Evaluation of the Benefits of Adaptive Physical Activity in Children and Adolescents With Osteogenesis Imperfecta (MOVE-OI)

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

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

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.

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.

blood count formula : leucocytes (giga/L), erythrocytes (tera/L), hemoglobin (g/L), hematocrit (%), platelets (giga/L)

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).

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)

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)

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)

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.

Centre de Référence des Maladies Rénales Rares - Hospices Civils de Lyon - Service de Néphrologie et Rhumatologie Pédiatriques - Hôpital Femme Mère Enfant