Familial male pseudohermaphroditism with normal Leydig cell function at puberty

Walter Meyer, B. S. Keenan, L. de Lacerda, I. J. Park, H. E. Jones, C. J. Migeon

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Four male pseudohermaphrodites from two families have been described. Although reared as females, at puberty, the timing, pattern, and degree of masculinization was similar to that of a normal male. No feminization occurred. They had normal testicular testosterone synthesis as judged by plasma testosterone, LH and FSH concentrations, as well as incubations of testicular minces with labeled precursors. Studies on cultured skin fibroblasts indicated adequate peripheral 5α-reductase and normal receptor affinity and capacity for dihydrotestosterone. The histology of the testis was suggestive of a primary testicular defect. A mosaic pattern was seen: some areas contained tubules with active spermiogenesis; other areas, only Sertoli cells. These male pseudohermaphrodites appear to have a defect in fetal testicular maturation in which inadequate fetal testosterone synthesis and defective differentiation of germinal elements occurred.

Original languageEnglish (US)
Pages (from-to)593-603
Number of pages11
JournalJournal of Clinical Endocrinology and Metabolism
Volume46
Issue number4
StatePublished - 1978

Fingerprint

XY Disorders of Sex Development 46
Leydig Cells
Puberty
Testosterone
Feminization
Defects
Histology
Sertoli Cells
Dihydrotestosterone
Spermatogenesis
Fibroblasts
Testis
Skin
Oxidoreductases
Plasmas

ASJC Scopus subject areas

  • Biochemistry
  • Endocrinology, Diabetes and Metabolism

Cite this

Meyer, W., Keenan, B. S., de Lacerda, L., Park, I. J., Jones, H. E., & Migeon, C. J. (1978). Familial male pseudohermaphroditism with normal Leydig cell function at puberty. Journal of Clinical Endocrinology and Metabolism, 46(4), 593-603.

Familial male pseudohermaphroditism with normal Leydig cell function at puberty. / Meyer, Walter; Keenan, B. S.; de Lacerda, L.; Park, I. J.; Jones, H. E.; Migeon, C. J.

In: Journal of Clinical Endocrinology and Metabolism, Vol. 46, No. 4, 1978, p. 593-603.

Research output: Contribution to journalArticle

Meyer, W, Keenan, BS, de Lacerda, L, Park, IJ, Jones, HE & Migeon, CJ 1978, 'Familial male pseudohermaphroditism with normal Leydig cell function at puberty', Journal of Clinical Endocrinology and Metabolism, vol. 46, no. 4, pp. 593-603.
Meyer W, Keenan BS, de Lacerda L, Park IJ, Jones HE, Migeon CJ. Familial male pseudohermaphroditism with normal Leydig cell function at puberty. Journal of Clinical Endocrinology and Metabolism. 1978;46(4):593-603.
Meyer, Walter ; Keenan, B. S. ; de Lacerda, L. ; Park, I. J. ; Jones, H. E. ; Migeon, C. J. / Familial male pseudohermaphroditism with normal Leydig cell function at puberty. In: Journal of Clinical Endocrinology and Metabolism. 1978 ; Vol. 46, No. 4. pp. 593-603.
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