Inhibin B and anti-Müllerian hormone, but not testosterone levels, are normal in infants with nonmosaic Klinefelter syndrome.

@article{Lahlou2004InhibinBA,
  title={Inhibin B and anti-M{\"u}llerian hormone, but not testosterone levels, are normal in infants with nonmosaic Klinefelter syndrome.},
  author={Najiba Lahlou and Ilene Fennoy and Jean-Claude Carel and Marc Roger},
  journal={The Journal of clinical endocrinology and metabolism},
  year={2004},
  volume={89 4},
  pages={
          1864-8
        }
}
  • N. Lahlou, I. Fennoy, +1 author M. Roger
  • Published 1 April 2004
  • Medicine, Biology
  • The Journal of clinical endocrinology and metabolism
Klinefelter syndrome is a major cause of infertility in the male. Nevertheless, pregnancies were recently obtained by intracytoplasmic injection of sperm retrieved by surgery or ejaculation, underscoring the need to understand the role of Sertoli and Leydig cell secretions during development. In 18 infants with prenatally diagnosed homogenous 47,XXY karyotype, blood samples were taken from birth to 3 yr of age. Inhibin B (INHB), anti-Müllerian hormone (AMH), testosterone, FSH, and LH levels… 
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Assessment of Leydig and Sertoli cell functions in infants with nonmosaic Klinefelter syndrome: insulin-like peptide 3 levels are normal and positively correlated with LH levels.
TLDR
In infants with KS, Leydig cells are normally sensitive to the LH proliferative effect, which may be prophetic of the postpubertal Sertoli cell resistance to FSH.
Sertoli cell markers in the diagnosis of paediatric male hypogonadism
TLDR
Measurement of serum AMH and inhibin B is helpful in assessing testicular function, without need for stimulation tests, and orientates the aetiological diagnosis of paediatric male hypogonadism.
Serum concentrations of Anti‐Müllerian Hormone (AMH) in 95 patients with Klinefelter syndrome with or without cryptorchidism
TLDR
Assessment of circulating AMH concentrations in patients with 47,XXY Klinefelter syndrome with or without cryptorchidism found no change in concentrations of anti‐Müllerian hormone.
Activating mutations in the luteinizing hormone receptor gene: a human model of non-follicle-stimulating hormone-dependent inhibin production and germ cell maturation.
TLDR
The results suggest that the pubertal FSH rise is not required for full expression of the two inhibin B genes and for the initiation of germ cell maturation, demonstrating that Leydig cells can produce significant amounts of the dimeric molecule.
Testicular anti-mullerian hormone secretion is stimulated by recombinant human FSH in patients with congenital hypogonadotropic hypogonadism.
TLDR
It is concluded that FSH stimulates AMH production in the testis when it is at a prepubertal stage, in agreement with the concept that during pubertal development and in adult life, the suppressive effect of LH-driven testicular androgens outweighs the stimulating effect of FSH on AMHproduction by Sertoli cells.
Anti-Müllerian Hormone and Its Clinical Use in Pediatrics with Special Emphasis on Disorders of Sex Development
TLDR
AMH can be used as a marker of premature ovarian insufficiency (POI) in both Turner Syndrome patients and in girls with cancer after treatment with alkylating gonadotoxic agents, and its usefulness has been proposed in the diagnosis of polycystic ovarian syndrome and ovarian granulosa cell tumors.
Hypothalamic and gonadal components of hypogonadism in boys with Prader-Labhart- Willi syndrome.
TLDR
Children with PWS display a specific form of combined hypothalamic (low LH) and peripheral (low inhibin B and high FSH) hypogonadism, suggesting a primary defect in Sertoli and/or germ cell maturation or an early germ cell loss.
Pituitary and testicular function in sons of women with polycystic ovary syndrome from infancy to adulthood.
TLDR
AMH concentrations are increased in prepubertal sons of women with PCOS, suggesting that these boys may show an increased Sertoli cell number or function during infancy and childhood, however, this does not seem to have a major deleterious effect on sperm production.
Testicular Function in Klinefelter Syndrome
TLDR
The testes in the adult KS male are, however, characterized by extensive fibrosis and hyalinization of the seminiferous tubules, and hyperplasia of the interstitium, but the tubules may show residual foci of spermatogenesis.
Endocrinological issues and hormonal manipulation in children and men with Klinefelter syndrome
  • M. Wosnitzer, D. Paduch
  • Medicine
    American journal of medical genetics. Part C, Seminars in medical genetics
  • 2013
TLDR
Early fertility preservation and hormonal supplementation may normalize pubertal development, prevent metabolic sequelae of hypogonadism, and have a positive effect on academic and social development.
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