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primary ovarian failure (40% of women before age 50 years), testicular failure (5%), and pernicious anemia (4%) The combination of Addison s disease and hypothyroidism is known as Schmidt s syndrome Tuberculosis as a leading cause of Addison s disease is relatively rare in the United States but common where tuberculosis is more prevalent Bilateral adrenal hemorrhage may occur during sepsis, heparin-associated thrombocytopenia or anticoagulation, or with antiphospholipid antibody syndrome It may occur in association with major surgery or trauma, presenting about 1 week later with pain, fever, and shock It may also occur spontaneously Adrenoleukodystrophy is an X-linked peroxisomal disorder causing accumulation of very long-chain fatty acids in the adrenal cortex, testes, brain, and spinal cord It may present at any age and accounts for one-third of cases of Addison s disease in boys Aldosterone deficiency occurs in 9% Hypogonadism is common Psychiatric symptoms often include mania, psychosis, or cognitive impairment Neurologic deterioration may be severe or mild (particularly in heterozygote women), mimics symptoms of multiple sclerosis, and can occur years after the onset of adrenal insufficiency Rare causes of adrenal insufficiency include lymphoma, metastatic carcinoma, coccidioidomycosis, histoplasmosis, cytomegalovirus infection (more frequent in patients with AIDS), syphilitic gummas, scleroderma, amyloid disease, and hemochromatosis Familial glucocorticoid deficiency is caused by a mutation in the gene encoding the adrenal ACTH receptor Triple A (Allgrove s) syndrome is characterized by variable expression of the following: adrenal ACTH resistance with cortisol deficiency, achalasia, alacrima, nasal voice, and neuromuscular disease of varying severity (hyperreflexia to spastic paraplegia) Cortisol deficiency usually presents in infancy but may not occur until the third decade of life Congenital adrenal hypoplasia causes adrenal insufficiency due to absence of the adrenal cortex; patients may also have hypogonadotropic hypogonadism, myopathy, and high-frequency hearing loss Patients with hereditary defects in adrenal enzymes for cortisol synthesis develop congenital adrenal hyperplasia due to ACTH stimulation The most common enzyme defect is P450c21 (21-hydroxylase) Patients with severely defective P450c21 enzymes manifest deficiency of mineralocorticoids (salt wasting) in addition to deficient cortisol and excessive androgens Women with milder enzyme defects have adequate cortisol but develop hirsutism in adolescence or adulthood and are said to have late-onset congenital adrenal hyperplasia (See Hirsutism section) Isolated hypoaldosteronism can be caused by various conditions Hyporeninemic hypoaldosteronism can be caused by renal tubular acidosis type IV and is commonly seen with diabetic nephropathy, hypertensive nephrosclerosis, tubulointerstitial diseases, and AIDS; hyperkalemia, hyperchloremia, and metabolic acidosis (see 21) are presenting signs Hyperreninemic hypoaldosteronism can be seen in patients with myotonic dystrophy, aldosterone synthase deficiency, and congenital adrenal hyperplasia Hyperkalemia, hypertension, and hypogonadism may present in adulthood in some patients with congenital adrenal hyperplasia (CYP17 defi-. how to read pdf file in asp.net c# Read and Extract PDF Text from C# / VB. NET applications - GemBox
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