Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 957
Filtrar
1.
Arch Sex Behav ; 53(5): 1695-1711, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38684620

RESUMEN

Several aspects of clinical management of 46,XX congenital adrenal hyperplasia (CAH) remain unsettled and controversial. The North American Disorders/Differences of Sex Development (DSD) Clinician Survey investigated changes, over the last two decades, in clinical recommendations by specialists involved in the management of newborns with DSD. Members of the (Lawson Wilkins) Pediatric Endocrine Society and the Societies for Pediatric Urology participated in a web-based survey at three timepoints: 2003-2004 (T1, n = 432), 2010-2011 (T2, n = 441), and 2020 (T3, n = 272). Participants were presented with two clinical case scenarios-newborns with 46,XX CAH and either mild-to-moderate or severe genital masculinization-and asked for clinical recommendations. Across timepoints, most participants recommended rearing the newborn as a girl, that parents (in consultation with physicians) should make surgical decisions, performing early genitoplasty, and disclosing surgical history at younger ages. Several trends were identified: a small, but significant shift toward recommending a gender other than girl; recommending that adolescent patients serve as the genital surgery decision maker; performing genital surgery at later ages; and disclosing surgical details at younger ages. This is the first study assessing physician recommendations across two decades. Despite variability in the recommendations, most experts followed CAH clinical practice guidelines. The observation that some of the emerging trends do not align with expert opinion or empirical evidence should serve as both a cautionary note and a call for prospective studies examining patient outcomes associated with these changes.


Asunto(s)
Hiperplasia Suprarrenal Congénita , Humanos , Femenino , Masculino , Encuestas y Cuestionarios , Recién Nacido , América del Norte , Adolescente , Pautas de la Práctica en Medicina , Trastornos del Desarrollo Sexual/cirugía , Adulto
2.
Bioethics ; 38(5): 460-468, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38470400

RESUMEN

This article argues for a ban on the performance of medically unnecessary genital normalizing surgeries as part of assigning a binary sex/gender to infants with intersex conditions on the basis of autonomy, regardless of etiology. It does this via a dis/analogy with the classic case in bioethics of Jehovah Witness (JW) parents' inability to refuse life-saving blood transfusions for their minor children. Both cases address ethical medical practice in situations where parents are making irreversible medical decisions on the basis of values strongly held, identity, and relationship-shaping values-such as religious beliefs or beliefs regarding the inherent value of binary sex/gender-amidst ethical pluralism. Furthermore, it takes seriously-as we must in the intersex case-that the restriction of parents' right to choose will likely result in serious harms to potentially large percentage of patients, their families, and their larger communities. I address the objection that parents' capacity to choose is restricted in the JW case on the basis of the harm principle or a duty to nonmaleficence, given that the result of parent choice would be death. I provide evidence that this is mistaken from how we treat epistemic uncertainty in the JW case and from cases in which clinicians are ethically obligated to restrict the autonomy of nonminor patients. I conclude that we restrict the parents' right to choose in the JW case-and should in the case of pediatric intersex surgery-to secure patient's future autonomy.


Asunto(s)
Transfusión Sanguínea , Trastornos del Desarrollo Sexual , Testigos de Jehová , Padres , Autonomía Personal , Humanos , Transfusión Sanguínea/ética , Masculino , Femenino , Trastornos del Desarrollo Sexual/cirugía , Negativa del Paciente al Tratamiento/ética , Cirugía de Reasignación de Sexo/ética , Lactante , Niño , Religión y Medicina , Toma de Decisiones/ética , Consentimiento Paterno/ética
3.
Arch Sex Behav ; 53(5): 1681-1694, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38383942

