Adrenal disorders insufficiency

Endocrine disorders Primary or secondary adrenal cortical insufficiency (hydrocortisone or cortisone is the drug of choice synthetic analogs may be used in conjunction with mineralocorticoids in infancy, mineralocorticoid supplementation is important) congenital adrenal hyperplasia nonsuppurative thyroiditis hypercalcemia associated with cancer. 

Endocrine disorders (e.g., primary or secondary adrenal cortical insufficiency)... 

Adrenal disorders Glucocorticoids are essential to preserve life in patients with chronic adrenal cortical insufficiency (Addison s disea.se) and are necessary in acute adrenal insufficiency associated with life-threatening shock, infection, or trauma. Glucocorticoids are also used in certain types of congenital adrenal hyperplasia, in which synthesis of abnormal forms of corticosteroids are stimulated by ACTO. In these conditions, administration of a potent synthetic glucocorticoid suppresses ACTH secretion sufficiendy to reduce the synthesis of the abnormal steroids. 

Secondary adrenal insufficiency occurs as a result of a pituitary gland dysfunction whereby decreased production and secretion of ACTH leads to a decrease in cortisol synthesis. Tertiary adrenal insufficiency is a disorder of the hypothalamus that results in decreased production and release of CRH, which, in turn, decreases pituitary ACTH production and release. In contrast to Addison s disease (i.e., primary adrenal insufficiency), aldosterone production is unaffected in the secondary and tertiary forms of the disease. Chronic adrenal insufficiency often has a good prognosis if diagnosed early and treated appropriately. 

Patients with autoimmune adrenal insufficiency may have other autoimmune disorders such as type 1 diabetes mel-litus and autoimmune thyroiditis. 

Endocrine disorders Thyroid hormone therapy in patients with concomitant diabetes mellitus or insipidus or adrenal insufficiency (Addison disease) exacerbates the intensity of their symptoms. 

Autoimmune polyglandular syndrome-Chron c autoimmune thyroiditis may occur in association with other autoimmune disorders. Treat patients with concomitant adrenal insufficiency with replacement glucocorticoids prior to initiation of treatment. Failure to do so may precipitate an acute adrenal crisis when thyroid hormone therapy is initiated. Patients with diabetes mellitus may require upward adjustments of their antidiabetic therapeutic regimens. Nontoxic diffuse goiter or nodular thyroid disease Use caution when administering levothyroxine to patients with nontoxic diffuse goiter or nodular thyroid disease in order to prevent precipitation of thyrotoxicosis. If the serum TSH is already suppressed, do not administer levothyroxine. 

Lower cortisol levels in the face of normal ACTH levels can reflect a relatively decreased adrenal output. Yet under circumstances of classic adrenal insufficiency, there is usually increased ACTH release compared to normal levels. Thus, in PTSD there may be an additional component of feedback on the pituitary that is acting to depress ACTH levels, making them appear normal. Indeed, elevations in ACTH would be expected not only from a reduced adrenal output but also from increased CRF stimulation (Baker et al. 1999 Bremner et al. 1997). On the other hand, the adrenal output in PTSD may be relatively decreased, but not substantially enough to affect ACTH levels, hi any event, the normal ACTH levels in PTSD in the context of the other findings suggest a more complex model of the regulatory influences on the pituitary in this disorder than reduced adrenal insufficiency. 

Substitution therapy for deficiency states acute or chronic adrenal insufficiency, congenital adrenal hyperplasia, and adrenal insufficiency secondary to pituitary insufficiency, nonendocrine disorders arthritis rheumatic carditis allergic, collagen, intestinal tract, liver, ocular, renal, shin diseases bronchial asthma cerebral edema malignancies PO 5-60 mg/day in divided doses. Intra-articular, Intralesional (acetate) 4-100 mg, repeated as needed. Intra-articular, Intralesional (sodium phosphate) 2-30 mg, repeated at 3-day to 3-week intervals, as needed. IM (acetate, sodium phosphate) 4-60 mg a day. 

