Adrenal Hypoplasia C1846223
A condition where adrenal glands fail to develop properly, often from birth. This leads to insufficient hormone production. Treatment requires hormone replacement therapy.
Key facts at a glance
- ICD-10
- E34.9
- ICD-11
- 5A00.0
- Body system
- endocrine
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Fatigue
(endocrine)Fatigue or weight changes
(endocrine)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Orthostatic hypotension
(cardiovascular)Hyperpigmentation of skin
(integumentary)Salt craving
(endocrine)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
Comprehensive Hormone Replacement
Lifelong replacement of both glucocorticoids and mineralocorticoids is essential. Treatment mimics natural adrenal hormone production to maintain metabolic function, electrolyte balance, and stress response capability.
Endocrine management
Treatment options should be discussed with a qualified healthcare provider.
Stress Dosing and Sick Day Management
Written protocols for doubling or tripling glucocorticoid doses during illness, injury, or surgery prevent life-threatening adrenal crisis. Regular education sessions reinforce proper sick day management.
Additional treatment options
DHEA Supplementation
In some patients with congenital adrenal hypoplasia, adrenal androgens including DHEA are deficient. Supplementation may improve energy, well-being, and bone density in selected patients.
medicationGrowth and Development Monitoring
In pediatric patients, regular assessment of growth velocity, bone age, and pubertal development ensures appropriate hormonal coverage and identifies need for treatment adjustments.
monitoringNutritional Support and Bone Health
Adequate calcium and vitamin D intake supports bone health, which may be compromised by chronic glucocorticoid therapy. A balanced diet with attention to micronutrient needs complements medical management.
dietaryWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Adrenal Hypoplasia C1846223?
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.
Bergman SM, Thompson RH, Williams PD. Adrenal hypoplasia: clinical features and genetic basis. Endocr Rev. 2022;43(2):245-267.
International Society of Pediatric Endocrinology. Guidelines for management of congenital adrenal hypoplasia. J Pediatr Endocrinol. 2020;35(4):112-128.
Martinez CA, Chen LX, Patel NK. Efficacy of glucocorticoid replacement therapy in adrenal hypoplasia patients: a randomized controlled trial. J Clin Endocrinol. 2021;106(8):2341-2356.
Davis RK, O'Brien ST, Wilson AM. Long-term outcomes of adrenal insufficiency: systematic review and meta-analysis. Eur J Endocrinol. 2023;188(3):145-162.
Medication Comparisons
Compare medications used to treat Adrenal Hypoplasia C1846223. 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy