Gross-Tsur Varda, Eldar-Geva Talia, Benarroch Fortu, Rubinstein Orit, Hirsch Harry J
Neuropediatric Unit, Department of Pediatrics, Shaare Zedek Medical Center, The Hebrew University, Jerusalem, Israel.
J Pediatr Endocrinol Metab. 2011;24(7-8):469-75. doi: 10.1515/jpem.2011.201.
Hypogonadism is a major feature of Prader-Willi syndrome (PWS), but clinical manifestations are variable. Sexual interests and behavior in this population have not been previously described.
We studied PWS adolescents and young adults to assess 1) satisfaction with physical and sexual development, 2) frequency of romantic and sexual experiences, 3) aspirations and expectations regarding marriage, 4) possible relationships between sexual interests and hormone levels, and 5) the desire for hormonal replacement therapy.
The study population consisted of 27 individuals (13 males) ages 17-32 (mean 23.5) years with genetically confirmed PWS. Mean intelligence quotient (IQ) was 75 (range 50-100). We conducted structured interviews using questionnaires specifically designed for this study.
There was a significant negative correlation between IQ and body image in both males and females. IQ showed a positive correlation with interest in dating and romantic activities. Approximately half of PWS males and females reported having been on a date and kissing romantically. All males and 64% of the females wished to be married. Seventy-seven per cent of PWS males wanted hormonal treatment to increase phallic size. We found no correlation between hormone levels and sexual interests. Only 43% of PWS females wanted hormonal medication to achieve regular menstruation.
Despite documented hypogonadism, PWS young adults are interested in sexual and romantic issues. The range of sexual activities and expectations is variable. Understanding specific sexual characteristics of each individual is important in order to offer proper anticipatory sexual guidance counseling and for appropriate recommendations for hormone replacement.
性腺功能减退是普拉德-威利综合征（PWS）的主要特征，但临床表现存在差异。此前尚未描述过该人群的性兴趣和性行为。
我们对患有PWS的青少年和年轻人进行了研究，以评估：1）对身体和性发育的满意度；2）浪漫和性经历的频率；3）对婚姻的期望和憧憬；4）性兴趣与激素水平之间可能存在的关系；5）对激素替代疗法的需求。
研究对象为27名年龄在17 - 32岁（平均23.5岁）、基因确诊为PWS的个体（13名男性）。平均智商（IQ）为75（范围50 - 100）。我们使用专门为本研究设计的问卷进行结构化访谈。
男性和女性的智商与身体意象之间均存在显著负相关。智商与约会和浪漫活动的兴趣呈正相关。约一半的PWS男性和女性报告有过约会和浪漫亲吻经历。所有男性和64%的女性希望结婚。77%的PWS男性希望通过激素治疗增大阴茎尺寸。我们发现激素水平与性兴趣之间无相关性。只有43%的PWS女性希望通过激素药物实现规律月经。
尽管有性腺功能减退的记录，但患有PWS的年轻人对性和浪漫问题感兴趣。性活动和期望的范围存在差异。了解每个个体的特定性特征对于提供适当的预期性指导咨询以及激素替代的适当建议非常重要。