RESUMEN

The traditional gender binary constitutes an integral aspect of Islamic social ethics, which has a pivotal role in shaping religious obligations, legal proceedings, and interpersonal judgments within Muslim communities. Within the familial sphere, this gender binary underscores fundamental responsibilities encompassing parenthood, filial duties, and inheritance rights. Recent years have witnessed a growing challenge to the traditional concept of the gender binary within Islamic societies. This shift is driven by increasing social libertarianism that emphasizes gender fluidity and individual choice. Hence, this article aims to critically scrutinize evolving discussions and controversies about the rights of intersex and transgender individuals, particularly issues relating to sex reassignment or gender-affirming surgery, marriage, and reproduction, from the perspective of the Sunni tradition of Islam. To support the various interpretations and insights presented here, a comprehensive and rigorous analysis is carried out on various religious texts and scholarly sources to elucidate the theological and jurisprudential positions on gender issues. It is thus concluded that Shariah offers greater flexibility in the treatment of intersex individuals compared to those with gender dysphoria because the intersex condition is viewed as a physical impairment that is not the choice of the afflicted individual. By contrast, in the case of individuals with gender dysphoria, they are willfully attempting to change their recognized biological sex, that God had naturally given to them at birth. Therefore, it is recommended that such transgender individuals deserve respectful psychological and social rehabilitation with help and guidance from religious authorities, their families, and communities.


Asunto(s)
Islamismo , Matrimonio , Derechos Sexuales y Reproductivos , Cirugía de Reasignación de Sexo , Personas Transgénero , Humanos , Cirugía de Reasignación de Sexo/legislación & jurisprudencia , Personas Transgénero/psicología , Matrimonio/legislación & jurisprudencia , Matrimonio/psicología , Masculino , Derechos Sexuales y Reproductivos/legislación & jurisprudencia , Femenino , Trastornos del Desarrollo Sexual/psicología , Trastornos del Desarrollo Sexual/cirugía
4.
J Equine Vet Sci ; 133: 105007, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38237706

RESUMEN

A two-year-old Arabian horse presented for abnormal external genitalia and dangerous stallion-like behavior was diagnosed with disorder of sexual development (DSD), also known as intersex/hermaphroditism. Standing 1-stage surgical procedure performed under sedation, and local anesthesia to concurrently eliminate stallion-like behavior, risk of neoplastic transformation of intraabdominal gonads, and to replace ambiguous external genital with a functional, and cosmetically more acceptable anatomy. Step-1) Laparoscopic abdominal exploration and gonadectomy; Step-2) Rudimentary penis resection and perineal urethrostomy. The horse tolerated surgery well (combined surgery time 185 min) with no complications. At macroscopic examination of the gonads, they resembled hypoplastic testis-like tissues. Microscopic examination confirmed presence of seminiferous tubules, Leydig and Sertoli/granulosa cells. Cytogenetic evaluation revealed a 64,XX karyotype, SRY-negative. The stallion-like behavior subsided within days post-operatively. Long-term follow-up revealed the genitoplasty site healed without urine scalding or urethral stricture. The owner satisfaction was excellent and the horse could be used post-surgery as an athlete.


Asunto(s)
Trastornos del Desarrollo Sexual , Enfermedades de los Caballos , Femenino , Masculino , Caballos , Animales , Manejo de Caso , Trastornos del Desarrollo Sexual/genética , Trastornos del Desarrollo Sexual/cirugía , Trastornos del Desarrollo Sexual/veterinaria , Gónadas , Cariotipificación/veterinaria , Cariotipo , Enfermedades de los Caballos/cirugía
5.
AMA J Ethics ; 25(6): E437-445, 2023 06 01.
Artículo en Inglés | MEDLINE | ID: mdl-37285298

RESUMEN

Genital reconstructive surgeries (GRS) are available for a variety of indications and populations, including transgender and gender diverse (TGD) individuals and those with intersex traits/differences in sex development (I/dsd). Despite the common outcomes of GRS for TGD and I/dsd individuals, decision making about this surgical care differs between these populations and across the lifespan. Sociocultural perspectives on sexuality and gender dominate the ethics of GRS, and reform is needed within clinical ethics to center the autonomy of TGD and I/dsd individuals in informed consent processes. Such changes are necessary to ensure justice in health care for all sex and gender diverse individuals across the lifespan.


