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Procysbi缓释胶囊2013年获美国FDA批准用于口服使用儿童和成人肾病型胱胺酸症的治疗
美国初始批准：1994
适应症和用法
PROCYSBI是胱氨酸消耗代理人表示对肾病胱氨酸病在成人和儿童6岁岁及以上的管理。
【用法用量】
PROCYSBI应规定由经验丰富的肾病胱氨酸病管理医生。
•吞咽胶囊或全部洒在食物或液体的建议后。与食物混合后经胃管（12 F或更大）的管理。
•总日剂量为1.3克/平方米/天，分成两次剂量给药，每12小时一次。
•采取PROCYSBI后至少2小时，至少30分钟后再吃。
•治疗目标是维持白细胞（WBC）胱氨酸的水平<1纳摩尔半胱胺酸/ mg蛋白或血浆中半胱胺浓度>0.1毫克/升。
由速释半胱胺切换到PROCYSBI
•每日总剂量PROCYSBI等于其之前的每日总剂量的速释半胱胺重酒石酸的。
初始剂量的半胱胺初治患者
•起始剂量：⅙至¼PROCYSBI的维持剂量.
•维持剂量：1.3克/平方米/天，分两次服用，每12小时
请参阅完整的处方信息进行管理上的细节。
剂型和规格
缓释胶囊：25毫克和75毫克
禁忌
过敏青霉胺
警告和注意事项
•埃勒斯 - 当洛综合征类：减少用量，如果皮肤和骨骼发生病变。
•皮疹：如果严重的皮疹，如多形红斑大疱或中毒性表皮坏死松解症发生时，请停止PROCYSBI。
•胃肠道：监视消化道溃疡和出血的症状。
•中枢神经系统：监测癫痫发作，嗜睡，嗜睡，抑郁和脑病。
•白细胞减少症和碱性磷酸酶升高级别：监视白细胞计数及碱性磷酸酶升高的水平。
•良性颅内压增高：用于监控和体征良性颅内压增高症状。
不良反应
最常见的不良反应（≥5％）是呕吐，腹部疼痛/不适，头痛，恶心，腹泻，厌食/食欲下降，口臭，乏力，头晕，皮肤的气味，和皮疹。
药物相互作用
PROCYSBI可施用与电解质（除碳酸氢盐）和必要的范可尼综合症，管理矿物替换以及维生素D和甲状腺激素.
特殊人群中使用
哺乳期妇女：哺乳，不建议同时服用PROCYSBI。
包装规格：
25mg胶囊/粒*60粒/胶囊/盒
75mg胶囊/粒*250粒/胶囊/盒
http://dailymed.nlm.nih.gov/dailymed/lookup.cfm?setid=b7b6e290-5168-46dc-9e7f-5995420ec1c1
PROCYSBI Rx
Generic Name and Formulations:
Cysteamine bitartrate 25mg, 75mg; delayed-release caps.
Company:
Raptor Pharmaceuticals, Inc.
Indications for PROCYSBI:
Management of nephropathic cystinosis.
Adults and Children's Dose:
<6yrs: not established. Take on an empty stomach. Swallow whole or sprinkle contents of cap on 4oz of food (eg, applesauce or berry jelly) or mix in 4oz of liquids (eg, orange or apple juice); also, may administer via feeding tube after mixing with 4oz of applesauce. ≥6yrs: New patients: start on ⅙ to ¼ of the maintenance dose; increase gradually over 4–6 weeks. Maintenance: 1.3g/m2/day, in 2 divided doses given every 12 hrs. Max 1.95g/m2/day. Goal of therapy: to maintain WBC cystine level <1nmol ½ cystine/mg protein or a plasma cysteamine concentration >0.1mg/L. Switching from immediate-release cysteamine: give previous total daily dose of immediate-release caps. Dose titration, measuring WBC cystine levels: see full labeling.
Pharmacological Class:
Cystine-depleting agent.
Contraindications:
Penicillamine hypersensitivity.
Warnings/Precautions:
Monitor for development of skin or bone lesions and interrupt dosing if occur; may restart at a lower dose. Discontinue if severe skin rash develops. Consider lowering the dose if severe GI symptoms develop. Evaluate and monitor for CNS symptoms; interrupt or adjust dose if severe symptoms, persist or progress. Monitor blood counts and alkaline phosphatase levels. Monitor for signs/symptoms of benign intracranial hypertension. Pregnancy (Cat.C). Nursing mothers: not recommended.
Adverse Reactions:
Vomiting, abdominal pain/discomfort, headaches, nausea, diarrhea, anorexia/decreased appetite, breath odor, fatigue, dizziness, skin odor, rash; skin or bone lesions, leukopenia, CNS effects (eg, somnolence, encephalopathy, seizures), GI ulceration/bleeding, interstitial nephritis, elevated alkaline phosphatase, pseudotumor cerebri, papilledema.
How Supplied:
Caps 25mg—60; 75mg—250 | 
