Glycogen storage disease type Ia is a genetic disease caused by glucose-6-phosphatase deficiency, which often causes hypoglycemia, hyperuricemia, hypertriglyceridemia, and lactic acidemia. Severe lactic acidosis is a serious complication of glycogen storage disease type Ia. This study aimed to analyze the clinical features and treatment of glycogen storage disease type Ia with severe lactic acidosis. We performed a retrospective analysis of the diagnosis and treatment of a patient with glycogen storage disease type Ia with severe lactic acidosis admitted to our department during the COVID-19 period and reviewed the related literature. The patient was admitted to our hospital for chest pain and vomiting of three days duration, which was accompanied by dyspnea for five hours. The patient has had a type Ia glycogen storage disease for 11 years, and arterial blood gas analysis revealed a pH of 7.192 and lactic acid levels of 26.77 mmol/L. However, the patient was diagnosed with type Ia glycogen storage disease alongside severe lactic acidosis. After 4 hours of hemodialysis and 36 hours of continuous blood purification, the patient had no shortness of breath, dyspnea, nausea, or vomiting. Biochemical retesting indicated a pH of 7.482, lactic acid levels of 7.41 mmol/L, and blood glucose levels of 10.7 mmol/L. Glycogen storage disease type Ia can lead to life-threatening severe lactic acidosis. Continuous renal replacement therapy is an effective treatment for severe lactic acidosis. Therefore, as prevention and control are being carried out during the COVID-19 period, attention should be given to the treatment of special populations, such as those with glycogen storage disease to prevent serious complications.
| Published in | American Journal of Pediatrics (Volume 7, Issue 4) |
| DOI | 10.11648/j.ajp.20210704.19 |
| Page(s) | 229-233 |
| Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
| Copyright |
Copyright © The Author(s), 2021. Published by Science Publishing Group |
Glycogen Storage Disease, Lactic Acidosis, Continuous Renal Replacement Therapy
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APA Style
Yu Luo, Fang Yang, Ding Liu, Zhiqiang Guo, Chongfeng Chen. (2021). Blood Purification Technology in the Treatment of Type Ia Glycogen Storage Disease Complicated with Severe Lactic Acidosis: A Case Report and Literature Review. American Journal of Pediatrics, 7(4), 229-233. https://doi.org/10.11648/j.ajp.20210704.19
ACS Style
Yu Luo; Fang Yang; Ding Liu; Zhiqiang Guo; Chongfeng Chen. Blood Purification Technology in the Treatment of Type Ia Glycogen Storage Disease Complicated with Severe Lactic Acidosis: A Case Report and Literature Review. Am. J. Pediatr. 2021, 7(4), 229-233. doi: 10.11648/j.ajp.20210704.19
AMA Style
Yu Luo, Fang Yang, Ding Liu, Zhiqiang Guo, Chongfeng Chen. Blood Purification Technology in the Treatment of Type Ia Glycogen Storage Disease Complicated with Severe Lactic Acidosis: A Case Report and Literature Review. Am J Pediatr. 2021;7(4):229-233. doi: 10.11648/j.ajp.20210704.19
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Download@article{10.11648/j.ajp.20210704.19,
author = {Yu Luo and Fang Yang and Ding Liu and Zhiqiang Guo and Chongfeng Chen},
title = {Blood Purification Technology in the Treatment of Type Ia Glycogen Storage Disease Complicated with Severe Lactic Acidosis: A Case Report and Literature Review},
journal = {American Journal of Pediatrics},
volume = {7},
number = {4},
pages = {229-233},
doi = {10.11648/j.ajp.20210704.19},
url = {https://doi.org/10.11648/j.ajp.20210704.19},
eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajp.20210704.19},
abstract = {Glycogen storage disease type Ia is a genetic disease caused by glucose-6-phosphatase deficiency, which often causes hypoglycemia, hyperuricemia, hypertriglyceridemia, and lactic acidemia. Severe lactic acidosis is a serious complication of glycogen storage disease type Ia. This study aimed to analyze the clinical features and treatment of glycogen storage disease type Ia with severe lactic acidosis. We performed a retrospective analysis of the diagnosis and treatment of a patient with glycogen storage disease type Ia with severe lactic acidosis admitted to our department during the COVID-19 period and reviewed the related literature. The patient was admitted to our hospital for chest pain and vomiting of three days duration, which was accompanied by dyspnea for five hours. The patient has had a type Ia glycogen storage disease for 11 years, and arterial blood gas analysis revealed a pH of 7.192 and lactic acid levels of 26.77 mmol/L. However, the patient was diagnosed with type Ia glycogen storage disease alongside severe lactic acidosis. After 4 hours of hemodialysis and 36 hours of continuous blood purification, the patient had no shortness of breath, dyspnea, nausea, or vomiting. Biochemical retesting indicated a pH of 7.482, lactic acid levels of 7.41 mmol/L, and blood glucose levels of 10.7 mmol/L. Glycogen storage disease type Ia can lead to life-threatening severe lactic acidosis. Continuous renal replacement therapy is an effective treatment for severe lactic acidosis. Therefore, as prevention and control are being carried out during the COVID-19 period, attention should be given to the treatment of special populations, such as those with glycogen storage disease to prevent serious complications.},
year = {2021}
}
TY - JOUR T1 - Blood Purification Technology in the Treatment of Type Ia Glycogen Storage Disease Complicated with Severe Lactic Acidosis: A Case Report and Literature Review AU - Yu Luo AU - Fang Yang AU - Ding Liu AU - Zhiqiang Guo AU - Chongfeng Chen Y1 - 2021/12/29 PY - 2021 N1 - https://doi.org/10.11648/j.ajp.20210704.19 DO - 10.11648/j.ajp.20210704.19 T2 - American Journal of Pediatrics JF - American Journal of Pediatrics JO - American Journal of Pediatrics SP - 229 EP - 233 PB - Science Publishing Group SN - 2472-0909 UR - https://doi.org/10.11648/j.ajp.20210704.19 AB - Glycogen storage disease type Ia is a genetic disease caused by glucose-6-phosphatase deficiency, which often causes hypoglycemia, hyperuricemia, hypertriglyceridemia, and lactic acidemia. Severe lactic acidosis is a serious complication of glycogen storage disease type Ia. This study aimed to analyze the clinical features and treatment of glycogen storage disease type Ia with severe lactic acidosis. We performed a retrospective analysis of the diagnosis and treatment of a patient with glycogen storage disease type Ia with severe lactic acidosis admitted to our department during the COVID-19 period and reviewed the related literature. The patient was admitted to our hospital for chest pain and vomiting of three days duration, which was accompanied by dyspnea for five hours. The patient has had a type Ia glycogen storage disease for 11 years, and arterial blood gas analysis revealed a pH of 7.192 and lactic acid levels of 26.77 mmol/L. However, the patient was diagnosed with type Ia glycogen storage disease alongside severe lactic acidosis. After 4 hours of hemodialysis and 36 hours of continuous blood purification, the patient had no shortness of breath, dyspnea, nausea, or vomiting. Biochemical retesting indicated a pH of 7.482, lactic acid levels of 7.41 mmol/L, and blood glucose levels of 10.7 mmol/L. Glycogen storage disease type Ia can lead to life-threatening severe lactic acidosis. Continuous renal replacement therapy is an effective treatment for severe lactic acidosis. Therefore, as prevention and control are being carried out during the COVID-19 period, attention should be given to the treatment of special populations, such as those with glycogen storage disease to prevent serious complications. VL - 7 IS - 4 ER -
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