Caring for Patients With Congenital Adrenal Hyperplasia Throughout the Lifespan.

Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder affecting cortisol and aldosterone biosynthesis, which can lead to virilization in fetuses with a 46,XX karyotype. 21-hydroxylase deficiency is the most common cause of CAH, accounting for 90-99% of all patients with the condition. The management of patients with CAH should be done with a multidisciplinary team, which would address all of the complex components of their care throughout their lifespans. Many multidisciplinary teams have adopted shared decision-making approaches to genital surgery in which parents and patients can be part of the decision-making process. Continued research is needed to best serve these patients throughout their lifespans.

Evolving indications for surgical intervention in patients with differences/disorders of sex development: Implications of deferred reconstruction.

Rare medical conditions are difficult to study due to the lack of patient volume and limited research resources, and as a result of these challenges, progress in the care of patients with these conditions is slow. Individuals born with differences of sex development (DSD) fall into this category of rare conditions and have additional social barriers due to the intimate nature of the conditions. There is also a lack of general knowledge in the medical community about this group of diverse diagnoses. Despite these limitations, progress has been made in the study of effective ways to care for patients who are born with chromosomal or anatomical differences of their internal reproductive organs or external genitalia. Advocacy groups have placed a spotlight on these topics and asked for a thoughtful approach to educate parents of newborns, medical providers, and the adolescents and young adults themselves as they mature.1 There is growing interest in the approaches to surgical reconstruction of the genitalia and the management of internal gonads, specifically the timing of procedures and the indications for those procedures.2 Advocates suggest deferring surgical procedures until the affected individual can participate in the decision-making process. This approach requires a roadmap for addressing the long-term implications of delayed surgical management. Presented here is a review of the specific issues regarding the complex management of the various categories of DSD.

Bleeding Patterns among Adolescents Using the Levonorgestrel Intrauterine Device: A Single Institution Review.

STUDY OBJECTIVE: To describe the bleeding patterns associated with the use of the levonorgestrel intrauterine device (IUD) in adolescents. DESIGN, SETTING, AND PARTICIPANTS: A retrospective chart review of postmenarchal adolescent patients ages 8-19 years who had the levonorgestrel IUD inserted at Phoenix Children's Hospital from 2012 to 2018. INTERVENTIONS: Insertion of the 52-mg and 13.5-mg levonorgestrel IUD. MAIN OUTCOME MEASURES: The rate of amenorrhea and other bleeding patterns after insertion of the levonorgestrel IUD and the factors that might predict those bleeding patterns. RESULTS: A total of 260 charts were identified with 221/260 (85.0%) patients choosing the 52-mg IUD and 39/260 (15.0%) patients choosing the 13.5-mg IUD to be inserted. Follow-up data were available for 166 patients. The overall rate of amenorrhea among IUD users was 39.8% (n = 66) with no difference between 52-mg and 13.5-mg IUD users (P = .656). Regularity and flow of menstrual cycle, history of bleeding disorder, history of developmental delay, and current treatment with testosterone for gender dysphoria before IUD insertion did not appear to have a significant effect on the rate of amenorrhea or bleeding patterns post-IUD insertion. CONCLUSION: The levonorgestrel IUD can be successfully used to control abnormal uterine bleeding and suppress menses in adolescents. Menstrual cycle characteristics pre-IUD insertion did not result in predictable post-IUD bleeding patterns.