Abstract
True precocious puberty (TPP) generally is considered to result from gonadotropin mediated premature gonadarche. However, this report concerns two brothers (46/XY) in whom TPP was caused by autonomous hypersecretion of testosterone (T) from interstitial cell hyperplasia (ICH). Salient clinical features were:
Basal sex hormone concentrations are listed in the table below:
Turnover studies revealed normal T clearance but excessive T production. Serum androgen concentrations were unresponsive to ACTH stimulation or Dexamethasone suppression. Excessive adrenal androgenesis was further excluded by split adrenal vein catheterization. Basal serum and 24-hour urine LH, FSH, and β-HCG concentrations were undetectable and there was no significant increase of RIA or bioassay LH/FSH in response to GnRH infusion. Testicular biopsy revealed patchy diffuse ICH, with mature spermatogenesis in the 6 y.o. These studies suggest that autonomous ICH may cause TPP in the absence of significant gonadotropin secretion.
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Schedewie, H., Reiter, E., DeVane, G. et al. AUTONOMOUS INTERSTITIAL CELL HYPERPLASIA AS A CAUSE OF FAMILIAL PRECOCIOUS PUBERTY. Pediatr Res 15, 77 (1981). https://doi.org/10.1203/00006450-198101000-00034
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DOI: https://doi.org/10.1203/00006450-198101000-00034