Evaluation of the Benefits of Adaptive Physical Activity in Children and Adolescents With Osteogenesis Imperfecta

Study Overview
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Brief Summary
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Osteogenesis imperfecta (OI) is a rare genetic disorder of increased bone fragility and low bone mass. It is conceivable that children and adolescents with OI are less active than healthy peers because of frequent fractures, immobilization,functionals limitations and no adapted physicals activity(APA). The hypothesis is that an Adapted physique activity could improve access of activity for patients with Osteogenesis Imperfecta (OI). The aim of the study is to evaluate benefice of APA,improve aerobic capacity, cardiovascular and bone benefits, and gain of quality of life. Children with OI between 6 and 18 years old will have a program of supervised adapted training program during one year. The program is adapted at each individual and without risk for the patient.

Official Title
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Evaluation of the Benefits of Adaptive Physical Activity in Children and Adolescents With Osteogenesis Imperfecta

Conditions
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Osteogenesis Imperfecta

Intervention / Treatment
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* Other: Adapted sports practices


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Child with osteogenesis imperfecta Child followed in the Reference centre for constitutional bone diseases in the Hôpital Femme Mère Enfant Parent (s) / legal guardian who has been informed of the study and has accepted participation in the study by signing the consent. Patient benefiting from a social security scheme Exclusion Criteria: Medical and surgical contraindications to adapted physical activity.

Ages Eligible for Study
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Minimum Age: 6 Years
Maximum Age: 17 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Prevention
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Adapted sports practice<br>patients with osteogenesis imperfect will practice adapted sport twice a week during 12 months in order to improve their aerobic capacity, cardiovascular and bone benefits, and gain of quality of life. | Other: Adapted sports practices<br>* Adapted sports practices for 30 minutes twice a week<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 6 minutes walk test | Distance traveled (in meters) in 6 minutes | Month 0 | 
| 6 minutes walk test | Distance traveled (in meters) in 6 minutes | Month 6 | 
| 6 minutes walk test | Distance traveled (in meters) in 6 minutes | Month 12 | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Weight | weight in kilograms will be measured with a scale | Month 0 | 
| Weight | weight in kilograms will be measured with a scale | Month 6 | 
| Weight | weight in kilograms will be measured with a scale | Month 12 | 
| Height | Size in meter measured with a meter | Month 0 | 
| Height | Size in meter measured with a meter | Month 6 | 
| Height | Size in meter measured with a meter | Month 12 | 
| body mass index | calculated with weight and height | Month 0 | 
| body mass index | calculated with weight and height | Month 6 | 
| body mass index | calculated with weight and height | Month 12 | 
| blood pressure | measurement of blood pressure (cm Hg) | Month 0 | 
| blood pressure | measurement of blood pressure (cm Hg) | Month 6 | 
| blood pressure | measurement of blood pressure (cm Hg) | Month 12 | 
| phosphocalcic biologic parameters | Ionosodium (mmol/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | potassium (mmol/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | calcium (mmol/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | chlore (mmol/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | bicarbonates (mmol/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | glucose (mmol/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | urea (mmol/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | creatinemia (mmol/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | phosphorus (mmol/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | proteinemia (g/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | Vitamin D (nmol/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | 1-25 OH vitamin D (pmol/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | PTH (ng/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | alkaline bone phosphatases (U/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | CTX (pg/ml)crosslaps | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | osteocalcin (µg/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | ferritin (µg/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | blood count formula : leucocytes (giga/L), erythrocytes (tera/L), hemoglobin (g/L), hematocrit (%), platelets (giga/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | calciuria (mmol/L) | Month 0 and Month 12 | 
| phosphocalcic biologic parameters | Ionosodium (mmol/L) potassium (mmol/L), calcium (mmol/L), chlore (mmol/L), bicarbonates (mmol/L), glucose (mmol/L), urea (mmol/L), creatinemia (mmol/L), phosphorus (mmol/L), proteinemia (g/L), Vitamin D (nmol/L), 1-25 OH vitamin D (pmol/L), PTH (ng/L), alkaline bone phosphatases (U/L), CTX (pg/ml)crosslaps, osteocalcin (µg/L), NFS, ferritin (µg/L), blood count formula : leucocytes (giga/L), erythrocytes (tera/L), hemoglobin (g/L), hematocrit (%), platelets (giga/L), calciuria (mmol/L), urinary creatinin (mmol/L). | Month 0 and Month 12 | 
| ration lean mass / fat mass | Dual-energy X-ray absorptiometry (DXA) | Month 0 | 
| ration lean mass / fat mass | Dual-energy X-ray absorptiometry (DXA) | Month 12 | 
| Respiratory Functional Exploration |  | Month 0 | 
| Respiratory Functional Exploration |  | Month 12 | 
| Thickness intima / media | echography | Month 0 | 
| Parameters of the connected watch : number of steps | data recovery of the connected watch | Month 0 and Month 6 and Month 12 | 
| Parameters of the connected watch : distance traveled | data recovery of the connected watch | Month 0 and Month 6 and Month 12 | 
| Parameters of the connected watch : calories consumption | data recovery of the connected watch | Month 0 and Month 6 and Month 12 | 
| Ratio thickness intima / media | echography | Month 12 | 
| Bone mineral density | Dual-energy X-ray absorptiometry (DXA) | Month 0 | 
| Bone mineral density | Dual-energy X-ray absorptiometry (DXA) | Month 12 | 
| Number of new fractures |  | Month 1 | 
| Number of new fractures |  | Month 3 | 
| Number of new fractures |  | Month 6 | 
| Number of new fractures |  | Month 9 | 
| Number of new fractures |  | Month 12 | 
| Quality of life questionnaire : PedsQL | Measurement Model for the Pedratric Quality of Life Inventory The 4 Multidimensional Scales and 3 Summary Scores are Scales Physical Functioning (8 items) Emotional Functioning (5 items) Social Functioning (5 items) School Functioning (5 items) Summary Scores Total Scale Score (23 items) Physical Health Summary Score (8 items) Psychosocial Health Summary Score (15 items) | Month 0 | 
| Quality of life questionnaire : PedsQL | Measurement Model for the Pedratric Quality of Life Inventory The 4 Multidimensional Scales and 3 Summary Scores are Scales Physical Functioning (8 items) Emotional Functioning (5 items) Social Functioning (5 items) School Functioning (5 items) Summary Scores Total Scale Score (23 items) Physical Health Summary Score (8 items) Psychosocial Health Summary Score (15 items) | Month 6 | 
| Quality of life questionnaire : PedsQL | Measurement Model for the Pedratric Quality of Life Inventory The 4 Multidimensional Scales and 3 Summary Scores are Scales Physical Functioning (8 items) Emotional Functioning (5 items) Social Functioning (5 items) School Functioning (5 items) Summary Scores Total Scale Score (23 items) Physical Health Summary Score (8 items) Psychosocial Health Summary Score (15 items) | Month 12 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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osteogenesis imperfecta, Adapted sport, Quality of life