Bone Microarchitecture in Men With Hemophilia

Not Applicable

Terminated

Interventions: Radiation: HR-pQCT
Other: medical data collection
Biological: Blood sample
Radiation: Dual energy X-ray absorptiometry

Brief Summary: Hemophilia A and B are hereditary sex-linked deficiencies of coagulation factors VIII and IX characterized by bleeding. Their modern therapy increases life expectancy and risk of age-related diseases, e.g., osteoporosis.

Hemophilia-specific risk factors impair formation of peak bone mass and accelerate bone loss. Fractures are more frequent in hemophilic men vs. age-matched men and induce bleeding which is aggravated by manipulations and surgical intervention.

The hypothesis of this study is that hemophilic men have poor bone microarchitecture (assessed by High-resolution peripheral quantitative computed tomography (HR-pQCT)) related to an imbalance between bone formation and resorption (assessed by bone turnover markers (BTM) and bone biomarkers).

The study aims to assess the difference in low trabecular number (Tb.N) at the distal radius between hemophilic men (cases) and age- height-weight-ethnicity and smoking-matched healthy men (controls). Correlation between BTM and Tb.N will be also studied.

Biologic markers of bone remodeling (C-terminal telopeptide of type I collagen (PINP), N-terminal propeptide of type I procollagen (CTX-I), periostin) will be studied.

Recruitment & Eligibility

Inclusion Criteria

Patients:

Healthy Mens:

Exclusion Criteria

Women
Not covered by health system
Vulnerable (adults unable to consent, protected under guardianship, prisoner)
Any blood coagulation abnormality other than severe hemophilia A or B
Having one of the following treatments against osteoporosis of more than 6 months : bisphosphonates, denosumab, teriparatide
With a chronical disease having a high impact on bone structure and no related to hemophilia disease, such as Cushing or Crohn diseases.

Arm && Interventions

Group	Intervention	Description
severe hemophilia A or B patients	Blood sample	30 patients with severe hemophilia A or B (Factor (F)VIII or Factor IX (FIX)≤ 1%) aged 20 to 60 years will be included in this study.
severe hemophilia A or B patients	HR-pQCT	30 patients with severe hemophilia A or B (Factor (F)VIII or Factor IX (FIX)≤ 1%) aged 20 to 60 years will be included in this study.
healthy men	medical data collection	Data of healthy men, matching in age- height-weight-ethnicity and smoking-matched with patient will be collected. These data are already available at the "Institut national de la santé et de la recherche médicale" (INSERM) research unit associated.
severe hemophilia A or B patients	Dual energy X-ray absorptiometry	30 patients with severe hemophilia A or B (Factor (F)VIII or Factor IX (FIX)≤ 1%) aged 20 to 60 years will be included in this study.

Primary Outcome Measures

Name	Time	Method
number of trabecular at distal tibia	3 months following the inclusion	The number of trabecular at distal tibia will be performed and compared to the values available for the healthy control group
number of trabecular at distal radius	3 months following the inclusion	The number of trabecular at distal radius will be performed and compared to the values available for the healthy control group

Secondary Outcome Measures

Name	Time	Method
reflection of bone strength	3 months following the inclusion	Micro-Finite Element Analysis (µFEA) reflection of bone strength will be performed and compared to the values available for the healthy control group
Sera bone remodeling biomarkers	3 months following the inclusion	This biomarkers will be performed: * PINP (ng/ml) * CTX-1 (ng/ml) * Periostin (ng/ml)
Trabecular Bone Score (TBS)	3 months following the inclusion	Trabecular Bone Score (TBS) will be performed and compared to the values available for the healthy control group The trabecular bone score is a measure of bone texture correlated with bone microarchitecture and a marker for the risk of osteoporosis