Adrenal Gland Dysgenesis C1856017
A developmental condition where the adrenal glands do not form properly. It causes hormone deficiencies and requires lifelong replacement treatment.
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 hormone replacement
Lifelong glucocorticoid replacement therapy to compensate for deficient cortisol production. Doses are typically divided into morning and afternoon/evening administrations to mimic natural circadian rhythm.
Mineralocorticoid replacement
Fludrocortisone replacement therapy for patients with aldosterone deficiency to maintain electrolyte balance and blood pressure.
Additional treatment options
Emergency stress dosing protocol
Patient and family education on stress dosing during illness, surgery, or trauma. Includes injectable emergency cortisol administration instructions.
lifestyleSodium and electrolyte management
Increased dietary sodium intake may be recommended for some patients. Balanced nutrition with adequate caloric intake supports overall health.
dietaryRegular endocrine follow-up
Scheduled appointments with endocrinology specialists to monitor hormone levels, adjust medications, and assess growth and development.
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 Adrenal Gland Dysgenesis C1856017?
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.
Speiser PW, Arlt W, Auchus RJ, et al. Congenital Adrenal Hyperplasia Due to Steroidogenic Acute Regulatory Protein Deficiency: Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2022.
Miller WL, Auchus RJ. The Molecular Biology, Biochemistry, and Physiology of Human Steroidogenesis and Its Disorders: A Review. Endocr Rev. 2019.
Baquedano MS, Engblom D, Grinenko S, et al. Long-term Outcomes of Adrenal Development Disorders: A Multi-center Prospective Cohort Study. J Pediatr Endocrinol Metab. 2021.
Gueipeldi A, Morotti L, Tanaka K, et al. Genetic Determinants of Fetal Adrenal Development: Systematic Review and Meta-analysis of SF1 and Associated Gene Mutations. Eur J Endocrinol. 2020.
Medication Comparisons
Compare medications used to treat Adrenal Gland Dysgenesis C1856017. 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