卡谷氨酸_Carbaglu_卡谷氨酸片Carglumic说明书
[caption id="attachment_37647" align="alignleft" width="300"]
Carbaglu dispersible tablets 200mg（卡谷氨酸片,カーバグル分散錠）[/caption]
药店国别：
产地国家：日本
处 方 药：是
所属类别：200毫克/片 60片/盒
包装规格：200毫克/片 60片/盒
计价单位：盒
生产厂家中文参考译名:Pola Pharma Inc
生产厂家英文名:Pola Pharma Inc
原产地英文商品名:CARBAGLU 200mg/Tablets 60Tablets/box
原产地英文药品名:Carglumic Acid
中文参考商品译名:CARBAGLU 200毫克/片 60片/盒
中文参考药品译名:卡谷氨酸
简介：
部分中文卡谷氨酸处方资料(仅供参考)
英文名：Carglumic Acid
商标名：CARBAGLU dispersible tablets
中文名：卡谷氨酸分散片
生产商：Pola Pharma Inc.
药品简介
高氨血症治疗：
批准日期：2016年11月
商标名：CARBAGLU dispersible tablets
一般名：Carglumic Acid[JAN]
化学名：(2S )-2-(Carbamoylamino)pentanedioic acid
分子式：C6H10N2O5
分子量：190.15
融点：159～163℃
性状：
它是白色粉末或无色晶体。该产品微溶于水，几乎不溶于有机溶剂。
批准条件：
·制定药品风险管理计划并适当实施。
·由于日本的临床试验数量极为有限，通过对制造和销售后的复审期间的所有管理案例进行使用结果调查，掌握使用该药物的患者的背景信息 ，尽快收集有关该药物安全性和有效性的数据，并采取必要措施正确使用该药物。
药用药理学：
动作N-乙酰谷氨酸合成酶(NAGS)缺乏症的机制是尿素循环障碍之一，一种常染色体隐性遗传病由NAGS基因突变。通过用于尿素循环氨甲酰磷酸合成酶I(CPSI)N-乙酰(NAG)的第一步骤的播放活化所需无法合成，表现出高氨血症。
甲基丙二酸血症，丙酸血症和异戊酸血症被分为有机酸代谢紊乱，一种常染色体隐性遗传性疾病引起的氨基酸代谢途径的酶缺乏症。 NAGS被累积的中间代谢物抑制，从而间歇性地表现出由于蛋白质分解代谢应激引起的高氨血症。
钙谷氨酸是NAG的结构类似物，其代表NAG活化CPSI并活化尿素循环以降低血氨浓度。
适应症：
由于以下疾病导致的高氨血症
·N-乙酰谷氨酸合成酶缺乏症
·异戊酸血症
·甲基丙二酸血症
·丙酸血症
用法与用量：
通常，每天每公斤体重100毫克至250毫克，每天分2至4次，使用时，将其分散在水中并口服给药。 之后，根据患者的情况相应地增加和减少。
包装：分散片
200毫克：5片，60片
(带干燥剂和聚丙烯瓶的聚乙烯盖)
New drug: Calguminic acid (Carbagre) Hyperammonemia treatment drug that activates urea circuit
On December 22, 2016, the hyperammonemia treatment drug calguminic acid (trade name Carbagul dispersible tablet 200 mg) was released. This product is approved for manufacture and marketing on September 28, 2016, and is listed on the drug price on November 18. Adaptation is "hyperammonemia caused by N-acetylglutamate synthase deficiency, isovaleric acidemia, methylmalonic acidemia, propionic acidemia". Beginning from 100 to 250 mg / kg per day, when it is used divided into 2 to 4 times a day, it is dispersed in water and administered orally. After that, it increases and decreases appropriately according to the state of the patient. Also, in order to prevent elevated blood ammonia levels due to diet, it is necessary to instruct patients to take as much as possible before meals.
