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Questions and Answers
What is the primary cause of Addison's disease in developing countries?
What is the primary cause of Addison's disease in developing countries?
- Hypocortisolism
- Autoimmune reaction
- Tuberculosis (correct)
- Adrenocortical hypofunction
What is the term used to describe the destruction or dysfunction of adrenal cortical tissue?
What is the term used to describe the destruction or dysfunction of adrenal cortical tissue?
- Hypocortisolism
- Chronic primary adrenocortical insufficiency (correct)
- Adrenocortical hypofunction
- Adrenal insufficiency
What is the term used to describe the failure of the adrenal glands to produce hormones, resulting from injury or surgery to the hypothalamus or pituitary gland?
What is the term used to describe the failure of the adrenal glands to produce hormones, resulting from injury or surgery to the hypothalamus or pituitary gland?
- Primary adrenocortical insufficiency
- Acute adrenal insufficiency
- Adrenal crisis
- Secondary adrenocortical insufficiency (correct)
What is the leading cause of Addison's disease in developed countries, including the US?
What is the leading cause of Addison's disease in developed countries, including the US?
What is the term used to describe the life-threatening complication of inadequately treated Addison's disease?
What is the term used to describe the life-threatening complication of inadequately treated Addison's disease?
What is the significance of Addison's disease?
What is the significance of Addison's disease?
Who provided the first detailed clinical description of CPAI?
Who provided the first detailed clinical description of CPAI?
What is the common term used to describe the ability of patients with Addison's disease to respond to stress?
What is the common term used to describe the ability of patients with Addison's disease to respond to stress?
What is a characteristic of Addisonian crisis?
What is a characteristic of Addisonian crisis?
What is the most common cause of Addison's disease (AD)?
What is the most common cause of Addison's disease (AD)?
What is a possible cause of chronic fatigue in patients with AD?
What is a possible cause of chronic fatigue in patients with AD?
What is a characteristic of autoimmune AD?
What is a characteristic of autoimmune AD?
What is a complication of adrenoleukodystrophy?
What is a complication of adrenoleukodystrophy?
What is a type of autoimmune disease that can occur in patients with AD?
What is a type of autoimmune disease that can occur in patients with AD?
What is a feature of localized autoimmune adrenal insufficiency?
What is a feature of localized autoimmune adrenal insufficiency?
What is the approximate percentage of cases of AD caused by tuberculosis in developed countries?
What is the approximate percentage of cases of AD caused by tuberculosis in developed countries?
What is the typical age range for Addison disease to develop in PGAD I patients?
What is the typical age range for Addison disease to develop in PGAD I patients?
Which of the following is NOT a complication of PGAD I?
Which of the following is NOT a complication of PGAD I?
What is the mode of inheritance of PGAD I?
What is the mode of inheritance of PGAD I?
What is the typical presentation of PGAD II?
What is the typical presentation of PGAD II?
Which of the following is associated with PGAD II?
Which of the following is associated with PGAD II?
What is the underlying abnormality in PGAD I?
What is the underlying abnormality in PGAD I?
Which of the following is NOT a feature of PGAD I?
Which of the following is NOT a feature of PGAD I?
What is the typical complication of PGAD I that affects the stomach?
What is the typical complication of PGAD I that affects the stomach?
What is the primary cause of Addison disease?
What is the primary cause of Addison disease?
At what percentage of total adrenocortical tissue dysfunction does the onset of Addison disease typically occur?
At what percentage of total adrenocortical tissue dysfunction does the onset of Addison disease typically occur?
What is the function of corticotropin-releasing hormone (CRH) in the HPA axis?
What is the function of corticotropin-releasing hormone (CRH) in the HPA axis?
Which of the following hormones is responsible for stimulating cortisol release from the adrenal glands?
Which of the following hormones is responsible for stimulating cortisol release from the adrenal glands?
What is the role of cortisol in the body's physiological response to stress?
What is the role of cortisol in the body's physiological response to stress?
What is the most common and specific antibody associated with autoimmune Addison disease?