Substitution therapy in deficiency states acute or chronic adrenai insufficiency, congenital adrenal hyperplasia, and adrenal insufficiency secondary to pituitary insufficiency nonendocrine disorders arthritis rheumatic carditis aiiergic, coiiagen, intestinai tract, liver, ocular, renal, shin diseases bronchiai asthma cerebrai edema maiignancies PO... 

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. 

Infants with salt-losing crisis and adrenal insufficiency in infancy may have adrenal hypoplasia congenita. This can be of two types recessive, for which the cause has not been defined and which affects mostly the fetal zone, and X-linked, which is caused by mutations in the DAX-1 gene, which (with steroidogenic factor-1) controls definitive zone development and steroidogenesis [71]. GC-MS analysis of patients with the disorder show variant patterns from absence of neonatal A5 steroids, appropriate for the recessive form [81], to extremely low cortisol production and transient 11/Lhy-droxylase deficiency, as evidenced through increased THS excretion (Malunowicz, personal communication). 

Hypercalcemia causes central nervous system depression, including coma, and is potentially lethal. Its major causes (other than thiazide therapy) are hyperparathyroidism and cancer with or without bone metastases. Less common causes are hypervitaminosis D, sarcoidosis, thyrotoxicosis, milk-alkali syndrome, adrenal insufficiency, and immobilization. With the possible exception of hypervitaminosis D, these latter disorders seldom require emergency lowering of serum calcium. A number of approaches are used to manage the hypercalcemic crisis. 

Some normal actions and some selected mechanisms of adrenocorticoids are described in this section. Understanding these actions aids the reader in better comprehending the results of adrenal insufficiency and the uses of adrenocorticoids as therapeutic agents in a variety of disorders. 

X-ALD is a clinically heterogeneous disorder that can result in progressive nervous system manifestations, adrenal insufficiency, and in-... 

Adrenal insufficiency is a rare disorder with a prevalence of only 4 to 11 cases per 100,000 population if untreated it is fatal. The disorder is classified as primary,secondary, or tertiary. Primary adrenal insujficiency, also known as... 

Addisons disease, results from progressive destruction or dysfunction of the adrenal glands by a local disease process or systemic disorder (Box 51-12), Worldwide, infectious diseases are the most common cause of primary adrenal insufficiency and include tuberculosis, fongal infections (histoplasmosis and cryptococcosis), and cytomegalovirus infections. Autoimmune adrenalitis accounts for more than 70% of cases reported in the Western world, with adrenal autoantibodies noted in more than 75% of cases,. The adrenal glands are atrophic, with loss of cortical cells but an intact medulla. Almost 50% of patients with autoimmune adrenalitis have an associated autoimmune disease with autoimmune thyroid disease the most common. 

A deficiency of ll -hydroxylase is the second most common form of CAH, with an incidence of 1 per 100,000 births, and is associated with manifestations of virilization, elevated concentrations of plasma androstenedione and DHEA-S, and hypertension. The mineralocorticoid-induced hypertension is caused by an elevation of DOC 11-deoxycortisol concentrations are markedly raised in subjects with this enzyme defect,The major distinguishing characteristic of this disorder from 21-hydroxylase deficiency, besides the elevated plasma concentrations of 11-deoxycortisol, is hypertension elicited by the salt retention caused by increased concentrations of DOC, A deficiency of 3 -hydroxysteroid dehydrogenase-isomerase has been reported and leads to an elevation in the ratio of 17a-hydroxypregnenolone to that of 17a-hydroxyprogesterone and to an increased ratio of DHEA to androstenedione. In severe forms of this rare disorder, female infants have pseudohermaphroditism, and male infants present with incomplete masculinization. Patients with this disorder usually present in early infancy with complete adrenal insufficiency including salt wasting. A late-onset form has also been reported in patients with premature pubarche with hirsutism, acne, and menstrual irregularities. The... 

Late-onset adrenal hyperplasia Cushing s syndrome Virilizing adrenal tumors Adrenocorticoid insufficiency Thyroid disorders Hypothyroidism Hyperthyroidism Pituitary disorders... 

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