Adrenal Disorders in Children
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Questions and Answers

What is the most common cause of autoimmune primary adrenal insufficiency?

Isolated adrenal insufficiency (Addison's disease)

Name one medication that can decrease steroid synthesis, potentially leading to adrenal insufficiency.

ketoconazole

Besides tuberculosis, name another infectious cause of primary adrenal insufficiency?

Histoplasmosis, HIV, or CMV

What is the name of the syndrome associated with adrenal hemorrhage?

<p>Waterhouse-Friderichsen syndrome</p> Signup and view all the answers

What is the name of the condition that includes the triad of achalasia, Addison disease, and alacrima?

<p>Allgrove syndrome/Triple A syndrome</p> Signup and view all the answers

What is a common cause of secondary adrenal insufficiency related to medication use?

<p>Withdrawal from glucocorticoid therapy</p> Signup and view all the answers

What is the underlying cause of the patient's hypoglycemia in the case study?

<p>The child's hypoglycemia is due to adrenal insufficiency.</p> Signup and view all the answers

What is the most likely cause of the child's adrenal insufficiency given his asthma treatment?

<p>Secondary adrenal insufficiency from the inhaled glucocorticoid</p> Signup and view all the answers

What are the two main sub-organs of the adrenal gland?

<p>Adrenal cortex and adrenal medulla</p> Signup and view all the answers

What role do mineralocorticoids play in the body, and give an example of one?

<p>Mineralocorticoids regulate sodium retention, potassium loss, and body fluid. An example is aldosterone.</p> Signup and view all the answers

How does cortisol affect blood glucose levels, particularly in fasting or starvation?

<p>Cortisol acts on the liver to maintain normal glucose levels, especially during fasting and starvation.</p> Signup and view all the answers

Describe the typical pattern of cortisol levels during the day.

<p>Cortisol levels typically peak in the early morning and reach their lowest point in the evening.</p> Signup and view all the answers

What is the function of the renin-angiotensin system in relation to aldosterone?

<p>The renin-angiotensin system regulates the release of aldosterone.</p> Signup and view all the answers

What are the two main parts of the adrenal gland?

<p>The adrenal cortex and adrenal medulla.</p> Signup and view all the answers

What is the primary hormone produced by the adrenal medulla?

<p>Adrenaline (epinephrine).</p> Signup and view all the answers

Name the three zones of the adrenal cortex.

<p>Zona glomerulosa, zona fasciculata, and zona reticularis.</p> Signup and view all the answers

What hormone is released by the juxtaglomerular apparatus in response to low renal perfusion?

<p>Renin.</p> Signup and view all the answers

Which hormone from the adrenal cortex is responsible for sodium retention and potassium wasting?

<p>Mineralocorticoids.</p> Signup and view all the answers

What stimulates the release of CRH (corticotropin-releasing hormone)?

<p>The hypothalamic–pituitary–adrenal axis.</p> Signup and view all the answers

What are the two main hormones released from the adrenal medulla?

<p>Adrenaline (epinephrine) and Noradrenaline (norepinephrine).</p> Signup and view all the answers

What is the primary function of glucocorticoids, such as cortisol?

<p>Protein catabolism, insulin resistance, immune response, hypertension, increased appetite and memory.</p> Signup and view all the answers

At what time of day does cortisol secretion typically peak?

<p>Between 7-9 o'clock in the morning.</p> Signup and view all the answers

What are the main effects of adrenaline on the cardiovascular system?

