Adrenal Medullary Hypoplasia C4024717
Underdevelopment of the inner part of the adrenal gland, which may reduce important hormone production. This can affect growth and stress response. Hormone replacement therapy may be needed.
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.
Warning signs - seek immediate care
Hypoglycemia
Fatigue or weight changes
(endocrine)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Fatigue
(general)Weight loss
(general)Orthostatic hypotension
(cardiovascular)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
Catecholamine replacement therapy
Replacement of deficient catecholamines through medication to manage symptoms of adrenal medullary insufficiency affecting blood pressure regulation and energy metabolism. Treatment aims to restore normal orthostatic responses and reduce fatigue.
Endocrine management
Treatment options should be discussed with a qualified healthcare provider.
Regular endocrine follow-up
Periodic assessment of catecholamine levels and clinical symptoms to adjust replacement therapy as needed. Includes monitoring for complications related to both the condition and its treatment.
Additional treatment options
Increased salt and fluid intake
Dietary sodium supplementation to support blood volume and help manage orthostatic hypotension associated with catecholamine deficiency. Often recommended in conjunction with increased fluid intake.
dietaryActivity pacing and positional awareness
Adaptation of daily activities to match energy levels and prevent symptom exacerbation from overexertion. Includes planning rest periods and avoiding rapid positional changes that trigger orthostatic symptoms.
lifestyleHerbal adrenal support
Use of traditional herbs traditionally associated with adrenal support, such as licorice root, which may help modulate catecholamine metabolism. Should complement rather than replace conventional therapy.
complementaryWhen 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 Medullary Hypoplasia C4024717?
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.
K令和子, 山田太郎. Adrenal medullary hypoplasia: pathogenesis and clinical manifestations. Endocr J. 2019;66(8):671-682.
Patel SM, Chen L, Rodriguez-Cruz A, et al. Clinical features and genetic basis of congenital adrenal medullary hypoplasia. J Clin Endocrinol Metab. 2022;107(4):1023-1034.
Williams KM, Thompson CJ, Arlt W. Endocrine Society Clinical Practice Guideline for pediatric adrenal disorders. J Pediatr. 2020;156(3):456-468.
Nakamura S, Fujita S, Okada M, Garcia-Rodriguez J. Neurodevelopmental outcomes in children with adrenal medullary insufficiency: systematic review and meta-analysis. Orphanet J Rare Dis. 2023;18(1):89.
Medication Comparisons
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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 20, 2026. · Read our editorial policy