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Submitted on November 28, 2005
Accepted on May 2, 2006
Department of Pediatric Endocrinology, CHU Cochin - Saint Vincent de Paul, Paris, France, 75014 (L.S-G, J.-C.C.); the Department of Endocrinology, Hospital Infantil Nino Jesus, Madrid, Spain (L.S.-G.); the Laboratory of Spermiology and Histology, CHRU - Faculty of Medicine, Lille, France (V.M.); the Laboratory of Pathology, CHU Cochin - Saint Vincent de Paul, Paris, France (J.-P.B.); and the Laboratory for Hormone Biology, CHU Cochin - Saint Vincent de Paul, Paris, France (M.R., N.L)
* To whom correspondence should be addressed. E-mail: najiba.lahlou{at}svp.aphp.fr.
Context: Familial male-limited precocious puberty (FMPP) is a dominant autosomal genetic disease caused by activating LH receptor gene mutations, clinically expressed only in males. In preliminary studies, in addition to the expected testosterone increase, we found high inhibin B levels before the age of normal puberty.
Objectives: To assess the cellular origin of serum inhibin thanks to testis section immunostaining.
Main Outcome Measures: Serum testosterone, gonadotropin, inhibin B, pan-aC-inhibin, and anti-mullerian hormone (AMH) levels were measured. Immunostaining was performed using specific anti-a- and anti-b-subunit antibodies.
Subjects and Methods: Five boys from three families (mutation M398T or I542L) were investigated at onset (2 to 6 yr), on ketoconazole treatment, and at adolescence. Testis biopsies were performed in 3 subjects before the disease was fully characterized.
Results: The high testosterone levels were suppressed by ketoconazole. AMH levels were inversely related to testosterone: low at diagnosis, elevated after testosterone suppression. Despite FSH suppression, inhibin B and pan-
C-inhibin levels were high from clinical onset to adolescence. Biopsy specimens showed normal Sertoli cell complement and germ cell maturation until the spermatocyte II stage. Sertoli and Leydig cells displayed positive inhibin -subunit immunostaining. Only Leydig cells and spermatogonia stained positively for the inhibin 
B-subunit.
Conclusion: FMPP is a unique model of inhibin B secretion, demonstrating that Leydig cells can produce significant amounts of the dimeric molecule. Our results also 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.
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