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Orthotopic liver transplantation in glycogen storage disease type la: Perioperative glucose and lactate homeostasis

Wilke, M. V. M. B., De Kleine, R. H., Wietasch, J. K. G., Van Amerongen, C. C. A., Blokzijl, H., Van Spronsen, F. J., Schwartz, I. V. D. & Derks, T. G. J., 25-Feb-2016, In : Journal of Inborn Errors of Metabolism and Screening. 4

Research output: Contribution to journal › Article › Academic › peer-review

APA

Wilke, M. V. M. B., De Kleine, R. H., Wietasch, J. K. G., Van Amerongen, C. C. A., Blokzijl, H., Van Spronsen, F. J., Schwartz, I. V. D., & Derks, T. G. J. (2016). Orthotopic liver transplantation in glycogen storage disease type la: Perioperative glucose and lactate homeostasis. Journal of Inborn Errors of Metabolism and Screening, 4. https://doi.org/10.1177/2326409816649599

Author

Wilke, Matheus V. M. B. ; De Kleine, Ruben H. ; Wietasch, J.K.G. ; Van Amerongen, Cynthia C. A. ; Blokzijl, Hans ; Van Spronsen, Francjan J. ; Schwartz, Ida V. D. ; Derks, Terry G. J. / Orthotopic liver transplantation in glycogen storage disease type la: Perioperative glucose and lactate homeostasis. In: Journal of Inborn Errors of Metabolism and Screening. 2016 ; Vol. 4.

Harvard

Wilke, MVMB, De Kleine, RH, Wietasch, JKG, Van Amerongen, CCA, Blokzijl, H, Van Spronsen, FJ, Schwartz, IVD & Derks, TGJ 2016, 'Orthotopic liver transplantation in glycogen storage disease type la: Perioperative glucose and lactate homeostasis', Journal of Inborn Errors of Metabolism and Screening, vol. 4. https://doi.org/10.1177/2326409816649599

Standard

Orthotopic liver transplantation in glycogen storage disease type la: Perioperative glucose and lactate homeostasis. / Wilke, Matheus V. M. B.; De Kleine, Ruben H.; Wietasch, J.K.G.; Van Amerongen, Cynthia C. A.; Blokzijl, Hans; Van Spronsen, Francjan J.; Schwartz, Ida V. D.; Derks, Terry G. J.

In: Journal of Inborn Errors of Metabolism and Screening, Vol. 4, 25.02.2016.

Research output: Contribution to journal › Article › Academic › peer-review

Vancouver

Wilke MVMB, De Kleine RH, Wietasch JKG, Van Amerongen CCA, Blokzijl H, Van Spronsen FJ et al. Orthotopic liver transplantation in glycogen storage disease type la: Perioperative glucose and lactate homeostasis. Journal of Inborn Errors of Metabolism and Screening. 2016 Feb 25;4. https://doi.org/10.1177/2326409816649599

BibTeX

@article{dda7cb58fa8e440587b64c6ae3d57fea,
title = "Orthotopic liver transplantation in glycogen storage disease type la: Perioperative glucose and lactate homeostasis",
abstract = "Glycogen storage disease type 1a (GSD 1a) is a rare inborn error of metabolism. It causes severe fasting intolerance and lactic acidosis due to the deficiency of glucose-6-phosphatase enzyme. Blood glucose and lactate concentrations from 2 patients with GSD 1a were retrospectively compared to a control group of patients with familial amyloid polyneuropathy. Carbohydrate intake and infusions were compared to experimental data based on stable isotope studies. Perioperative lactate concentrations were significantly higher in our 2 patients with GSD 1a (median 15.0 mmol/L; range 9.9-22.0 mmol/L) versus 8 controls. In one patient, despite normal blood glucose concentrations, lactate acidosis was probably caused by a combination of the disease itself, insufficient (par)enteral carbohydrate intake, Ringer lactate infusions, and circulatory insufficiency. Patients with GSD 1a carry an increased risk of lactic acidosis during orthotopic liver transplantation compared to non-GSD patients. Multidisciplinary perioperative care is essential to prevent significant complications.",
keywords = "Familial amyloid polyneuropathy, Glucose blood, Glycogen storage disease type la, Hypoglycemia, Lactic acidemia, Liver transplantation, glucose, lactic acid, adult, article, bleeding, carbohydrate intake, clinical article, controlled study, female, glucose homeostasis, glycogen storage disease type 1, glycogen storage disease type 1a, human, hypoglycemia, lactic acidosis, liver transplantation, male, priority journal, retrospective study, young adult",
author = "Wilke, {Matheus V. M. B.} and {De Kleine}, {Ruben H.} and J.K.G. Wietasch and {Van Amerongen}, {Cynthia C. A.} and Hans Blokzijl and {Van Spronsen}, {Francjan J.} and Schwartz, {Ida V. D.} and Derks, {Terry G. J.}",
year = "2016",
month = feb,
day = "25",
doi = "10.1177/2326409816649599",
language = "English",
volume = "4",
journal = "Journal of Inborn Errors of Metabolism and Screening",
issn = "2326-4098",
publisher = "SAGE Publications Inc.",

