首页|52例性发育异常儿童的临床特点和诊治分析

52例性发育异常儿童的临床特点和诊治分析

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目的 分析性发育异常(DSD)儿童的临床诊治特点,进一步提高DSD的诊治水平.方法 回顾性分析2019年I月至2021年11月在本院泌尿外科住院的52例性发育异常患儿的临床资料,将患儿分为A组(46,XX DSD,11例)、B组(46,XY DSD,20例)和C组(性染色体DSD,21例),总结分析三组患儿的临床诊断及治疗特点.结果 C组的年龄低于A、B组,差异有统计学意义(P<0.05).A组未进行性腺活检率、有阴道子宫率高于B、C组,B组性腺病理学为睾丸组织率、有前列腺囊率高于A、C组.差异有统计学差异(P<0.001).A组:性别认定9例,包括尿道成形术2例,拟青春期行阴道成形术1例,糖皮质激素治疗+阴蒂整形术4例,糖皮质激素治疗1例,糖皮质激素治疗+拟青春期阴道成形、阴蒂整形术1例.B组:性别认定13例,其中性别改变5例,均为社会性别女性改为男性,包括尿道成形术8例,卵睾切除+尿道成形术1例,卵睾切除+前列腺囊切除+尿道成形术1例,双侧性腺切除+阴蒂整形术1例,双侧性腺切除+生长激素治疗1例.C组:性别认定19例,尿道成形术11例,混合性腺切除+盆腔囊性包块切除+尿道成形术3例,双侧性腺切除+阴蒂整形术2例,混合性腺切除+尿道成形术3例.结论 多学科诊治模式下制定个体化治疗可全面、全程、有效为DSD患儿提供诊疗思路,值得临床推广.
Clinical diagnosis and treatment of 52 children with disorder of sex development
Objective To analyze the clinical characteristics of diagnosis and treatment of dis-order of sex development(DSD)in children,and further improve the diagnosis and treatment of DSD.Methods The clinical data of 52 inpatients with DSD of Urology of Hunan Children's Hospital from January 2019 to November 2021 were retrospectively analyzed,summarize and analyze the characteristics of clinical diagnosis and treatment.The patients were divided into group A(46,XX DSD,11 cases),group B(46,XY DSD,20 cases)and group C(sex chromosome DSD,21 cases),and the clinical di-agnosis and treatment characteristics were summarized and analyzed.Results The age of group C was less than that of groups A and B,and the difference was statistically significant(P<0.05).The rate of no gonadal biopsy and vaginal uterus in group A was higher than that in group B and C,the rate of gonadal pathology was testicular tissue and the rate of prostate sac in group B was higher than that in group A and C.The difference was statistically significant(P<0.001).Group A:9 cases were gen-der identified,including 2 cases of urethroplasty,1 case of quasi-adolescent vaginoplasty,4 cases of glucocorticoid therapy+clitoridplastic surgery,1 case of glucocorticoid therapy+quasi-adolescent vaginoplasty and clitoridplastic surgery.Group B:There were 13 cases of gender identification,inclu-ding 5 cases of gender change,all of which were female to male,including 8 cases of urethroplasty,1 case of ootestectomy+urethroplasty,1 case of ootestectomy+prostatic cyst resection+urethroplasty,1 case of bilateral gonadectomy+clitoridectomy,and 1 case of bilateral gonadectomy+growth hormone therapy.Group C:19 cases of gender identification,11 cases of urethroplasty,3 cases of mixed gona-dectomy+pelvic cystic mass resection+urethroplasty,2 cases of bilateral gonadectomy+clitoridecto-my,3 cases of mixed gonadectomy+urethroplasty.Conclusions Individualized treatment under multidisciplinary diagnosis and treatment mode can provide comprehensive,whole-process and effective diagnosis and treatment ideas for children with disorder of sex development,which is worth promoting.

ChildSexual DevelopmentMultidisciplinary Diagnosis and Treatment

胡建军、赵夭望、童方运、祖建成、何天衢、陈毅夫

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湖南省儿童医院泌尿外科,长沙 410007

儿童 性发育 多学科诊疗

湖南创新型省份建设专项临床医疗技术创新引导项目湖南省儿童泌尿生殖疾病临床医学研究中心国家临床重点专科建设项目

2021SK505252021SK4017湘卫医发[2022]2号

2024

国际泌尿系统杂志
中华医学会,湖南省医学会

国际泌尿系统杂志

CSTPCD
影响因子:0.414
ISSN:1673-4416
年,卷(期):2024.44(2)
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