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The male gonad, in turn, normally produces hormones such as testosterone and Mullerian inhibiting substance (MIS) that regulate differentiation of the internal and external genitalia, thus determining phenotypic sex. When an abnormality develops in any of the above three processes, an intersex infant with ambiguous genitalia results from the incongruent genetic, gonadal, and phenotypic sex. Clinically, such 46XY males with intersex abnormalities present challenges for gender assignment, timely surgical intervention, and appropriate hormonal therapy. <\/jats:p>","DOI":"10.1146\/annurev.med.45.1.505","type":"journal-article","created":{"date-parts":[[2002,7,27]],"date-time":"2002-07-27T11:45:04Z","timestamp":1027770304000},"page":"505-524","source":"Crossref","is-referenced-by-count":44,"title":["MALE SEX DETERMINATION: Current Concepts of Male Sexual Differentiation"],"prefix":"10.1146","volume":"45","author":[{"given":"Michael L.","family":"Gustafson, M.D","sequence":"first","affiliation":[{"name":"Division of Pediatric Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts 02114"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia K.","family":"Donahoe, M.D","sequence":"additional","affiliation":[{"name":"Division of Pediatric Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts 02114"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"22","reference":[{"key":"b1","volume-title":"Langman's Medical Embryology.","author":"Sadler TW","year":"1990"},{"key":"b2","first-page":"67","volume":"32","author":"Witschi E","year":"1948","journal-title":"Contrib. 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