Asunto(s)
Trastornos del Desarrollo Sexual , Personas Transgénero , Transexualidad , Masculino , Femenino , Humanos , Trastornos del Desarrollo Sexual/cirugía , Identidad de Género , Genitales/cirugía , Toma de Decisiones
6.
Cad Saude Publica ; 39(1): e00066322, 2023.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-36753094

RESUMEN

Intersex children in Brazil are still subjected to "normalizing" surgical procedures and subsequent bodily interventions to make their bodies conform to binary views of sex. Resolution n. 1,664/2003 of the Brazilian Federal Council of Medicine legitimizes interventions upon intersex bodies, being the only national normative instrument that address the subject. However, the demands of international intersex political activism have denounced how early childhood interventions for sex designation mutilate children's bodies and violate a number of human rights. This research discusses how early, irreversible, and normalizing procedures performed without the intersex person's consent are human rights violations. Based on the concept of epistemic (in)justice, we first look at the disputes surrounding the evidence that underpin medical practices. We demonstrate how such procedures violate human rights to health, body integrity, autonomy, and sexual and reproductive rights, analyzing which strategies were put into place to prevent them. We propose that intersex people be at the center of decisions regarding their bodies, that non-surgical paths be discussed with patients and their family members, and that early, invasive, mutilating, harmful, cosmetic, and unconsented surgical interventions on intersex children be prohibited. Guiding tools must introduce changes into its regulatory bias to, from an interdisciplinary perspective, include bioethical and human rights bodies, as well as intersex activists.


No Brasil, crianças intersexo ainda são submetidas a procedimentos para designação de sexo binário no nascimento e a intervenções corporais subsequentes. A Resolução nº 1.664/2003, do Conselho Federal de Medicina, legitima intervenções sobre as corporalidades intersexo, se constituindo como o único instrumento normativo nacional que trata sobre o tema. No entanto, as demandas advindas do ativismo político internacional intersexo vêm expondo o quanto as intervenções precoces na infância para a designação de um sexo binário mutilam os corpos das crianças e violam uma série de direitos humanos. Esta pesquisa visa identificar como os procedimentos precoces, irreversíveis e normalizadores, realizados sem o consentimento da pessoa intersexo, revelam-se violadores de direitos humanos. Sob as lentes do conceito de (in)justiça epistêmica, partimos das disputas em torno da produção de evidências que embasam as práticas médicas. Demonstramos como esses procedimentos violam os direitos humanos à saúde, à integridade corporal e à autonomia e os direitos sexuais e reprodutivos, e analisamos quais têm sido as estratégias para evitar essas violações. Propomos que pessoas intersexo estejam no centro das decisões sobre o próprio corpo e que sejam debatidos, junto a pacientes e familiares, caminhos não cirúrgicos e proibidas intervenções precoces, invasivas, mutilatórias, prejudiciais, cosméticas e não consentidas nos corpos de crianças intersexo. A proposição de mudanças em instrumentos norteadores que deixem de regular esses corpos é necessária para, a partir de uma perspectiva interdisciplinar, incluir instâncias bioéticas e de direitos humanos, assim como pessoas do ativismo político intersexo.


En Brasil, los niños intersexuales todavía están sujetos a procedimientos de asignación de sexo binario al nacer y a intervenciones corporales posteriores. La Resolución nº 1.664/2003, del Consejo Federal de Medicina, asegura las intervenciones sobre corporalidades intersexuales y es el único instrumento normativo nacional sobre el tema. Sin embargo, las demandas que surgieron desde el activismo político internacional intersexual plantean cómo las intervenciones tempranas en la infancia para la asignación de género binario mutilan el cuerpo de los niños y vulneran una serie de derechos humanos. Esta investigación tiene por objetivo identificar cómo los procedimientos tempranos, irreversibles y normalizadores, realizados sin el consentimiento de la persona intersexual producen violadores de los derechos humanos. Con base en el concepto de (in)justicia epistémica, partimos de las disputas en torno a la producción de evidencia que subyace a las prácticas médicas. Demostramos cómo estos procedimientos vulneran los derechos humanos a la salud, la integridad y autonomía corporales, y los derechos sexuales y reproductivos, además, analizamos qué estrategias se han utilizado para evitarlos. Debatimos que las personas intersexuales deben estar en el centro de las decisiones sobre sus propios cuerpos y que se discutan con pacientes y familiares formas no quirúrgicas e intervenciones tempranas, invasivas, mutiladoras, dañinas, cosméticas y no consensuales en los cuerpos de los niños intersexuales. Los cambios en los instrumentos rectores para que dejen de regular estos cuerpos son necesarios para que, desde una perspectiva interdisciplinaria, se incluyan instancias de bioética y derechos humanos, así como a personas del activismo político intersexual.


