英文药名：PULMOZYME Inhalation Solution（Dornase Alfa[Genetical Recombination]） 中文药名：重组脱氧核糖核酸酶吸入溶液 生产厂家：中外制药 プルモザイム吸入液2.5mg 治疗类别名称 重组人DNA降解酶制剂 一般名 ドルナーゼ　アルファ（遺伝子組換え） （Dornase Alfa（Genetical Recombination））（JAN） 本質 Dornase alpha是重组人DNA酶I，在中国仓鼠卵巢细胞中产生。Dornase阿尔法，260个氨基酸残基（分子式：C1321H1995N339O396S9）是由糖蛋白。 分子量 約37,000 条件批准 从在日本的管理经验就已经极其有限，在复试期间，表演的安全性和疗效的上市后研究，以注册该药物的患者的管理。其中，充分考虑到是在长期给药时的安全性和有效性。 药效药理 本剂选择性水解酶的DNA，具有降低含有大量的DNA粘性脓性分泌物的效果。 适应病症 改善囊性纤维化肺功能 用法用量 本品溶液经压缩空气驱动的雾化器产生气溶胶，大多数病人每日吸入1次（2.5mg）（雾化吸入：每次5万～10万U（溶于生理盐水）），每天3～4次，一般连续用药4～6天。 气管内滴入：每次5万U（溶于2mL生理盐水），适量滴入。 一些研究提示，本品对较大年龄（20岁以上）的病人疗效比年龄小的差，较大年龄的病人每天须要两倍的剂量，而另一些研究认为，1天大于2.5mg两倍的剂量并无任何益处。 包装规格 吸入溶液 2.5mg：2.5毫升×30安瓿（铝枕式包装1袋6安瓿×5袋） 制造厂商 中外制药有限公司 完整处方来源附件：http://www.info.pmda.go.jp/go/pack/2290704G1024_1_03/ Pulmozyme(dornase alfa)Inhalation Solution Pulmozyme® (dornase alfa) Inhalation Solution is a sterile, clear, colorless, highly purified solution of recombinant human deoxyribonuclease I (rhDNase), an enzyme which selectively cleaves DNA. Pulmozyme is administered by inhalation of an aerosol mist produced by a compressed air driven nebulizer system. Launched in 1994, Pulmozyme was the first new therapeutic drug for the management of cystic fibrosis in over 30 years. Status Daily administration of Pulmozyme in conjunction with standard therapies is indicated in the management of cystic fibrosis patients to improve pulmonary function. In 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. Important Safety Information Pulmozyme should not be used in patients who are allergic to any of its ingredients. Pulmozyme should be used along with standard therapies for cystic fibrosis. When starting Pulmozyme therapy, patients may experience change in or loss of their voice, discomfort in the throat, chest pain, red watery eyes, rash, dizziness, fever, or runny nose. These side effects are usually mild and short-lived. There is a limited amount of information available concerning the usage of Pulmozyme in patients who are younger than 5 years. The decision to use Pulmozyme in these patients is made after considering the potential for a benefit in lung function or in decreasing the risk of respiratory tract exacerbations. The safety of Pulmozyme given by daily inhalation for 2 weeks has been studied using 98 CF patients with 65 of them aged 3 months to <5 years (younger group) and 33 aged 5 years to ≤10 years (older group). The PARI BABY™ reusable nebulizer (which uses a face mask instead of a mouthpiece) was used in patients who were unable to show that they could breathe in or out using their mouth throughout the entire treatment period. There were more reports of cough, moderate to severe cough, runny nose, and rash in the younger group of patients. Other reported events tended to be of mild to moderate severity. The kind of side effects reported was similar to that of Pulmozyme when used in the larger trials that studied older patients. Proposed Mechanism of Action In vitro, Pulmozyme cleaves extracellular DNA in mucus of cystic fibrosis patients, reducing the adhesiveness and viscoelasticity of the mucus. Disease Education Cystic fibrosis is a life-threatening disease involving a genetic mutation that disrupts the cystic fibrosis transmembrane regulator protein. This protein acts on an ion-specific channel that modulates salt and water transport. This ion-channel defect leads to poorly hydrated, thick, mucous secretions in the airways and severely impaired mucociliary clearance. Impairments in these vital lung defense mechanisms typically begin in early childhood. Chronic secondary infections invariably occur, resulting in progressive lung dysfunction and deterioration. Cystic fibrosis affects other organ systems as well, especially the digestive tract and reproductive organs. Respiratory failure accounts for approximately 90% of deaths in patients with cystic fibrosis. According to the U.S. Cystic Fibrosis Foundation, the median average life expectancy for patients with cystic fibrosis is more than 37 years. Cystic fibrosis is an inherited, recessive disease which is more common in Caucasians。