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Surgical treatment of disorders of sexual development
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Rafael V. Pieretti, Patricia K. Donahoe
Male pseudohermaphroditism occurs in 46,XY genetic males with deficient masculinization of the external genitalia due to insufficient testosterone production, conversion, or inadequate target organ response. Many patients with male pseudohermaphroditism have been raised as males. However, if the female gender is chosen, gonadectomy should be done at the time of perineal reconstruction. The patient with an absent or rudimentary vagina usually requires only a clitoroplasty and labioscrotal reduction. The labioscrotal folds should be partially reduced during the first procedure and dilatation or a substitute vaginoplasty planned for the late adolescent or early adult years. Patients with testicular feminization in whom an introitus is often present may have this dilated with bougies at a later age to form a functional vagina.
Variation of sex differentiation
Published in Joseph S. Sanfilippo, Eduardo Lara-Torre, Veronica Gomez-Lobo, Sanfilippo's Textbook of Pediatric and Adolescent GynecologySecond Edition, 2019
Anne-Marie Amies Oelschlager, Margarett Shnorhavorian
Gonadal surgery: The purposes of gonadectomy include treating or preventing a gonadal tumor, or decreasing virilization in a patient who identifies female with testes.77 For patients with Y chromatin present, there may be an increased risk of gonadal tumor development. The risk of gonadal malignancy is based on the underlying etiology, with dysgenetic gonadal tissue conferring the greatest risk (Table 7.7). The highest risk of tumor development is in dysgenetic gonadal tissue; therefore when there is no potential for fertility or hormonal function, gonadectomy is recommended.78 For patients with a low risk of tumor, including androgen insensitivity syndrome, retaining the testes will allow spontaneous pubertal progression with a low risk of tumor development through adolescence.79 Given the low risk of tumor with CAIS, current recommendation is that gonadectomy should be delayed until the patient can consent to the procedure.
Developmental abnormalities of the genitalia: intersex, hypospadias, and cryptorchidism
Published in J Kellogg Parsons, E James Wright, The Brady Urology Manual, 2019
Management:2Gender assignment (historically female).Gonadectomy or orchidopexy.Screening for Wilms’ tumor.
Chronic exposure to dim light at night disrupts cell-mediated immune response and decreases longevity in aged female mice
Published in Chronobiology International, 2022
Jennifer A. Liu, James C. Walton, Jacob R. Bumgarner, William H. Walker, O. Hecmarie Meléndez-Fernández, A. Courtney DeVries, Randy J. Nelson
In our study, mice were unmated and remained gonadally intact throughout the duration of their lifespan; these factors have previously been demonstrated to affect lifespan and longevity (Garratt, 2020; Benedusi, 2015). In female mice, reproductive changes and decline are detectable by 13–14 months of age (Nelson et al. 1995), and accelerates such that 80% of female mice are acyclic or exhibit irregular cycles at 17 months of age and 100% by 25 months (Frick et al., 2000). Thus, because the mice in our study were assigned to experimental lighting groups at approximately 20 months of age, it is likely that the majority experienced reproductive decline prior to the experimental lighting exposure. We did not gonadectomize aged mice as we addressed the experimental question of how exposure to chronic lighting conditions would affect otherwise typically aging mice. Whether gonadectomy would alter the outcome of our study, implicating a role for gonadal steroids across the lifespan, is an interesting question that should be answered in future studies.
Neoplasia in Turner syndrome: a retrospective cohort study in a tertiary referral centre in Belgium
Published in Acta Clinica Belgica, 2022
Cas Dejonckheere, Carolien Moyson, Francis de Zegher, Leen Antonio, Griet Van Buggenhout, Brigitte Decallonne
This cohort study has several limitations, such as a relatively small sample size, compiled from a single tertiary referral centre, and its retrospective nature. Statistically, about 2,300 women in Belgium have Turner syndrome, thus we estimate that this study comprises about 5% of them. The most recent data from included patients could date back up to 10 years, which could potentially introduce bias. This was the case for only a small portion of included women, however, since annual evaluation is recommended. On the other hand, this might have led to earlier diagnosis and treatment of certain tumours. The fairly low mean age in our cohort implicates an incomplete age coverage and thus tumour spectrum, potentially leading to an underestimation of the actual tumour risk. However, the use of SIRs as a means of comparing with national incidence rates, partially corrects for this. With respect to the risk of gonadoblastoma and/or dysgerminoma, gonadectomy was performed at a young age in the majority of patients, when tumour incidence is still low (i.e. for prophylactic reasons). Moreover, some analyses took place more than a decade ago, using older and thus outdated detection techniques. Therefore, the actual risk might have been underestimated. Despite these limitations, we reason that our study is an accurate representation of the Belgian situation. A national TS database, linked with the national cancer registry, could help to better map the neoplasia risk among Belgian women with TS.
Molecular study and genotype–phenotype in Chinese female patients with 46, XY disorders of sex development
Published in Gynecological Endocrinology, 2021
Junke Xia, Jing Wu, Chen Chen, Zhenhua Zhao, Yanchuan Xie, Zhouxian Bai, Xiangdong Kong
After diagnosis, the first aspect to be considered is gender re-assignment. For patients in childhood (P.4 and P.9), gender re-assignment was performed according to their parents’ decision. For patients aged above 11–12 years old who had developed their own consciousness, gender re-assignment was performed based on the joint decisions of the patients and his parents/guardians. All the patients refused to change gender after careful consideration of the medical and psychosocial issues. Gonadectomy is advised at an early age to avoid the potential risk of malignancy and the psychosocial difficulties in subjecting an adolescent female to gonadectomy [32]. When gonadectomy is performed before puberty, estrogen replacement is necessary to induce puberty [10]. In patients who maintained the gonads, a careful monitoring including US or MRI has been suggested [33].