46 Xx Disorder Of Sex Development Induced By Endogenous Maternal Derived Androgen C5679875
A condition where hormones from the mother cross the placenta and cause atypical development in an XX fetus. It is present at birth. Management involves pediatric specialists and may include hormone therapy.
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.
Ambiguous genitalia
(reproductive)Generalized symptoms
(reproductive)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Clitoral hypertrophy
(reproductive)Labial fusion
(reproductive)Early pubarche
(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
Prenatal Androgen Monitoring
Careful monitoring during pregnancy when maternal androgen excess is identified. Includes fetal monitoring and maternal hormone level tracking.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Maternal Androgen Suppression
Carefully managed anti-androgen therapy during pregnancy when appropriate, balancing maternal and fetal benefits. Requires specialist oversight.
Additional treatment options
Long-term Pediatric Endocrine Follow-up
Ongoing assessment of growth, development, and hormonal function throughout childhood and adolescence to address any lingering effects of prenatal androgen exposure.
monitoringDevelopmental and Family Psychological Support
Support for developmental milestones, school adjustment, and family understanding of the condition. Addresses questions about fertility and long-term outcomes.
psychologicalCorrective Surgery if Indicated
Surgical intervention for anatomical variations present at birth, typically performed when consistent with family wishes and in centers experienced with DSD.
surgicalWhen 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 Disorder Of Sex Development Induced By Endogenous Maternal Derived Androgen C5679875?
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, Nihoul-Fékété C, Audi L. Management of 46,XX disorders of sex development: Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2020.
Pache TD, de Jong FH, Fauser BC. Androgen excess in pregnancy: Fetal programming and 46,XX sexual development. Endocr Rev. 2018.
Sultan C, Gaspari L, Maimoun L. Outcomes of prenatal androgen exposure treatment in 46,XX DSD patients: A systematic review and meta-analysis. Horm Res Paediatr. 2022.
Lee PA, Houk CP, Ahmed SF. Consensus statement on management of intersex disorders: 46,XX DSD with maternal androgen excess. Pediatrics. 2019.
Medication Comparisons
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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 25, 2026. · Read our editorial policy