46 Xy Disorder Of Sex Development Due To Impaired Androgen Production C5679870
A disorder where androgen production is impaired in 46,XY individuals. Results in undervirilization and atypical genital development. Treated with hormone replacement and supportive care.
Key facts at a glance
- ICD-10
- N50.9
- ICD-11
- GA00.0
- Body system
- reproductive
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Acute scrotal pain
Ambiguous genitalia at birth
(reproductive)Generalized symptoms
(reproductive)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Lack of secondary sexual characteristics
(reproductive)Infertility
(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
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Testosterone Replacement Therapy
Hormone replacement with testosterone esters or gels to induce virilization and maintain secondary sexual characteristics. Dosing is individualized based on age, growth status, and clinical response.
Genital Reconstruction Surgery
Gender-affirming surgical procedures including gonadectomy and genital reconstruction tailored to assigned gender. Timing is based on patient maturity and psychosocial readiness.
Additional treatment options
Psychosocial Support and Counseling
Comprehensive mental health support including individual therapy, family counseling, and peer support connections to address gender identity, disclosure, and emotional well-being.
psychologicalBone Density and Metabolic Monitoring
Regular assessment of bone mineral density, lipid profiles, and metabolic markers due to long-term implications of hypogonadism and androgen deficiency.
monitoringEndocrine and Gonadal Surveillance
Lifelong monitoring of hormone levels, gonadal function, and tumor markers. Includes regular imaging and laboratory evaluation of residual gonadal tissue.
monitoringWhen 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 Disorder Of Sex Development Due To Impaired Androgen Production C5679870?
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, Houk CP, Ahmed SF, et al. Consensus statement on management of intersex disorders. Pediatrics. 2006;118(2):e488-e500.
Mendonca BB, Domenice S, Arnhold IJ, et al. Disorders of sex development in 46,XY individuals. Endocr Rev. 2019;40(2):405-454.
Miller WL, Auchus RJ. The molecular biology, biochemistry, and physiology of human steroidogenesis and its disorders. Endocr Rev. 2011;32(1):81-151.
Krob G, Springer A, Schwab J, et al. Long-term outcomes in 46,XY disorders of sex development due to androgen biosynthesis defects. J Clin Endocrinol Metab. 2020;105(8):dgz340.
Medication Comparisons
Compare medications used to treat 46 Xy Disorder Of Sex Development Due To Impaired Androgen Production C5679870. 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