Page 227 - Petelin, Ana, et al. Eds. 2019. Zdravje otrok in mladostnikov / Health of Children and Adolescents. Zbornik povzetkov z recenzijo / Book of Abstracts. Koper: Založba Univerze na Primorskem/University of Primorska Press
P. 227
ects of respiratory exercises in children with spinal muscular otrok in mladostnik – okolje | child and adolescent - environment
atrophy
Anton Zupan1, Tina Barašin2, Martina Sardoč2, Mitja Slapar3
1 University Rehabilitation Institute Republic of Slovenia – Soča, Linhartova 51,
1000 Ljubljana, Slovenia
2 Muscular distrophy association of Slovenia, Linhartova 1, 1000 Ljubljana, Slovenia
3 Alma Mater Europaea - ECM, Slovenska cesta 17, 2000 Maribor, Slovenia
Introduction: Spinal muscular atrophy (SMA) is an autosomal recessive neuro-
degenerative disease in childhood characterized by the degeneration and loss
of lower motor neurons in the front horn cells of the spinal cord, causing pro-
gressive proximal weakness and atrophy of the skeletal muscles. The cause of
SMA is homozygous mutation in the SMN1 gene.
Methods: The aim of the study was to investigate the effects of respiratory ex-
ercises on pulmonary function in children with SMA. Nine children with SMA
participated in the study were included in a 2-week rehabilitation program. In
addition to other daily therapies, they had an additional 20 minutes of respira-
tory training. The measurements were performed with the Vitalograph In2itive
spirometer in a seated and lying position.
Results: A slight improvement in pulmonary function occurred after treatment
was completed. Children with a mild form of SMA had better results than chil-
dren with a more severe form of SMA. Children who had a higher body mass
index (ITM) had a higher VC than children with a lower BMI.
Discussion and conclusion: The weakness of respiratory muscles and conse-
quent restrictive respiratory failure is the main cause of morbidity and mor-
tality of children with SMA. In our study there was a slight improvement of
pulmonary function in SMA children after 14 days of respiratory training. We
conclude that respiratory training in children with SMA is helpful and advisable.
Keywords: spinal muscular atrophy, respiratory exercises, pulmonary
capacity
225
atrophy
Anton Zupan1, Tina Barašin2, Martina Sardoč2, Mitja Slapar3
1 University Rehabilitation Institute Republic of Slovenia – Soča, Linhartova 51,
1000 Ljubljana, Slovenia
2 Muscular distrophy association of Slovenia, Linhartova 1, 1000 Ljubljana, Slovenia
3 Alma Mater Europaea - ECM, Slovenska cesta 17, 2000 Maribor, Slovenia
Introduction: Spinal muscular atrophy (SMA) is an autosomal recessive neuro-
degenerative disease in childhood characterized by the degeneration and loss
of lower motor neurons in the front horn cells of the spinal cord, causing pro-
gressive proximal weakness and atrophy of the skeletal muscles. The cause of
SMA is homozygous mutation in the SMN1 gene.
Methods: The aim of the study was to investigate the effects of respiratory ex-
ercises on pulmonary function in children with SMA. Nine children with SMA
participated in the study were included in a 2-week rehabilitation program. In
addition to other daily therapies, they had an additional 20 minutes of respira-
tory training. The measurements were performed with the Vitalograph In2itive
spirometer in a seated and lying position.
Results: A slight improvement in pulmonary function occurred after treatment
was completed. Children with a mild form of SMA had better results than chil-
dren with a more severe form of SMA. Children who had a higher body mass
index (ITM) had a higher VC than children with a lower BMI.
Discussion and conclusion: The weakness of respiratory muscles and conse-
quent restrictive respiratory failure is the main cause of morbidity and mor-
tality of children with SMA. In our study there was a slight improvement of
pulmonary function in SMA children after 14 days of respiratory training. We
conclude that respiratory training in children with SMA is helpful and advisable.
Keywords: spinal muscular atrophy, respiratory exercises, pulmonary
capacity
225
