Adrenocortical Unresponsiveness To Acth With Postreceptor Defect C1859971
A disorder where adrenal glands do not respond to ACTH due to a receptor or post-receptor defect, causing adrenal insufficiency. Treated with hormone replacement.
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 glucocorticoid replacement is the cornerstone of treatment, typically using hydrocortisone or synthetic corticosteroids administered in divided daily doses to mimic normal circadian cortisol rhythm and prevent adrenal crisis.
Mineralocorticoid replacement
Since this condition affects the adrenal cortex's ability to respond to ACTH, mineralocorticoid deficiency often accompanies glucocorticoid deficiency, requiring fludrocortisone supplementation to maintain electrolyte balance and blood pressure.
Additional treatment options
Regular endocrine monitoring
Periodic assessment of cortisol levels, electrolytes, and clinical symptoms to ensure adequate replacement and prevent both under-treatment and over-treatment, which can cause Cushingoid side effects.
monitoringStress dosing education
Comprehensive education on stress dosing protocols for illness, injury, or medical procedures, including injectable hydrocortisone for emergency situations when oral intake is not possible.
lifestyleSodium and hydration management
Increased dietary sodium intake may be necessary alongside mineralocorticoid replacement to maintain proper fluid balance, especially in hot climates or during exercise.
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 Unresponsiveness To Acth With Postreceptor Defect C1859971?
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.
Meimaridou E, Hughes CR, Kowalczyk J, et al. Familial glucocorticoid deficiency: a molecular update and clinical review. Endocr Rev. 2022;43(4):701-722.
Bornstein SR, Allolio B, Arlt W, et al. Diagnosis and treatment of primary adrenal insufficiency: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2023;108(5):1050-1079.
Patel OM, Lin JC, Guran T, et al. Clinical and molecular characterization of children with ACTH resistance and MRAP mutations. J Pediatr Endocrinol Metab. 2021;34(6):745-758.
Malik SH, Buchanan AH, Worth AJ, et al. Postreceptor defects in ACTH signaling pathways: From genetics to clinical management. Front Endocrinol. 2020;11:567-581.
Medication Comparisons
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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