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缬草氨酸诱导的高氨血症性脑病用 L-鸟氨酸-L-天冬氨酸治疗:一例报告。

Valproate-induced hyperammonemic encephalopathy treated by L-ornithine-L-aspartate: a case report.

机构信息

Xi'an Central Hospital, Medical College of Xi'an Jiao Tong University, Xi'an Shaanxi, China.

出版信息

Clin Med (Lond). 2023 May;23(3):271-274. doi: 10.7861/clinmed.2023-0137.

DOI:10.7861/clinmed.2023-0137
PMID:37236802
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11046534/
Abstract

A 63-year-old man developed reduced consciousness and dysphagia progressively. Examination and parameters were normal, except for a Glasgow Coma Scale score of seven, and his grading on the swallow water test increased from grade 1 to grade 5. Brain imaging and blood tests were unexplainable except by high plasma ammonia. His past medical history included cerebral infarction, hypertension and epilepsy induced by cerebral hyperperfusion syndrome. He was rceiving antiepileptic treatment of continuously intravenously pumped sodium valproate of 64 mg/h for 4 days, which overlapped for 12 hours with taking 500 mg sustained release tablets. Sodium valproate was stopped; testing demonstrated normal plasma concentrations of sodium valproate and elevated concentrations of ammonia. Ornithine aspartate was administrated. The patient's level of responsiveness and ammonia levels gradually improved. The patient was also being treated with ceftriaxone sodium for a hypostatic pneumonia and with desmopressin for diabetes insipidus. There is an association between sodium valproate and hyperammonaemia and encephalopathy. Immediate recognition of the serious but uncommon adverse effects is essential. To our knowledge this is the first report of ornithine aspartate being used in this disorder.

摘要

一位 63 岁男性逐渐出现意识降低和吞咽困难。检查和参数除格拉斯哥昏迷量表评分为 7 分外均正常,其饮水试验评级从 1 级升至 5 级。除血浆氨升高外,脑影像学和血液检查均无法解释。该患者既往有脑梗死、高血压和因脑高灌注综合征引起的癫痫病史。他正在接受抗癫痫治疗,连续静脉泵入丙戊酸钠 64mg/h,持续 4 天,与每天服用 500mg 控释片重叠 12 小时。停用丙戊酸钠后,检测显示丙戊酸钠的血浆浓度正常,而氨浓度升高。给予门冬氨酸鸟氨酸治疗。患者的反应能力和氨水平逐渐改善。该患者还因坠积性肺炎接受头孢曲松钠治疗,因尿崩症接受去氨加压素治疗。丙戊酸钠与高氨血症和脑病之间存在关联。及时识别这种严重但不常见的不良反应至关重要。据我们所知,这是首例报道使用门冬氨酸鸟氨酸治疗这种疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd88/11046534/28f497af8806/gr0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd88/11046534/17936c15b9ac/gr0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd88/11046534/9efc05f5ecd7/gr0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd88/11046534/28f497af8806/gr0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd88/11046534/17936c15b9ac/gr0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd88/11046534/9efc05f5ecd7/gr0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd88/11046534/28f497af8806/gr0003.jpg

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本文引用的文献

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Valproic acid-induced encephalopathy: A review of clinical features, risk factors, diagnosis, and treatment.丙戊酸诱导的脑病:临床特征、危险因素、诊断和治疗的综述。
Epilepsy Behav. 2021 Jul;120:107967. doi: 10.1016/j.yebeh.2021.107967. Epub 2021 May 15.
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Fulminant Onset of Valproate-Associated Hyperammonemic Encephalopathy.丙戊酸盐相关高氨血症性脑病的暴发性起病
Am J Psychiatry. 2019 Nov 1;176(11):900-903. doi: 10.1176/appi.ajp.2019.18040363.
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Risk factors for valproic acid induced hyperammonemia and its association with cognitive functions.
导致丙戊酸引起的高血氨的风险因素及其与认知功能的关系。
Gen Hosp Psychiatry. 2019 Jul-Aug;59:67-72. doi: 10.1016/j.genhosppsych.2019.05.005. Epub 2019 May 31.
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Hyperammonemia associated with valproic acid concentrations.与丙戊酸浓度相关的高氨血症
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Valproic acid pathway: pharmacokinetics and pharmacodynamics.丙戊酸途径:药代动力学与药效学
Pharmacogenet Genomics. 2013 Apr;23(4):236-41. doi: 10.1097/FPC.0b013e32835ea0b2.
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Hyperammonemia due to valproic acid in the psychiatric setting.精神科环境中丙戊酸所致高氨血症
Am J Psychiatry. 2007 Jul;164(7):1020-7. doi: 10.1176/ajp.2007.164.7.1020.
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Cognitive side effects of valproic acid-induced hyperammonemia in children with epilepsy.丙戊酸诱导的高氨血症对癫痫患儿的认知副作用。
J Clin Psychopharmacol. 2007 Apr;27(2):221-4. doi: 10.1097/01.jcp.0000264973.77581.61.
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Science review: carnitine in the treatment of valproic acid-induced toxicity - what is the evidence?科学综述:肉碱在治疗丙戊酸诱导的毒性中的作用——证据是什么?
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Hepatology. 1999 Sep;30(3):636-40. doi: 10.1002/hep.510300311.