性发育异常性腺肿瘤患者术后生存质量评估研究

摘要

Objective: To investigate the quality of life (QOL) in patients after operation with of sex development disorders with gonadal tumors,and propose some suggestions of long-term care. Methods: Twenty four patients after operation of disorders of sex development with gonadal tumors diagnosed in Peking Union Medical College Hospital from January 2006 to December 2015 were enrolled in the study. All patients were followed-up and provided psychological support after surgery. WHOQOL-BREF questionnaires including physical,psychological,and social relationship and environmental domains were used for QOL evaluation. Results: The age of initial treatment in DSD patients with gonadal tumors was (19.25±4.75) years old (ranging from 13 to 29 years). All individuals are phenotypic female and were closely followed-up for (4.79±2.36) years (ranging from 1 to 10 years). There were 15 (62.50%) 46,XY pure gonadal dysgenesis (46,XY PGD),6 (25.00%) androgen insensitivity syndrome (AIS),3 (12.50%) XO/XY gonadal dysgenesis (Mixed GD) patients involved. QOL scores of psychological domain in different DSD categories were lower than that of other domains,and Mixed GD patients carried the lowest psychological QOL score. DSD patients with tumors had better QOL scores than general DSD patients and the general urban adult population (P<0.05). There was no significantly difference between the QOL scores of DSD patients with benign (n=11) and malignant tumors (n=13) (P>0.05). Conclusions: With optimal care,including close follow-ups and psychological support,DSD patients with gonadal tumors are expected to achieve ideal QOL.%目的 评估性发育异常(DSD)性腺肿瘤患者术后生存质量(QOL),以期对该疾病的长期健康管理提供参考. 方法 对2006年1月至2015年12月北京协和医院妇产科收治的24例DSD肿瘤患者进行术后随访并提供心理支持,使用WHOQOL-BREF量表(包含生理领域、心理领域、社会关系领域、环境领域4部分)评估QOL. 结果 DSD肿瘤患者初治平均年龄为(19.25±4.75)岁(13~29岁),社会性别均为女性,术后均规律随访(4.79±2.36)年(1~10年)至今.纳入15例(62.50%)46,XY单纯性腺发育不全(46,XY PGD),6例(25.00%)雄激素不敏感综合征(AIS),3例(12.50%)XO/XY性腺发育不全(Mixed GD),各疾病类型DSD肿瘤患者QOL心理领域评分均低于其他领域,Mixed GD心理领域评分最低.DSD肿瘤患者QOL评分高于一般DSD患者及一般成年城市人口,差异有统计学意义(P<0.05).患者中良性肿瘤11例,恶性肿瘤13例,两组间QOL评分比较,差异无统计学意义(P>0.05). 结论 通过接受长期的、个体化的、优化的手术药物治疗和心理支持,DSD肿瘤患者可获得较理想的QOL.