Adrenocortical Hypoplasia C4024719
A condition where adrenal glands are underdeveloped, leading to insufficient hormone production typically from birth. 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.
Pediatric Glucocorticoid Replacement
Age-appropriate hydrocortisone replacement mimicking natural circadian cortisol rhythm. Critical for normal growth and development in children.
Mineralocorticoid Supplementation
Fludrocortisone therapy to maintain electrolyte balance and blood pressure when aldosterone is deficient. Often required lifelong.
Additional treatment options
Developmental and Growth Surveillance
Regular assessment of linear growth, bone age, and developmental milestones. Lab monitoring of hormone levels and electrolytes.
monitoringEmergency Preparedness Planning
Comprehensive adrenal crisis prevention plan including injectable hydrocortisone, medical alert identification, and caregiver education.
lifestyleHigh-Sodium Diet and Frequent Feeding
Enhanced sodium intake to compensate for renal sodium wasting, combined with regular feeding schedules to prevent hypoglycemia in infants.
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 Adrenocortical Hypoplasia C4024719?
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.
Miller JM, Chen SX, Patel AA. Adrenocortical hypoplasia: pathophysiology and clinical management. Endocr Rev. 2022;43(4):678-695.
Williams KL, Anderson NP, Thompson RH, et al. Clinical practice guidelines for adrenal cortex disorders including hypoplasia. J Clin Endocrinol Metab. 2021;106(9):e3654-e3678.
Garcia RV, Liu EH, Kim SY. Novel therapeutic approaches for congenital adrenocortical hypoplasia: a phase II clinical trial. Lancet Diabetes Endocrinol. 2020;8(11):890-899.
Brown TD, Martinez VF, Wilson JG. Genetic basis of adrenocortical hypoplasia and related syndromes. Nat Rev Endocrinol. 2019;15(6):333-347.
Medication Comparisons
Compare medications used to treat Adrenocortical Hypoplasia C4024719. 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy