先天性纯红细胞再生障碍性贫血患儿肥胖发生情况及影响因素分析

Prevalence and risk factors of obesity in children with Diamond-Blackfan anemia

目的探讨先天性纯红细胞再生障碍性贫血(Diamond-Blackfan anemia,DBA)患儿的体重指数分布情况,并分析DBA患儿肥胖发生的相关因素。方法选取2003年1月至2020年12月于中国医学科学院北京协和医学院血液病医院儿童血液病诊疗中心就诊的DBA患儿为研究对象,收集患儿的临床资料、治疗方案,以及于随访时间点前后1周内测量的身高、体重并计算体重指数,分析DBA患儿肥胖发生的影响因素。结果129例DBA患儿中,男性80例(62.0%),女性49例(38.0%),中位测量年龄为49(范围:3~189)个月,肥胖发生率为14.7%(19/129)。多因素logistic回归分析发现,无核糖体蛋白基因突变与DBA患儿肥胖发生关系密切(调整后OR=3.63,95%CI:1.16~11.38,调整后P=0.027)。在激素依赖DBA患儿中,肥胖发生与激素开始治疗年龄、激素治疗时间、激素维持剂量无关(P>0.05)。结论DBA患儿肥胖发生率较高,无核糖体蛋白基因突变与DBA患儿肥胖的发生密切相关。

Objective To investigate the distribution of body mass index(BMI)and risk factors for obesity in children with Diamond-Blackfan Anemia(DBA).Methods The children with DBA who attended National Clinical Research Center for Blood Diseases,Institute of Hematology&Blood Diseases Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,from January 2003 to December 2020 were enrolled as subjects.The related clinical data and treatment regimens were recorded.The height and weight data measured within 1 week before or after follow-up time points were collected to calculate BMI.The risk factors for obesity were determined by multivariate regression analysis in children with DBA.Results A total of 129 children with DBA were enrolled,among whom there were 80 boys(62.0%)and 49 girls(38.0%),with a median age of 49 months(range 3-189 months).The prevalence rate of obesity was 14.7%(19/129).The multivariate logistic regression analysis showed that the absence of ribosomal protein gene mutation was closely associated with obesity in children with DBA(adjusted OR=3.63,95%CI:1.16-11.38,adjusted P=0.027).In children with glucocorticoid-dependent DBA,obesity was not associated with age of initiation of glucocorticoid therapy,duration of glucocorticoid therapy,and maintenance dose of glucocorticoids(P>0.05).Conclusions There is a high prevalence rate of obesity in children with DBA,and the absence of ribosomal protein gene mutation is closely associated with obesity in children with DBA.