Endocrine treatment of gender-dysphoric/gender-incongruent persons : an Endocrine Society clinical practice guideline

Objective: To update the "Endocrine Treatment of Transsexual Persons: An Endocrine Society Clinical Practice Guideline," published by the Endocrine Society in 2009. Participants: The participants include an Endocrine Societyappointed task force of nine experts, a methodologist, and a medical writer. Evidence: This evidence-based guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation approach to describe the strength of recommendations and the quality of evidence. The task force commissioned two systematic reviews and used the best available evidence from other published systematic reviews and individual studies. Consensus Process: Group meetings, conference calls, and e-mail communications enabled consensus. Endocrine Society committees, members and cosponsoring organizations reviewed and commented on preliminary drafts of the guidelines. Conclusion: Gender affirmation is multidisciplinary treatment in which endocrinologists play an important role. Gender-dysphoric/gender-incongruent persons seek and/or are referred to endocrinologists to develop the physical characteristics of the affirmed gender. They require a safe and effective hormone regimen that will (1) suppress endogenous sex hormone secretion determined by the persons genetic/gonadal sex and (2) maintain sex hormone levels within the normal range for the persons affirmed gender. Hormone treatment is not recommended for prepubertal gender-dysphoric/gender-incongruent persons. Those clinicians who recommend gender-affirming endocrine treatments-appropriately trained diagnosing clinicians (required), a mental health provider for adolescents (required) and mental health professional for adults (recommended)-should be knowledgeable about the diagnostic criteria and criteria for gender-affirming treatment, have sufficient training and experience in assessing psychopathology, and be willing to participate in the ongoing care throughout the endocrine transition. We recommend treating gender-dysphoric/gender-incongruent adolescents who have entered puberty at Tanner Stage G2/B2 by suppression with gonadotropin-releasing hormone agonists. Clinicians may add gender-affirming hormones after a multidisciplinary team has confirmed the persistence of gender dysphoria/gender incongruence and sufficient mental capacity to give informed consent to this partially irreversible treatment. Most adolescents have this capacity by age 16 years old. We recognize that there may be compelling reasons to initiate sex hormone treatment prior to age 16 years, although there is minimal published experience treating prior to 13.5 to 14 years of age. For the care of peripubertal youths and older adolescents, we recommend that an expert multidisciplinary team comprised of medical professionals and mental health professionals manage this treatment. The treating physician must confirm the criteria for treatment used by the referring mental health practitioner and collaborate with them in decisions about gender-affirming surgery in older adolescents. For adult gender-dysphoric/gender-incongruent persons, the treating clinicians (collectively) should have expertise in transgender-specific diagnostic criteria, mental health, primary care, hormone treatment, and surgery, as needed by the patient. We suggest maintaining physiologic levels of gender-appropriate hormones and monitoring for known risks and complications. When high doses of sex steroids are required to suppress endogenous sex steroids and/or in advanced age, clinicians may consider surgically removing natal gonads along with reducing sex steroid treatment. Clinicians should monitor both transgender males (female to male) and transgender females (male to female) for reproductive organ cancer risk when surgical removal is incomplete. Additionally, clinicians should persistently monitor adverse effects of sex steroids. For gender-affirming surgeries in adults, the treating physician must collaborate with and confirm the criteria for treatment used by the referring physician. Clinicians should avoid harming individuals (via hormone treatment) who have conditions other than gender dysphoria/gender incongruence and who may not benefit from the physical changes associated with this treatment

Hormone therapy in transgender adults is safe with provider supervision; A review of hormone therapy sequelae for transgender individuals

Introduction: Some providers report concern for the safety of transgender hormone therapy (HT). Methods: This is a systematic literature review of HT safety for transgender adults. Results: Current literature suggests HT is safe when followed carefully for certain risks. The greatest health concern for HT in transgender women is venous thromboembolism. HT among transgender men appears to cause polycythemia. Both groups experienced elevated fasting glucose. There is no increase in cancer prevalence or mortality due to transgender HT. Conclusion: Although current data support the safety of transgender HT with physician supervision, larger, long-term studies are needed in transgender medicine

17-β-hydroxysteroid dehydrogenase type 3 deficiency: Identifying a rare cause of 46, XY female phenotype in adulthood

Objective: To review a case of differences of sexual differentiation (DSD) in an adult female patient and highlight the potential challenges and complexity in diagnosing and managing these conditions. Methods: We review a case of a 21-year-old 46, XY (+SRY) female presenting with primary amenorrhea and the clinical course leading to her suspected and confirmed diagnosis. Results: A 21-year-old 46, XY (+SRY) female presented with primary amenorrhea at age 21. Based on her clinical presentation, she was initially diagnosed with partial androgen insensitivity syndrome, but ultimately found to have 17-β-HSD3 deficiency due to a homozygous mutation in the HSD17B3 gene. Molecular confirmation of her condition, which may not always be performed in DSD cases, provided her a more accurate and individualized treatment plan as well as personal risk assessment for her family members. Conclusion: 17-β-HSD3 deficiency is reported to be a rare cause of female 46, XY DSD but may be overlooked in adult patients due to clinical similarities with androgen insensitivity and lack of genetic testing in suspected cases. It is essential to have a multidisciplinary team to help confirm the diagnosis and provide comprehensive care to affected individuals. Keywords: Differences of sexual differentiation, Androstenedione, 17-β-hydroxysteroid dehydrogenase type