Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Class |
|---|---|
| North-China Pharmaceutical Company, China | UNKNOWN |
Not provided
Not provided
Not provided
There is no confirmed drug therapy for plastic bronchitis. The study wish to test the effectiveness and safety of sirolimus (rapamycin) in patients with lymphatic plastic bronchitis.
Plastic bronchitis is a rare respiratory disorder characterized by formation of gelatinous or rigid cast of bronchial tree. Confirmed drug therapy for plastic bronchitis is lacking. Recently, part of plastic bronchitis has been found to have dysfunction of lymphatic circulation in the lung. Pulmonary lymphatic perfusion syndrome (PLPS) is regarded as a major mechanism of lymphatic plastic bronchitis. Sirolimus (rapamycin) has not been studied in plastic bronchitis, however, it has been shown effective in several lymphatic disorders, such as lymphangioleiomyomatosis, generalized lymphatic anomaly (GLA) including lymphangiomatosis, etc. We wish to test the effectiveness of sirolimus in lymphatic plastic bronchitis.
Not provided
Not provided
Not provided
Not provided
| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Sirolimus | Experimental | Patients in sirolimus group will receive sirolimus for 6 months. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Sirolimus | Drug | Patients will receive sirolimus for 6 months. |
|
| Measure | Description | Time Frame |
|---|---|---|
| changes of pulmonary lymphatic perfusion distribution | quantitatively measured with 68Ga-NEB positron emission tomography (PET) at baseline and the end of study | 6 months |
| changes of coughing score | measured by coughing VAS score (0-10) at baseline, 3 months and the end of study | 6 months |
| Measure | Description | Time Frame |
|---|---|---|
| changes of pulmonary function (FEV1, FVC) | measured by spirometry at baseline, 3 months and the end of study | 6 months |
| changes of six minutes walking distance | measured by six minutes walking test at baseline, 3 months and the end of study |
Not provided
Inclusion Criteria:
Exclusion Criteria:
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Kai-Feng Xu | Contact | 010-69155039 | xukf@pumch.cn |
| Name | Affiliation | Role |
|---|---|---|
| Kai-Feng Xu, MD | Peking Union Medical College Hospital | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Peking Union Medical College Hospital | Recruiting | Beijing | Beijing Municipality | 100730 | China |
The protocol and clinical study report will be shared.
Data will be shared between time of completion of the study and time of publication of the study.
contact principle investigator Dr Kai-Feng Xu via xukf@pumch.cn
Not provided
Not provided
| ID | Term |
|---|---|
| D020123 | Sirolimus |
| ID | Term |
|---|---|
| D018942 | Macrolides |
| D007783 | Lactones |
| D009930 | Organic Chemicals |
Not provided
Not provided
Ten lymphatic bronchitis patients are planned to be recruited for the study. Sirolimus will be administered for 6 months.
Not provided
Not provided
Not provided
Not provided
| 6 months |
| change of health-related quality of life | measure by St George Respiratory Questionnaire at baseline, 3 months and the end of study | 6 months |
| changes of breathlessness score | measured by Borg scale (0-10) at baseline, 3 months and the end of study | 6 months |
| Number of participants with treatment-related adverse events as assessed by CTCAE v4.0 | Common Terminology Criteria for Adverse Events (CTCAE) was used to collect adverse effects at baseline and through study completion | 6 months |