Adrenal Disorders in Children Flashcards
Q: What are the primary hormones secreted by the adrenal glands?
A:
1- Mineralocorticoids (Aldosterone): Regulates sodium and potassium levels.
2- Glucocorticoids (Cortisol): Promotes gluconeogenesis, suppresses the immune system, and influences mood.
3- Androgens: Regulate bone, muscle, genitalia, and pubertal development.
4- Catecholamines (Adrenaline and Noradrenaline): Trigger the “fight or flight” response.
Q: Name the adrenal disorders caused by increased hormone secretions.
A:
Cushing’s Syndrome
Hyperaldosteronism
Phaeochromocytoma
Q: Name the adrenal disorders caused by decreased hormone secretions.
Adrenal cortical insufficiency
Congenital Adrenal Hyperplasia (CAH)
Addison’s Disease
Q: What is the primary cause of Cushing’s syndrome in children?
A:
Long-term glucocorticoid treatment, such as steroid therapy for nephrotic syndrome.
May also result from adrenocorticoid tumors or pituitary adenomas.
Q: How is an adrenal crisis managed?
A:
1- Urgent IV hydrocortisone administration.
2- 2 mL/kg bolus of 10% dextrose if glucose < 3 mmol/L.
3- Maintenance fluids: 0.9% saline + 5% glucose (100 mL/kg/day).
4- Careful monitoring and correction of electrolyte imbalances.
5- ECG monitoring for hyperkalaemia.
6- Administer IV calcium gluconate for severe hyperkalaemia.
Q: How is congenital adrenal hyperplasia (CAH) diagnosed and managed?
A:
- Diagnosed via newborn screening on Day 5 (low 21-hydroxylase levels).
- Treated with age-appropriate doses of hydrocortisone and fludrocortisone.
- Families receive emergency kits with IM hydrocortisone and Glucogel.
- Regular growth monitoring and psychological support.
Q: What test confirms Addison’s disease?
ACTH stimulation (Synacthen) test.
Q: What is the hallmark presentation of Addison’s disease in children?
Hyperpigmentation and associations with autoimmune conditions like T1DM.
Q: What are the sick day rules for managing adrenal disorders?
Increase glucocorticoid dosing during illness or stress as per BSPED guidelines.
Use emergency IM hydrocortisone when oral intake is compromised.
Q: What are the long-term monitoring requirements for children with congenital adrenal hyperplasia?
1- 6-month reviews, more frequently during rapid growth/puberty.
2- Monitor height, weight, and signs of under/over replacement.
3- Assess bone age and density using left wrist X-rays.