Human beings with adifference in sexual development (DSD) often underwent gender reassignment surgery during early childhood. However, the medical decision was often not congruent with the gender identity that affected persons developed later on. To represent the interests of affected persons, an interdisciplinary guideline in cooperation with support groups was written. The revision of the first version of the guideline, published in 2016, was edited by 18professional societies and working groups as well as 3support groups. Aliterature search was performed for each of the 12chapters. Recommendations and statements created by the working groups were voted on during four consensus conferences. The guideline highlights the right of self-determination of affected persons. In this context, new legal requirements are reported. Other than necessary primary diagnostics, medical procedures should be postponed. Most important is the psychological support of parents and patients. Tumor risk of the gonads and protection of fertility are analyzed and discussed in detail. The content of the guideline represents aparadigm shift in dealing with human beings with adifference of sexual development. Projects as DSD Care and Empower-DSD help to promote the practical implementation of the guideline's recommendations.
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