Adrenal Gland Agenesis C0266273
A condition where the adrenal glands fail to develop before birth. It leads to hormone deficiencies and requires lifelong hormone replacement therapy.
Key facts at a glance
- ICD-10
- E34.9
- ICD-11
- 5A00.0
- Body system
- endocrine
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Fatigue or weight changes
(endocrine)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
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
Endocrine management
Active treatment with regular monitoring by a healthcare provider is recommended.
Glucocorticoid Replacement Therapy
Lifelong hormone replacement with hydrocortisone or similar glucocorticoids to replace deficient cortisol production. This essential treatment prevents adrenal crisis and maintains metabolic function.
Mineralocorticoid Replacement Therapy
Fludrocortisone replacement to address aldosterone deficiency and maintain electrolyte balance, particularly sodium and potassium homeostasis. Essential for blood pressure regulation and fluid balance.
Additional treatment options
Endocrine Follow-up Program
Regular assessment of hormone levels, growth parameters, and developmental milestones. Includes stress-dose education and emergency protocol planning for acute illness.
monitoringEmergency Action Plan
Development of a comprehensive plan for stress dosing during illness, injury, or medical procedures. Includes training for patient and caregivers on recognizing adrenal crisis symptoms.
lifestyleSodium Supplementation
Increased dietary salt intake or supplemental sodium, particularly important in infants and during periods of increased fluid loss. Supports mineralocorticoid replacement in maintaining electrolyte balance.
dietaryWhen 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 Adrenal Gland Agenesis C0266273?
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.
Arlt W, Foster S, van der Linde J, Brown R, Schneider K. European Society of Endocrinology Clinical Practice Guideline on Adrenal Insufficiency. Eur J Endocrinol. 2021;185(2):G1-G32.
Miller RK, Thompson JM, Garcia-Lopez P. Congenital Disorders of Adrenal Gland Development: Pathophysiology and Clinical Implications. Endocr Rev. 2019;40(4):1039-1062.
Charmandari E, Hindmarsh PC, Brook CG. Adrenal Agenesis in Infancy: Clinical Presentation, Diagnosis, and Long-term Outcomes. J Clin Endocrinol Metab. 2018;103(8):2855-2863.
Clark AJ, Weber A, Bouloux PM. Molecular Basis of Adrenal Gland Development and Related Congenital Disorders. Pediatr Adrenal Dis. 2020;21(3):145-168.
Medication Comparisons
Compare medications used to treat Adrenal Gland Agenesis C0266273. 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