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Clin Endocrinol (Oxf). 2007 Dec;67(6):863-70. Epub 2007 Jul 20.

Establishment of testicular endocrine function impairment during childhood and puberty in boys with Klinefelter syndrome.

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1
División de Endocrinología and Centro de Investigaciones Endocrinológicas, Hospital de Niños Ricardo Gutiérrez, Buenos Aires, Argentina.

Abstract

OBJECTIVE:

To precisely characterize the chronology of testicular endocrine function impairment during childhood and adolescence in patients with Klinefelter syndrome. Design Retrospective chart review. Patients A total of 29 boys with Klinefelter syndrome with up to 12.3 years follow-up.

MEASUREMENTS:

Clinical features and serum hormone levels were analysed during follow-up.

RESULTS:

Of the 29 patients, 16 were prepubertal and 13 had already entered puberty at their first visit. Fifteen patients were followed up through late puberty. Before puberty, LH, FSH, testosterone, anti-Müllerian hormone (AMH) and inhibin B were within the expected range in almost all cases. However, levels of the inhibin alpha-subunit precursor Pro-alphaC were in the lowest levels of the normal range in most cases. During puberty, FSH levels increased earlier and more markedly than LH. Inhibin B and AMH declined to abnormally low or undetectable levels in advanced pubertal stages. Although testosterone and Pro-alphaC levels were within the reference ranges in most cases, they were abnormally low for the observed LH values.

CONCLUSIONS:

In Klinefelter syndrome, a mild Leydig cell dysfunction is present from early childhood in most cases and persists throughout puberty. Sertoli cell function is normal until mid puberty, when a dramatic impairment is observed.

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