}

RIS

TY - JOUR

T1 - Orthotopic liver transplantation in glycogen storage disease type la: Perioperative glucose and lactate homeostasis

AU - Wilke, Matheus V. M. B.

AU - De Kleine, Ruben H.

AU - Wietasch, J.K.G.

AU - Van Amerongen, Cynthia C. A.

AU - Blokzijl, Hans

AU - Van Spronsen, Francjan J.

AU - Schwartz, Ida V. D.

AU - Derks, Terry G. J.

PY - 2016/2/25

Y1 - 2016/2/25

N2 - Glycogen storage disease type 1a (GSD 1a) is a rare inborn error of metabolism. It causes severe fasting intolerance and lactic acidosis due to the deficiency of glucose-6-phosphatase enzyme. Blood glucose and lactate concentrations from 2 patients with GSD 1a were retrospectively compared to a control group of patients with familial amyloid polyneuropathy. Carbohydrate intake and infusions were compared to experimental data based on stable isotope studies. Perioperative lactate concentrations were significantly higher in our 2 patients with GSD 1a (median 15.0 mmol/L; range 9.9-22.0 mmol/L) versus 8 controls. In one patient, despite normal blood glucose concentrations, lactate acidosis was probably caused by a combination of the disease itself, insufficient (par)enteral carbohydrate intake, Ringer lactate infusions, and circulatory insufficiency. Patients with GSD 1a carry an increased risk of lactic acidosis during orthotopic liver transplantation compared to non-GSD patients. Multidisciplinary perioperative care is essential to prevent significant complications.

AB - Glycogen storage disease type 1a (GSD 1a) is a rare inborn error of metabolism. It causes severe fasting intolerance and lactic acidosis due to the deficiency of glucose-6-phosphatase enzyme. Blood glucose and lactate concentrations from 2 patients with GSD 1a were retrospectively compared to a control group of patients with familial amyloid polyneuropathy. Carbohydrate intake and infusions were compared to experimental data based on stable isotope studies. Perioperative lactate concentrations were significantly higher in our 2 patients with GSD 1a (median 15.0 mmol/L; range 9.9-22.0 mmol/L) versus 8 controls. In one patient, despite normal blood glucose concentrations, lactate acidosis was probably caused by a combination of the disease itself, insufficient (par)enteral carbohydrate intake, Ringer lactate infusions, and circulatory insufficiency. Patients with GSD 1a carry an increased risk of lactic acidosis during orthotopic liver transplantation compared to non-GSD patients. Multidisciplinary perioperative care is essential to prevent significant complications.

KW - Familial amyloid polyneuropathy

KW - Glucose blood

KW - Glycogen storage disease type la

KW - Hypoglycemia

KW - Lactic acidemia

KW - Liver transplantation

KW - glucose

KW - lactic acid

KW - adult

KW - article

KW - bleeding

KW - carbohydrate intake

KW - clinical article

KW - controlled study

KW - female

KW - glucose homeostasis

KW - glycogen storage disease type 1

KW - glycogen storage disease type 1a

KW - human

KW - hypoglycemia

KW - lactic acidosis

KW - liver transplantation

KW - male

KW - priority journal

KW - retrospective study

KW - young adult

U2 - 10.1177/2326409816649599

DO - 10.1177/2326409816649599

M3 - Article

VL - 4

JO - Journal of Inborn Errors of Metabolism and Screening

JF - Journal of Inborn Errors of Metabolism and Screening

SN - 2326-4098

ER -

ID: 118535296

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