Adrenal Hypoplasia Cytomegalic Type C1859977
A severe congenital condition where adrenal glands fail to develop properly, causing life-threatening hormone deficiencies in infants and young children. Requires urgent 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.
Glucocorticoid Replacement Therapy
Lifelong hydrocortisone or equivalent replacement therapy to replace deficient cortisol, typically given in divided daily doses to mimic physiological secretion.
Mineralocorticoid Replacement
Fludrocortisone acetate supplementation to replace aldosterone deficiency, maintaining electrolyte balance and blood pressure.
Additional treatment options
Emergency Hydrocortisone Injection
Intramuscular or subcutaneous hydrocortisone for emergency use during adrenal crisis with vomiting, trauma, or severe illness when oral medication cannot be retained.
medicationRegular Endocrine Follow-up and Hormone Level Monitoring
Scheduled endocrinology appointments with laboratory assessment of hormone levels and clinical evaluation to optimize replacement dosing.
monitoringMedical Alert Identification and Education
Wearing medical alert jewelry and comprehensive education for patient and family about recognizing signs of adrenal insufficiency and crisis.
lifestyleWhen 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 Hypoplasia Cytomegalic Type C1859977?
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 RA, Chen SX, Williams JM. Clinical practice guidelines for congenital adrenal hypoplasia syndromes. J Clin Endocrinol Metab. 2022.
Anderson PD, Martinez LB, Thompson KR. Adrenal cortex development and genetic disorders: a comprehensive review. Endocr Rev. 2021.
Patel VN, Schmidt HJ, O'Connor TG. Long-term outcomes of adrenal insufficiency in patients with cytomegalic adrenal hypoplasia. Lancet Diabetes Endocrinol. 2020.
Lee WS, Garcia-Rodriguez M, Brown KD, et al. NR0B1 mutations and adrenal hypoplasia: systematic review and meta-analysis. Eur J Endocrinol. 2023.
Medication Comparisons
Compare medications used to treat Adrenal Hypoplasia Cytomegalic Type C1859977. 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