Lymphangioleiomyomatosis, Tuberous Sclerosis Complex
Conditions
Keywords
urine
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.
Interventions
OTHER24h urine
Urine samples will be collected at home over 24 hours
OTHERurine sample
A urine sample of 20cc will be collected
Sponsors
University Hospital, Tours
Study design
Observational model
CASE_CONTROL
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
FEMALE
Age
18 Years to No maximum
Healthy volunteers
Yes
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| 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
| Measure | Time frame | Description |
|---|---|---|
| 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 |
| urinary quantity of cystatin C | inclusion visit | comparison of urine cystacin C quantity in the 3 groups |
| 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 |
| FEV1 measurement | inclusion visit | most recent FEV1 measurement as % of predicted value in lymphangioleiomyomatosis patients |
| urinary quantity C and N-telopeptides of type 1 collagen | inclusion visit | comparison in the 3 groups |
Countries
France
Outcome results
None listed