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Chapter-091a 46,XX Disorders of Sexual Development

BOOK TITLE: ESI Manual of Clinical Endocrinology

Author
1. C Shivaprasad
ISBN
9789351526476
DOI
10.5005/jp/books/12535_114
Edition
2/e
Publishing Year
2015
Pages
8
Author Affiliations
1. MS Ramaiah Medical College, Bengaluru, Karnataka, India, Vydehi Institute of Medical Sciences, Bengaluru, Karnataka, India, Vydehi Institute of Medical Sciences and research Centre, Bengaluru, Karnataka, India
Chapter keywords
Masculinization, ovotesticular DSD, XX testicular DSD, gonadal dysgenesis, glucocorticoid replacement, congenital adrenal hyperplasia

Abstract

The 46, XX DSDs cause masculinization of the genetically female fetus and occur due to exposure to androgens during certain critical times of development. Clinical features and presentations of disorders of ovarian development, as well as disorders of androgen synthesis are discussed. The disorders of ovarian development include ovotesticular DSD, XX testicular DSD and gonadal dysgenesis. The disorders of androgen synthesis are fetal: congenital adrenal hyperplasia, fetoplacental disorders of androgen excess and maternal disorders of androgen excess. These can be diagnosed on the basis of genital ambiguity, family history of DSD etc. Treatment of CAH comprises glucocorticoid replacement, maintaining fluid and electrolyte balance and vaginal reconstruction surgeries. The factors influencing the gender assignment includes diagnosis, genital appearance, surgical options, need for lifelong replacement therapy, the potential for fertility, views of the family etc.

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