Adrenogenital Syndrome
Adrenogenital syndrome usually refers to congenital adrenal hyperplasia, an inherited enzyme disorder that reduces cortisol production and can increase adrenal androgen production.
Key takeaways
- Severity ranges from salt-wasting illness in infancy to milder later presentations with early puberty, excess hair growth or irregular periods.
- Diagnosis uses hormone tests and, when appropriate, genetic testing; newborn screening identifies many classic cases.
- Treatment balances adequate hormone replacement against over-treatment and requires specialist monitoring through life.
The listings below are not suitable without confirmation of the specific enzyme disorder and an individual endocrine plan.
Why presentations differ
The amount of residual enzyme activity determines whether aldosterone as well as cortisol is deficient. Salt-wasting disease can cause dehydration, low sodium and shock. Excess androgen may affect genital development, growth, puberty, menstrual cycles and fertility.
Long-term management
Glucocorticoid replacement suppresses excess adrenal stimulation while replacing cortisol; mineralocorticoid and salt may also be required. Monitoring includes growth, blood pressure, electrolytes and hormone markers. Doses need temporary adjustment during illness or surgery under sick-day rules.
When to seek urgent care
Follow the emergency plan and seek urgent help for repeated vomiting, severe weakness, dehydration, confusion, collapse or inability to take replacement medicine.