Leydig cell hypofunction resulting in male pseudohermaphroditism

Peter Lee, J. A. Rock, T. R. Brown, K. M. Fichman, C. J. Migeon, H. W. Jones

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Abstract

An 11-year-old patient with male pseudohermaphroditism presented with essentially normal-appearing female external genitalia. When examined, inguinal gonads, redundant foreskin, and some posterior labial fusion were found. Evaluation revealed basal testosterone (T) levels ranging from 65 to 107 ng/dl with slightly elevated serum gonadotropin levels (luteinizing hormone [LH]: 76 ng/ml, and follicle-stimulating hormone [FSH]:568 ng/ml). Neither T nor its precursors increased with human chorionic gonadotropin (hCG) stimulation. However, progesterone (P), 17-hydroxyprogesterone (17-OHP), and cortisol (F) responses to adrenocorticotropic hormone (ACTH) were normal. Androgen binding and 5α-reductase activity in cultured genital skin fibroblasts were normal. These data, plus the microscopic finding of a markedly reduced number of Leydig cells, strongly suggest that the male pseudohermaphroditism in this patient was due to inadequate Leydig cell function unrelated to LH receptors.

Original languageEnglish (US)
Pages (from-to)675-679
Number of pages5
JournalUnknown Journal
Volume37
Issue number5
Publication statusPublished - Jan 1 1982

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All Science Journal Classification (ASJC) codes

  • Reproductive Medicine
  • Obstetrics and Gynecology

Cite this

Lee, P., Rock, J. A., Brown, T. R., Fichman, K. M., Migeon, C. J., & Jones, H. W. (1982). Leydig cell hypofunction resulting in male pseudohermaphroditism. Unknown Journal, 37(5), 675-679.