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The other patient, a male pseudo\u2010hermaphrodite, was found at autopsy to have typical lipoid adrenal hyperplasia and ectopic adrenal tissue adjacent to an intra\u2010abdominal testicle. Complete vagina, uterus, and fallopian tubes were present in addition to the Wolffian structures. This unique observation supports the view that steroids may be necessary for M\u00fcllerian inhibitory factor to induce regression of M\u00fcllerian structures. The segregation of 27 autosomal markers was studied in one affected and five unaffected sibs. Genetic linkage to HLA, MNS, and GPT is unlikely. In addition, the affected sib is heterozygote for a haplotype of chromosome 1 which includes the Rh, Fy, PGM\u20101 systems. Determination of fetal gender by the combined use of ultrasonography and amniocentesis is suggested for prenatal diagnosis and improved risk counselling.<\/jats:p>","DOI":"10.1002\/ajmg.1320250218","type":"journal-article","created":{"date-parts":[[2005,6,11]],"date-time":"2005-06-11T20:09:08Z","timestamp":1118520548000},"page":"319-325","source":"Crossref","is-referenced-by-count":4,"title":["Familial lipoid adrenal hyperplasia: Genetic marker data and an approach to prenatal 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