Adrenocortical insufficiency Addison

Drugs with mineralocorticoidlike activity (aldosterone agonists) are frequently administered as replacement therapy whenever the natural production of mineralo-corticoids is impaired. Mineralocorticoid replacement is usually required in patients with chronic adrenocortical insufficiency (Addison disease), following adrenalectomy, and in other forms of adrenal cortex hypofunction. These conditions usually require both mineralocorticoid and glucocorticoid replacement. 

Chronic primary adrenocortical insufficiency (Addison s disease)... 

IV Diseases where high potassium levels may be encountered hyperkalemia renal failure and conditions in which potassium retention is present oliguria or azotemia anuria crush syndrome severe hemolytic reactions adrenocortical insufficiency (untreated Addison disease) adynamica episodica hereditaria acute dehydration heat cramps hyperkalemia from any cause early postoperative oliguria except during Gl drainage. 

Cortisone acetate and hydrocortisone are usually the corticoids of choice for replacement therapy in patients with primary adrenocortical insufficiency (such as Addison s disease), or after adrenalectomy where both glucocorticoid and mineralo-corticoid replacement is needed. In secondary adrenal insufficiency, associated with inadequate corticotrophin (ACTH) secretion, glucocorticoid replacement alone is usually adequate [62]. 

Clinically, ACTH stimulation of the adrenals is used diagnostically to detect adrenal insufficiency plasma cortisol levels are measured before and 60 minutes following an intravenous injection of cosyntropin. Adrenocortical insufficiency is known as Addison s disease Addison s classic description, in 1855, namely general debility, remarkable feebleness of the heart, irritability of the stomach, and a peculiar change of the colour of the skin , summarizes the clinical features of this disease, which is uniformly fatal if undetected and untreated. Therapeutically, corticotropin therapy has been essentially abandoned in favor of the direct administration of glucocorticoids. However, ACTH is still rarely used in the treatment of the infantile spasm seizure disorder. 

Diagnosis and Treatment of Disturbed Adrenal Function Adrenocortical Insufficiency Chronic (Addison s Disease)... 

Cortisol. Cortisol, secreted by the adrenal cortex in response to adrenocorticotropic hormone (ACTH), stimulates gluconeogenesis and increases the breakdown of protein and fat. Patients with Cushing s syndrome have increased cortisol owing to a tumor or hyperplasia of the adrenal cortex and may become hyperglycemic. In contrast, people with Addisons disease have adrenocortical insufficiency because of destruction or atrophy of the adrenal cortex and may exhibit hypoglycemia. ... 

Figure 51-12 Basal plasma ACTH concentrations in primary and secondary adrenocortical insufficiency. (From Irvine WJ, Toft AD, Feek CM.Addison s disease. In JamesVHZ ed.The adrenal gland. New York Raven Press, 1979 131.)...

Hypoaldosteronism is rare and usually is associated with low renin status, diabetes, complete heart block, or severe postural hypotension, or it may occur postoperatively following tumor removal. Hypoaldosteronism may be part of a larger adrenal insufficiency or be the only defect the patient has. In nonselective hypoaldosteronism, the etiology of the low aldosterone is most likely generalized adrenocortical insufficiency (see Addison s disease). In selective hypoaldosteronism, the etiology is usually a specific defect in the stimulation of adrenal aldosterone secretion (21-hydroxylase deficiency being most common) or a defect in peripheral aldosterone action (decreased aldosterone receptors). 

In addition to the clinical observations, a number of biochemical results may point towards adrenocortical insufficiency. These are hyponatraemia. hypcrkalacmia and elevated serum ureti, and may be seen in many patients with Addison s Disease. 

Addison s disease is a rare, potentially fatal condition due to insufficient production of both aldosterone and cortisol caused by atrophy of the adrenal glands. It is characterised by low blood pressure, loss of sodium, weight loss and pigmentation of mucosal membranes. Adrenocortical insufficiency also results from pituitary failure with loss of adrenocorticotrophic hormone (ACTH) production. 

Use of OxyContin is associated with increased potential risks and it should be used only with caution in the following conditions acute alcoholism adrenocortical insufficiency (e.g. Addison s disease) CNS depression or coma delirium tremens debilitated patients kyphoscoliosis associated with respiratory depression myxedema or hypothyroidism prostatic hypertrophy or urethral stricture severe impairment of hepatic, pulmonary or renal function and toxic psychosis. 

Synacthen (tetracosactrin) is a synthetic peptide having ACTH activity. It is used in the diagnosis of adrenocortical hypo-function. Injection of synacthen should normally result in an increase in plasma cortisol levels after 30 minutes as a result of stimulation of the adrenal gland. An impaired response suggests adrenocortical hypofunction. A further test may then be performed in order to distinguish between primary adrenocortical hypofunction (Addison s disease) and secondary adrenocortical hypofunction. This consists of three injections of synacthen on successive days. An improvement in cortisol production indicates secondary adrenocortical hypofunction. No improvement occurs if there is primary adrenocortical insufficiency. 

Of 62 initially autoantibody-negative patients treated with interferon alfa for chronic hepatitis C for a mean of 8 months, three developed antibodies to 21b-hydroxylase, a sensitive assay of adrenocortical autoimmunity (528). However, there were no cases of Addison s disease or subclinical adrenal insufficiency. This study suggested that the adrenal cortex is another potential target organ of autoimmune effects of interferon alfa, along with thyroid and pancreatic islet cells. 

Figure 51-13 Serum cortisol response to 0.25 mg of cosyntropin (ACTH) in nine normal individuals (normal adrenal), eight patients with hypopituitarism (secondary adrenal insufficiency), and seven patients with Addison s disease (primary adrenal insufficiency). (From Spechart P, Nicoloff J, Bethune J. Screening for adrenocortical insufl ciency with cosyntropin [synthetic ACTH], Arch Intern Med 1971 128 761. Copyright 1971, American Medical Association.)...

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