Clinical trial · Observational
A National Registry on Chinese Patients With Lymphangioleiomyomatosis
A National Registry on Clinical Manifestations, Genetics, Interventions, and Outcomes in Chinese Patients With Lymphangioleiomyomatosis (LAM-CHINA)
- Source
- ClinicalTrials.gov
- Retrieved
- Sep 8, 2026
- Layer
- normalized (units and labels harmonized; values unchanged)
- Run
- ING-CLINICALTRIALS-20260908-000001
Summary
Brief summary (as posted)
Pulmonary lymphangioleiomyomatosis (LAM), a disease characterized by diffuse cystic changes in the lung, is a rare disorder that affects almost exclusively women. The main objectives of this study are to accurately evaluate the prevalence of LAM, the status of disease, the diagnosis and treatment, the quality of care, and the health related outcomes in China.
Conditions
Conditions (1)
Free-text conditions as registered, with the CancerIndex entity they were reconciled to and the match type.
| Condition (as posted) | Mapped entity | Match | Confidence |
|---|---|---|---|
| Pulmonary Function | — | UNRESOLVED | — |
Interventions
Interventions (0)
Data not yet available
Design
Arms and outcomes
Arms (0)
[]Primary outcomes (1)
- measure
- Change in spirometry (FEV1 and FVC) of lymphangioleiomyomatosis patients.
- timeFrame
- 10 years
- description
- Spirometry will be evaluated at baseline and through study completion, an average of 3 years.
Secondary outcomes (4)
- measure
- Change of diffusing capacity of the lung.
- timeFrame
- 10 years
- description
- Pulmonary function tests will be evaluated at baseline and through study completion, an average of 3 years.
- measure
- Annual incidence of major health outcomes: hemoptysis, pneumothorax, chylothorax, and spontaneous hemorrhage of kidney angiomyolipoma (AML).
- timeFrame
- 10 years
- description
- The number of occurrences will be recorded through study completion, an average of 3 years.
Eligibility
Eligibility (as posted)
- Sex
- Female
Show eligibility criteria text
Inclusion Criteria: 1. Gender: female. 2. Age: no limitation. 3. Diagnosis meets one of the following criteria, (1) definite or probable diagnosis of LAM based on ATS/JRS and ERS criteria. (2) Investigators recommend including of the patient. Exclusion Criteria: 1. Suspected LAM patients without other supporting evidence for LAM diagnosis. 2. No diffuse cystic lesions in the lung. 3. Patients with bilateral cystic lung lesions but the LAM diagnosis cannot be established. 4. Without signed informed consent. 5. Difficult to follow up.
References
Publications (2)
- DERIVEDWang YY, Zou LP, Xu KF, Xu WS, Zhang MN, Lu Q, Tian XL, Pang LY, He W, Wang QH, Gao Y, Liu LY, Chen XQ, Ma SF, Chen HM, Dun S, Yang XY, Luo XM, Huang LL, Li YF. Long-term safety and influence on growth in patients receiving sirolimus: a pooled analysis. Orphanet J Rare Dis. 2024 Aug 15;19(1):299. doi: 10.1186/s13023-024-03243-5. PMID 39148107
- DERIVEDXu W, Yang C, Cheng C, Wang Y, Hu D, Huang J, He Y, Wang J, Chen K, Yang L, Zhou W, Zhang T, Liu S, Dai J, Meng S, Li X, Yang Y, Wang ST, Feng R, Zhang W, Zhang H, Wang L, Tian X, Xu KF. Determinants of Progression and Mortality in Lymphangioleiomyomatosis. Chest. 2023 Jul;164(1):137-148. doi: 10.1016/j.chest.2023.02.026. Epub 2023 Feb 18. PMID 36801466