scholarly journals 46, XY DSD (Disorder of Sex Development) : Diagnosis dan Tatalaksananya.

2021 ◽  
Vol 37 (1) ◽  
pp. 26-35
Author(s):  
Mildi Felicia ◽  
Beto Suhartono
Keyword(s):  
Surgical Management ◽  
Neonatal Period ◽  
System Development ◽  
Disorders Of Sex Development ◽  
Genital Organ ◽  
Optimal Care ◽  
Disorder Of Sex Development ◽  
Sex Development ◽  
Pregnancy History ◽  
Complete Physical Examination

Disorder of Sex Development (DSD) is a congenital disorder that occurs in the development of chromosomes, gonads, and internal or external genital organ. DSD of 46 XY is a condition where the children with XY genotype is not able to have complete virilization of external genital. Determination phase is an initial phase of reproductive system development. Disruption of this phase may potentially lead to DSD.  Optimal care for children with Disorder of Sex Development requires a multidisciplinary team starting since neonatal period. Family and pregnancy history, complete physical examination and assessment of genital organ are the first step of ensuring the diagnose. Management of children with DSD are focusing on gender determination, hormone support therapy and surgical management. On the other hand, Children with XY genotype should be raised as a boy however if there is no responsive evidence in administration of androgen the children should be raised as a girl. Subsequent to prescribe the gender of the children, surgical management is a required treatment for removing unnecessary genital afterward. Keywords: Disorders of Sex Development, DSD, 46 XY DSD

scholarly journals Ovotesticular Disorder of Sex Development: An Unusual Presentation

2019 ◽  
Vol 9 ◽  
pp. 34 ◽  
Author(s):  
Meltem Özdemir ◽  
Rasime Pelin Kavak ◽  
Ihsan Yalcinkaya ◽  
Kursat Guresci
Keyword(s):  
Unusual Case ◽  
Disorders Of Sex Development ◽  
Ambiguous Genitalia ◽  
Normal Male ◽  
Genital Organ ◽  
Rare Form ◽  
Disorder Of Sex Development ◽  
Breast Enlargement ◽  
Sex Development ◽  
Bilateral Breast Enlargement

Disorder of sex development is an inclusive term that refers to any problem where the genital organ is atypical in relation to chromosomes or gonads. Ovotesticular disorder of sex development, which is formerly known as “true hermaphroditism,” is the most rare form among all disorders of sex development in humans. It is characterized by the simultaneous presence of both ovarian and testicular tissues in the same individual and characteristically presents with ambiguous genitalia in neonates or infants. Herein, we present an unusual case of a 19-year-old individual with phenotypically nearly normal male genitalia who presented with the complaint of bilateral breast enlargement.

scholarly journals THE ROLE OF UROLOGISTS IN MANAGEMENT OF DISORDERS OF SEX DEVELOPMENT

2012 ◽  
Vol 19 (1) ◽  
Author(s):  
Ilham Wahyudi ◽  
Irfan Wahyudi ◽  
Kanadi Sumadipradja ◽  
Jose RL Batubara ◽  
Arry Rodjani
Keyword(s):  
Multidisciplinary Team ◽  
Disorders Of Sex Development ◽  
Androgen Insensitivity Syndrome ◽  
Therapeutic Management ◽  
Gender Assignment ◽  
Disorder Of Sex Development ◽  
Sex Development ◽  
Diagnostic Management ◽  
One Year

Objective: To evaluate disorder of sex development (DSD) profile at Cipto Mangunkusumo Hospital (RSCM), the management profile, and the role of urologist on diagnostic and therapeutic management. Material & method: We retrospectively collected data from medical record of all DSD cases managed by pediatric endocrinologist, urologist, obstetric gynaecologist at RSCM from January 2002 up to December 2009. 2006 IICP criteria was used as classification. The management profile and the role of urologist were evaluated. Results: there were 133 DSD cases with the majority of cases was congenital adrenal hyperplasia (CAH) followed by androgen insensitivity syndrome (AIS). Most of the cases were diagnosed before one year old and other on pubertal period. Karyotyping, laboratory examination, ultrasonography, genitography, uretrocystoscopy, kolposcopy, diagnostic laparascopy were performed as diagnostic management. Gender assignment was performed by multidisciplinary team. Masculinizing surgery, feminizing surgery, and gonadectomy was done as therapeutic management. Conclusion: The majority case on RSCM’s DSD profile was CAH. The management was performed by multidisciplinary team. Gender assignment decision should be based upon thorough diagnostic evaluation. The urologist has important role on diagnostic and therapeutic management. Keywords: Disorder of sex development, diagnostic management, gender assignment, therapeutic management, urologist.