N-acetylglutamate synthetase (NAGS) deficiency is an autosomal recessive genetic disease that causes a urea circuit abnormality. The synthesis of N-acetylglutamic acid (NAG) necessary for activation of carbamyl phosphate synthase I (CPS I) which is the first step of the urea cycle becomes impossible, causing excessive accumulation of ammonia.
Both isovaleric acidemia, methylmalonic acidemia and propionic acidemia, which are organic acid metabolic disorders, develop hyperammonemia due to enzyme deficiency in the amino acid metabolism pathway. The accumulated intermediary metabolite, organic acid antagonizes NAGS activity, thereby showing the same pathological condition as NAGS deficiency.
With these hyperammonemias, irreversible disorders such as neurological disorder may occur if rapid and appropriate treatment is not performed, which may cause developmental injury and death. For this reason, it is important to control the blood ammonia level quickly by lowering the blood ammonia concentration during treatment. Although treatment varies depending on the condition, drug therapy using dietary and nutritional management such as protein restriction and L-arginine (Algin U) and sodium phenylbutyrate (Bufenyl) which promote excretion of blood ammonia to the outside is the basis ing. However, a number of cases have been reported in the current situation where control of blood ammonia levels is insufficient.
Calgulmic acid is a structural analogue of NAG that activates CPS I and is an agent that decreases blood ammonia concentration by activating the urea cycle. Since it was approved overseas in Europe in 2003 and in the US in 2010, it has been approved in 42 countries worldwide by June 2016.
In Japan, a development request was submitted from the Japanese Society for Congenital Metabolic Disorders, and in April 2010 I got a high reputation for "unapproved drugs for non-approved drugs with high medical necessity" and for this approval It was connected. In addition, this drug is designated as a drug for rare diseases in November 2014.
In domestic and overseas clinical trials, pay attention to side effects such as psychiatric disorder (such as elevated condition), dysgeusia, hyperhidrosis etc. are recognized. In addition, it is also necessary to pay attention to the fact that it is the condition of approval to conduct the usage result survey for all cases, for a certain period of time, because the cases of clinical trials in Japan are limited.
Carbaglu dispersible tablets 200mg（卡谷氨酸片,カーバグル分散錠）[/caption]
药店国别：
产地国家：日本
处 方 药：是
所属类别：200毫克/片 60片/盒
包装规格：200毫克/片 60片/盒
计价单位：盒
生产厂家中文参考译名:Pola Pharma Inc
生产厂家英文名:Pola Pharma Inc
原产地英文商品名:CARBAGLU 200mg/Tablets 60Tablets/box
原产地英文药品名:Carglumic Acid
中文参考商品译名:CARBAGLU 200毫克/片 60片/盒
中文参考药品译名:卡谷氨酸
简介：
部分中文卡谷氨酸处方资料(仅供参考)
英文名：Carglumic Acid
商标名：CARBAGLU dispersible tablets
中文名：卡谷氨酸分散片
生产商：Pola Pharma Inc.
