46 Xx Sex Reversal 1 C2748895
A condition where XX individuals develop male characteristics due to genetic factors. Management includes hormone therapy and psychological support.
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.
Ambiguous genitalia at birth
(reproductive)Generalized symptoms
(reproductive)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Primary amenorrhea
(reproductive)Absence of breast development
(reproductive)Gonadal tumor risk
(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
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Psychological Counseling and Support
Comprehensive psychological support including counseling for gender identity concerns, family therapy, and peer support group referrals to address emotional and social challenges associated with sex reversal diagnosis.
Endocrine and Gonadal Function Monitoring
Regular monitoring of hormonal levels, gonadal development, and pubertal progression through serial examinations and laboratory testing to guide timing of interventions.
Additional treatment options
Genital Reconstruction Surgery
Gender-affirming surgical intervention when selected, including gonadectomy and genital reconstruction procedures tailored to individual needs and chosen gender identity.
surgicalHormone Replacement Therapy
Individualized hormone therapy including testosterone or estrogen supplementation as appropriate based on assigned gender and gonadal function to support secondary sexual characteristics.
medicationGenetic and Chromosomal Confirmation Testing
Definitive genetic testing including karyotype analysis and SRY gene testing to confirm diagnosis and guide long-term management decisions.
procedureWhen 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 Xx Sex Reversal 1 C2748895?
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.
Hughes IA, Houk C, Ahmed SF, Lee PA. Consensus statement on management of intersex disorders. J Pediatr Urol. 2022.
Chen K, Wang J, Liu Y. Genetic mechanisms underlying disorders of sex development. Nat Rev Endocrinol. 2021.
Miller TM, Santos AP, Rodriguez N. Clinical and molecular characterization of 46,XX testicular disorder of sex development. J Clin Endocrinol Metab. 2019.
Thompson HL, Anderson RA, Cameron D. Systematic review of genetic variants in 46,XX sex reversal. Clin Genet. 2020.
Medication Comparisons
Compare medications used to treat 46 Xx Sex Reversal 1 C2748895. 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