Congenital Adrenal Hyperplasia: Female Pseudohermaphroditism and Virilization

2009 ◽  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adrenal Hyperplasia ◽  
Female Pseudohermaphroditism

OCCURRENCE OF CONGENITAL ADRENAL HYPERPLASIA IN HALF SISTERS

PEDIATRICS ◽  
1961 ◽  
Vol 28 (6) ◽  
pp. 946-949
Author(s):  
Geoffrey C. Robinson ◽  
James R. Miller ◽  
Hamish W. McIntosh
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Autosomal Recessive ◽  
Mutant Gene ◽  
Adrenal Hyperplasia ◽  
Female Pseudohermaphroditism ◽  
Recessive Genes

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.

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
Keyword(s):  
Adverse Effects ◽  
Congenital Adrenal Hyperplasia ◽  
Diagnostic Error ◽  
Adrenal Hyperplasia ◽  
Adrenogenital Syndrome ◽  
General Change ◽  
Female Pseudohermaphroditism ◽  
Sex Assignment ◽  
Before And After ◽  
Special Circumstances

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.

Female pseudohermaphroditism caused by maternal congenital adrenal hyperplasia

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

926: Dorsal Nerve Preservation in Modified Reduction Clitoroplasty in Congenital Adrenal Hyperplasia (CAH)

2007 ◽  
Vol 177 (4S) ◽  
pp. 307-307
Author(s):  
Ariella Hochsztein ◽  
Rebecca Baergen ◽  
Emily Loyd ◽  
Jie Chen ◽  
Diane Felsen ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adrenal Hyperplasia ◽  
Nerve Preservation ◽  
Dorsal Nerve
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