Assessment of Leydig and Sertoli cell functions in @entity1 with nonmosaic XXXX : insulin-like peptide 3 levels are normal and positively correlated with LH levels.

@entity1 @entity251 @entity4810 @entity252 @entity32407 @entity22567
CONTEXT: @entity252 ( @entity252 ) is the most common @entity22567 and a major cause of @entity4810 . In adult patients, serum inhibin B and anti-Mullerian-hormone (AMH) are undetectable, @entity251 secretion is often impaired, and the tubules are depleted of germ cells. Before puberty, inhibin B, AMH, and @entity251 levels are within the normal range. OBJECTIVE: Sertoli and Leydig cell secretions, including @entity32407 ( @entity32407 ), were evaluated in @entity1 with nonmosaic XXY karyotype to assess testicular function soon after birth. DESIGN: The study was conducted in four University Pediatric Departments from the United States and France. SUBJECTS: Sixty-eight prenatally diagnosed @entity1 aged 2-750 d were enrolled. MAIN OUTCOME MEASURES: Serum FSH, LH, inhibin B, AMH, and @entity32407 were measured by immunoassay, and @entity251 was measured by tandem mass-spectrometry. RESULTS: In @entity1 with @entity252 , @entity32407 levels transiently increased at 2-3 months of age and were significantly correlated with @entity251 (Spearman r = 0.57) and LH (Spearman r = 0.73) levels. They did not differ from controls. @entity251 levels were within the normal range, but most of them were below the median of controls. Inhibin B and AMH levels were also within normal range. Inhibin B was correlated with FSH (Spearman r = 0.49). AMH was not correlated with FSH or @entity251 . FSH levels were above normal in 25% of @entity1 , despite normal inhibin B levels. CONCLUSIONS: In @entity1 with @entity252 , Leydig cells are normally sensitive to the LH proliferative effect. In contrast, the Sertoli cell sensitivity to FSH is questionable, which may be prophetic of the postpubertal Sertoli cell resistance to FSH.
