Leydig cell hypofunction resulting in male pseudohermaphroditism

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

doi:10.1016/s0015-0282(16)46281-3

Leydig cell hypofunction resulting in male pseudohermaphroditism

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

College of Medicine and Health Sciences

Johns Hopkins University School of Medicine

Research output: Contribution to journal › Article › peer-review

38 Scopus citations

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 language	British English
Pages (from-to)	675-679
Number of pages	5
Journal	Unknown Journal
Volume	37
Issue number	5
DOIs	https://doi.org/10.1016/s0015-0282(16)46281-3
State	Published - 1982

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title = "Leydig cell hypofunction resulting in male pseudohermaphroditism",

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.",

author = "Lee, {P. A.} and Rock, {J. A.} and Brown, {T. R.} and Fichman, {K. M.} and Migeon, {C. J.} and Jones, {H. W.}",

note = "Funding Information: Received September 8, 1981; revised and accepted January 27,1982. *Supported in part by USPHS grants AM-00180 and RR-00052. tReprint requests: Peter A. Lee, M.D., Ph.D., Children's Hospital of Pittsburgh, 125 DeSoto Street, Pittsburgh, Pennsylvania 15213. *Division of Pediatric Endocrinology, Department of Pediatrics, The Johns Hopkins University School of Medicine. §Division of Reproductive Endocrinology, Department of Obstetrics and Gynecology, The Johns Hopkins University School of Medicine. IIDepartment of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh.",

year = "1982",

doi = "10.1016/s0015-0282(16)46281-3",

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pages = "675--679",

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T1 - Leydig cell hypofunction resulting in male pseudohermaphroditism

AU - Lee, P. A.

AU - Rock, J. A.

AU - Brown, T. R.

AU - Fichman, K. M.

AU - Migeon, C. J.

AU - Jones, H. W.

N1 - Funding Information: Received September 8, 1981; revised and accepted January 27,1982. *Supported in part by USPHS grants AM-00180 and RR-00052. tReprint requests: Peter A. Lee, M.D., Ph.D., Children's Hospital of Pittsburgh, 125 DeSoto Street, Pittsburgh, Pennsylvania 15213. *Division of Pediatric Endocrinology, Department of Pediatrics, The Johns Hopkins University School of Medicine. §Division of Reproductive Endocrinology, Department of Obstetrics and Gynecology, The Johns Hopkins University School of Medicine. IIDepartment of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh.

PY - 1982

Y1 - 1982

N2 - 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.

AB - 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.

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Leydig cell hypofunction resulting in male pseudohermaphroditism

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