17 alpha-hydroxylase deficiency surgery
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Congenital adrenal hyperplasia due to 17 alpha-hydroxylase deficiency Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
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17 alpha-hydroxylase deficiency surgery On the Web |
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American Roentgen Ray Society Images of 17 alpha-hydroxylase deficiency surgery |
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Risk calculators and risk factors for 17 alpha-hydroxylase deficiency surgery |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]
Overview
The predominant therapy for ambigous genitalia in congenital adrenal hyperplasia due to 17 alpha-hydroxylase deficiency is surgical correction.
Surgery
- The most difficult management decisions are posed by the more ambiguous genetic (XY) males. Most who are severely undervirilized, looking more female than male, are raised as females with surgical removal of the nonfunctional testes. If raised as males, a brief course of testosterone can be given in infancy to induce growth of the penis. Surgery may be able to repair the hypospadias. The testes should be salvaged by orchiopexy if possible. Testosterone must be replaced in order for puberty to occur and continued throughout adult life.