HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Ovotesticular Disorders of Sex Development

Conditions of sexual ambiguity in which the individual possesses gonadal tissues of both sexes, tissues from the OVARY and the TESTIS. There can be a testis on one side and an ovary on the other (lateral), or there may be combined ovarian and testicular tissue (ovotestes) on each side (bilateral). The karyotype may be 46,XX; 46,XY; or a mosaic of 46,XX/46,XY. These disorders have historically been called true hermaphroditism.
Also Known As:
46,XX Gonadal Dysgenesis, Complete, Sry-Positive; 46,XX True Hermaphroditism, Sry-Positive; Familial True Hermaphroditism; Familial XX True Hermaphroditism; Ovotesticular DSD; Ovotesticular Disorder Of Sex Development; True Hermaphroditism; XX Male Syndrome, Sry-Positive; DSD, Ovotesticular; DSDs, Ovotesticular; Familial True Hermaphroditisms; Hermaphroditism, Familial True; Hermaphroditisms, Familial True; Hermaphroditisms, True; Ovotesticular DSDs; True Hermaphroditism, Familial; True Hermaphroditisms; True Hermaphroditisms, Familial; XX Male Syndrome, Sry Positive; Hermaphroditism, True
Networked: 75 relevant articles (0 outcomes, 7 trials/studies)

Disease Context: Research Results

Related Diseases

1. Gonadal Dysgenesis (Gonadal Agenesis)
2. Disorders of Sex Development (Intersexuality)
3. Androgen-Insensitivity Syndrome (Testicular Feminization)
4. XX Disorders of Sex Development 46 (Female Pseudohermaphroditism)
5. Congenital Adrenal Hyperplasia (Hyperplasia, Congenital Adrenal)

Experts

1. Rey, Rodolfo A: 2 articles (01/2016 - 01/2014)
2. Hersmus, R: 2 articles (09/2010 - 05/2008)
3. Oosterhuis, J W: 2 articles (09/2010 - 05/2008)
4. Wolffenbuttel, K P: 2 articles (09/2010 - 05/2008)
5. Akira, Shigeo: 1 article (01/2022)
6. Ichikawa, Tomoko: 1 article (01/2022)
7. Kato, Ryoko: 1 article (01/2022)
8. Kuwabara, Yoshimitsu: 1 article (01/2022)
9. Matsuda, Shigeru: 1 article (01/2022)
10. Nakao, Kimihiko: 1 article (01/2022)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Ovotesticular Disorders of Sex Development:
1. H-Y AntigenIBA
2. EnzymesIBA
3. Testosterone (Sustanon)FDA Link
01/01/2017 - "In a neonate with genital ambiguity due to ovotesticular DSD, testicular AMH and testosterone production respectively appear to account for the initial arrest of ovarian development and subsequent rapid hyperfunction of the contralateral ovary after ovotestis removal.
."
07/01/1980 - "The blood plasma testosterone content was determined by radioimmunological method in patients with hirsutism, premature sexual maturation, false male hermaphroditism, true hermaphroditism, mixed gonad dysgenesis, adrenogenital syndrome, and also in some mothers of patients suffering from intersexualism and in healthy persons. "
10/01/2023 - "Testosterone replacement, commonly used for Klinefelter syndrome, androgen insensitivity syndrome, 5-alpha reductase deficiency, gonadal dysgenesis, or ovotesticular DSD, may cause acne."
01/01/2002 - "Sequencing of exons 2-8 of the androgen receptor after study of testosterone precursors following human chorionic gonadotrophin stimulation is recommended when gonadal dysgenesis and true hermaphroditism can be excluded."
01/01/2016 - "Serum AMH measurements are useful, together with testosterone determination, in the diagnosis of patients with ambiguous genitalia: both are low in patients with gonadal dysgenesis, including ovotesticular disorders of sex development, testosterone is low but AMH is in the normal male range or higher in patients with disorders of androgen synthesis, and both hormones are normal or high in patients with androgen insensitivity. "
4. Complement System Proteins (Complement)IBA
5. Androgen Receptors (Androgen Receptor)IBA
01/01/2002 - "Sequencing of exons 2-8 of the androgen receptor after study of testosterone precursors following human chorionic gonadotrophin stimulation is recommended when gonadal dysgenesis and true hermaphroditism can be excluded."
09/01/1997 - "Diagnoses included Drash syndrome with Wilms tumor in infancy (3 patients), partial androgen insensitivity resulting from androgen receptor mutations (2), true hermaphroditism (2), chromosomal aberration (1), deficiency of antimüllerian hormone (1), gonadal dysgenesis (1), partial 5 alpha-reductase deficiency caused by a novel point mutation (1), and XX-male syndrome (1). "
04/01/2019 - "Abbreviations: 5α-RD2: 5α-Reductase type 2; AIS: androgen insensitivity syndrome; AMH: antimullerian hormone; AMHR: antimullerian hormone receptor; AR: androgen receptor gene; CAH: congenital adrenal hyperplasia; CAIS: complete AIS; CAH: congenital adrenal hyperplasia; CHH: congenital hypogonadotropic hypogonadism; CXORF6: chromosome X open reading frame 6 gene; CYP19A1: cytochrome P450 family 19 subfamily A member 1 gene; DHT: dihydrotestosterone; DMRT1: double sex and mab-3 related transcription factor 1 gene; DSD: disorders of sexual development; GD: gonadal dysgenesis; HGMD: human gene mutation database; IH: isolated hypospadias; MAMLD1: mastermind like domain containing 1 gene; MIS: mullerian inhibiting substance; NTD: N-terminal domain; OT DSD: ovotesticular DSD; PAIS: partial AIS; SOX9: SRY-related HMG-box 9 gene; SRY: sex-determining region Y gene; STAR: steroidogenic acute regulatory protein gene; SRD5A2: steroid 5 alpha-reductase 2 gene; T DSD: testicular DSD; T: testosterone; WNT4: Wnt family member 4 gene; WT1: Wilms tumor 1 gene; Δ4: androstenedione."
6. Protein Isoforms (Isoforms)IBA
7. Nitric Oxide Synthase (NO Synthase)IBA
8. ImmunosorbentsIBA
9. N 30IBA
10. AndrogensIBA

Therapies and Procedures

1. Castration
2. Laparotomy
3. Orchiopexy
4. Hysterectomy
5. Hormone Replacement Therapy (Therapy, Hormone Replacement)