This Article Full Text (PDF) Submit a related Letter to the Editor Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Reprints, Permissions and Rights Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by GORDAN, G. S. Articles by WINCH, G. A. Search for Related Content PubMed PubMed Citation Articles by GORDAN, G. S. Articles by WINCH, G. A.

A SYNDROME OF GONADAL DYSGENESIS: A VARIETY OF OVARIAN AGENESIS WITH ANDROGENIC MANIFESTATIONS^*

The Endocrine Clinic of the Department of Medicine, University of California School of Medicine San Francisco, California
Department of Obstetrics and Gynecology, University of California School of Medicine San Francisco, California

THE condition now generally known as ovarian agenesis has been thoroughly denned by Varney, Kenyon and Koch, 1942 (1), Albright, Smith and Fraser, 1942 (2), Wilkins and Fleischmann, 1944 (3) and Lisser, Curtis, Escamilla and Goldberg, 1947 (4). The syndrome consists of: a) primary amenorrhea, b) lack of development of primary and secondary sexual characteristics, c) increased urinary gonadotropin titer, often with d) short stature (usually 53–57 inches) and e) associated congenital anomalies, of which webbed neck, cubitus valgus, and coarctation of the aorta are the most frequent (Turner, 1938 (5)). The genital maldevelopment consists of: a) atrophic external genitalia, including the clitoris, b) rudimentary uterus, c) somewhat small, but complete, fallopian tubes and d) ovarian anlagen represented by elongated streak-like structures, white and glistening, on the posterior surface of each broad ligament, extending parallel to the tubes, from the uterine cornu to the fimbriated end. Histologically, these ovarian anlagen contain onty rudimentary stroma and no primordial follicles or germinal epithelium.

^* Part of the cost of these studies was defrayed by a grant from Ciba Pharmaceutical Products, Inc., Summit, New Jersey. Chorionic gonadotropin (APL®) and Premarin® were generously furnished by Ayerst Laboratories, New York, N. Y.

Received April 6, 1954.

This article has been cited by other articles:

				R. B. Greenblatt, O. M. de Acosta, E. Vazquez, and D. F. Mullins Jr. ORAL MUCOSAL SMEARS IN DETECTION OF GENETIC SEX JAMA, June 23, 1956; 161(8): 683 - 685. [Abstract] [PDF]