TY - JOUR
T1 - "True agonadism"
T2 - A misnomer?
AU - Parks, Gary A.
AU - Dumars, Kenneth W.
AU - Limbeck, George A.
AU - Quinlivan, W. Leslie
AU - New, Maria I.
N1 - Funding Information:
From the Department of Pediatrics, The New York Hospital-Cornell Medical Center, and the Departments of Pediatrics and Obstetrics" and Gynecology, University of California. Supported in part by Grant liD 72, National Institutes of Health, United States Public Health Service. Presented in part at the 41st and 43rd Annual Meetings of the Society for Pediatric Research, 1971 and 1973. *Reprint address: Department of Pediatrics, The New York HospitaI-Cornell Medical Center. 525 E. 68th St., New York. N. Y. 10021 ** Recipient of Career Scientist A wardo \['theH ealth Research Council of the City of New York under contract U-2204.
PY - 1974/3
Y1 - 1974/3
N2 - A 3 1/2-year-old "girl" whose condition had been diagnosed as "true agonadism" underwent endocrine evaluation, which suggested the presence of Leydig cells. The child was considered to be female, although born with mild phallic enlargement and posterior labial fusion. At one year of age, evaluation revealed a, 46,XY karyotype; at exploratory laparotomy, no gonads, uterus, Fallopian tubes, or Wolffian remnants were observed. Base-line gonadotropins (FSH, 0.72 ng.per milliliter; LH, 1.9 ng. per milliliter), were not elevated and a significant rise in plasma testosterone (63 to 169 ng. per 100 ml.) followed stimulation with HCG; both features suggested the presence of testosterone-secreting Leydig cells. The obsence of Müllerian anlagen suggests that at some time during fetal development Müllerian-inhibiting factor was secreted by testes. "True agonadism" is probably a misnomer and actually represents that form of male pseudohermaphroditism with a scant amount of functioning testicular tissue present.
AB - A 3 1/2-year-old "girl" whose condition had been diagnosed as "true agonadism" underwent endocrine evaluation, which suggested the presence of Leydig cells. The child was considered to be female, although born with mild phallic enlargement and posterior labial fusion. At one year of age, evaluation revealed a, 46,XY karyotype; at exploratory laparotomy, no gonads, uterus, Fallopian tubes, or Wolffian remnants were observed. Base-line gonadotropins (FSH, 0.72 ng.per milliliter; LH, 1.9 ng. per milliliter), were not elevated and a significant rise in plasma testosterone (63 to 169 ng. per 100 ml.) followed stimulation with HCG; both features suggested the presence of testosterone-secreting Leydig cells. The obsence of Müllerian anlagen suggests that at some time during fetal development Müllerian-inhibiting factor was secreted by testes. "True agonadism" is probably a misnomer and actually represents that form of male pseudohermaphroditism with a scant amount of functioning testicular tissue present.
UR - https://www.scopus.com/pages/publications/0015953744
U2 - 10.1016/S0022-3476(74)80720-1
DO - 10.1016/S0022-3476(74)80720-1
M3 - Article
C2 - 4272854
AN - SCOPUS:0015953744
SN - 0022-3476
VL - 84
SP - 375
EP - 380
JO - Journal of Pediatrics
JF - Journal of Pediatrics
IS - 3
ER -