OPEN-LABEL AZTREONAM LYSINATE FOR INHALATION IN CYSTIC FIBROSIS

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目及 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。所列机构为 研究中心，而研究中心不一定是研究者所在的机构。 本研究是一项开放标签、多中心研究，受试者曾参加过两项既往III期AI研究（CP-AI-004或CP-AI-005）之一。 患者将在CP-AI-004/005中分配的相同方案（每日两次[BID]或每日三次[TID]）中接受75 mg赖氨酸氨曲南吸入剂（AI）治疗。 将从美国最多70家CF中心入组最多370例患者。 每例患者的研究将持续约6个月。 患者将接受长达三个28天的AI疗程，每个疗程之后将有28天的停药期。 前两个AI课程是强制性的;第三个课程将由医生决定。 研究期间，患者可根据需要接受任何抗假单胞菌抗生素（口服、IV或吸入）治疗，IV氨曲南除外。 本研究的主要目的是评价囊性纤维化（CF）患者反复暴露于吸入用赖氨酸氨曲南（AI）的安全性。 次要目的包括评价重复暴露于AI对微生物学和疾病相关终点的影响。 将通过监测不良事件、气道反应性、生命体征以及血清化学和血液学来评价安全性。 将通过痰液中PA CFU密度、其他细菌病原体和真菌病原体的消失或出现以及氨曲南对PA的最小抑菌浓度（MIC）评价微生物学。 还将评价其他终点，包括FEV 1、FVC和FEF 25-75、CF量表修订版（CFQ-R）评估的临床症状、住院、体重、因CF疾病而缺课或工作天数以及静脉注射抗生素的时间。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. This study is an open-label, muticenter study in patients who participated in one of two previous Phase 3 AI studies (CP-AI-004 or CP-AI-005). Patients will receive treatment with 75 mg Aztreonam Lysinate for Inhalation (AI) in the same regiment (twice daily [BID] or three times daily [TID] to which they were assigned in CP-AI-004/005. Up to 370 patients will be enrolled from up to 70 CF centers throughout the United States. This study will last approximately 6 months for each patient. Patients will receive up to three 28-day courses of AI, each of which will be followed by a 28-day off drug period. The first two AI courses are mandatory; the third course will be at the physician's discretion. During the study, patient may be treated as needed with any antipseudomonal antibiotics (oral, IV or inhaled) with the exception of IV Aztreonam. The primary objective of the study is to evaluate the safety of repeated exposure to Aztreonam Lysinate for Inhalation (AI) in cystic fibrosis (CF) patients. Secondary objective include evaluation of the effects of repeated exposure to AI in microbiology and disease-related endpoints. Safety will be evaluated by monitoring adverse events, airway reactivity, vital signs, and serum chemistry and hematology. Microbiology will be evaluated by PA CFU density in sputum, disappearance or appearance of other bacterial pathogens and fungal pathogens, and minimum inhibitory concentration (MIC) of Aztreonam for PA. Other endpoints will also be evaluated to include FEV1, FVC, and FEF 25-75, clinical symptoms as assessed by the CF Questionnaire-Revised (CFQ-R), hospitalizations, body weight, missed school or work days due to CF illness, and time to intravenous antibiotics.