Lymphangioleiomyomatosis, a Study on Cathepsin K

Completed

• Conditions: Tuberous Sclerosis Complex; Lymphangioleiomyomatosis
• Interventions: Other: 24h urine; Other: urine sample

• Registration Number: NCT05323370
• Lead Sponsor: University Hospital, Tours

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.

Detailed Description

Not available

Study Timeline

• Study First Submitted: 2022-04-04
• Study First Submitted QC: 2022-04-04
• Study First Posted: 2022-04-12
• Study Start: 2022-05-31
• Primary Completion: 2022-10-30
• Study Completion: 2022-10-30
• Status Verified: 2023-03
• Last Update Submitted: 2023-03-28
• Last Update Posted: 2023-03-29

Recruitment & Eligibility

• Status: COMPLETED
• Sex: Female
• Target Recruitment: 19

Inclusion Criteria

Not provided

Exclusion Criteria

Not provided

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Patientes with tuberous sclerosis complex without Lymphangioleiomyomatosis	24h urine	no intervention administered. Recruitment in neurology department
Patientes with tuberous sclerosis complex without Lymphangioleiomyomatosis	urine sample	no intervention administered. Recruitment in neurology department
Patientes with lymphangioleiomyomatosis	24h urine	no intervention administered. Recruitment in pneumology department
Patientes with lymphangioleiomyomatosis	urine sample	no intervention administered. Recruitment in pneumology department
Healthy women volunteers	urine sample	no intervention administered. Recruitment in clinical investigation centre
Healthy women volunteers	24h urine	no intervention administered. Recruitment in clinical investigation centre

Primary Outcome Measures

Name	Time	Method
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

Name	Time	Method
urinary quantity of cystatin C	inclusion visit	comparison of urine cystacin C quantity in the 3 groups
FEV1 measurement	inclusion visit	most recent FEV1 measurement as % of predicted value in lymphangioleiomyomatosis patients
serum level of chondroitin sulphates	inclusion visit	measurement 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
urinary quantity C and N-telopeptides of type 1 collagen	inclusion visit	comparison in the 3 groups
renal angiolipomas	inclusion visit	measurement of the size of renal angiomyolipomas in lymphangioleiomyomatosis patients

Trial Locations

Locations (1)

University hospital; 🇫🇷 Tours, France