INVESTIGADORES
BERGADÁ Ignacio
artículos
Título:
Are Klinefelter boys hypogonadal?
Autor/es:
REY RA; GOTTLIEB S; PASQUALINI T; BASTIDA MG; GRINSPON RP; CAMPO SM; BERGADÁ I
Revista:
ACTA PAEDIATRICA
Editorial:
WILEY-BLACKWELL PUBLISHING, INC
Referencias:
Lugar: Londres; Año: 2011 vol. 100 p. 830 - 838
ISSN:
0803-5253
Resumen:
Male hypogonadism implies decreased function of one or more testicular cell population,
i.e. germ, Leydig and / or Sertoli cells. In the normal prepubertal boy, Sertoli cells are very
active, as indicated by high anti-Mu¨ llerian hormone (AMH) and inhibin B secretion,
whereas the functional activity of Leydig cells is minimal, as evidenced by low testosterone
production, and germ cells do not undergo the full spermatogenic process. Klinefelter
syndrome is the most frequent cause of hypogonadism in the adult male. In this review,
we discuss whether the gonadal failure is already established during infancy and childhood.
In Klinefelter syndrome, there is increased germ cells degeneration from mid-foetal life ?
resulting in a decreased number at birth ? which persists during infancy and childhood and
becomes dramatic during puberty. Controversial results exist in the literature regarding
Leydig cell function in Klinefelter boys: while some authors have found normal to low
testosterone levels in infancy and childhood, others have reported normal to high values.
Sertoli cell products AMH and inhibin B are normal in prepubertal boys and only decline
during mid- to late puberty.
i.e. germ, Leydig and / or Sertoli cells. In the normal prepubertal boy, Sertoli cells are very
active, as indicated by high anti-Mu¨ llerian hormone (AMH) and inhibin B secretion,
whereas the functional activity of Leydig cells is minimal, as evidenced by low testosterone
production, and germ cells do not undergo the full spermatogenic process. Klinefelter
syndrome is the most frequent cause of hypogonadism in the adult male. In this review,
we discuss whether the gonadal failure is already established during infancy and childhood.
In Klinefelter syndrome, there is increased germ cells degeneration from mid-foetal life ?
resulting in a decreased number at birth ? which persists during infancy and childhood and
becomes dramatic during puberty. Controversial results exist in the literature regarding
Leydig cell function in Klinefelter boys: while some authors have found normal to low
testosterone levels in infancy and childhood, others have reported normal to high values.
Sertoli cell products AMH and inhibin B are normal in prepubertal boys and only decline
during mid- to late puberty.
active, as indicated by high anti-Mu¨ llerian hormone (AMH) and inhibin B secretion,
whereas the functional activity of Leydig cells is minimal, as evidenced by low testosterone
production, and germ cells do not undergo the full spermatogenic process. Klinefelter
syndrome is the most frequent cause of hypogonadism in the adult male. In this review,
we discuss whether the gonadal failure is already established during infancy and childhood.
In Klinefelter syndrome, there is increased germ cells degeneration from mid-foetal life ?
resulting in a decreased number at birth ? which persists during infancy and childhood and
becomes dramatic during puberty. Controversial results exist in the literature regarding
Leydig cell function in Klinefelter boys: while some authors have found normal to low
testosterone levels in infancy and childhood, others have reported normal to high values.
Sertoli cell products AMH and inhibin B are normal in prepubertal boys and only decline
during mid- to late puberty.
Conclusion: Klinefelter syndrome is a primary hypogonadism affecting all testicular
cell populations. Germ cells are affected from foetal life, and a severe depletion occurs at
puberty. Leydig cell function may be normal or mildly affected in foetal and early postnatal
life. Sertoli cell function is not impaired until mid- to late puberty, as reflected by normal
AMH and inhibin B in Klinefelter boys.Klinefelter syndrome is a primary hypogonadism affecting all testicular
cell populations. Germ cells are affected from foetal life, and a severe depletion occurs at
puberty. Leydig cell function may be normal or mildly affected in foetal and early postnatal
life. Sertoli cell function is not impaired until mid- to late puberty, as reflected by normal
AMH and inhibin B in Klinefelter boys.