SYNDROME OF RUDIMENTARY OVARIES WITH ESTROGENIC INSUFFICIENCY AND INCREASE IN GONADOTROPINS

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Abstract
FOR MANY years it seemed reasonable, when a patient presented retardation of growth and lack of genital development, to accept these two phenomena as being due to a primary hypophyseal lesion, provided that cretinism, severe disorders of nutrition and other conditions with general impairment could be excluded. The knowledge gained concerning the relations between the anterior pituitary gland and the gonads, and particularly the investigations of the hormones of these glands in the urine, have made possible a better understanding and explanation of several of these syndromes. Worthy of mention in this respect are the initial discoveries made by Zondek, and Smith and Engle, showing definitely the hypophyseal-gonadal relations. Subsequently, as a result of these investigations, biological tests have appeared which allowed the identification and measure of certain hypophyseal hormones and illustrated many concepts of the physiopathology of these conditions. Based mainly on the application of these biological tests, two groups of American investigators, Varney and his collaborators (1942) (33) and Albright and his co-workers (1942) (1) established the clinical and hormonal characteristic of a new syndrome and published various observations on this subject. The former authors called it “Association of short stature, retarded sexual development and high urinary gonadotropin titers in women”, the latter “A syndrome characterized by primary ovarian insufficiency and decreased stature. A report of 11 cases with a digression on hormonal control of axillary and pubic hair”. Later, more cases, with ovarian biopsies, were presented by Wilkins and Fleischmann (1944) (34–35). These investigators called this condition “ovarian agenesis”.