Asunto(s)
Trastornos del Desarrollo Sexual , Preescolar , Humanos , Niño , Brasil , Trastornos del Desarrollo Sexual/cirugía , Derechos Humanos , Justicia Social
7.
J Pediatr Endocrinol Metab ; 36(3): 234-241, 2023 Mar 28.
Artículo en Inglés | MEDLINE | ID: mdl-36630604

RESUMEN

Intersex/Disorders/Differences of sex development conditions have been recognized for millennia. An organized approach was adopted in the 1960-70s using the philosophy that gender identity was fluid and malleable. Consequences of this approach were the lack of disclosure, stigmatization, and excessive surgery to "normalize" the genitalia. Often this led to quality of life issues for those patients. There have been many modifications in approach since then to avoid the problems noted. There is consensus on many of these changes (e.g. disclosure) but continued controversy on others (e.g. the benefits of early surgery). This review summarizes the historical context and the current areas of consensus and controversy.


Asunto(s)
Trastornos del Desarrollo Sexual , Identidad de Género , Humanos , Masculino , Femenino , Calidad de Vida , Trastornos del Desarrollo Sexual/cirugía , Consenso , Genitales
10.
Horm Res Paediatr ; 96(2): 228-237, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-35691284

RESUMEN

Informed decision-making and considerations of the child's best interest offer a starting place for building informed and lifelong discussions that promote the long-term interests and the well-being of individuals experiencing differences in sex development (DSD) or intersex traits. Parents require sufficient information and support to understand what "doing something and doing nothing" really means when learning about nonsurgical options. This may take the form of health literacy support, asking how parents are given access to meaningful and understandable information, as well as psychosocial support and psychological care. Timely psychological assessment and interventions that support informed decision-making actions are an essential aspect of holistic care for children and youth with DSD and their parents. Without actionable tools or approaches, parents cannot make informed decisions about their child's health and, as such, health literacy is a key attribute to aid decision making for both parents and children. As individuals with DSD become increasingly adept at building resourcefulness and gathering and applying knowledge about their bodies, limiting irreversible surgeries in childhood can afford wider life choices. To this end, an educated and informed comprehensive and helpful multidisciplinary group understands and embodies, as a whole team, the need for compassionate, emotionally supportive, and validating care in all interactions with parents of children and individuals with DSD. The paper draws on the primary author's experiences working with the charity, dsdfamilies, concluding with actionable approaches that include supporting personal knowledge through health literacy, examining team-based psychological care, and psychosocial approaches across the lifespan.


Asunto(s)
Trastornos del Desarrollo Sexual , Padres , Niño , Adolescente , Humanos , Padres/psicología , Trastornos del Desarrollo Sexual/cirugía , Trastornos del Desarrollo Sexual/psicología , Desarrollo Sexual
11.
Int J Impot Res ; 35(1): 72-77, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-35031713

RESUMEN

Surgical interventions on infants with intersex characteristics are considered justified by some on the grounds that they carry a high risk of intolerable stigma. However, public understanding of intersex and its medicalization are under-researched. We review recent qualitative and quantitative studies of the understandings of intersex and its medicalization among people who have no particular professional or public experience of intersex. First, such laypeople reason about clinical dilemmas by drawing on values in similar ways as expert healthcare professionals do. Second, laypeople can over-estimate the utility of current 'umbrella terms,' including intersex, for people with direct familial experience of intersex. Third, beliefs about good and bad effects of medical intervention are affected by framing intersex as either a medical condition or the natural basis for a social identity. Fourth, sexual identity is the best evidenced predictor of opinions about early surgical intervention and its legal limitation on human rights grounds. We argue that possible stigmatizing reactions from the public may not be a solid basis on which to justify early surgical intervention on intersex characteristics.


