BACKGROUND Sunitinib,a multi-targeted tyrosine kinase inhibitor(TKI),has been approved for the salvage treatment of gastrointestinal stromal tumors(GIST).Hyperammonemic encephalopathy is a rare but severe complication...BACKGROUND Sunitinib,a multi-targeted tyrosine kinase inhibitor(TKI),has been approved for the salvage treatment of gastrointestinal stromal tumors(GIST).Hyperammonemic encephalopathy is a rare but severe complication of sunitinib use.Here,we present the case of a 66-year-old male with metastatic GIST without underlying liver cirrhosis who developed sunitinib-induced hyperammonemic encephalopathy.CASE SUMMARY A 66-year-old male with metastatic GIST was admitted because of reduced consciousness.Imatinib was administered as the first-line systemic therapy.He experienced repeated episodes of peritonitis due to tumor perforation,and surgery was performed.Progressive disease was confirmed based on increased liver metastasis,and sunitinib was initiated as a salvage treatment.However,23 d after the third course of sunitinib,he presented to the emergency room with an episode of altered consciousness and behavioral changes.Based on the patient clinical history and examination findings,sunitinib-induced encephalopathy was suspected.Sunitinib was discontinued,and the patient was treated for hyperammonemia.The patient had a normal level of consciousness four days later,and the serum ammonia level gradually decreased.No further neurological symptoms were reported in subsequent follow-ups.CONCLUSION TKI-induced hyperammonemic encephalopathy is potentially life-threatening.Patients receiving TKIs experiencing adverse reactions should undergo systemic evaluation and prompt treatment.展开更多
Hepatic encephalopathy is a medical phenomenon that is described as a neuropsychiatric manifestation of chronic or acute liver disease that is characterized by psychomotor,intellectual and cognitive abnormalities with...Hepatic encephalopathy is a medical phenomenon that is described as a neuropsychiatric manifestation of chronic or acute liver disease that is characterized by psychomotor,intellectual and cognitive abnormalities with emotional/affective and behavioral disturbances.This article focuses on the underlying mechanisms of the condition and the differences between hepatic encephalopathy and noncirrhotic hyperammonemic encephalopathy.Hepatic encephalopathy is a serious condition that can cause neurological death with brain edema and intracranial hypertension.It is assumed that approximately 60%-80% of patients with liver cirrhosis develop hepatic encephalopathy This review explores the complex mechanisms that lead to hepatic encephalopathy.However,noncirrhotic hyperammonemic encephalopathy is not associated with hepatic diseases and has a completely different etiology.Noncirrhotic hyperammonemic encephalopathy is a severe occurrence that is connected with multiple pathogeneses.展开更多
目的探讨丙戊酸钠致高血氨脑病不良反应的发生情况及临床特点,为临床安全用药提供参考。方法计算机检索CNKI、WanFang Data、VIP、PubMed和Web of Science数据库,搜集丙戊酸钠致高血氨脑病的中英文文献病例报道,检索时限为建库至2023年3...目的探讨丙戊酸钠致高血氨脑病不良反应的发生情况及临床特点,为临床安全用药提供参考。方法计算机检索CNKI、WanFang Data、VIP、PubMed和Web of Science数据库,搜集丙戊酸钠致高血氨脑病的中英文文献病例报道,检索时限为建库至2023年3月,提取相关信息进行统计分析。结果纳入37篇文献,共41例患者,其中男性28例(68.3%),女性13例(31.7%),年龄5~78岁,中位年龄41岁。大多数患者均无基础疾病,实验室检查血氨浓度均高于正常范围,临床主要表现为神经系统损害如认知障碍、嗜睡、昏迷等意识障碍;经停药、换药及对症治疗后患者意识均逐渐恢复,血氨浓度恢复正常。结论丙戊酸钠致高血氨脑病临床上不易识别和发现,应用此类药物时应密切监测患者临床表现及血药浓度,一旦发现异常应及时对症处理,保障患者用药安全。展开更多
BACKGROUND Ornithine transcarbamylase deficiency(OTCD)is an X-linked inherited disorder and characterized by marked elevation of blood ammonia.The goal of treatment is to minimize the neurological damage caused by hyp...BACKGROUND Ornithine transcarbamylase deficiency(OTCD)is an X-linked inherited disorder and characterized by marked elevation of blood ammonia.The goal of treatment is to minimize the neurological damage caused by hyperammonemia.OTCD can be cured by liver transplantation(LT).Post-transplant patients can discontinue anti-hyperammonemia agents and consume a regular diet without the risk of developing hyperammonemia.The neurological damage caused by hyperammonemia is almost irreversible.CASE SUMMARY An 11.7-year-old boy presented with headache,vomiting,and altered consciousness.The patient was diagnosed with late-onset OTCD.After nitrogen scavenging treatment and a protein-free diet,ammonia levels were reduced to normal on the third day of admission.Nevertheless,the patient remained in a moderate coma.After discussion,LT was performed.Following LT,the patient’s blood ammonia and biochemical indicators stabilized in the normal range,he regained consciousness,and his nervous system function significantly recovered.Two months after LT,blood amino acids and urine organic acids were normal,and brain magnetic resonance imaging showed a decrease in subcortical lesions.CONCLUSION LT can significantly improve partial neurological impairment caused by late-onset OTCD hyperammonemic encephalopathy,and LT can be actively considered when early drug therapy is ineffective.展开更多