Gonadal dysgenesis in disorders of sex development: Diagnosis and surgical management

2016 ◽  
Vol 12 (6) ◽  
pp. 411-416 ◽  
Author(s):  
K.P. Wolffenbuttel ◽  
R. Hersmus ◽  
H. Stoop ◽  
K. Biermann ◽  
P. Hoebeke ◽  
...  
Keyword(s):  
Surgical Management ◽  
Gonadal Dysgenesis ◽  
Disorders Of Sex Development ◽  
Sex Development

scholarly journals First experience of a single-stage simultaneous single access endoscopic surgery in a child with a disorder of sex development

2013 ◽  
Vol 94 (2) ◽  
pp. 247-250
Author(s):  
N R Akramov ◽  
A K Zakirov
Keyword(s):  
Endoscopic Surgery ◽  
Abdominal Cavity ◽  
Disorders Of Sex Development ◽  
Point Of View ◽  
Single Stage ◽  
Service Needs ◽  
Disorder Of Sex Development ◽  
Single Access ◽  
Sex Development ◽  
Simultaneous Surgery

A clinical case and treatment of a disorder of sex development (46XX, ovotesticular type) who underwent a simultaneous surgery including diagnostic laparoscopy using the single access endoscopic surgery followed by gonadectomy and the single-stage feminizing genitoplasty, is described. Endocrinology service needs for determination of treatment approach in children with disorders of sex development are closely entwined with capabilities of endoscopic surgery, particularly laparoscopy. It’s necessary to quickly determine the gonadal structure due to the high risk of malignancies while examining these patients. The most useful method is the direct visualization of the gonads in the abdominal cavity by laparoscopy. In these cases the single access endoscopic surgery technique reduces the trauma and is welcomed in pediatrics. From our point of view, the preference for the patients with disorders of sex development management with expected gonadal dysgenesis must be given to a single access endoscopic surgery.

The ESPU/SPU standpoint on the surgical management of Disorders of Sex Development (DSD)

2014 ◽  
Vol 10 (1) ◽  
pp. 8-10 ◽  
Author(s):  
P. Mouriquand ◽  
A. Caldamone ◽  
P. Malone ◽  
J.D. Frank ◽  
P. Hoebeke
Keyword(s):  
Surgical Management ◽  
Disorders Of Sex Development ◽  
Sex Development

Surgical management of hypospadias in cases with concomitant disorders of sex development

2019 ◽  
Vol 35 (5) ◽  
pp. 611-617
Author(s):  
Takanori Ochi ◽  
Asuka Ishiyama ◽  
Yuta Yazaki ◽  
Hiroshi Murakami ◽  
Masahiro Takeda ◽  
...  
Keyword(s):  
Surgical Management ◽  
Disorders Of Sex Development ◽  
Sex Development

scholarly journals Disorders of Sex Development: Management of Gender Assignment in a Preterm Infant with Intrauterine Growth Restriction

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Lucy D. Mastrandrea ◽  
Christine H. Albini ◽  
Ralph J. Wynn ◽  
Saul P. Greenfield ◽  
Luther K. Robinson ◽  
...  
Keyword(s):  
Preterm Infant ◽  
Intrauterine Growth Restriction ◽  
Disorders Of Sex Development ◽  
Definitive Diagnosis ◽  
Growth Restriction ◽  
Gender Assignment ◽  
Disorder Of Sex Development ◽  
Intrauterine Growth ◽  
Sex Development ◽  
And Gender

We describe how a gender specialist team managed the case of a disorder of sex development in a preterm infant where definitive diagnosis and gender assignment were delayed due to complications of prematurity, anemia, and severe intrauterine growth restriction.

scholarly journals Rare variant of ovotesticular disorder of sex development diagnosed due to injury-induced rupture of the reproductive gland in a patient with SRY-negative 46,ХХ karyotype (clinical case)

2021 ◽  
Vol 21 (4) ◽  
pp. 98-102
Author(s):  
A. B. Okulov ◽  
E. A. Volodko ◽  
O. Yu. Latyshev ◽  
D. N. Godlevsky ◽  
E. V. Timokhovich ◽  
...  
Keyword(s):  
Rare Variant ◽  
Clinical Case ◽  
Disorders Of Sex Development ◽  
Emergency Operation ◽  
Ambiguous Genitalia ◽  
Traumatic Rupture ◽  
Disorder Of Sex Development ◽  
Sex Development ◽  
Derivatives Of ◽  
Reproductive Gland

The clinical case of a rare variant of disorder of sex development (DSD) is described. This disorder was diagnosed with an emergency operation for the traumatic rupture of the gonad. A patient (14 years old) with a male phenotype and lack of muller duct derivatives had a female SRY negative karyotype (46,XX) and an ovotesticular gonad structure as a result of duplication in the regulatory zone of the SOX9 gene. Ovotesticular disorders of sex development with karyotype 46,XX, as a rule, are accompanied by an ambiguous genitalia and derivatives of the muller structures. Early diagnosis of the described variant of DSD was difficult due to the development of male type genitalia. Timely identification of DSD including the presented option of DSD, is possible during routine examinations of the urologist with mandatory ultrasound examination of the scrotum and pelvis.

scholarly journals 46,XY ovotesticular disorders of sex development: A therapeutic challenge

2017 ◽  
Vol 9 (4) ◽  
Author(s):  
Maria-Grazia Scarpa ◽  
Massimo Di Grazia ◽  
Gianluca Tornese
Keyword(s):  
Multidisciplinary Approach ◽  
Disorders Of Sex Development ◽  
Consensus Meeting ◽  
Gender Assignment ◽  
Disorder Of Sex Development ◽  
Therapeutic Challenge ◽  
Sex Development ◽  
Patient Gender

46,XY ovotesticular disorder of sex development is extremely rare and indicates the presence of both testis and ovary in the same patient. Gender assignment in newborns represents a therapeutic challenge. We describe and comment on our multidisciplinary approach, ten years after the Chicago consensus meeting on disorder of sex development.

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