新型吸入溶液Pulmozyme(dornase alpha)获美国FDA批准上市。本品是30多年来第一个处理CF的新治疗药物，与标准治疗联用适用于处理囊性纤维化病人以改善肺功能
处方修改日期：2014年12月 公司：Genentech, Inc.
PULMOZYME（脱氧核糖核酸酶 dornase alfa）吸入溶液，供吸入用
最初美国批准：1993年
目前的主要变化
剂量和给药方法 12/2014
作用机理
PULMOZYME是重组人脱氧核糖核酸酶I（rhDNase），选择性切割DNA。在临床前体外研究中，PULMOZYME水解CF患者的痰液的DNA，并减少痰粘弹性酶，在CF患者气道有贡献粘稠脓性分泌物潴留既减少了肺功能和感染的病情加重。化脓性肺分泌物中含有非常高浓度的由退化响应于感染积累白细胞释放胞外DNA。
适应症和用法
PULMOZYME是与标准疗法的囊性纤维化（CF）患者的管理，以改善肺功能一起指示的重组DNA酶的酶。
用法用量
推荐剂量为1个2.5毫克一次性使用安瓿吸入，每日一次使用建议的喷雾器。
部分患者可受益于每日两次给药。
剂型和规格
吸入溶液：2.5毫克/毫升2.5单用安瓿
禁忌症
PULMOZYME是禁忌在病人已知过敏dornase阿尔法，中国仓鼠卵巢细胞产物，或该产品的任何部分。
警告和注意事项
无。
不良反应
最常见的不良反应，在CF患者的临床试验看出（在与PULMOZYME安慰剂治疗的患者≥3％存在的）是：语音改建，咽炎，皮疹，喉炎，胸痛，结膜炎，鼻炎，减少的≥10肺活量％，发烧和呼吸困难。
包装规格/储存与处理
PULMOZYME提供在：
含有6单次使用的安瓿每2.5毫升安瓿中含有2.5毫克dornase阿尔法5箔囊30单元箱（1毫克/毫升）：NDC50242-100-40。
储存和处理
商店PULMOZYME在冷藏条件下（2℃〜8°C/36°F至46°F）在其保护膜避光，不要超出印在安瓿使用期限内使用。在他们的保护膜袋存放未使用的安瓿在冷藏条件下。冷藏PULMOZYME在运输过程中，不要暴露在室温为24小时的总时间。

完整处方来源附件：
1）：http://www.pulmozyme.com/hcp/cf-treatment/pulmozyme-moa.html
2）：https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=d8c78a7e-ff99-48f3-8952-643ec2ea0f86
The only FDA-approved mucus-altering therapy, Pulmozyme plays a distinct role in the fight against cystic fibrosis (CF)
Important Safety Information
Pulmozyme is contraindicated in patients with known hypersensitivity to dornase alfa, Chinese Hamster Ovary cell products, or any component of the product.
The most common adverse reactions associated with the use of Pulmozyme include: voice alteration, pharyngitis, rash, laryngitis, chest pain, conjunctivitis, rhinitis, decrease in FVC of ≥ 10%, fever, dyspepsia, and dyspnea. There have been no reports of anaphylaxis attributed to the administration of Pulmozyme. Mild to moderate urticaria and mild skin rash have been observed and have been transient.
For further information, please see the Pulmozyme full Prescribing Information.
IndicationPulmozyme (dornase alfa) is indicated for daily administration in conjunction with standard therapies for the management of cystic fibrosis (CF) patients to improve pulmonary function.
In CF patients with an FVC ≥ 40% of predicted, daily administration of Pulmozyme has also been shown to reduce the risk of respiratory tract infections requiring parenteral antibiotics.
Pediatric UseThe safety and effectiveness of Pulmozyme have been established in pediatric patients 5 years of age and older. The safety of Pulmozyme, 2.5 mg by inhalation, was studied with 2 weeks of daily administration in 65 patients with cystic fibrosis aged 3 months to < 5 years. While clinical trial data are limited in pediatric patients younger than 5 years of age, the use of Pulmozyme should be considered for pediatric CF patients who may experience potential benefit in pulmonary function or who may be at risk of respiratory tract infection.
The safety of Pulmozyme, 2.5 mg by inhalation, was studied with 2 weeks of daily administration in 98 pediatric patients with cystic fibrosis 3 months to 10 years of age (65 aged 3 months to < 5 years, 33 aged 5 to ≤ 10 years). The PARI BABY™ reusable nebulizer (which uses a facemask instead of a mouthpiece) was utilized in patients unable to demonstrate the ability to inhale or exhale orally throughout the entire treatment period (54/65, 83% of the younger; and 2/33, 6% of the older patients). Overall, the nature of adverse reactions was similar to that seen in the placebo-controlled trials in older patients. The number of patients reporting cough was higher in the younger age group as compared to the older age group (29/65, 45%; compared to 10/33, 30%) as was the number reporting moderate to severe cough (24/65, 37%; compared to 6/33, 18%). The number of patients reporting rhinitis was higher in the younger age group as compared to the older age group (23/65, 35%; compared to 9/33, 27%) as was the number reporting rash (4/65, 6% as compared to 0/33, 0%).
*囊性纤维化(CF)是一种累及遍及全身的腺体的遗传状态。因此CF可侵犯许多不同器官。其症状常累及肺和消化系统。肺中的阻塞、感染是CF中最常见的特征。
在CF中，肺中浓厚的粘液阻塞呼吸道并使细菌感染和发生炎症。白细胞，中性粒细胞被送至肺与这些感染斗争。当中性粒细胞死亡时，释放DNA和弹性酶，时粘液进一步变脓稠，并加重呼吸道的阻塞、感染和炎症。Pulmozyme是人工制备的天然存在物质被称为脱氧核糖核酸酶DNase。尽管不能治愈CF，但是Pulmozyme可能有助于治疗这种情况。酶将中性粒细胞释放的DNA "切割"。Pulmozyme在1994年已经推向失常，是30多年来第一个处理CF的新治疗药物。
1993年12月30日美国FDA批准Genentech公司的Pulmozyme(dornase alpha)吸入溶液与标准治疗联用适用于处理囊性纤维化病人以改善肺功能。病人用力肺活量(FVC)≥40%预期值，每天给予Pulmozyme也曾显示减低需肠道外抗生素的呼吸道感染的危险。尚未证实病人每天给药治疗12个月以上的安全性和有效性。