Asunto(s)
Trastornos del Desarrollo Sexual , Humanos , Trastornos del Desarrollo Sexual/cirugía , Identidad de Género
12.
J Relig Health ; 62(2): 797-818, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36070127

RESUMEN

Management of newborns with disorders of sex development (DSD), especially in deciding the need for a sex assignment surgery, is a complex matter. It is associated with many bioethical issues, such as concerns about the rights and welfare of the newborns and the reliability of parents' consent to the paternalistic disposition of physicians in making the best decisions. This paper, containing interviews with six medical experts and three religious' experts, aims to raise awareness of the multidisciplinary approach, which uses a combination of medicine, religion, and ethics in managing children with DSD, particularly in Malaysia, to avoid unnecessary psychological, biological, emotional, and societal ramifications.


Asunto(s)
Trastornos del Desarrollo Sexual , Humanos , Niño , Recién Nacido , Malasia , Reproducibilidad de los Resultados , Trastornos del Desarrollo Sexual/psicología , Trastornos del Desarrollo Sexual/cirugía , Discusiones Bioéticas , Islamismo , Religión y Medicina
13.
J Pediatr Urol ; 18(4): 492.e1-492.e8, 2022 08.
Artículo en Inglés | MEDLINE | ID: mdl-35668006

RESUMEN

BACKGROUND: SRD5A2 deficiency leads to incomplete masculinization of individuals with a 46 XY karyotype. A definitive diagnosis in early infancy facilitates decisions concerning choice of sex of rearing and management. AIM: To review the clinical presentation, diagnosis, treatment and outcome of children with 46 XY DSD due to SRD5A2 deficiency at a Paediatric Gender Clinic. STUDY DESIGN AND METHODS: Retrospective review of cases of SRD5A2 deficiency (2000-15) managed with a standard protocol at a multidisciplinary clinic. Demographic data, clinical presentation, physical findings, investigations (hormonal profile, imaging, genitoscopy), psychological evaluation (child, family), medical and surgical management, outcome and follow up were collated and analyzed. RESULTS: There were 12 cases aged 3 days-14 years at presentation, 3 had parental consanguinity. Eight were reared as males and 4 as females. Specialist referral was sought for hypospadias (5), atypical genitalia (5) or incongruent pubertal masculinization (2). All had chordee, symmetrical inguinoscrotal gonads, rugose labioscrotum and proximal hypospadias (perineoscrotal -9, perineal -3). Both pubertal cases had significant masculinization and no gynecomastia. The median testosterone/dihydrotestosterone ratio was 22.1(IQR-8.6-55.7). Despite a classical phenotype, four (2 prepubertal, 2 pubertal) had a ratio <10. Genitoscopy showed urogenital sinus remnant (4) and hypoplastic verumontanum (5). Sex reassignment was done in 4. Surgical management was staged and completed by 4 years in those with infantile presentation. Besides correction of chordee and urethroplasty in 11, other procedures included orchidopexy (5), excision of a urogenital sinus remnant (4) and correction of penoscrotal transposition (4). The urethroplasty was single staged in 3. All operated cases were followed up (mean age at last follow up - 10.63 years, mean follow up period - 7.25 years). The overall cosmetic result was satisfactory, but the phallic structure remained relatively small across prepubertal period. Uroflowmetry curves were normal in 9. All showed penile tumescence/erection and two peripubertal cases had typical secondary sexual characters. All cases, including those with sex reassignment, have a well-adjusted male psyche. DISCUSSION AND CONCLUSION: The diagnosis, management and longitudinal follow up of cases of SRD5A2 deficiency at a multidisciplinary gender clinic is presented. Diagnostic dilemmas with low T/DHT ratios remained in a third of cases. Most were diagnosed in infancy and assigned a male sex of rearing, all underwent staged masculinizing genitoplasty. Those with sex reassignment also fared well with comprehensive management after family counseling.


