Abstract
This paper reports on testicular histopathology in 2 Japanese 46,XY patients with congenital lipoid adrenal hyperplasia who underwent gonadectomy at ages 12 and 17 years, respectively. Both patients were completely feminized, although hCG stimulated serum testosterone levels differed (case 1, <0.1 → <0.1 ng/ml; case 2, < 0.5 → 1.3 ng/ml. Case 1: The interstitium contained increased number of grossly swollen Leyding cells filled with numerous lipid droplets. Seminiferous tubules were normal in diameter (115.9±5.9 μm) with age-appropriate number of spermatogonia (0-11/tubule) and well developed Sertoli cells. There were no spermatocytes, spermatids, or sperms. Case 2: The intersitium contained mildy enlarged Leydig cells with dispersed lipid droplets. Seminiferous tubules were normal in diameter (88.7±18.7 μm) and contained age-appropriate number of spermatogonia (0-9/tubule and well developed Sertoli cells. A small number of spermatocytes were found, developed Sertoli cells. A small number of spermatocytes were found, although no spermatids or sperms were identified. We conclude that the defect is characteristically addressed in the testis, consisting hampered germ cell maturation, and apparently spared Sertoli cells.
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Ogata, T., Matsuo, N., Aya, M. et al. TESTICULAR, HISTOPATHOLOGY IN CONGENITAL LIPOID ADRENAL HYPERPLASIA: A LIGHT AND ELECTION MICROSCOPIC STUDY. Pediatr Res 33 (Suppl 5), S22 (1993). https://doi.org/10.1203/00006450-199305001-00114
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DOI: https://doi.org/10.1203/00006450-199305001-00114