28 Questions
What is the main difference between primary adrenal insufficiency and secondary adrenal insufficiency?
Primary adrenal insufficiency is due to a problem in the adrenal gland, whereas secondary adrenal insufficiency is due to a problem elsewhere in the body.
What is Waterhouse-Friderichsen syndrome?
A type of acute adrenal insufficiency
What is the term for a tumor of the adrenal cortex?
Adrenocortical adenoma
What is Conn syndrome?
A type of hyperaldosteronism
What is the term for destruction of the adrenal gland due to various causes?
Addison disease
What type of cells can give rise to pheochromocytoma?
Chromaffin cells
What is the primary cause of hyperpigmentation in Addison disease?
Overproduction of proopiomelanocortin
What is the primary symptom of Addisonian crisis?
Sudden and severe pain in the back, abdomen, or legs
What is the purpose of ACTH stimulation test in diagnosing Addison disease?
To stimulate the production of cortisol in response to ACTH
What is the primary treatment for hypovolemia and hypotension in Addison disease?
IV fluids and vasopressors
What is the primary cause of adrenal crisis in Addison disease?
Sudden increase in cortisol demand
What is the primary role of fludrocortisone in the treatment of Addison disease?
To replace aldosterone
What is the most common cause of Addison disease in high-income countries?
Autoimmune destruction
Which adrenal gland layer is responsible for aldosterone production?
Zona glomerulosa
What is the result of aldosterone levels dropping in Addison disease?
Hyperkalemia and hypotension
Which of the following is NOT a clinical manifestation of Addison disease?
Hypervolemia and hypertension
What is the primary cause of Addison disease in other countries?
Infectious due to tuberculosis
What is the genetic cause of Sturge-Weber Syndrome?
Somatic mutations in the GNAQ gene
What is a common symptom of pheochromocytoma?
Palpitations
What is the consequence of untreated hypertension caused by pheochromocytoma?
Hemorrhage and ischemia
What is the term for the increased pressure in the blood vessels due to pheochromocytoma?
Secondary hypertension
What is the name of the tumor that causes pheochromocytoma?
Paraganglioma
What is the primary function of CXCL12 in neuroblastoma?
To migrate towards organs
Which symptom of neuroblastoma is dependent on the location of the metastatic tumor?
Breathing difficulties
What is the primary diagnostic test used to measure the size and location of a neuroblastoma tumor?
CT scan
What is the typical treatment for neuroblastoma with metastasis?
Chemotherapy and bone marrow transplantation
Which of the following is a symptom of neuroblastoma in the bone marrow?
Abnormal RBC, platelets, and WBC
What is the primary diagnostic marker used to detect neuroblastoma?
Homovanillic acid (HMA) and vanillylmandelic acid (VMA)
Study Notes
Adrenal Gland Dysfunction
- Primary adrenal insufficiency occurs when the adrenal gland cannot produce enough hormones, particularly aldosterone and cortisol, due to damage to the adrenal cortex.
Primary Adrenal Insufficiency
- Can be either acute or chronic
- Acute: Waterhouse-Friderichsen syndrome
- Chronic: Addison disease
Addison Disease
- Caused by progressive destruction of the adrenal gland from various causes
- Symptoms dependent on the adrenal gland layer(s) destroyed
- Zona fasciculata destruction: cortisol levels drop, leading to hypoglycemia, and hyperpigmentation
- Zona glomerulosa destruction: aldosterone levels drop, leading to hyperkalemia, hyponatremia, hypovolemia, hypotension, and metabolic acidosis
Addison Disease: Clinical Manifestations
- Symptoms worsen with stressors such as injury, surgery, or infection, leading to Addisonian crisis
Diagnosis of Addison Disease
- Cortisol measurements
- ACTH measurements
- ACTH stimulation test
- Aldosterone and renin levels
- Adrenal CT/MRI
- 21-hydroxylase antibodies
Treatment of Addison Disease
- Hormone replacement therapy:
- Low cortisol: corticosteroids
- Low aldosterone: fludrocortisone
- Hypovolemia/hypotension: IV fluids and vasopressors
- Hypoglycemia: IV dextrose
Hyperaldosteronism
- Primary: Conn syndrome
- Treatment: hormone replacement therapy with fludrocortisone
Pheochromocytoma
- Classic signs and symptoms: secondary hypertension, headache, palpitations, diaphoresis, and stroke
- Metastasis: lymph nodes, liver, bones, and bone marrow
Neuroblastoma
- Symptoms: fever, weight loss, sweating, and fatigue
- Other symptoms dependent on location of metastatic tumor
- Diagnosis: urinary and serum homovanillic acid (HMA) and vanillylmandelic acid (VMA) measurements, CT scan, and complete blood count
- Treatment: depends on size and stage of neuroblastoma, may include chemotherapy, medication, and bone marrow transplantation
Learn about the etiologies, pathophysiology, clinical features, laboratory findings, diagnostic approaches, and treatment of adrenal gland dysfunction. Understand primary adrenal insufficiency, hyperfunction, hypofunction, and neoplasms.
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