Bone Microarchitecture in Men With Hemophilia

Hospices Civils de Lyon10 enrolled

Overview

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.

Study Type

INTERVENTIONAL

Allocation

NON_RANDOMIZED

Purpose

DIAGNOSTIC

Masking

NONE

Enrollment

Conditions

Severe Hemophilia A Osteoporosis

Interventions

HR-pQCTRADIATION

Patients will have a unique HR-pQCT scanner imaging to study their bone microarchitecture

Blood sampleBIOLOGICAL

Patients will have a unique blood sampling of 10 mL for the measurements of sera PINP, CTX-I, periostin levels

Dual energy X-ray absorptiometryRADIATION

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 collectionOTHER

Data of healthy men is already available

Eligibility

Sex: MALEMin age: 20 YearsMax age: 60 Years

Medical Language ↔ Plain English

Inclusion Criteria: Patients: * Men aged 20 to 60 years * Severe hemophilia A or B (FVIII or FIX\<1%) * Regular followed up in Lyon Hemophilia Center * Ability to give free and informed consent * Person capable of actively participating in radiological examinations Healthy Mens: * Aged 20 to 60 years 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.

Outcomes

Primary Outcomes

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