Musculoskeletal Diseases

All Conditions

Diseases and Abnormalities at or Before Birth

Neoplasms

Nervous System Diseases

Skin and Connective Tissue Diseases

Nutritional and Metabolic Diseases

Symptoms and General Pathology

Behaviors and Mental Disorders

Rare Diseases

Scoliosis

Congenital Abnormalities

Spinal Diseases

Neurofibromatoses

Neurofibromatosis 1

Neurofibroma

Osteochondrodysplasias

Mucopolysaccharidosis IV

Spinal Curvatures

Bone Diseases

Nerve Sheath Neoplasms

Neoplasms, Nerve Tissue

Neoplasms by Histologic Type

Syndrome

Neoplastic Syndromes, Hereditary

Neurocutaneous Syndromes

Heredodegenerative Disorders, Nervous System

Neurodegenerative Diseases

Genetic Diseases, Inborn

Bone Diseases, Developmental

Quality of Life

Neurofibromatosis

Neurofibromatosis Type 1

Mucopolysaccharidosis Type IV

Thomas Johan Kibsgård, MD PhD

Observation study about physical activity, motor competence, pulmonary function, and health related quality of life in children, surgically treated for early onset scoliosis

Early Onset Scoliosis (EOS) defines patients who develop deformities of the spine and/or thorax in the embryologic development or in the early childhood. The exact prevalence of EOS in Norway is unknown, but it is assumed to be approximately 200-300 new EOS patients every year. EOS is a severe condition, whereby some of the most common and also most severe consequences of the deformity are severe pulmonary problems. The patients may require extensive orthopedic treatment from early childhood and until maturity to avoid serious consequences with severe pulmonary problems and shortening of life.

EOS' secondary problems, including severe consequences on the pulmonary function might contribute to enhanced inactivity. Inactivity contributes to further negative impact on development of the musculoskeletal system, motor competence and pulmonary function. Considering the severity of EOS and children's reduced life expectancy, it is extremely important that EOS children achieve the favorable impact of physical activity during their growth. Insufficient level of activity might worsen the prognosis, thus counteracting the treatment's ambitions. The investigator's aim is to extend the knowledge about level of activity, motor competence, pulmonary function, and health related quality of life in Norwegian children, surgically treated for EOS.

Physical Activity, Motor Competence, Pulmonary Function, and Quality of Life in Children With Severe Spinal Disease

Physical Activity will be measured objective by use of an accelerometer (used over 7 days), recorded as Activity Counts/ minutes

Children \>8 years will also be assessed by use of plethysmography. Airway resistance will be measured, recorded as the ratio of driving pressure divided by flow through the airways

Children \>8 years will also be assessed by use of plethysmography. Residual volume (liters) and total lung capacity (liters) will be measured.

Children \>8 years will be assessed by use of diffusion capacity test. Diffusing capacity or transfer factor of the lung for carbon monoxide (CO) (DLCO) will be measured.

All participants will go through spirometry. Vital capacity (VC), Forced vital capacity (FVC), Forced expiratory volume (FEV) at timed intervals of 0.5, 1.0 (FEV1), 2.0, and 3.0 seconds and maximal voluntary ventilation (MVV) will be measured (liters)

All participants will go through spirometry. Forced expiratory flow 25-75% (FEF 25-75) will be measured (liters/second)

Motor competence will be assessed by use of Movement Assessment Battery of Children- 2. edition

Health related quality of life will be assessed by use of the Early Onset Scoliosis 24-item Questionnaire (EOSQ-24). Total score, ranged 0 (worst) to 100 (best), including 11 subdomain scores, ranged 0 (worst) to 100 (best) will be calculated.

Among participants \> 16 years, health related quality of life will be assessed by use of the Scoliosis Research Society 22- item (SRS-22) questionnaire. Total score and 5 subdomain scores, all ranged 5 (best) to 1 (worst), will be calculated.

A physiotherapist will assess the active shoulder range of motion, categorized as normal, active range of motion \>50% of perceived motion, or active range of motion \<50% of perceived motion. shoulder flexion, extension, abduction, and rotation will be measured.

Physical Activity will be subjective measured by use of a questionnaire regarding participants' everyday activity. Participants range their activity in several sports activities from never (1) to several days a week (4).The questionnaire is previous used in a national survey study regarding physical activity among children and adolescents in Norway.

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization	Link
Physical Activity, Motor Competence, Pulmonary Function, and Quality of Life in Children With Severe Spinal Disease	NCT03494829	Scoliosis; Idio... Scoliosis; Juve... Scoliosis Neuro... Scoliosis in Ne... Scoliosis; Cong... Scoliosis in Sk... Scoliosis Assoc... Scoliosis; Cong... Scoliosis		1 Year - 20 Years	Oslo University Hospital
Physical Activity, Motor Competence, Pulmonary Function, and Quality of Life in Children With Severe Spinal Disease	NCT03494829	Scoliosis; Idio... Scoliosis; Juve... Scoliosis Neuro... Scoliosis in Ne... Scoliosis; Cong... Scoliosis in Sk... Scoliosis Assoc... Scoliosis; Cong... Scoliosis		1 Year - 20 Years	Oslo University Hospital

Spots Global Cancer Trial Database for scoliosis in neurofibromatosis