Adrenocortical insufficiency

Adrenocortical insufficiency Organ transplants Liver disease Adrenogenital syndrome Nephrotic syndrome Acute spinal cord injury Hyp ere alemia Hematologic disorders Myasthenia gravis Neoplastic disease... 

ACTH is contraindicated in patients with adrenocortical insufficiency or hyperfunction, allergy to pork or pork products (corticotropin is obtained from porcine... 

The ghicocorticoids are used as replacement therapy for adrenocortical insufficiency, to treat allergic reactions, collagen diseases (eg, systemic lupus erythematosus), dermatologic conditions, rheumatic disorders, shock, and other conditions (see Display 50-1). The anti-inflammatory activity of these hormones make them valuable as anti-inflammatories and as immunosuppressants to suppress inflammation and modify the immune response... 

Adrenoleukodystrophy is an X-linked dysmyelinative disorder caused by mutations in the ABCD1 gene, which encodes the peroxisomal integral membrane ALD protein, a member of the ATP binding cassette transporter family. These mutations result in impaired clearance of plasma very-long-chain fatty acids. Affected males may present with symmetrical distal axonal polyneuropathy, adrenocortical insufficiency or CNS demyelination, while occasional heterozygous women demonstrate deficits suggestive of multiple sclerosis [56]. Manipulation of dietary fatty acid intake has some minimal therapeutic effect, while bone marrow transplantation has diminished deficits in a few patients. (See in Ch. 41.)... 

Corticosteroids are generally recommended, but there is no convincing evidence of adrenocortical insufficiency in thyroid storm their benefits may be attributed to their antipyretic action and stabilization of blood pressure. 

Fluid and electrolyte abnormalities Hypercalcemia Volume depletion Water intoxication Adrenocortical insufficiency Drug-induced Opiates Antibiotics Antifungals Cl obstruction... 

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. 

Hypersensitivity to these agents depressed sodium or potassium serum levels marked kidney and liver disease or dysfunction suprarenal gland failure hyperchloremic acidosis adrenocortical insufficiency severe pulmonary obstruction with inability to increase alveolar ventilation since acidosis may be increased (dichlorphenamide) cirrhosis (acetazolamide, methazolamide) long-term use in chronic noncongestive angle-closure glaucoma. 

Pantothenic acid deficiency manifests itself by symptoms of neuromuscular degeneration and adrenocortical insufficiency. 

In the treatment of secondary adrenocortical insufficiency, lower doses of cortisol are generally effective, and fluid and electrolyte disturbances do not have to be considered, since patients with deficient corticotrophin secretion generally do not have abnormal function of the zona glomerulosa. Since cortisol replacement therapy is required for life, adequate assessment of patients is critical to avoid the serious long-term consequences of excessive or insufficient treatment. In many cases, the doses of glucocorticoid used in replacement therapy are probably too high. Patients should ideally be administered three or more doses daily. To limit the risk of osteoporosis, replacement therapy should be carefully assessed on an individual basis and overtreatment avoided. 

Metyrapone is used in the differential diagnosis of both adrenocortical insufficiency and Cushing s syndrome (hypercortisolism). The drug tests the functional competence of the hypothalamic-pituitary axis when the adrenals are able to respond to corticotrophin that is, when primary adrenal insufficiency has been ruled out. 

In patients with longstanding hypothyroidism and those with ischemic heart disease, rapid correction of hypothyroidism may precipitate angina, cardiac arrhythmias, or other adverse effects. For these patients, replacement therapy should be started at low initial doses, followed by slow titration to full replacement as tolerated over several months. If hypothyroidism and some degree of adrenal insufficiency coexist, an appropriate adjustment of the corticosteroid replacement must be initiated prior to thyroid hormone replacement therapy. This prevents acute adrenocortical insufficiency that could otherwise arise from a thyroid hormone-induced increase in the metabolic clearance rate of adrenocortical hormones. 

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. 

Diagnostic agent to detect adrenocortical insufficiency in suspected individual CAS Registry 16960-16-0 (1-24)- tetracosapeptide Ser-Tyr-Ser-Met- Glu-His-Phe-Arg- Trp-GIy-Lys-Pro- Val-GIy-Lys-Lys- Arg-Arg-Pro-Val- Lys-Val-Tyr-Pro NA... 

DIAGNOSIS AND TREATMENT OF DISTURBED ADRENAL FUNCTION Adrenocortical Insufficiency... 

Chronic adrenocortical insufficiency is characterized by weakness, fatigue, weight loss, hypotension, hyperpigmentation, and inability to maintain the blood glucose level during fasting. In such individuals, minor noxious, traumatic, or infectious stimuli may produce acute adrenal insufficiency with circulatory shock and even death. 

When acute adrenocortical insufficiency is suspected, treatment must be instituted immediately. Therapy consists of large amounts of parenteral hydrocortisone in addition to correction of fluid and electrolyte abnormalities and treatment of precipitating factors. 

This compound, a potent steroid with both glucocorticoid and mineralocorticoid activity, is the most widely used mineralocorticoid. Oral doses of 0.1 mg two to seven times weekly have potent salt-retaining activity and are used in the treatment of adrenocortical insufficiency associated with mineralocorticoid deficiency. These dosages are too small to have important anti-inflammatory or antigrowth effects. 

Treatment of adrenocortical insufficiency. chronic primary and chronic secondary... 

Ascorbic acid depleted in adrenal cortex on stimulation by ACTH Biotin and vitamin A adrenocortical insufficiency noted in biotin and vitamin A deficiency... 

Ascorbic acid may be required for steroid hormone biosynthesis depleted from adrenal cortex on cortical secretion Biotin adrenocortical insufficiency noted in biotin deficiency... 

Substitution therapy is used in cases of primary and secondary adrenocortical insufficiency the aim is to provide glucocorticoids and mineralocorticoids in physiological amounts, and the better the dosage regimen is adapted to the individual s needs, the less the chance of adverse effects (1). 

Withdrawal symptoms disappear if the glucocorticoid is resumed, but as a rule they will in any case vanish spontaneously within a few days. More serious consequences can ensue, however, in certain types of cases and if adrenal cortical atrophy is severe. In patients treated with corticoids for the nephrotic syndrome and apparently cured, the syndrome is particularly likely to relapse on withdrawal of therapy if the adrenal cortex is atrophic (SEDA-3,305). In some cases, acute adrenocortical insufficiency after glucocorticoid treatment has actually proved fatal. It is advisable to withdraw long-term glucocorticoid therapy gradually so that the cortex has sufficient opportunity to recover. Table 5 lists methods of... 

Relative adrenocortical insufficiency can follow withdrawal of corticotropin treatment (presumably because the cortex has adapted itself to a constant high level of stimulation) and can persist for some months. Glucocorticoid substitution has to be provided during this period. The risk of this effect can be reduced by keeping the dose of corticotropin as low as possible. 

Fosfestrol is an unusual agent used in Japan for the treatment of prostatic carcinoma but not accepted by experts in Europe. Described as an estrogen, in European studies it had a high incidence of complications, including fluid retention (16%), myocardial infarction (10%), and thromboembolism (6.3%). A case of adrenocortical insufficiency has now been documented in Japan, involving a 59-year-old man who had taken the drug for 10 years (3). 

Iida H, Miyamoto I, Noda Y, Sawaki M, Nagai Y. Adrenocortical insufficiency associated with long-term high-dose fosfestrol therapy for prostatic carcinoma. Intern Med 1999 38(10) 804-7. 

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