药品简介
高氨血症治疗：
批准日期：2016年11月
商标名：CARBAGLU dispersible tablets
一般名：Carglumic Acid[JAN]
化学名：(2S )-2-(Carbamoylamino)pentanedioic acid
分子式：C6H10N2O5
分子量：190.15
融点：159～163℃
性状：
它是白色粉末或无色晶体。该产品微溶于水，几乎不溶于有机溶剂。
批准条件：
·制定药品风险管理计划并适当实施。
·由于日本的临床试验数量极为有限，通过对制造和销售后的复审期间的所有管理案例进行使用结果调查，掌握使用该药物的患者的背景信息 ，尽快收集有关该药物安全性和有效性的数据，并采取必要措施正确使用该药物。
药用药理学：
动作N-乙酰谷氨酸合成酶(NAGS)缺乏症的机制是尿素循环障碍之一，一种常染色体隐性遗传病由NAGS基因突变。通过用于尿素循环氨甲酰磷酸合成酶I(CPSI)N-乙酰(NAG)的第一步骤的播放活化所需无法合成，表现出高氨血症。
甲基丙二酸血症，丙酸血症和异戊酸血症被分为有机酸代谢紊乱，一种常染色体隐性遗传性疾病引起的氨基酸代谢途径的酶缺乏症。 NAGS被累积的中间代谢物抑制，从而间歇性地表现出由于蛋白质分解代谢应激引起的高氨血症。
钙谷氨酸是NAG的结构类似物，其代表NAG活化CPSI并活化尿素循环以降低血氨浓度。
适应症：
由于以下疾病导致的高氨血症
·N-乙酰谷氨酸合成酶缺乏症
·异戊酸血症
·甲基丙二酸血症
·丙酸血症
用法与用量：
通常，每天每公斤体重100毫克至250毫克，每天分2至4次，使用时，将其分散在水中并口服给药。 之后，根据患者的情况相应地增加和减少。
包装：分散片
200毫克：5片，60片
(带干燥剂和聚丙烯瓶的聚乙烯盖)
New drug: Calguminic acid (Carbagre) Hyperammonemia treatment drug that activates urea circuit
On December 22, 2016, the hyperammonemia treatment drug calguminic acid (trade name Carbagul dispersible tablet 200 mg) was released. This product is approved for manufacture and marketing on September 28, 2016, and is listed on the drug price on November 18. Adaptation is "hyperammonemia caused by N-acetylglutamate synthase deficiency, isovaleric acidemia, methylmalonic acidemia, propionic acidemia". Beginning from 100 to 250 mg / kg per day, when it is used divided into 2 to 4 times a day, it is dispersed in water and administered orally. After that, it increases and decreases appropriately according to the state of the patient. Also, in order to prevent elevated blood ammonia levels due to diet, it is necessary to instruct patients to take as much as possible before meals.
N-acetylglutamate synthetase (NAGS) deficiency is an autosomal recessive genetic disease that causes a urea circuit abnormality. The synthesis of N-acetylglutamic acid (NAG) necessary for activation of carbamyl phosphate synthase I (CPS I) which is the first step of the urea cycle becomes impossible, causing excessive accumulation of ammonia.
Both isovaleric acidemia, methylmalonic acidemia and propionic acidemia, which are organic acid metabolic disorders, develop hyperammonemia due to enzyme deficiency in the amino acid metabolism pathway. The accumulated intermediary metabolite, organic acid antagonizes NAGS activity, thereby showing the same pathological condition as NAGS deficiency.
With these hyperammonemias, irreversible disorders such as neurological disorder may occur if rapid and appropriate treatment is not performed, which may cause developmental injury and death. For this reason, it is important to control the blood ammonia level quickly by lowering the blood ammonia concentration during treatment. Although treatment varies depending on the condition, drug therapy using dietary and nutritional management such as protein restriction and L-arginine (Algin U) and sodium phenylbutyrate (Bufenyl) which promote excretion of blood ammonia to the outside is the basis ing. However, a number of cases have been reported in the current situation where control of blood ammonia levels is insufficient.
Calgulmic acid is a structural analogue of NAG that activates CPS I and is an agent that decreases blood ammonia concentration by activating the urea cycle. Since it was approved overseas in Europe in 2003 and in the US in 2010, it has been approved in 42 countries worldwide by June 2016.
In Japan, a development request was submitted from the Japanese Society for Congenital Metabolic Disorders, and in April 2010 I got a high reputation for "unapproved drugs for non-approved drugs with high medical necessity" and for this approval It was connected. In addition, this drug is designated as a drug for rare diseases in November 2014.
In domestic and overseas clinical trials, pay attention to side effects such as psychiatric disorder (such as elevated condition), dysgeusia, hyperhidrosis etc. are recognized. In addition, it is also necessary to pay attention to the fact that it is the condition of approval to conduct the usage result survey for all cases, for a certain period of time, because the cases of clinical trials in Japan are limited.
用药温馨提示：当您服用此药物时，需定期接受医疗专业人士的检查，以便随时针对其药效、副作用等情况进行监测。本网站所包含的信息旨在为患者提供帮助，不能代替医学建议和治疗。
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