors of metabolism affecting the synthesis or breakdown of glycogen in the liver. This study, for the first time, systematically assessed vitamin B(12)status in a large cohort of hepatic GSD patients.Methods: Plasma vitamin B-12, total plasma homocysteine (tHcy) and methylmalonic acid concentrations were measured in 44 patients with hepatic GSDs and compared to 42 healthy age- and gender-matched controls. Correlations of vitamin B(12)status with different disease markers of GSDs (including liver transaminase activities and triglycerides) as well as the vitamin B(12)intake were studied.Results: GSD patients had significantly higher plasma vitamin B(12)concentrations than healthy controls (p= 0.0002). Plasma vitamin B(12)concentration remained elevated in GSD patients irrespective of vitamin B(12)intake. Plasma vitamin B(12)concentrations correlated negatively with triglyceride levels, whereas no correlations were detected with liver transaminase activities (GOT and GPT) in GSD patients. Merging biomarker data of healthy controls and GSD patients showed a positive correlation between vitamin B(12)status and liver function, which suggests complex biomarker associations. A combined analysis of biomarkers permitted a reliable clustering of healthy controls versus GSD patients.Conclusions: Elevated plasma concentration of vitamin B-12(irrespective of B(12)intake) is a common finding in patients with hepatic GSD. The negative correlation of plasma vitamin B(12)with triglyceride levels suggests an influence of metabolic control on the vitamin B(12)status of GSD patients. Elevated vitamin B(12)was not correlated with GOT and GPT in our cohort of GSD patients. Merging of data from healthy controls and GSD patients yielded positive correlations between these biomarkers. This apparent dichotomy highlights the intrinsic complexity of biomarker associations and argues against generalizations of liver disease and elevated vitamin B(12)in blood. Further studies are needed to determine whether the identified associations are causal or coincidental, and the possible impact of chronically elevated vitamin B(12)on GSD.

AB - Background: Hepatic glycogen storage diseases (GSDs) are inborn errors of metabolism affecting the synthesis or breakdown of glycogen in the liver. This study, for the first time, systematically assessed vitamin B(12)status in a large cohort of hepatic GSD patients.Methods: Plasma vitamin B-12, total plasma homocysteine (tHcy) and methylmalonic acid concentrations were measured in 44 patients with hepatic GSDs and compared to 42 healthy age- and gender-matched controls. Correlations of vitamin B(12)status with different disease markers of GSDs (including liver transaminase activities and triglycerides) as well as the vitamin B(12)intake were studied.Results: GSD patients had significantly higher plasma vitamin B(12)concentrations than healthy controls (p= 0.0002). Plasma vitamin B(12)concentration remained elevated in GSD patients irrespective of vitamin B(12)intake. Plasma vitamin B(12)concentrations correlated negatively with triglyceride levels, whereas no correlations were detected with liver transaminase activities (GOT and GPT) in GSD patients. Merging biomarker data of healthy controls and GSD patients showed a positive correlation between vitamin B(12)status and liver function, which suggests complex biomarker associations. A combined analysis of biomarkers permitted a reliable clustering of healthy controls versus GSD patients.Conclusions: Elevated plasma concentration of vitamin B-12(irrespective of B(12)intake) is a common finding in patients with hepatic GSD. The negative correlation of plasma vitamin B(12)with triglyceride levels suggests an influence of metabolic control on the vitamin B(12)status of GSD patients. Elevated vitamin B(12)was not correlated with GOT and GPT in our cohort of GSD patients. Merging of data from healthy controls and GSD patients yielded positive correlations between these biomarkers. This apparent dichotomy highlights the intrinsic complexity of biomarker associations and argues against generalizations of liver disease and elevated vitamin B(12)in blood. Further studies are needed to determine whether the identified associations are causal or coincidental, and the possible impact of chronically elevated vitamin B(12)on GSD.

KW - glycogen storage disease

KW - glycogen

KW - vitamin B-12

KW - cobalamin

KW - liver transaminases

KW - vitamin B(12)intake

KW - BOVINE SERUM-ALBUMIN

KW - METHYLMALONIC ACID

KW - INTRINSIC-FACTOR

KW - HYPERVITAMINEMIA B12

KW - LIPID-METABOLISM

KW - COBALAMIN

KW - DEFICIENCY

KW - FOLATE

KW - AGE

KW - HOMOCYSTEINE

U2 - 10.3390/jcm9082326

DO - 10.3390/jcm9082326

M3 - Journal article

VL - 9

JO - Journal of Clinical Medicine

JF - Journal of Clinical Medicine

SN - 2077-0383

IS - 8

M1 - 2326

ER -