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Willem de Ronde, Anne de Haan and Madeleine L Drent

Context

Klinefelter syndrome (KS) is a genetic disorder, characterized by an XXY karyotype, hypergonadotrophic hypogonadism and infertility, which affects ∼1 in 700 men. KS has also been associated with neuropsychological alterations.

Objective

To investigate whether quality of life (QoL) is reduced in patients with KS on androgen treatment.

Design

Observational study.

Methods

Questionnaires (RAND-36) were sent to 46 KS patients on androgen treatment who regularly visit the outpatient clinic of an academic medical centre. QoL scores were compared with a Dutch male reference group.

Results

Forty (87%) questionnaires were returned which were suitable for evaluation. KS patients had significantly lower (worse) scores on eight of the nine domains of the RAND questionnaire compared with the male reference group. In KS patients, higher education was associated with significantly better QoL scores.

Conclusions

QoL is reduced in patients with KS on androgen treatment.

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Willem de Ronde, Albert Hofman, Huibert A P Pols and Frank H de Jong

Objective: The origin of oestrogens in men is only partly understood. From infusion studies with radioactively labelled hormones, we know that oestradiol (E2) and oestrone (E1) are either directly secreted by the testes and adrenal glands or peripherally produced from testicular or adrenal androgens.

Design and methods: We determined E2, E1, androstenedione, testosterone and dehydroepiandroster-one sulphate (DHEAS) in 292 elderly men and 367 postmenopausal women. We considered post-menopausal women as men without testes, assuming that the postmenopausal ovary is not endocrinologically active and that the testes do not contribute to circulating levels of DHEAS. Subjects were stratified by DHEAS levels to adjust for differences in DHEAS levels between sexes. For men and women separately, mean levels of E2, E1, androstenedione and testosterone were calculated per DHEAS stratum. The relative direct and indirect contributions of the testes to steroid levels in men were calculated by the formula [(Cm −Cf)/Cm] × 100%, in fwhich Cm and Cf represent the mean concentrations of the steroid in men and women respectively.

Results: The relative contributions (%) of the testes to hormone levels per DHEAS stratum (<2, 2–4, 4–6 and >6 μmol/l) respectively were, for E2, 72%, 60%, 52% and 44%; for E1, 54%, 47%, 35% and 34%; for androstenedione, 14%, 4%, 12% and 0%; and, for testosterone, 88%, 88%, 87% and 83%.

Conclusions: We conclude that in elderly men dependent on DHEAS levels, 44–72% of E2 and 34–54% of E1 originate directly or indirectly from the testes.