Asunto(s)
Trastornos del Desarrollo Sexual , Hipospadias , Humanos , Femenino , Masculino , Hipospadias/genética , Desarrollo Sexual , Trastornos del Desarrollo Sexual/diagnóstico , Trastornos del Desarrollo Sexual/genética , Trastornos del Desarrollo Sexual/cirugía , Identidad de Género , Pene , Virilismo , Proteínas de la Membrana , 3-Oxo-5-alfa-Esteroide 4-Deshidrogenasa/genética
14.
Hist Philos Life Sci ; 44(2): 28, 2022 Jun 08.
Artículo en Inglés | MEDLINE | ID: mdl-35674937

RESUMEN

The history of the field of intersex bodies/bodies with variations of sex development (VSD) reflects the ongoing tension between sociomedical attempts to control uncertainty and reduce the duration of corporeal uncertainty by means of early diagnosis and treatment, and the embodied subjects who resist or challenge these attempts, which ultimately increase uncertainty. Based on various qualitative studies in the field of intersex, this article describes three temporal sociomedical approaches that have evolved over the last decade and aims to address the uncertainty surrounding intersex/VSD bodies. These approaches are (1) the corrective-concealing approach, which includes early surgeries and hormone therapies intended to "correct" intersex conditions and the deliberate concealment of the ambiguity and uncertainty associated with intersex conditions; (2) the preventive approach, which involves early genetic diagnostic methods aimed at regulating or preventing the recurrence of hereditary conditions under the umbrella of VSD; and (3) the wait-and-see approach, which perceives intersex bodies as natural variations and encourages parents to take time, wait, and give their children the right to bodily autonomy. A comparison of these approaches from biopolitical, phenomenological, and pragmatic perspectives reveals that time is an essential social agent in addressing and controlling uncertainty, a gatekeeper of social norms and social and physical orders, and, on the other hand, a sociopolitical agent that enables creative social change.


Asunto(s)
Trastornos del Desarrollo Sexual , Niño , Trastornos del Desarrollo Sexual/diagnóstico , Trastornos del Desarrollo Sexual/cirugía , Técnicas Genéticas , Humanos , Cambio Social
15.
Vet Med Sci ; 8(3): 953-958, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-35156328

RESUMEN

INTRODUCTION: Hermaphroditism is less frequently reported in dogs and is often associated with infertility. CASE REPORT: An 8-month-old French bulldog weighing 5 kg with an enlarged structure protruding from the vulva was clinically diagnosed with hermaphroditism. Physical, hormonal assay, computed tomography, and gross and histological studies were done in addition to successfully detailed surgical correction. On physical examination, the dog showed the presence of an os-clitoris protruded from the vulvar labia. Hormonal levels of estradiol, testosterone, and progesterone were 6.39 pg/ml, 0.4 ng/ml, and 8.67ng/ml, respectively. Surgical removal of internal gonadal tissues and os clirectomy operations were conducted after the exploratory laparotomy. The removed gonadal tissues were identical to that of a female with testicles instead of ovaries, according to a gross examination. Histological examination confirmed gonads as testis, with inactive seminiferous tubules and epididymis attached to uterine horns. CONCLUSION: The congenital anomalies in the present case were diagnosed as male pseudohermaphroditism (MPH). Surgical correction was performed, and the owner's satisfaction was achieved.


Asunto(s)
Trastorno del Desarrollo Sexual 46,XY , Trastornos del Desarrollo Sexual , Enfermedades de los Perros , Animales , Clítoris/anomalías , Clítoris/patología , Clítoris/cirugía , Trastorno del Desarrollo Sexual 46,XY/veterinaria , Trastornos del Desarrollo Sexual/diagnóstico , Trastornos del Desarrollo Sexual/cirugía , Trastornos del Desarrollo Sexual/veterinaria , Enfermedades de los Perros/diagnóstico , Enfermedades de los Perros/patología , Enfermedades de los Perros/cirugía , Perros , Estradiol , Femenino , Masculino , Testículo
16.
BMJ Case Rep ; 15(2)2022 Feb 08.
Artículo en Inglés | MEDLINE | ID: mdl-35135794

