Ravicti

Ravicti在肝脏和肾脏中与其他物质结合，帮助消除体内的氮。过量的氮可导致高氨血症，即血液中氨含量升高。氨在血液和组织中循环时具有很强的毒性，可能导致永久性脑损伤、昏迷或死亡。

Ravicti有助于预防尿素循环障碍的成人和儿童体内氨的积累。这种药物不能治疗高氨血症。

Ravicti通常与低蛋白饮食一起使用，并且有时会配合膳食补充剂。

Ravicti 可能会影响您的神经系统。如果您出现以下任何症状，请立即联系医生：困惑、头痛、记忆力问题、听力问题、呕吐、味觉改变、手脚麻木或刺痛感，或者感到异常困倦或头晕。

如果您出现对Ravicti的过敏反应迹象，请立即寻求紧急医疗帮助：

• 荨麻疹；
• 咳嗽、喘息、呼吸困难；感觉要昏倒；面部、嘴唇、舌头或喉咙肿胀。

如果您有任何高氨血症（高氨血症）的迹象，请立即致电您的医生：

• 乏力，缺乏精力；
• 思维问题，行为改变，感到易怒；
• 呼吸困难；
• 进食问题；或
• 癫痫发作（抽搐）。

Ravicti可能会影响您的神经系统。如果您出现以下任何症状，请立即致电您的医生：

• 嗜睡，异常疲倦；
• 记忆问题；
• 有头晕的感觉，像要昏倒；
• 困惑，持续的头痛，呕吐；
• 手脚麻木、刺痛或烧灼感；
• 味觉改变；或
• 听力问题。

Ravicti的常见副作用可能包括：

• 偶尔头痛；
• 恶心，呕吐，腹泻；
• 食欲不振，胀气，胃痛；
• 眩晕，疲倦；
• 皮疹；或
• （2岁以下儿童）发热，感冒或流感症状，咳嗽，皮肤上小的丘疹。

这并非完整的副作用列表，其他副作用也可能发生。关于副作用，请咨询您的医生以获取医疗建议。

如果您对甘油苯丁酸过敏，则不应使用Ravicti。

为了确保Ravicti对您是安全的，请告知您的医生是否曾经患有：

• 肝脏或肾脏疾病；

• 胰腺疾病；

• 胃肠道疾病，

• 一种叫做NAGS（N-乙酰谷氨酸合成酶）缺乏症的疾病；或

• 如果您正在使用其他药物。

目前尚不清楚甘油苯丁酸是否会危害未出生的婴儿。如果您怀孕或计划怀孕，请告知您的医生。

如果您怀孕了，您的姓名可能会被列入妊娠登记簿。这是为了跟踪妊娠结果并评估甘油苯丁酸对婴儿的任何影响。

在使用Ravicti期间，您不应该进行母乳喂养。

有时同时使用某些药物可能不安全。某些其他药物会影响您血液中的氨水平，使Ravicti的效果降低或导致高氨血症。

许多药物可以与甘油苯丁酸发生相互作用。这包括处方药和非处方药、维生素以及草药产品。这里并未列出所有可能的相互作用。请告知您的医生您目前正在使用的所有药物以及您开始或停止使用的任何药物。

Take Ravicti exactly as prescribed by your doctor. Follow all directions on your prescription label and read all medication guides or instruction sheets. Your doctor may occasionally change your dose.

Ravicti is usually taken 3 times per day. Take with food or infant formula. If you are breastfeeding a baby who is taking this medicine, give each dose right before nursing.

Measure Ravicti liquid carefully. Use the dosing syringe provided, or use a medicine dose-measuring device (not a kitchen spoon).

Ravicti may be given through a nasogastric (NG) or gastric feeding tube as follows: Measure the correct dose in the oral syringe, attach it to the feeding tube and push the plunger down to empty the syringe into the tube. Flush the tube with 10 milliliters (mL) of water and allow the tube to drain. Follow this with 10 mL more of water to wash the contents down.

If you switch from sodium phenylbutyrate to Ravicti, your dose will not be the same. Follow your doctor's dosing instructions very carefully.

Glycerol phenylbutyrate doses are based on body surface area (height and weight). Your dose needs may change if you gain or lose weight or if you are still growing.

