PSYCHOLOGIC STUDIES BEFORE AND AFTER CLITORIDECTOMY IN FEMALE PSEUDOHERMAPHRODITISM CAUSED BY CONGENITAL VIRILIZING ADRENAL HYPERPLASIA

PEDIATRICS ◽  
1958 ◽  
Vol 21 (5) ◽  
pp. 832-839
Author(s):  
Alan K. Rosenwald ◽  
Joseph H. Handlon ◽  
Ira M. Rosenthal ◽  
John S. Hyde ◽  
I. Pat Bronstein

Psychobogic tests were performed with five female pseudohermaphrodites with congenital adrenal hyperplasia both before and some time after clitoridectomy. Of these children, four had been reared as girls. The child who had been reared as a boy underwent change of sex assignment at the age of 6 years, after performance of the initial psychologic tests. None of the children showed evidence of significant psychopathology before clitoridectomy. None showed evidence of deleterious psychologic effects after the operation. The child whose sex was changed at the age of 6 years apparently made a good adjustment and showed no adverse effects as evaluated by psychologic tests. If there is no significant psychopathology, clitoridectomy may be performed without fear of deleterious psychologic effects in female pseudohermaphrodites who have been reared as girls. While, in general, change of sex assignment should not be done in any hermaphrodite beyond the infantile age period, female pseudohermaphrodites with adrenogenital syndrome who have been reared as boys, because of an original diagnostic error, should be individually evaluated with regard to possible change of sex assignment. Despite the apparent success in the case reported here with its unusual special circumstances, the psychologic hazards involved will make reassignment of sex inadvisable in most of these cases.

PEDIATRICS ◽  
1961 ◽  
Vol 28 (6) ◽  
pp. 946-949
Author(s):  
Geoffrey C. Robinson ◽  
James R. Miller ◽  
Hamish W. McIntosh

The occurrence of female pseudohermaphroditism due to congenital adrenal hyperplasia in half sisters is described. It is concluded that the disease is transmitted by autosomal recessive genes. The mother, herself a heterozygote, was unfortunate in that her two husbands each were heterozygous for the mutant gene in question.


1979 ◽  
Vol 95 (3) ◽  
pp. 418-420 ◽  
Author(s):  
Hiroshi. Kai ◽  
Osamu. Nose ◽  
Yoshihiko. Iida ◽  
Jiro. Ono ◽  
Tokuzo. Harada ◽  
...  

2021 ◽  
Vol 16 (3) ◽  
pp. 245-266
Author(s):  
Irma Th. Rass

Glucocorticoid (GC) preparations are used in medicine for more than 70 years as the most powerful anti-inflammatory drugs also possessing immunosuppressive, anti-allergic, and antitoxic properties. However, administration of these unique preparations is associated with nearly inevitable severe adverse effects and a difficulty of their withdrawal. These adverse effects are caused not by toxicity of GC preparations, but are manifestations of their hormonal features. GC preparations are synthetic analogs of GC hormones which directly or indirectly participate in the regulation of virtually all reactions and processes in the body. Nevertheless, in clinical practice there is no index of tissue provision with GCs and real need in these hormones (or preparations). In this paper, blood tyrosine level was shown to characterize the tissue provision with GCs on two models: adrenalectomy in rats and the replacement GC therapy in congenital adrenal hyperplasia. Determination of blood tyrosine level made it possible to reveal the insufficiency of tissue provision with GGs in patients with bronchial asthma during the period of attacks. In patients with systemic lupus erythematosus, GC preparations were shown to be favorable on the background of increased blood tyrosine, i.e. on the insufficient tissue provision with GCs, and until the normalization of blood tyrosine, i.e. until the compensation of the hormonal insufficiency. On the background of normal blood tyrosine GC preparations in SLE were ineffective and side effects appeared rapidly. These observations allowed me to propose blood tyrosine level as a laboratory parameter for monitoring GC therapy. The present paper consists of two parts: I) short reviews of the literature prerequisites for the proposal; II) the description of the author’s studies on blood tyrosine behavior in comparison with delivery and efficiency of glucocorticoid hormones or preparations in experiment (adrenalectomy in rats) and in some pathologies: congenital adrenal hyperplasia, bronchial asthma, and systemic lupus erythematosus.


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