RESUMEN

A young adult patient with 46XX congenital adrenal hyperplasia (CAH) presented with recurrent painful haematuria. CAH was diagnosed at birth following ambiguous genitalia. Hormonal treatment was started, female gender was assigned and feminising genitoplasty was planned, however the patient was lost to follow-up. Gender dysphoria started to occur during childhood which prompted the family to raise the patient as a boy. He eventually identified himself as a male. Examination revealed a male phenotype with severely virilised genitalia. Imaging studies confirmed the presence of uterus with low confluent urogenital sinus. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed, and the troublesome symptoms were cured.


Asunto(s)
Hiperplasia Suprarrenal Congénita , Trastornos del Desarrollo Sexual , Hiperplasia Suprarrenal Congénita/complicaciones , Hiperplasia Suprarrenal Congénita/cirugía , Trastornos del Desarrollo Sexual/complicaciones , Trastornos del Desarrollo Sexual/cirugía , Femenino , Genitales , Genitales Femeninos , Humanos , Histerectomía , Masculino
18.
BJU Int ; 129(3): 394-405, 2022 03.
Artículo en Inglés | MEDLINE | ID: mdl-33587786

RESUMEN

OBJECTIVES: To report the long-term follow-up outcomes of masculinizing surgery in disorders/differences of sex development (DSD), including both physicians' and patients' perspectives on appearance and functional outcome, including sexuality. PATIENTS AND METHODS: In total, 1040 adolescents (age ≥16 years) and adults with a DSD took part in this multicentre cross-sectional clinical study in six European countries in 2014/2015. Of those, 150 living in other than the female gender had some kind of masculinizing surgery: hypospadias repair, orchidopexy, breast reduction and/or gonadectomy. The study protocol included medical data collection, an optional genital examination, and patient-reported outcomes including satisfaction with appearance and current sexual functioning. RESULTS: Diagnoses included partial and mixed gonadal dysgenesis (45,XO/46,XY; n = 38), Klinefelter syndrome/46,XX males (n = 57), and various 46,XY DSDs (n = 42; e.g. partial androgen insensitivity syndrome, severe hypospadias) and 13 with other diagnoses. Of the participants, 84 underwent hypospadias surgery, 86 orchidopexy, 52 gonadectomy and 32 breast reduction (combinations possible). Physicians evaluated anatomical appearance at genital examination as poor in approximately 11% of patients. After hypospadias surgery, 38% of participants reported that they were (very) dissatisfied with anatomical appearance and 20% with function. The physician and patient evaluations were moderately correlated (r = 0.43). CONCLUSION: The majority of participants were neutral to satisfied with the appearance and function in the long-term after masculinizing surgery. Given the initial severe phenotype and a risk of unsatisfactory results after masculinizing surgery in DSD, treatment should be handled by experienced multidisciplinary teams in order to optimize the postoperative results.


Asunto(s)
Trastornos del Desarrollo Sexual , Hipospadias , Adolescente , Estudios Transversales , Trastornos del Desarrollo Sexual/genética , Trastornos del Desarrollo Sexual/cirugía , Femenino , Humanos , Hipospadias/cirugía , Masculino , Medición de Resultados Informados por el Paciente , Desarrollo Sexual
19.
Semin Pediatr Surg ; 30(4): 151078, 2021 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-34412885

RESUMEN

Differences/disorders of sex development (DSD) are a heterogeneous group of congenital conditions in which the development of chromosomal, gonadal, or anatomical sex is atypical. Patients usually present during the newborn period but occasionally some cases remain unrecognized until later in infancy or even adolescence. Genital appearance, psychosocial support, sociocultural background, gender identity development, and genetic and biochemical analysis in addition to ethical and legal implications need to be considered when deciding on the appropriate treatment strategy. Surgeons are important members of the multidisciplinary expert teams involved in the initial approach and long-term follow-up. Surgical care of DSD patients is one of the main challenges. Recommendations regarding the opportunity and timing of surgical procedures are still under discussion. Surgical procedures are aimed to reduce urologic problems, prevent the risk of gonadal germ-cell cancer, and facilitate sexual function and reproduction. Providing its excellent visualization, access to pelvic structures and less postoperative adhesion MIS has been an important tool in the diagnosis and treatment of DSD. The role of MIS will be summarized in: 1) Gonadal biopsy / gonadectomy 2) Treatment of urogenital sinus/vaginoplasty 3) Vaginal Replacement 4) Resection / treatment of Mullerian structures.