<p>Vasodilatation and tachycardia.</p> Signup and view all the answers

Study Notes

Adrenal Disorders in Children

  • Adrenal glands are located above the kidneys
  • Divided into two sub-organs: cortex and medulla
  • Adrenal cortex secretes steroid hormones: glucocorticoids, mineralocorticoids, and androgens
  • Adrenal medulla secretes catecholamines (epinephrine/adrenaline and norepinephrine)

Adrenal Gland Function

  • Mineralocorticoids: regulate sodium retention and potassium loss, balancing body fluid
  • Glucocorticoids: act as anti-inflammatory agents and affect metabolism
  • Androgens: regulate growth/development of genitalia and puberty
  • Adrenaline (epinephrine): increases heart rate and blood pressure
  • Norepinephrine: constricts arterioles

Glucocorticoids (Cortisol)

  • Crucial for many physiological processes, including cardiovascular function and blood pressure regulation
  • Influences carbohydrate metabolism, maintaining normal glucose levels
  • Plays a role in immune function, reducing inflammation and suppressing the immune system
  • Cortisol levels typically peak in the early morning and are lowest in the evening. This is a typical pattern.

Aldosterone

  • A mineralocorticoid regulating sodium and potassium balance
  • Plays a key role in the renin-angiotensin-aldosterone system (RAAS) which regulates blood pressure.
  • Kidneys are major participants in the regulation process

Adrenal Disorders

  • Primary adrenal insufficiency:
    • Hereditary: Congenital adrenal hyperplasia, others
    • Autoimmune: Addison's disease, polyglandular autoimmune syndromes (type 1 & 2)
    • Infectious: Tuberculosis, systemic fungal infections, HIV, CMV
    • Medications: reduced steroid synthesis (e.g., ketoconazole), increased steroid metabolism (e.g., rifampin)
    • Miscellaneous: hemorrhage, Triple A syndrome (Allgrove syndrome)
  • Secondary/Tertiary adrenal insufficiency:
    • Isolated ACTH deficiency, panhypopituitarism (congenital or acquired)
    • Hypothalamic/pituitary disorders, tumors, surgery, radiation therapy (possibly infections related to CNS)
    • Withdrawal from glucocorticoid therapy, inadequate replacement, infant born to a mother on steroids, surgical tumor removal (Cushing's disease)

Congenital Adrenal Hyperplasia (CAH)

  • Autosomal recessive condition
  • Most common cause of primary adrenal insufficiency
  • High incidence of 21-hydroxylase deficiency which affects the ability to produce cortisol and aldosterone.
  • Neonatal screening for this is recommended.
  • Prenatal therapy is essential to prevent genital problems in affected females

Addison's Disease

  • Rare autoimmune disorder
  • Autoimmune destruction of adrenal glands
  • Leads to inadequate production of adrenal hormones causing insufficiency
  • Clinical symptoms include:
    • Fasting hypoglycemia
    • Nausea/vomiting and diarrhea
    • Weight loss/severe anorexia
    • Fatigue, lethargy, muscle weakness
    • Hyperpigmentation (darkening of skin)
    • Hypotension, shock, even death

Cushing's Syndrome

  • Excess cortisol secretion, potentially from:
    • ACTH-secreting tumor of the pituitary
    • Neoplasm within adrenal cortex
    • Ectopic ACTH secretion by a malignant growth outside the adrenal gland
    • Prolonged steroid use
  • Clinical presentation includes:
    • Truncal obesity
    • Moon face
    • Buffalo hump (fat accumulation in the upper back)
    • Acne, hirsutism
    • Abdominal striae (stretch marks)
    • Hypertension
    • Psychiatric disturbances
    • Osteoporosis
    • Amenorrhea
    • Diabetes

Diagnostic Workup

  • Medical history and physical examination are crucial
  • Measurements including blood pressure, serum electrolytes, glucose, and cortisol levels
  • Imaging tests and hormonal assessments to confirm the diagnosis
  • ACTH stimulation tests

Treatment

  • Treatment for adrenal disorders varies significantly based on the underlying cause.
  • Medications: hydrocortisone, fludrocortisone
  • Possible surgery for neoplasia

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Description

This quiz covers the anatomy and functions of adrenal glands, specifically in children. It discusses the roles of adrenal cortex and medulla, along with the hormones they secrete and their physiological effects. Test your knowledge on the impact of these hormones on growth, metabolism, and cardiovascular function.

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