Clinical follow-up analysis of multidisciplinary treatment of children with spinal muscular atrophy.
10.3760/cma.j.cn112140-20220221-00138
- Author:
Yu XIA
1
;
Yi Jie FENG
1
;
Mei YAO
1
;
Jia Ning JIN
1
;
Jia WEI
1
;
Yi Qin CUI
1
;
Ling Shuang WANG
1
;
Ting Ting CHEN
2
;
Xiao Yang CHEN
2
;
Hai Bing LI
3
;
Jing Fang XU
3
;
Qi LONG
4
;
Yuan JIANG
5
;
Jin Ling LIU
5
;
Jin Gan LOU
6
;
Feng GAO
1
;
Shan Shan MAO
1
Author Information
1. Department of Neurology, the Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, China.
2. Department of Developmental Behavior, the Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, China.
3. Department of Orthopedics, the Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, China.
4. Department of Clinial Nutrition, the Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, China.
5. Department of Respiratory, the Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, China.
6. Department of Gastroenterology, the Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, China.
- Publication Type:Journal Article
- MeSH:
Child;
Male;
Female;
Humans;
Child, Preschool;
Scoliosis;
Retrospective Studies;
Follow-Up Studies;
Muscular Atrophy, Spinal;
Malnutrition
- From:
Chinese Journal of Pediatrics
2022;60(11):1134-1139
- CountryChina
- Language:Chinese
-
Abstract:
Objective: To analyze the follow-up and clinical effect of multidisciplinary treatment on the children with spinal muscular atrophy (SMA). Methods: The clinical data including nutritional status, respiratory function, bone health and motor function of 45 children with SMA who received multidisciplinary management 1-year follow-up in the Children's Hospital, Zhejiang University School of Medicine from July 2019 to October 2021 were retrospectively collected. Comparisons before and after management were performed using paired-samples t-test or Wilcoxon rank-sum test, etc. Results: The age of 45 patients (25 boys and 20 girls) was 50.4 (33.6, 84.0) months at the enrollment, with 6 cases of type 1, 22 cases of type 2, and 17 cases of type 3 respectively. After the multidisciplinary management, the cases of SMA patients with malnutrition decreased from 22 to 12 (P=0.030), the level of vitamin D were significantly increased ((45±17) vs. (48±14) nmol/L, t=-4.13, P<0.001). There was no significant difference in the forced vital capacity %pred, the forced expiratory volume at 1 second %pred, and the peak expiratory flow %pred ((76±19)% and (76±21)%, (81±18)% and (79±18)%, (81±21)% and (78±17)%; t=-0.24, 1.36, 1.21; all P>0.05). The Cobbs angle of scoliosis also improved significantly (8.0°(0°, 13.0°) vs. 10.0°(0°, 18.5°), Z=-3.01, P=0.003). The Hammersmith functional motor scale expanded scores of children with SMA type 2 and type 3 both showed significant elevation (11.0 (8.0, 18.0) vs. 11.0 (5.0, 18.5) scores, 44.0 (36.5, 53.0) vs. 44.0 (34.0, 51.5) scores, Z=2.44, 3.11, P=0.015, 0.002). Conclusion: Multidisciplinary management is beneficial for delaying the progression of the multi-system impairments of SMA patients, such as malnutrition, restrictive ventilation dysfunction and scoliosis.
