Abstract
Background: 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.
Objectives: 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.
Methods: 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.
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 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.
Conclusion: 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.
©2011 by Walter de Gruyter Berlin Boston
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- Commentary
- Using genetic tests for talent identification in sports: too soon to be true
- Letter to the Editors
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- Meetings
- Meetings Calendar
Articles in the same Issue
- Editorial
- Pitfalls for bone age measurement
- Bone disease and cytokines in idiopathic hypercalciuria: a review
- Cord blood adiponectin and infant growth at one year
- Plasma visfatin and adiponectin concentrations in physically active adolescent girls: relationships with insulin sensitivity and body composition variables
- Gender differences exist in the association of leptin and adiponectin levels with insulin resistance parameters in prepubertal Arab children
- Development and linear growth in diabetic children receiving insulin pigment
- Genotypes of p53 codon 72 correlate with age at onset of type 1 diabetes in a sex-specific manner
- A prospective clinical pilot-trial comparing the effect of an optimized mixed diet versus a flexible low-glycemic index diet on nutrient intake and HbA1c levels in children with type 1 diabetes
- Low-dose insulin therapy in patients with cystic fibrosis and early-stage insulinopenia prevents deterioration of lung function: a 3-year prospective study
- Short communication
- Italian pediatric data support hypothesis that simultaneous epidemics of type 1 diabetes and type 2 diabetes/metabolic syndrome/obesity are polar opposite responses (i.e., symptoms) to a primary inflammatory condition
- Adult height after growth hormone treatment in Japanese children with idiopathic growth hormone deficiency: analysis from the KIGS Japan database
- Growth patterns of large, small, and appropriate for gestational age infants: impacts of long-term breastfeeding: a retrospective cohort study
- Body image and sexual interests in adolescents and young adults with Prader–Willi syndrome
- Growth hormone secretory pattern in non-obese children and adolescents with Prader-Willi syndrome
- The effect of corrective surgery on serum IGF-1, IGFBP-3 levels and growth in children with congenital heart disease
- Patient preference for a new growth hormone injection device: results of an open-label study in Japanese pediatric patients
- DXA surrogates for visceral fat are inversely associated with bone density measures in adolescent athletes with menstrual dysfunction
- Evaluation of bone density in girls with precocious and early puberty during treatment with GnRH agonist
- Comparative analysis of clinical, biochemical and genetic aspects associated with bone mineral density in small for gestational age children
- Outcome of gonadotropin-releasing analog treatment for children with central precocious puberty: 15-year experience in southern Thailand
- Ovarian function in adolescents with McCune-Albright syndrome
- Hypothalamic-pituitary-adrenal axis suppression in asthmatic children on inhaled and nasal corticosteroids – more common than expected?
- Clinical characteristics of adrenocortical tumors in children
- Preimplantation genetic diagnosis (PGD) – prevention of the birth of children affected with endocrine diseases
- The usefulness of ultrasound in follow-up of a patient with dyshormonogenetic congenital hypothyroidism
- Accelerated carpal bone maturation in juvenile idiopathic arthritis: pitfall for bone age measurement
- Case reports
- Hypokalaemic paralysis in a 15-year-old boy with 11β-hydroxylase deficiency
- Structural chromosome disruption of the NR3C2 gene causing pseudohypoaldosteronism type 1 presenting in infancy
- Continuous subcutaneous hydrocortisone infusion (CSHI) in a young adolescent with congenital adrenal hyperplasia (CAH)
- Causes and consequences of abandoning one-stage bilateral adrenalectomy recommended in primary pigmented nodular adrenocortical disease – case presentation
- Short stature in a patient with familial glucocorticoid deficiency
- Biochemical evaluation of an infant with hypoglycemia resulting from a novel de novo mutation of the GLUD1 gene and hyperinsulinism-hyperammonemia syndrome
- Entire prophet of Pit-1 (PROP-1) gene deletion in an Indian girl with combined pituitary hormone deficiencies
- Diabetes in young – unusual case presentation
- Patient reports
- Two cases of 22q11.2 deletion syndrome with anorectal anomalies and growth retardation
- A 3-year-old boy with ovotestes: gender reassignment and surgical management
- Case reports
- A 5-year-old boy with atrophic autoimmune thyroiditis caused by thyroid-stimulation blocking antibodies
- Small nuclear ribonucleoprotein polypeptide N quantitative methylation analysis in infants with central hypotonia
- Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome (APECED) due to AIRET16M mutation in a consanguineous Greek girl
- Pulmonary hypertension and reopening of the ductus arteriosus in an infant treated with diazoxide
- Commentary
- Using genetic tests for talent identification in sports: too soon to be true
- Letter to the Editors
- Insulin resistance and the metabolic syndrome in obese children
- Graves’ disease in a Down’s syndrome patient responds well to radioiodine rather than antithyroid drugs
- Aortic intima-media thickness and lipid profile in neonates born to mothers with pre-eclampsia: weak or strong relations?
- Meetings
- Meetings Calendar