OPEN-LABEL AZTREONAM LYSINATE FOR INHALATION IN CYSTIC FIBROSIS

这个子项目是利用由NIH/NCRR资助的中心拨款提供的资源的许多研究子项目之一。子项目和调查员(PI)可能从另一个NIH来源获得了主要资金，因此可能会出现在其他CRISE条目中。列出的机构是针对中心的，而不一定是针对调查员的机构。这项研究是一项开放标签的多中心研究，对象是参加了之前两项3期人工智能研究(CP-AI-004或CP-AI-005)之一的患者。患者将在CP-AI-004/005中分配到的同一军团(每天两次[BID]或每天三次[TID])接受75毫克氨曲南吸入(AI)治疗。多达370名患者将从全美多达70个CF中心登记入选。这项研究将为每个患者持续大约6个月。患者将接受最多三个28天的人工智能疗程，每个疗程之后将有28天的停药期。前两个人工智能课程是必修课；第三个课程将由医生自行决定。在研究期间，患者可以根据需要使用除静脉注射氨曲南外的任何抗假单抗(口服、静脉注射或吸入)治疗。本研究的主要目的是评估囊性纤维化(CF)患者反复吸入氨曲南(AI)的安全性。次要目标包括评估反复暴露于AI对微生物学和疾病相关终点的影响。安全性将通过监测不良事件、呼吸道反应性、生命体征以及血清化学和血液学进行评估。微生物学将通过痰中PA菌落密度、其他细菌和真菌病原体的消失或出现以及氨曲南对PA的最低抑菌浓度(MIC)进行评估。还将评估其他终点，包括FEV1、FVC和FEF 25-75、修订后的CF问卷(CFQ-R)评估的临床症状、住院情况、体重、因CF疾病而旷课或上班的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.