The Experts below are selected from a list of 234 Experts worldwide ranked by ideXlab platform
Antonia Paula Marquesdefaria - One of the best experts on this subject based on the ideXlab platform.
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Female Pseudohermaphroditism due to classical 21 hydroxylase deficiency in a girl with turner syndrome
Clinical Genetics, 2008Co-Authors: Andrea Trevas Macielguerra, Gil Guerra, Sofia Helena Valente De Lemos Marini, Maria Tereza Matias Baptista, Antonia Paula MarquesdefariaAbstract:We report on a rare case of Female Pseudohermaphroditism due to classical 21-hydroxylase deficiency associated with Turner syndrome (45,X/46,XX). Difficulties in the management of both diseases are briefly discussed. We regard this rare combination as a coincidental occurrence.
Andrea Trevas Macielguerra - One of the best experts on this subject based on the ideXlab platform.
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Female Pseudohermaphroditism due to classical 21 hydroxylase deficiency in a girl with turner syndrome
Clinical Genetics, 2008Co-Authors: Andrea Trevas Macielguerra, Gil Guerra, Sofia Helena Valente De Lemos Marini, Maria Tereza Matias Baptista, Antonia Paula MarquesdefariaAbstract:We report on a rare case of Female Pseudohermaphroditism due to classical 21-hydroxylase deficiency associated with Turner syndrome (45,X/46,XX). Difficulties in the management of both diseases are briefly discussed. We regard this rare combination as a coincidental occurrence.
A. Di Benedetto - One of the best experts on this subject based on the ideXlab platform.
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The Anterior Sagittal Transanorectal Approach: A Modified Approach to 1-Stage Clitoral Vaginoplasty in Severely Masculinized Female Pseudohermaphrodites-Preliminary Results
The Journal of Urology, 1997Co-Authors: V. Di Benedetto, M. Gioviale, V. Bagnara, S. Cacciaguerra, A. Di BenedettoAbstract:ABSTRACTPurpose: We present a modified 1-stage clitoral vaginoplasty technique for severely masculinized Female Pseudohermaphroditism involving an anterior sagittal transanorectal approach with the patient prone after clitoroplasty according to the Passerini-Glazel procedure.Material and Methods: An anterior sagittal transanorectal approach with protective colostomy was performed in 2 patients with severely masculinized Female Pseudohermaphroditism and a normal rectum. The anorectal sphincteric mechanism was divided only in the anterior midline, and the perineal body and rectum were opened in the anterior rectal wall, providing excellent exposure of the urogenital sinus. The vagina was easily and fully separated from the urogenital sinus, the site of vaginal attachment to the urethra was sutured, and anastomosis was created between the vaginal neo-introitus and vagina. The rectum, perineal body and anterior sphincteric mechanism were reconstructed.Results: Cosmetic and anatomical results are satisfactory....
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THE ANTERIOR SAGITTAL TRANSANORECTAL APPROACH: A SEVERELY MASCULINIZED Female PSEUDOHERMAPHRODITES-PRELIMINARY RESULTS MODIFIED APPROACH TO 1-STAGE CLITORAL VAGINOPLASTY IN
1997Co-Authors: V. Di, M. Gioviale, V. Bagnara, A. Di BenedettoAbstract:Purpose: We present a modified 1-stage clitoral vaginoplasty technique for severely masculinized Female Pseudohermaphroditism involving an anterior sagittal transanorectal approach with the patient prone after clitoroplasty according to the Passerini-Glaze1 procedure. Material and Methods: An anterior sagittal transanorectal approach with protective colostomy was performed in 2 patients with severely masculinized Female Pseudohermaphroditism and a normal rectum. The anorectal sphincteric mechanism was divided only in the anterior midline, and the perineal body and rectum were opened in the anterior rectal wall, providing excellent exposure of the urogenital sinus. The vagina was easily and fully separated from the urogenital sinus, the site of vaginal attachment to the urethra was sutured, and anastomosis was created between the vaginal neo-introitus and vagina. The rectum, perineal body and anterior sphincteric mechanism were reconstructed. Results: Cosmetic and anatomical results are satisfactory. The vaginal neo-introitus is located just below the urethral meatus, the clitoris appears almost normal and in the vulvar region a mucous lining is present in the front wall of the perineum between the clitoris and vagina. Convalescence was uneventful. The patients had normal bowel control after colostomy closure and no urinary incontinence. Conclusions: Our modified technique favors easy and safe posterior anastomosis between the vaginal neo-introitus and vagina under direct vision. Furthermore, suturing the vaginal stump is easier than in the original technique, since the approach to the vagina is posterior, not transvesical. Kcr Worn: vagina; clitoris; adrenogenital hyperplasia, congenital; Pseudohermaphroditism Different surgical solutions have been proposed for the treatment of severely masculinized Female Pseudohermaphroditism but they have technical problems associated with complex surgical procedures and poor cosmetic results.'q First described in 1989 by Passerini-Glazel, 1-stage clitoral vaginoplasty has great advantages over other surgical techniques.' Nevertheless, we believe that in the 1-stage procedure retrovesical dissection between the urethra and vagina, closure of the vaginal stump and anastomosis between the vaginal neo-introitus and vagina are not easy to perform and do not allow optimal vision of the operative field. Our technique is a modification of the Passerini-Glaze1 procedure. It enables dissection of the vagina from the urethra, suture of the vaginal stump, and anastomosis of the vaginal neointroitus and vagina under direct vision. MATERIAL AND METHODS
Gil Guerra - One of the best experts on this subject based on the ideXlab platform.
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Female Pseudohermaphroditism due to classical 21 hydroxylase deficiency in a girl with turner syndrome
Clinical Genetics, 2008Co-Authors: Andrea Trevas Macielguerra, Gil Guerra, Sofia Helena Valente De Lemos Marini, Maria Tereza Matias Baptista, Antonia Paula MarquesdefariaAbstract:We report on a rare case of Female Pseudohermaphroditism due to classical 21-hydroxylase deficiency associated with Turner syndrome (45,X/46,XX). Difficulties in the management of both diseases are briefly discussed. We regard this rare combination as a coincidental occurrence.
Sofia Helena Valente De Lemos Marini - One of the best experts on this subject based on the ideXlab platform.
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Female Pseudohermaphroditism due to classical 21 hydroxylase deficiency in a girl with turner syndrome
Clinical Genetics, 2008Co-Authors: Andrea Trevas Macielguerra, Gil Guerra, Sofia Helena Valente De Lemos Marini, Maria Tereza Matias Baptista, Antonia Paula MarquesdefariaAbstract:We report on a rare case of Female Pseudohermaphroditism due to classical 21-hydroxylase deficiency associated with Turner syndrome (45,X/46,XX). Difficulties in the management of both diseases are briefly discussed. We regard this rare combination as a coincidental occurrence.
