LAM-CAK: Lymphangioleiomyomatosis, a Study on Cathepsin K
Study Details
Study Description
Brief Summary
This is a physiopathological case-control, non-interventional, monocentric study of adult patients with lymphangioleiomyomatosis. The controls are patients followed in neurology at the CHU of Tours for a tuberous sclerosis complex without lymphangioleiomyomatosis, the healthy volunteers are women with neither pulmonary nor renal pathology and recruited at the clinical investigation centre of the CHU of Tours.
| Condition or Disease | Intervention/Treatment | Phase |
|---|---|---|
|
Study Design
Arms and Interventions
| Arm | Intervention/Treatment |
|---|---|
| Patientes with lymphangioleiomyomatosis no intervention administered. Recruitment in pneumology department |
Other: 24h urine
Urine samples will be collected at home over 24 hours
Other: urine sample
A urine sample of 20cc will be collected
|
| Patientes with tuberous sclerosis complex without Lymphangioleiomyomatosis no intervention administered. Recruitment in neurology department |
Other: 24h urine
Urine samples will be collected at home over 24 hours
Other: urine sample
A urine sample of 20cc will be collected
|
| Healthy women volunteers no intervention administered. Recruitment in clinical investigation centre |
Other: 24h urine
Urine samples will be collected at home over 24 hours
Other: urine sample
A urine sample of 20cc will be collected
|
Outcome Measures
Primary Outcome Measures
- urine cathepsin K proteins levels [inclusion visit]
comparison of urine cathepsin K protein levels in the 3 groups
- urine cathepsin K activity [inclusion visit]
comparison of urine cathepsin K activity in the 3 groups
Secondary Outcome Measures
- urinary quantity of cystatin C [inclusion visit]
comparison of urine cystacin C quantity in the 3 groups
- urinary quantity C and N-telopeptides of type 1 collagen [inclusion visit]
comparison in the 3 groups
- serum level of chondroitin sulphates [inclusion visit]
measurement in lymphangioleiomyomatosis patients
- renal angiolipomas [inclusion visit]
measurement of the size of renal angiomyolipomas in lymphangioleiomyomatosis patients
- FEV1 measurement [inclusion visit]
most recent FEV1 measurement as % of predicted value in lymphangioleiomyomatosis patients
- VEGF-D measurement [inclusion visit]
most recent measurement of VEGF-D blood levels in lymphangioleiomyomatosis patients
- T-score measurement [inclusion visit]
T-score measurement of the most recent bone densitometry in lymphangioleiomyomatosis patients
Eligibility Criteria
Criteria
Inclusion criteria for patients with lymphangioleiomyomatosis
-
Age ≥ 18 years
-
Female carrier of lymphangioleiomyomatosis according to ERS criteria
Inclusion criteria for healthy female volunteers
-
Age ≥ 18 years
-
Women with no history of lymphangioleiomyomatosis, pulmonary, renal or osteoporotic disease
Inclusion criteria for tuberous sclerosis complex patients without lymphangioleiomyomatosis
-
Age ≥ 18 years
-
Woman followed for tuberous sclerosis complex for whom a chest CT scan less than 3 years old does not objectify a pulmonary cyst. (= without lymphangioleiomyomatosis)
Non-inclusion criteria
-
Woman under judicial protection
-
Woman with a urinary tract infection within 15 days
-
Person who objected to the data processing
Contacts and Locations
Locations
| Site | City | State | Country | Postal Code | |
|---|---|---|---|---|---|
| 1 | University hospital | Tours | France | 37044 |
Sponsors and Collaborators
- University Hospital, Tours
Investigators
- Principal Investigator: Sylvain MARCHAND-ADAM, University Hospital, Tours
Study Documents (Full-Text)
None provided.More Information
Publications
None provided.- DR220020