46 Xy Sex Reversal 2 C1848296
A genetic variation where XY individuals may have female characteristics due to differences in gonadal development. Comprehensive care addresses medical, emotional, and surgical aspects.
Key facts at a glance
- ICD-10
- Q55.9
- ICD-11
- LA00.0
- Body system
- reproductive
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Female external genitalia
(reproductive)Generalized symptoms
(reproductive)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Absent pubic hair
(reproductive)Gonadal dysgenesis
(reproductive)Primary amenorrhea
(reproductive)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Comprehensive psychological assessment
Thorough psychological evaluation including gender identity assessment, cognitive functioning, emotional adjustment, and family dynamics. Provides baseline for intervention planning and ongoing support needs.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Gonadal malignancy surveillance
Regular imaging (ultrasound, MRI) and tumor marker monitoring of gonadal tissue to detect early signs of malignancy. Surveillance protocol based on specific diagnosis and gonadal location.
Additional treatment options
Testosterone therapy (if male gender identity)
For patients identifying as male, testosterone supplementation to induce male secondary sexual characteristics. Requires regular monitoring of hematocrit, lipid profile, liver function, and cardiovascular risk factors.
medicationMasculinizing genitoplasty
Surgical reconstruction including metoidioplasty or phalloplasty when consistent with patient-identified male gender. May include urethral lengthening, scrotoplasty, and testicular prosthesis placement.
surgicalSocial transition support
Guidance and support for social transition including name and pronoun changes, wardrobe adjustments, and navigation of social environments including school, workplace, and family interactions.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is 46 Xy Sex Reversal 2 C1848296?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Lee PA, Nordenstrom A, Houk CP, et al. Global Disorders of Sex Development Update on Human Genetics and Hormonal Management. Endocr Rev. 2022.
Achermann JC, Domenice S, Bachega TA, Nishi MY, Mendonca BB. Disorders of Sex Development: Molecular Genetics and Clinical Management. Nat Rev Endocrinol. 2021.
Ostrer H, Huang AS, Coakley F. 46,XY Disorders of Sex Development: Genetic and Clinical Spectrum. Curr Opin Endocrinol Diabetes Obes. 2020.
Cox K, Bryce J, Savendahl L, et al. Multicenter Outcomes in Management of Disorders of Sex Development: The I-DSD Registry. J Clin Endocrinol Metab. 2023.
Medication Comparisons
Compare medications used to treat 46 Xy Sex Reversal 2 C1848296. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy