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Progressive weakness, anemia, hypoglycemia, and hyperpigmentation of the skin. Vomiting, apneic spells, cyanosis, vascular collapse, and cardiovascular shock may be consequential.

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Addison Disease; Adrenal Insufficiency; Adrenal Hypoplasia/Aplasia.

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First described in 1849 by Thomas Addison, the English physician, in patients suffering from tuberculosis with suprarenal involvement.

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Most cases are sporadic, but depending on the etiology solid evidence exists for autosomal recessive and X-linked recessive forms.

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Complete or partial deficit of mineralocorticoids and glucocorticoid hormones, excreted by the zonae fasciculata (aldosterone) and glomerulosa (glucocorticoids) of the adrenal cortex, caused by different mechanisms. Autoimmune mechanisms (which may be associated with other autoimmune diseases, e.g. chronic lymphocytic thyroiditis, hyperthyroidism, diabetes mellitus, celiac disease), adrenal aplasia, X-linked congenital adrenal hypoplasia, cytomegalic adrenocortical hypoplasia, familial hypoadrenocorticism, pituitary gland hypoplasia, isolated ACTH deficiency, X-linked adrenoleukodystrophy, and Waterhouse Friderichsen Syndrome (fulminant form caused by meningococcemia). Dysfunction of the adrenal cortex leads to interrupted hypothalamic and anterior pituitary gland feedback inhibition, resulting in continuous and uninhibited secretion of ACTH. Cleavage of the prohormone pro-opiomelanocortin in the adenohypophysis results in simultaneous secretion of ACTH and melanocyte-stimulating hormone (MSH). The elevated MSH level is responsible for the typical bronze hyperpigmentation in patients with primary adrenal insufficiency.

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Vomiting, cyanosis, apneic spells, hypoglycemia, seizures, and vascular collapse are the usual presenting symptoms of an adrenal crisis. Cardiovascular collapse may occur. Plasma and urinary concentrations of all adrenal steroids are low. Serum glucose level usually is low. Sodium plasma concentrations are decreased (but rarely <120 mmol/l) but potassium plasma concentrations are increased (but rarely >7.0 mmol/l). Moderate metabolic acidosis with plasma bicarbonate levels between 15 and 20 mmol/l is common. BUN typically is elevated. Urinary excretion of sodium and chloride is increased, whereas potassium excretion is decreased. Plasma cortisol levels—if measured before treatment—are low. However, the final diagnosis of adrenocortical insufficiency is based on the finding of an absent or decreased response in adrenal steroid synthesis to stimulation with external (intravenous or intramuscular) ACTH. Increased serum concentrations of cortisol and aldosterone are signs of a functional adrenal cortex. Under normal conditions, cortisol synthesis increases by a factor of two to five times above normal levels after administration of ACTH. Failure of aldosterone serum level to double within 30 minutes of ACTH administration indicates a problem with mineralocorticoid synthesis. A normal ACTH stimulation test result excludes the diagnosis of primary adrenocortical insufficiency. The adrenal glands may appear enlarged on abdominal CT scan or sonography in patients with Addison disease secondary to infection, hemorrhage, or malignancies involving the adrenals. Primary adrenal insufficiency results in atrophic adrenal glands secondary to inactivity. Idiopathic autoimmune adrenocortical insufficiency usually resulting from autoimmune lymphocytic infiltration of the adrenal cortex is the most common cause of Addisonian syndrome, accounting for approximately 80% of cases. Another significant percentage results from adrenal cortex destruction by tuberculosis.

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Idiopathic autoimmune Addison disease may occur in isolation or in combination with other autoimmune diseases, such as diabetes mellitus type I, systemic lupus erythematosus, Hashimoto thyroiditis, ...

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