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| ID | Type | Description | Link |
|---|---|---|---|
| 2019-A03281-56 | Other Identifier | ID-RCB |
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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| severe hemophilia A or B patients | Experimental | 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 | Other | 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. |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| HR-pQCT | Radiation | Patients will have a unique HR-pQCT scanner imaging to study their bone microarchitecture |
|
| Measure | Description | Time Frame |
|---|---|---|
| number of trabecular at distal tibia | The number of trabecular at distal tibia will be performed and compared to the values available for the healthy control group | 3 months following the inclusion |
| number of trabecular at distal radius | The number of trabecular at distal radius will be performed and compared to the values available for the healthy control group | 3 months following the inclusion |
| Measure | Description | Time Frame |
|---|---|---|
| Sera bone remodeling biomarkers | This biomarkers will be performed:
| 3 months following the inclusion |
| reflection of bone strength | Micro-Finite Element Analysis (µFEA) reflection of bone strength will be performed and compared to the values available for the healthy control group |
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Inclusion Criteria:
Patients:
Healthy Mens:
Exclusion Criteria:
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hôpital Cardio-Vasculaire et Pneumologique | Bron | 69677 | France |
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| ID | Term |
|---|---|
| D006467 | Hemophilia A |
| D010024 | Osteoporosis |
| ID | Term |
|---|---|
| D025861 | Blood Coagulation Disorders, Inherited |
| D001778 | Blood Coagulation Disorders |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
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| ID | Term |
|---|---|
| D001800 | Blood Specimen Collection |
| D015502 | Absorptiometry, Photon |
| ID | Term |
|---|---|
| D013048 | Specimen Handling |
| D019411 | Clinical Laboratory Techniques |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
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| Blood sample | Biological | Patients will have a unique blood sampling of 10 mL for the measurements of sera PINP, CTX-I, periostin levels |
|
| Dual energy X-ray absorptiometry | Radiation | A dual energy X-ray absorptiometry scan of lumbar spine, hip, distal radius and whole body, body composition, lateral spine will be performed. |
|
| medical data collection | Other | Data of healthy men is already available |
|
| 3 months following the inclusion |
| Trabecular Bone Score (TBS) | 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 | 3 months following the inclusion |
| D020147 | Coagulation Protein Disorders |
| D006474 | Hemorrhagic Disorders |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D001851 | Bone Diseases, Metabolic |
| D001847 | Bone Diseases |
| D009140 | Musculoskeletal Diseases |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
| D011677 | Punctures |
| D013514 | Surgical Procedures, Operative |
| D008919 | Investigative Techniques |
| D011859 | Radiography |
| D003952 | Diagnostic Imaging |
| D003720 | Densitometry |
| D010783 | Photometry |
| D002623 | Chemistry Techniques, Analytical |