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False claims of having an intersex condition have been observed in print, video, Internet media, and in live presentations. Claims of being intersexed in publicly accessible media were examined and evidence that they were false was considered sufficiently conclusive in 37 cases. Falsity was most often detected due to medical implausibility and/or inconsistency, but sometimes also using information from third-party or published sources. The majority, 26/37, of cases were natal males; 11/37 were natal females. Almost all (34/37) were transgendered, living, or aspiring to live, in their non-natal sex or as socially intergender. The most commonly claimed diagnosis was ovotesticular disorder ("true hermaphroditism") due to chimerism, an actually uncommon cause of authentic intersexuality. Motivations for pretending to be intersexed were inferred from statements and behaviors and were varied. Some such pretenders appear to be avoiding the external or internalized stigma of an actual transgendered condition. Some appear, similarly to persons with factitious disorder, to be seeking attention and/or the role of a sick, disadvantaged, or victimized person. Some showed evidence of paraphilia, most frequently autogynephilia, and, in several cases, paraphilic diaperism. For some cases, such claims had been accepted as authentic by journalists or social scientists and repeated as true in published material. [ABSTRACT FROM AUTHOR]

Complete androgen insensitivity syndrome is a rare disorder. Primary amenorrhea beyond 14 years of age without, and 16 years along with developed secondary sexual characters should raise suspicion of this entity as a cause. Inguinal examination may confirm presence of inguinal gonads. The absence of female internal genitalia on imaging, absence of pubic/axillary hair, and chromosomal analysis clinch the diagnosis. Surgical excision of gonads to prevent malignancy, estrogen supplementation, and vaginoplasty along with psychological counselling remain the mainstay of treatment. [ABSTRACT FROM AUTHOR]

Background: The aim of this article is to review the essential concepts, current terminologies and classification, management guidelines and the rationale of gender assignment in different types of differences/disorders of sexual development. Main body: The basics of the present understanding of normal sexual differentiation and psychosexual development were reviewed. The current guidelines, consensus statements along with recommendations in management of DSD were critically analyzed to formulate the review. The classification of DSD that is presently in vogue is presented in detail, with reference to old nomenclature. The individual DSD has been tabulated based on various differential characteristics. Two schemes for analysis of DSD types, based on clinical presentation, karyotype and endocrine profile has been proposed here. The risk of gonadal malignancy in different types of DSD is analyzed. The rationale of gender assignment, therapeutic options, and ethical dimension of treatment in DSD is reviewed in detail. Conclusion: The optimal management of different types of DSD in the present era requires the following considerations: (1) establishment of a precise diagnosis, employing the advances in genetic and endocrine evaluation. (2) A multidisciplinary team is required for the diagnosis, evaluation, gender assignment and follow-up of these children, and during their transition to adulthood. (3) Deeper understanding of the issues in psychosexual development in DSD is vital for therapy. (4) The patients and their families should be an integral part of the decision-making process. (5) Recommendations for gender assignment should be based upon the specific outcome data. (6) The relative rarity of DSD should prompt constitution of DSD registers, to record and share information, on national/international basis. (7) The formation of peer support groups is equally important. The recognition that each subject with DSD is unique and requires individualized therapy remains the most paramount. [ABSTRACT FROM AUTHOR]

Background and objective: Differences of sexual development (DSD) affect the development of internal reproductive organs and external genitalia. Ectopic kidney is a rare and challenging pathology causing amongst others incontinence and recurrent urinary tract infections. Those pathologies may in certain cases be an indication for surgery. This manuscript aims to evaluate the role of robot-assisted laparoscopy in the surgical treatment of patients with ectopic kidneys or DSD. Materials and methods: A prospective database is maintained in a tertiary referral center with all robotic surgeries performed in children. From this database, a prospective series of robot-assisted resection of embryologic remnants located in the pelvis was extracted: resection of a prostatic utricle cyst, removal of ectopic non-functional kidneys, and resection of a hemi-uterus. Results: From an initial database including 72 patients, six patients met the inclusion criteria. Three male patients presenting with utricle cysts, two young girls presenting with ectopic kidneys, and one young boy with pelvic embryological remnants of the uterus, were further evaluated. Conclusion: Surgical treatment of patients with DSD is safe, feasible, and a good indication for robot-assisted laparoscopic surgery, as both deep dissection and reconstruction in a limited surgical field are requested. [ABSTRACT FROM AUTHOR]

Background: Neonatal-onset Denys–Drash syndrome (NODDS) is a distinctive clinical entity and has a poor renal and life outcome. Early diagnosis of NODDS is important for managing disorders of sexual development and determining assigned gender. Although patients with NODDS and congenital nephrotic syndrome of the Finnish type (CNF) present with nephrotic syndrome in neonatal life or infancy, the clinical course of NODDS and factors distinguishing these diseases at onset is unknown. Methods: We performed a retrospective cohort study of patients with NODDS and CNF between 1997 and 2017. Patients with nephrotic syndrome and WT1 or NPHS1 mutations with neonatal onset (within 30 days) were eligible. Results: We studied eight patients with NODDS and 15 with CNF. The median serum creatinine level at onset in the NODDS group was significantly higher (1.85 mg/dL) than that in the CNF group (0.15 mg/dL; P = 0.002). The median placental/fetal weight ratio in the NODDS and CNF group was 41.8% and 21.0%, respectively (P = 0.001). Kaplan–Meier analysis showed that the median number of days for progression to ESRD from onset in the NODDS and CNF groups was 6 and 910 days, respectively (P < 0.001). All patients in the NODDS group were alive at follow-up. Only one patient in the CNF group died of cardiac complications during follow-up. Conclusion: CNS, renal dysfunction at onset, and a relatively large placenta are prominent signs of NODDS. Prognosis for patients with NODDS is satisfactory if appropriate and active management is performed. [ABSTRACT FROM AUTHOR]

This article explores the relationship between gender, technology, language, and how infants and children born with disorders of sexual development are shaped into intelligible members of the community. The contemporary medical model maintains that children ought to be both socially and surgically assigned and reared as one particular gender. Gender scholar Suzanne Kessler rejects this position and argues for the acceptance of greater genital variability through the use of language. Using a Heideggerian lens, the main question I seek to answer in this article is: does Kessler's approach succeed in its aim to better treat individuals born with disorders of sexual development? I argue that Kessler is successful in offering practical solutions for persons with intersexed conditions to exist and flourish as intelligible members of the community, but that her project ultimately relies on power to "challenge forth" greater acceptance of genital variance. Building on the work of Kessler and Heidegger, I argue that a better approach to making intelligible the existence of an infant born with a disorder of sexual development is not to rely on the manipulation of language, but to instead reinvigorate a sense of the sacred in response to having an intersex condition. [ABSTRACT FROM AUTHOR]

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Disorders of sexual development (DSD) are a complex group of disorders to manage, not only because the diagnosis is difficult, but also because it involves many factors related to the psychosexual well being, the social stigma associated with the disorder, confusion and reluctance on the part of medical professionals in dealing with this issue, catastrophic experience of parents of these children and difficult interpersonal relationships between parents and children born with this disorder. This brief review attempts to throw light on the recent advances in understanding and management of these disorders to help improve immediate and long term care of these patients and their families. [ABSTRACT FROM AUTHOR]

Copyright of Monatsschrift Kinderheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Monatsschrift Kinderheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Monatsschrift Kinderheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Monatsschrift Kinderheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)