Asunto(s)
Trastornos del Desarrollo Sexual , Neoplasias de Células Germinales y Embrionarias , Trastornos del Desarrollo Sexual/diagnóstico , Trastornos del Desarrollo Sexual/cirugía , Femenino , Identidad de Género , Humanos , Masculino , Procedimientos Quirúrgicos Mínimamente Invasivos , Desarrollo Sexual
20.
J Sex Med ; 18(10): 1797-1806, 2021 10.
Artículo en Inglés | MEDLINE | ID: mdl-34419416

RESUMEN

BACKGROUND: Secondary vaginal stenosis may occur after reconstruction of genital malformations in childhood or after failed vaginal aplasia repair in adults. AIM: This study focusses on the results of the surgical treatment of these patients in our multidisciplinary transitional disorders/differences of sex development team of pediatric surgeons and gynecologists. METHODS: A retrospective analysis was carried out on adult and female identified disorders/differences of sex development patients with vaginal stenoses treated between 2015 and 2018 in a single center with revision vaginoplasty. The underlying type of malformation, the number and surgical techniques of vaginoplasties in infancy, techniques of revision of the stenotic vagina, vaginal length and caliber, possibility of sexual intercourse, and temporary vaginal dilatation. A review of literature with regard to recommended surgical techniques of revision vaginoplasties was accomplished. OUTCOMES: To describe the surgical technique, the main outcome measures of this study are vaginal calipers after revision vaginoplasty as well as ability for sexual intercourse. RESULTS: Thirteen patients presented with vaginal stenosis with a median age of 19 years (range 16-31). All patients had one or more different types of vaginoplasties in their medical history, with a median age at first vaginoplasty of 15 months (0-233). Underlying anatomical conditions were urogenital sinus (n = 8), vaginal agenesis (n = 2), persistent cloacae (n = 2), and cloacal exstrophy (n = 1). The main symptoms were disability of sexual intercourse in 13 patients due to stenotic vaginal tissue. The most frequently performed surgical technique was partial urogenital mobilization with a perineal or lateral flaps (n = 10), followed by bowel vaginoplasty (n = 2), in 1 patient a revision vaginoplasty failed due to special anatomical conditions. In a median follow-up of 11 months, all but one patient presented with physiological vaginal length and width, and normal sexual intercourse in those with a partnership. CLINICAL IMPLICATIONS: Perineal flap with partial urogenital mobilization should be considered as a treatment of choice in severe cases of distal vaginal stenosis and after multiple failed former vaginoplasties, while bowel vaginoplasty should be reserved only for cases of complete cicatrization or high located stenosis of the vagina. STRENGTHS & LIMITATIONS: The strength of this study is the detailed description of several cases while the retrospective character is a limitation. CONCLUSION: In patients after feminizing genital repair, perineal flap with partial urogenital mobilization provides a normal anatomical outcome and allows unproblematic sexual intercourse. Ellerkamp V, Rall KK, Schaefer J, et al. Surgical Therapy After Failed Feminizing Genitoplasty in Young Adults With Disorders of Sex Development: Retrospective Analysis and Review of the Literature. J Sex Med 2021;18:1797-1806.


Asunto(s)
Coito , Trastornos del Desarrollo Sexual , Adolescente , Adulto , Niño , Constricción Patológica , Trastornos del Desarrollo Sexual/cirugía , Femenino , Procedimientos Quirúrgicos Ginecológicos , Humanos , Estudios Retrospectivos , Vagina/cirugía , Adulto Joven
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA
...