What is the most common and specific antibody associated with autoimmune Addison disease?
What is the consequence of a deficiency of functional suppressor T lymphocytes in autoimmune Addison disease?
What is the consequence of a deficiency of functional suppressor T lymphocytes in autoimmune Addison disease?
What is the significance of detecting antibodies to steroid 21-hydroxylase in patients without symptomatic Addison disease?
What is the significance of detecting antibodies to steroid 21-hydroxylase in patients without symptomatic Addison disease?
What percentage of patients with AD experience an addisonian crisis when they first see a health professional?
What percentage of patients with AD experience an addisonian crisis when they first see a health professional?
What is the percentage of patients with CPAI who exhibit hyperpigmentation of the skin and mucous membranes?
What is the percentage of patients with CPAI who exhibit hyperpigmentation of the skin and mucous membranes?
What is the typical duration of time that hyperpigmentation precedes other clinical manifestations of AD?
What is the typical duration of time that hyperpigmentation precedes other clinical manifestations of AD?
What is the normal range of cortisol levels in the morning, when baseline cortisol levels are normally highest each day?
What is the normal range of cortisol levels in the morning, when baseline cortisol levels are normally highest each day?
What is the primary test used to diagnose AD, which assesses the functional capacity of the adrenal cortex to synthesize cortisol?
What is the primary test used to diagnose AD, which assesses the functional capacity of the adrenal cortex to synthesize cortisol?
What is the dose of synthetic ACTH (cosyntropin) used in the rapid ACTH stimulation test?
What is the dose of synthetic ACTH (cosyntropin) used in the rapid ACTH stimulation test?
How many times does a normal adrenal cortex release its basal plasma cortisol output within 15-30 minutes of ACTH treatment?
How many times does a normal adrenal cortex release its basal plasma cortisol output within 15-30 minutes of ACTH treatment?
At what time interval after cosyntropin administration are two more blood samples drawn for cortisol assay and aldosterone determination?
At what time interval after cosyntropin administration are two more blood samples drawn for cortisol assay and aldosterone determination?
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Study Notes
Addison's Disease
- Also known as chronic primary adrenocortical insufficiency (CPAI), a potentially life-threatening neuroendocrine or hormonal disorder and chronic metabolic condition.
- Caused by destruction or dysfunction of adrenal cortical tissue.
- Characterized by diminished glucocorticoid (cortisol) and, less commonly, mineralocorticoid (aldosterone) and adrenal androgen function.
History
- First detailed clinical description by Thomas Addison in 1855.
- Before 1950, CPAI usually resulted from an infection of the adrenal glands, with tuberculosis (TB) being the leading cause.
- In developed countries, non-specific destruction of the adrenal glands by cells of the immune system (autoimmune reaction) is the most common cause.
Causes and Risk Factors
- Insults to the adrenal glands that cause AD are multiple.
- 80% of cases are caused by gradual destruction of the outer layer of the adrenal gland (adrenal cortex) by the body's immune system, referred to as autoimmune AD.
- Other causes include:
- Tuberculosis (10% of cases in developed countries)
- Adrenoleukodystrophy (X-linked recessive disorder)
- Hypogonadism (small gonads resulting in ovarian or testicular failure)
- Polyglandular autoimmune disease (PGAD)
Pathophysiology
- The HPA axis regulates the secretion of cortisol from the adrenal glands in response to stress.
- Corticotropin-releasing hormone (CRH) stimulates adrenocorticotropic hormone (ACTH) release, which stimulates cortisol release.
- Addison disease specific antibodies are responsible for the autoimmune destruction of the adrenal cortex.
Diagnosis
- Hyperpigmentation of the skin and mucous membranes is a clinical hallmark of AD, present in 90% of patients.
- Low plasma cortisol (< 3 µg/dL) between 6-8 A.M. strongly suggests AD.
- Definitive diagnosis rests on the assessment of the functional capacity of the adrenal cortex to synthesize cortisol using the rapid ACTH stimulation test.
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