Bone Microarchitecture in Young Cystic Fibrosis Patients

Study Overview
=================
Brief Summary
-----------------
Patients with cystic fibrosis are at risk of developing low bone mineral density (BMD) potentially leading to pathological fractures at adult age. Recent data from our center and others have suggested that low BMD could be observed very early in life. However, quantitative bone abnormalities found out by Dual X-ray absorptiometry (DXA) need to be confronted to qualitative evaluation of bone microarchitecture (surrogate of bone strength). High-Resolution peripheral quantitative computed tomography (HR-pQCT) is a recent technology with very high spatial resolution. Images obtained with this technic are considered as virtual bone biopsies. It enables an accurate bones' cortical and trabecular surfaces exploration in a three-dimensional manner, and therefore provides informations on bone microarchitecture as well as bone density. The aim of this study is to evaluate bone microarchitecture of paediatric patients matched to sex-age-pubertal status-healthy volunteers. In the meantime, biological markers will be collected and DXA (Dual-energy x-ray absorptiometry) will be performed in order to explore potential correlations HR-pQCT parameters.

Official Title
-----------------
Evaluation of the Bone Microarchitecture in a Young Cystic Fibrosis Patients Using High-Resolution Peripheral Quantitative Computed Tomography

Conditions
-----------------
Cystic Fibrosis

Intervention / Treatment
-----------------
* Other: scan examination HR-pQCT


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Cystic Fibrosis patient of both sex Pubertal patient Age ≥10 years and ≤18 years on the date of informed consent FEV1(forced expiratory volume at one second ) ≥ 60% of predicted normal for age, gender and height Patient on a clinical stable period Exclusion Criteria: Unable to maintain arm and/or leg immobile for 3 minutes History of solid organ transplantation Participation in the same time to a clinical trial Acute pulmonary exacerbation at the time of evaluation

Ages Eligible for Study
-----------------
Minimum Age: 10 Years
Maximum Age: 18 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
Accepts Healthy Volunteers

Study Plan
=================
How is the study designed?
-----------------

Design Details

Primary Purpose: Diagnostic
Allocation: Non-Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Healthy volunteers<br>Volunteers realize a HR-pQCT scanner | Other: scan examination HR-pQCT<br> <br> |
| Experimental: Cystic Fibrosis patient<br>Patients realize a HR-pQCT scanner | Other: scan examination HR-pQCT<br> <br> |

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Total Tibial Bone Mass Density measured by High-Resolution peripheral Quantitative Computed Tomography |  | at the inclusion visit J0 | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| total radial bone mass density |  | at the inclusion visit J0 | 
| Trabecular bone micro-architecture at tibia and radial sites |  | at the inclusion visit J0 | 
| Biological markers : 1) bone markers : parathyroid hormone (PTH), Calcifediol (25(OH)D3), osteocalcin, Endocrinal markers : IGF-1 (insulin like growth factor ), IGFBP-3, leptin, adiponectin, visfatin, resistin |  | at the inclusion visit J0 | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
Cystic Fibrosis, High-Resolution peripheral Quantitative Computed Tomography, Bone Mass Density