儿童脊髓性肌萎缩症的肺功能变化及临床意义  

Changes in Pulmonary Function and Clinical Significance in Children with Spinal Muscular Atrophy

作　　者：许淑争 马碧泓 邵巧燕[1] 张晓燕[1] 施晓容[1] XU Shuzheng;MA Bihong;SHAO Qiaoyan;ZHANG Xiaoyan;SHI Xiaorong(Department of Pediatrics,The First Affiliated Hospital of Fujian Medical University,Fuzhou 350005,China)

机构地区：[1]福建医科大学附属第一医院儿科,福州350005

出　　处：《福建医科大学学报》2023年第3期211-216,共6页Journal of Fujian Medical University

摘　　要：目的探讨儿童脊髓性肌萎缩症(SMA)的肺功能变化及临床意义。方法选取2022年1—12月住院治疗的28例SMA患儿为研究对象,患儿年龄5~14岁,入院后均进行肺功能检测,均采用肺功能检测仪测定用力肺活量(FVC)、第一秒用力呼气容积(FEV1)、1秒率(FEV1/FVC)、最大呼气流量(PEF)等指标,比较不同临床分型、不同肌力、有无脊柱侧弯的SMA患儿的肺功能损伤程度。结果28例中,男童17例,女童11例;年龄9.00(7.25,12.38)岁;病程8.0(6.0,10.5)a。根据临床分型进行分组,1型0例、2型22例、3型6例、4型0例,均存在不同程度肺功能损害,均呈限制性通气功能障碍。3型患儿的FVC、FEV1、PEF均显著高于2型,差别均有统计学意义(P<0.05)。L1组(下肢近端肌力0~2级)患儿的FVC、FEV1、PEF显著低于L2组(下肢近端肌力3~5级),U1组(上肢近端肌力0~2级)患儿的FVC、FEV1、PEF亦显著低于U2组(上肢近端肌力3~5级),差别均有统计学意义(P<0.05)。脊柱侧弯组患儿的FVC、FEV1、PEF均低于脊柱无侧弯组,差别均有统计学意义(P<0.05)。3组病程长短不同的患儿,FVC、FEV1、FEV1/FVC、PEF组间比较,差别均无统计学意义(P>0.05)。结论肺功能检查可作为评估SMA患儿病情严重程度的重要部分,有助于对其开展多学科个体化管理,指导改善SMA患儿预后。Objective It is to investigate the changes of pulmonary function in children with spinal muscular atrophy(SMA)and its clinical significance.Methods A total of 28 children with SMA who were hospitalized from January to December 2022 were selected for this study.All children underwent lung function test after admission.Forced vital capacity(FVC),forced expiratory volume in one second(FEV1),the ratio of forced expiratory volume in one second to all expiratory volume(FEV1/FVC),and peak expiratory flow(PEF)were measured by pulmonary function tester.The degree of pulmonary function impairment in children with spinal muscular atrophy with different clinical subtypes,different muscle strengths and with or without scoliosis were compared.Results There were 28 children with SMA,including 17 boys and 11 girls,with a mean age of 9.00(7.25,12.38)years and a course of disease of 8.0(6.0,10.5)years.Grouped according to clinical staging,0 cases of type 1 SMA,22 cases of type 2 SMA and 6 cases of type 3 SMA had different degrees of pulmonary function impairment and all showed restrictive ventilation dysfunction.The FVC,FEV1 and PEF of the children with type 3 SMA were significantly higher than those of children with type 2 SMA,and the differences between the two groups were statistically significant(P<0.05).FVC,FEV1,and PEF in group L1(proximal muscle strength of lower limbs of 0-2 grade)were significantly lower than those in group L2(proximal muscle strength of lower limbs 3-5 grade),and those in group U1(proximal muscle strength of upper limbs of 0-2 grade)were also significantly lower than those in group U2(proximal muscle strength of upper limbs 3-5 grade),with statistically significant differences(P<0.05).The FVC,FEV1,and PEF of children in the scoliosis group were lower than those in the non-scoliosis group,and the differences were statistically significant(P<0.05).There was no statistical significance in FVC,FEV1,FEV1/FVC and PEF among the three groups with different courses of disease(P>0.05).Conclusion Pulmonary function

关 键 词：肺功能 脊髓性肌萎缩症 肌力 脊柱侧弯 

分 类 号：R746.4[医药卫生—神经病学与精神病学]