17-beta-hydroxysteroid dehydrogenase deficiency surgical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Abdulkerim Yassin, M.B.B.S[2]
Overview
Surgical therapy is guided by the risk of germ cell tumor development and to allow for proper gender assignment.
Surgical therapy
- 17-beta-hydroxysteroid dehydrogenase deficiency has a 28 % risk of germ cell tumor in disorder of sexual development. Consequently, close monitoring is warranted for an individual who is raised as a male rather than removing the gonads at the time of diagnosis.[1]
- Surgical treatment can also be used to treat anatomic changes due to the hormonal disorder. Patients with this disease may present with ambiguous or female genitalia, despite most of them developing male gender identity.
- Some studies have suggested that early surgical treatment may improve outcomes. Feminizing genitoplasty usually requires less surgical interventions than masculinizing genitoplasty. Outcomes vary greatly in both cases. Many patients may present with risk of neoplasia associated with the neovagina, while regarding the masculinizing genitoplasty's results are heavily associated with the amount of erectile tissue and degree of hypospadias.[2]
References
- ↑ "The Clinical and Molecular Heterogeneity of 17βHSD-3 Enzyme Deficiency - FullText - Hormone Research in Paediatrics 2010, Vol. 74, No. 4 - Karger Publishers".
- ↑ Melnik B, Hollmann J, Hofmann U, Yuh MS, Plewig G (1990). "Lipid composition of outer stratum corneum and nails in atopic and control subjects". Arch Dermatol Res. 282 (8): 549–51. doi:10.1007/BF00371952. PMID 2082839.