文摘BACKGROUND Sunitinib,a multi-targeted tyrosine kinase inhibitor(TKI),has been approved for the salvage treatment of gastrointestinal stromal tumors(GIST).Hyperammonemic encephalopathy is a rare but severe complication of sunitinib use.Here,we present the case of a 66-year-old male with metastatic GIST without underlying liver cirrhosis who developed sunitinib-induced hyperammonemic encephalopathy.CASE SUMMARY A 66-year-old male with metastatic GIST was admitted because of reduced consciousness.Imatinib was administered as the first-line systemic therapy.He experienced repeated episodes of peritonitis due to tumor perforation,and surgery was performed.Progressive disease was confirmed based on increased liver metastasis,and sunitinib was initiated as a salvage treatment.However,23 d after the third course of sunitinib,he presented to the emergency room with an episode of altered consciousness and behavioral changes.Based on the patient clinical history and examination findings,sunitinib-induced encephalopathy was suspected.Sunitinib was discontinued,and the patient was treated for hyperammonemia.The patient had a normal level of consciousness four days later,and the serum ammonia level gradually decreased.No further neurological symptoms were reported in subsequent follow-ups.CONCLUSION TKI-induced hyperammonemic encephalopathy is potentially life-threatening.Patients receiving TKIs experiencing adverse reactions should undergo systemic evaluation and prompt treatment.
文摘Hepatic encephalopathy is a medical phenomenon that is described as a neuropsychiatric manifestation of chronic or acute liver disease that is characterized by psychomotor,intellectual and cognitive abnormalities with emotional/affective and behavioral disturbances.This article focuses on the underlying mechanisms of the condition and the differences between hepatic encephalopathy and noncirrhotic hyperammonemic encephalopathy.Hepatic encephalopathy is a serious condition that can cause neurological death with brain edema and intracranial hypertension.It is assumed that approximately 60%-80% of patients with liver cirrhosis develop hepatic encephalopathy This review explores the complex mechanisms that lead to hepatic encephalopathy.However,noncirrhotic hyperammonemic encephalopathy is not associated with hepatic diseases and has a completely different etiology.Noncirrhotic hyperammonemic encephalopathy is a severe occurrence that is connected with multiple pathogeneses.
文摘目的探讨丙戊酸钠致高血氨脑病不良反应的发生情况及临床特点,为临床安全用药提供参考。方法计算机检索CNKI、WanFang Data、VIP、PubMed和Web of Science数据库,搜集丙戊酸钠致高血氨脑病的中英文文献病例报道,检索时限为建库至2023年3月,提取相关信息进行统计分析。结果纳入37篇文献,共41例患者,其中男性28例(68.3%),女性13例(31.7%),年龄5~78岁,中位年龄41岁。大多数患者均无基础疾病,实验室检查血氨浓度均高于正常范围,临床主要表现为神经系统损害如认知障碍、嗜睡、昏迷等意识障碍;经停药、换药及对症治疗后患者意识均逐渐恢复,血氨浓度恢复正常。结论丙戊酸钠致高血氨脑病临床上不易识别和发现,应用此类药物时应密切监测患者临床表现及血药浓度,一旦发现异常应及时对症处理,保障患者用药安全。
基金Supported by the Sanming Project of Medicine in Shenzhen,No.SZSM201812005。
文摘BACKGROUND Ornithine transcarbamylase deficiency(OTCD)is an X-linked inherited disorder and characterized by marked elevation of blood ammonia.The goal of treatment is to minimize the neurological damage caused by hyperammonemia.OTCD can be cured by liver transplantation(LT).Post-transplant patients can discontinue anti-hyperammonemia agents and consume a regular diet without the risk of developing hyperammonemia.The neurological damage caused by hyperammonemia is almost irreversible.CASE SUMMARY An 11.7-year-old boy presented with headache,vomiting,and altered consciousness.The patient was diagnosed with late-onset OTCD.After nitrogen scavenging treatment and a protein-free diet,ammonia levels were reduced to normal on the third day of admission.Nevertheless,the patient remained in a moderate coma.After discussion,LT was performed.Following LT,the patient’s blood ammonia and biochemical indicators stabilized in the normal range,he regained consciousness,and his nervous system function significantly recovered.Two months after LT,blood amino acids and urine organic acids were normal,and brain magnetic resonance imaging showed a decrease in subcortical lesions.CONCLUSION LT can significantly improve partial neurological impairment caused by late-onset OTCD hyperammonemic encephalopathy,and LT can be actively considered when early drug therapy is ineffective.
