D'Alberton Franco, Assante Maria Teresa, Foresti Maura, Balsamo Antonio, Bertelloni Silvano, Dati Eleonora, Nardi Laura, Bacchi Maria Letizia, Mazzanti Laura
Pediatric Endocrinology Unit, Department of Pediatrics, S. Orsola-Malpighi University Hospital of Bologna, Bologna, Italy.
Adolescent Medicine, Division of Pediatrics, Department of Obstetrics, Gynecology and Pediatrics, University Hospital, Pisa, Italy.
J Sex Med. 2015 Jun;12(6):1440-9. doi: 10.1111/jsm.12884. Epub 2015 Apr 20.
Progressive care improvement for differences of sex development (DSD), regarding diagnosis communication, psychological, medical and surgical management has been claimed.
To assess clinical management, quality of life (QoL) and the general psychosocial adjustment of individuals with 46,XY DSD. Some differences related to age at diagnosis are investigated.
Cross-sectional study using standardized questionnaires.
Forty-three Caucasian females with 46,XY DSD (self declared diagnoses: complete androgen insensitivity syndrome, n = 34; complete gonadal dysgenesis, n = 1; 5α-reductase deficiency, n = 4; Leydig cell hypoplasia, n = 1; unknown diagnosis, n = 3; age years: 31.5 ± 9.6 [range 18-57 years]).
University Hospitals.
Subjects were required to fill in questionnaires (ABCL, WHOQOL, dedicated 17-item questionnaire). Academic and socioeconomic data were compared with those of the Italian population. QoL and psychological data were compared with those of a comparison group (46,XX healthy females: n = 43; age, years: 34.5 ± 9.7, range 22-51 years).
Present sample of women living with 46,XY DSD were well adapted and were higher achievers than controls, both in educational and professional life. They showed good QoL, but they appeared less satisfied in psychological and social areas. They had borderline mean scores and statistically higher scores than the comparison group for depression, anxiety, internalizing and externalizing problems. Younger persons living with a 46,XY DSD showed better psychosocial adjustment than older ones. Younger women showed lower age at diagnosis communication. Psychological support was more often proposed at the time of diagnosis communication to younger individuals, and they undertook it more frequently than older ones.
Italian people living with 46,XY DSD were well adapted and successful; they reported a good QoL but showed higher degree of psychological distress than the comparison group. Lower psychological distress in younger women could indicate some positive effects of changes in management.
对于性发育差异（DSD）在诊断沟通、心理、医学和手术管理方面的渐进性护理改善已有相关主张。
评估46,XY DSD个体的临床管理、生活质量（QoL）和总体心理社会适应情况。研究了一些与诊断年龄相关的差异。
使用标准化问卷的横断面研究。
43名患有46,XY DSD的白种女性（自我申报诊断：完全雄激素不敏感综合征，n = 34；完全性腺发育不全，n = 1；5α-还原酶缺乏症，n = 4；莱迪希细胞发育不全，n = 1；诊断不明，n = 3；年龄：31.5±9.6[范围18 - 57岁]）。
大学医院。
受试者被要求填写问卷（ABCL、世界卫生组织生活质量问卷、专门的17项问卷）。将学术和社会经济数据与意大利人群的数据进行比较。将生活质量和心理数据与对照组（46,XX健康女性：n = 43；年龄：34.5±9.7，范围22 - 51岁）的数据进行比较。
目前患有46,XY DSD的女性样本适应良好，在教育和职业生活方面比对照组取得更高成就。她们显示出良好的生活质量，但在心理和社会领域似乎不太满意。她们在抑郁、焦虑、内化和外化问题方面的平均得分处于临界水平，且在统计学上高于对照组。患有46,XY DSD的年轻个体比年长个体表现出更好的心理社会适应。年轻女性在诊断沟通时的年龄较低。在诊断沟通时，更常向年轻个体提供心理支持，且她们比年长个体更频繁地接受心理支持。
患有46,XY DSD的意大利人适应良好且成功；他们报告生活质量良好，但与对照组相比显示出更高程度的心理困扰。年轻女性较低的心理困扰可能表明管理变化产生了一些积极影响。
