McCune-Albright综合征患儿生活质量及其影响因素分析  

Quality of life in children with McCune-Albright syndrome and its influencing factors

作　　者：郝会民 丁敏利 马晓翠 郭亚楠[1] 陈永兴 苏畅[3] 卫海燕[1] Hao Huimin;Ding Minli;Ma Xiaocui;Guo Yanan;Chen Yongxing;Su Chang;Wei Haiyan(Department of Endocrinology,Genetics and Metabolism,Children′s Hospital Affiliated to Zhengzhou University,Henan Children′s Hospital,Zhengzhou Children′s Hospital,Henan Key Laboratory of Pediatric Genetics and Metabolic Diseases,Zhengzhou 450000,China;Department of Child Healthcare,Children′s Hospital Affiliated to Zhengzhou University,Henan Children′s Hospital,Zhengzhou Children′s Hospital,Zhengzhou 450000,China;Department of Endocrinology,Genetics and Metabolism Center,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China)

机构地区：[1]郑州大学附属儿童医院,河南省儿童医院,郑州儿童医院内分泌遗传代谢科,河南省遗传代谢重点实验室,郑州450000 [2]郑州大学附属儿童医院,河南省儿童医院,郑州儿童医院儿童保健科,郑州450000 [3]国家儿童医学中心,首都医科大学附属北京儿童医院内分泌遗传代谢科,北京100045

出　　处：《中华实用儿科临床杂志》2023年第7期528-531,共4页Chinese Journal of Applied Clinical Pediatrics

基　　金：河南省重点研发与推广专项(科技攻关)项目(212102310438、212102310439)。

摘　　要：目的调查McCune-Albright综合征(MAS)患儿的生活质量及影响因素, 为提高患儿生活质量提供科学依据。方法回顾性分析2015年6月至2021年12月在河南省儿童医院诊断及随访的31例MAS儿童(MAS组)的临床资料, 同时按照1∶1匹配同年龄、同性别健康儿童37例(健康对照组), 应用儿童生活质量普适性核心量表(PedsQL?4.0)进行调查及对比分析, 统计学方法采用独立样本t检验、χ^(2)检验及多元回归分析。结果与健康对照组比较, MAS组患儿生理功能(t=2.092, P<0.05)、情感功能(t=2.373, P<0.05)、总分(t=2.360, P<0.05)均明显降低, 差异均有统计学意义。多元回归分析显示, 生理功能与每年阴道出血次数(t=-2.367, P<0.05)、首次骨折年龄呈负相关(t=-2.606, P<0.05)。社交功能与骨折次数呈负相关(t=-2.481, P<0.05)。结论 MAS儿童总体生活质量低, 以生理功能、情感功能较明显, 每年阴道出血次数、首次骨折年龄、骨折次数可能是患儿生活质量降低的影响因素。Objective To investigate the quality of life in children with McCune-Albright syndrome(MAS)and its influencing factors,so as to provide scientific basis for improving their quality of life.Methods The clinical data of 31 children with MAS diagnosed(MAS group)and followed up in Henan Children′s Hospital from June 2015 to December 2021 were retrospectively analyzed.During the same period,37 healthy age and sex-matched children at a ratio of 1∶1 were recruited as healthy control group.The children′s Quality of Life Universal Core Scale(PedsQL™4.0)was used for the investigation and comparative analysis.Statistical analysis was performed using the independent sample t test,Chi-square test and multiple regression analysis.Results Compared with the healthy control group,the physiological function(t=2.092,P<0.05),emotional function(t=2.373,P<0.05)and total score(t=2.360,P<0.05)of MAS group significantly decreased.Multiple regression analysis showed that physiological function was negatively correlated with the annual number of vaginal bleeding(t=-2.367,P<0.05)and the age of first fracture(t=-2.606,P<0.05).Social function was negatively correlated with the number of fractures(t=-2.481,P<0.05).Conclusions The overall quality of life of MAS children is low,especially the quality of physiological function and emotional function.The annual number of vaginal bleedings,the age of the first fracture and the number of fractures are influencing factors for the reduction of the quality of life of children with MAS.

关 键 词：儿童 MCCUNE-ALBRIGHT综合征 生活质量 

分 类 号：R725[医药卫生—儿科]