Endocrine Abstracts

Background: Hypogonadism is a major feature of Prader–Willi syndrome, but clinical manifestations are variable. Sexual interests and behavior in this population have not been previously described.

Objectives: We studied PWS adolescents and young adults to assess: i) satisfaction with physical and sexual development ii) frequency of romantic and sexual experiences, iii) aspirations and expectations regarding marriage, iv) investigate the relation between sexual interests and hormone levels, and v) assess the desire for hormonal replacement therapy.

Methods: The study population consisted of 27 individuals (13 males) ages 17 to 32 (mean 23.5) years with genetically confirmed PWS. Mean IQ was 75 (range 50–100). We conducted structured interviews using questionnaires specifically designed for this study.

Results: 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 gone on a date and kissing romantically. All males and 64% of the females wished to be married. Seventy-seven percent 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

Conclusions: 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.