• Clinical and Experimental Reproductive Medicine
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• v.33 no.2
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• pp.133-138
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• 2006

Female phenotype of a 46,XY male may originates from male pseudohermaphroditism due to $17{\alpha}$-hydroxylase deficiency. Lack of cortisol increases adrenocorticotropic hormone (ACTH) and mineralocorticoid production, leading to low renin hypertention and hypokalemia. A 41-year-old phenotypic female presented primary amenorrhea and hypertension. In the hormonal profile, the levels of serum estradiol, testosterone, rennin, and cortisol were decreased and ACTH and deoxycorticosterone were increased. Laparoscopic bilateral gonadectomy was performed, and corticosteroid, antihypertensive drugs, and estrogen were administered. We report this case with a brief review of the literatures.

• Archives of Plastic Surgery
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• v.32 no.1
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• pp.110-116
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• 2005

Male pseudohermaphroditism is individuals with Y chromosome whose external appearances fail to develop as expected as a normal male. Cytogenetic form of male pseudohermaphroditism was 46XY but external genital forms were variable. We reviewed data from 7 patients with male pseudohermaphroditism operated in Dong-A University Hospital from January 1996 to August 2002. They all had severe form of hypospadias (pseudovaginal perineoscrotal type). Only 1 of 7 cases was operated urethroplasty by one stage method, and all others by two stage method. Patients with mild chordee were operated by one stage method and patients with severe chordee were operated by two stage method. Complete chordee release was done by Z-plasty or FTSG on the first stage and posterior urethroplasty was done at level of mid-dorsum of shaft at the same time. Anterior urethroplasty was done completely on the second stage. Results were good from the viewpoint of shape and urination. Urethral fistula occurred in two cases, but other complications (infection, urethral stricture, etc.) didn't occur. In conclusion, two stage method was compatible to correct hypospadias in male pseudohermaphroditism, because hypospadias in male pseudohermaphroditism is in severe form.