Recoverable, Record-High Lactic Acidosis in a Patient with Glycogen Storage Disease Type 1: A Mixed Type A and Type B Lactate Disorder
Joint Authors
Heyman, Samuel N.
Oster, Yonatan
Wexler, Isaiah D.
Fried, Elchanan
Source
Issue
Vol. 2016, Issue 2016 (31 Dec. 2016), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2016-11-16
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
A 17-year-old patient with GSD type 1a (von Gierke disease) was hospitalized with an extremely elevated serum lactate following an intercurrent infection and interruption of his frequent intake of carbohydrates.
The patient developed shock, oliguric renal failure, and cardiorespiratory failure requiring mechanical ventilation and inotropes.
At the peak of metabolic decompensation and clinical instability, serum lactate reached a level of 47.6 mmol/L which was accompanied by a severe anion gap metabolic acidosis with a pH of 6.8 and bicarbonate of 4 meq/L.
The patient was stabilized with massive infusions of sodium bicarbonate (45 meq/h) and glucose and recovered without the need for dialysis.
This patient illustrates pathophysiologic mechanisms involved in the development of extreme mixed type A and type B lactic acidemia, reflecting altered metabolic pathways in GSD type 1, combined with tissue hypoperfusion.
The rationale for the specific interventions in this case is outlined.
American Psychological Association (APA)
Oster, Yonatan& Wexler, Isaiah D.& Heyman, Samuel N.& Fried, Elchanan. 2016. Recoverable, Record-High Lactic Acidosis in a Patient with Glycogen Storage Disease Type 1: A Mixed Type A and Type B Lactate Disorder. Case Reports in Medicine،Vol. 2016, no. 2016, pp.1-5.
https://search.emarefa.net/detail/BIM-1101107
Modern Language Association (MLA)
Oster, Yonatan…[et al.]. Recoverable, Record-High Lactic Acidosis in a Patient with Glycogen Storage Disease Type 1: A Mixed Type A and Type B Lactate Disorder. Case Reports in Medicine No. 2016 (2016), pp.1-5.
https://search.emarefa.net/detail/BIM-1101107
American Medical Association (AMA)
Oster, Yonatan& Wexler, Isaiah D.& Heyman, Samuel N.& Fried, Elchanan. Recoverable, Record-High Lactic Acidosis in a Patient with Glycogen Storage Disease Type 1: A Mixed Type A and Type B Lactate Disorder. Case Reports in Medicine. 2016. Vol. 2016, no. 2016, pp.1-5.
https://search.emarefa.net/detail/BIM-1101107
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1101107