You may need to follow a special diet while using Ravicti. Follow all instructions of your doctor or dietitian. Learn about the foods to eat or avoid to help control your condition.

A buildup of ammonia in the blood can quickly cause brain injury or death. You will need frequent blood tests. Every person with a urea cycle disorder should remain under the care of a doctor.

Your name may be listed on a Urea Cycle Disorder registry. This is to collect information about people with these disorders and to evaluate the effects of treatment.

Store Ravicti at room temperature away from moisture and heat.

Usual Adult Dose of Ravicti for Urea Cycle Disorders:

Initial Doses:
Administer orally in 3 equally divided dosages rounded up to the nearest 0.5 mL:
-Phenylbutyrate-Naive Patients: 4.5 to 11.2 mL/m2/day (5 to 12.4 g/m2/day)
-Phenylbutyrate-Naive Patients with Some Residual Enzyme Activity: 4.5 mL/m2/day
-Patients Switching from sodium phenylbutyrate, use the following conversion:
--Tablets: Total daily dose of glycerol phenylbutyrate (mL)=total daily dose of sodium phenylbutyrate tablets (g) x 0.86
--Powder: Total daily dose of glycerol phenylbutyrate (mL)=total daily dose of sodium phenylbutyrate powder (g) x 0.81

MAINTENANCE DOSES: Follow clinically and with plasma ammonia levels, especially with changing doses; for elevated plasma ammonia, increase dose to maintain fasting plasma ammonia to less than half the upper limit of normal; if the phenylacetate (PAA) to phenylacetylglutamine (PAGN) ratio and urinary phenylacetylglutamine (U-PAGN) measurement are available, this will help guide dosing; product labeling may be consulted for additional information

MAXIMUM DOSE: 17.5 mL (19 g) per day

Usual Pediatric Dose of Ravicti for Urea Cycle Disorders:

Initial Doses:
-Patients less than 2 years: Administer in 3 or more equally divided dosages, rounded up to the nearest 0.1 mL
-Patients 2 years or older: Administer in 3 equally divided dosages, rounded up to the nearest 0.5 mL
Phenylbutyrate-Naive Patients: 4.5 to 11.2 mL/m2/day (5 to 12.4 g/m2/day)
Phenylbutyrate-Naive Patients with Some Residual Enzyme Activity: 4.5 mL/m2/day
Patients Switching from sodium phenylbutyrate, use the following conversion:
--Tablets: Total daily dose of glycerol phenylbutyrate (mL)=total daily dose of sodium phenylbutyrate tablets (g) x 0.86
--Powder: Total daily dose of glycerol phenylbutyrate (mL)=total daily dose of sodium phenylbutyrate powder (g) x 0.81

MAINTENANCE DOSES:
-Patients 6 years or older: Follow clinically and with plasma ammonia levels, especially with changing doses; for elevated plasma ammonia, increase dose to maintain fasting plasma ammonia to less than half the upper limit of normal; if the phenylacetate (PAA) to phenylacetylglutamine (PAGN) ratio and urinary phenylacetylglutamine (U-PAGN) measurement are available, this will help guide dosing; product labeling may be consulted for additional information
-Patients less than 6 years: Follow above guidance, however, if obtaining fasting ammonia is problematic due to frequent feedings, adjust the dose to keep the first ammonia of the morning below the upper limit for age

MAXIMUM DOSE: 17.5 mL (19 g) per day

Comments:
-When determining the starting dose, consider the patient's residual urea synthetic capacity, dietary protein requirements, and diet adherence; may consider an initial estimated daily dose of 0.6 mL per gram of dietary protein ingested per 24-hour period.
-Must use with dietary protein restriction and in some cases, with dietary supplements (e.g., essential amino acids, arginine, citrulline, protein-free calorie supplements).
-Not indicated for the treatment of acute hyperammonemia as more rapid interventions should be used.
-Safety and efficacy for treatment of N-acetylglutamate synthase (NAGS) deficiency has not been established.

Use: As a nitrogen-binding agent for chronic management of patients with urea cycle disorders (UCDs) who cannot be managed by dietary protein restriction and/or amino acid supplementation alone.

尽快服用药物，但如果接近您下次服药的时间，则跳过漏服的剂量。不要一次性服用两剂。

立即寻求紧急医疗救助。