The quality of life in children with spinal muscular atrophy: a case–control study

BMC Pediatrics

Research

Nahid Khosroshahi¹  Sareh Hosseinpour²  Masood Ghahvechi Akbari³  Homa Ghabeli⁴  Gholamreza Zamani⁴  Mahmoud Reza Ashrafi⁴  Morteza Heidari⁴  Reza Shervin Badv⁴  Mahmoud Mohammadi⁴  Roya Haghighi⁴  Elham Pourbakhtyaran⁴ 

[1] Department of Pediatric Neurology, Bahrami Children’s Hospital, Tehran University of Medical Sciences, Tehran, Iran;Department of Pediatric Neurology, Vali-E-Asr Hospital, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran;Department of Physical Medicine and Rehabilitation, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran;Pediatrics Center of Excellence, Department of Pediatric Neurology, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran;

关键词: Spinal muscular atrophy;    Health-related quality of life;    Quality of life;    Children;   

DOI  :  10.1186/s12887-022-03751-y

 received in 2022-05-17, accepted in 2022-11-14,  发布年份 2022

来源: Springer

【 摘 要 】

ObjectivesThis study aimed to analyze the health-related quality of life (HRQoL) of patients with spinal muscular atrophy (SMA) based on the type of SMA, demographic and clinical features and compare HRQoL of these patients with a matched healthy control group. MethodsThis was a case–control study of Patients with SMA in Iran. Sixty-six patients with SMA type II and III aged 8–18 years and also 264 healthy age, sex, and socio-economic matched individuals were enrolled. To assess the quality of life, we used the Persian version of the KIDSCREEN-27.ResultsThe health-related quality of life between children with type II and type III SMA was not significant in all 5 subscales. However, HRQoL in healthy children was significantly higher than in SMA children in all 5 subscales.ConclusionThe quality of life in children with SMA was lower than the healthy control group in all subscales, and physical well-being and psychosocial aspects are the main domains of life impaired by SMA disease. However, no significant difference between the quality of life in children with SMA type II and type III was observed. 

【 授权许可】

CC BY   
© The Author(s) 2022