[Special studies on endocrine diseases--Addison's disease].
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Addison Disease↗
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[Urinary elimination of 17-ketosteroids and dehydroisoandrosterone in patients with various endocrine diseases (Addison's disease, Cushing's disease, Cushing's syndrome, precocious puberty caused by congenital adrenal hyperplasia, acromegaly)].
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17-Ketosteroids↗
Autoimmunity in Addison's disease.
Addison's disease has a low incidence and is most frequently the result of an autoimmune disease in developed countries. Addison's disease can present as an isolated entity or in combination with other autoimmune diseases: Addison's disease can be part of the distinct polyglandular autoimmune syndromes APS I and II. Autoantibodies in patients with isolated Addison's disease are directed against the enzymes involved in steroid synthesis, P45oc21, P45oscc and P45oc17. Addison's disease, both isolated and in the context of APS II, has been associated with the haplotype HLA-A1, -B8 and DR3. The value of the increased expression of these molecules on adrenocortical cells could point towards an infectious pathogenesis. Given the prevalence, up to 80 %, of autoantibodies in Addison's disease as well as the high predictive value for developing the disease when antibodies are present (41% in three years), we advise screening high-risk populations, such as patients with other autoimmune endocrinopathies or their relatives for the presence of these antibodies. The adrenocortical function of patients positive for antibodies should be followed yearly.
Addison Disease↗
Corticotroph cell hyperplasia in a patient with Addison disease: case report.
A woman with Addison disease developed hyperpigmentation, headache, and nausea despite conventional replacement therapy with cortisone. Excessively elevated plasma adrenocorticotropic hormone (ACTH) with absence of response to administration of corticotropin-releasing factor (CRF), and roentgenological evidence of enlargement of the sella turcica, as well as detection of enlarged pituitary gland on magnetic resonance images, led to a diagnosis of ACTH-producing microadenoma, which was removed by transsphenoidal microsurgery. The specimen obtained at surgery evidenced corticotroph hyperplasia, as demonstrated by immunohistochemical staining for ACTH. Fine structure exhibited densely granulated cells with a few bundles of microfilaments and an abundance of large lysosomal bodies. Surgical removal of the hyperplasia alleviated the patient's symptoms, and hyperpigmentation faded remarkably. Her plasma ACTH level returned to normal, has remained normal for more than 3 years, and responds adequately to CRF administration.
Addison Disease↗
X-linked adrenoleukodystrophy in patients with idiopathic Addison disease.
UNLABELLED: The two main causes of primary adrenal disease are tuberculosis and auto-immune adrenal destruction. The latter is responsible for about 70% of the cases of primary adrenal insufficiency (Addison disease). Commonly referred to as a rare cause of adrenal failure is X-linked adrenoleukodystrophy (ALD), a demyelinating peroxisomal disorder affecting 1: 20,000 Caucasian males. Albeit primary adrenal insufficiency is a rare entity per se, we decided to study patients with idiopathic Addison disease and establish the frequency of ALD as a cause of adrenal insufficiency. The biochemical defect of ALD was found in 5 out of 24 patients. The small number of cases in our series led us to include in our analysis the published results of two other groups of investigators. This analysis indicates that the proportion of cases in which Addison disease is attributable to ALD is age dependent. It is highest when the adrenal insufficiency manifests before 15 years. This study clearly demonstrates that the proportion of ALD in patients presenting primary adrenal insufficiency has been under-estimated. CONCLUSION: Addison disease manifesting during the first decade of life has a high likelihood of being the first sign of X-linked adrenoleukodystrophy.
Addison Disease↗
Familial X-linked Addison disease as an expression of adrenoleukodystrophy (ALD): elevated C26 fatty acid in cultured skin fibroblasts.
Adrenoleukodystrophy (ALD) is a fatal X-linked disorder of very long chain fatty acid (VLCFA) metabolism manifested by disease of the central and peripheral nervous systems and the adrenals. X-linked Addison disease alone, as an expression of ALD, has not been previously reported. We present the results of our study of a unique family among whom clinically apparent Addison disease without neurologic involvement has occurred in affected males, and spastic paraparesis has occurred in female carriers. The presence of ALD was confirmed by VLCFA determination in cultured skin fibroblasts. A comparison group of patients with Addison disease on a putative immunopathogenic basis was normal.
Addison Disease↗
Effect of glucocorticoid replacement therapy on bone mineral density in patients with Addison disease.
OBJECTIVE: To study the influence of glucocorticoid replacement therapy on bone mineral density. DESIGN: Cross-sectional. SETTING: University hospital in the Netherlands. PATIENTS: 91 patients with Addison disease who had been receiving glucocorticoid replacement therapy for a mean of 10.6 years (range, 0.5 to 36.5 years). MEASUREMENTS: Bone mineral density of the lumbar spine and both femoral necks using a dual-energy x-ray absorptiometer and basal serum concentrations of adrenocorticotropin, gonadal hormones, and adrenal androgens. RESULTS: Decreased bone mineral density (less than 2 standard deviations [SD] of the mean value of an age-matched reference population) was found in 10 of 31 men (32%; 95% Cl, 17% to 51%) and in 4 of 60 women (7%; Cl, 2% to 16%). No statistically significant differences were found between men and women with regard to age, duration of glucocorticoid substitution, or glucocorticoid dose, either in absolute quantities or when expressed per kilogram of body weight. However, in men with decreased bone mineral density, the daily hydrocortisone dose per kilogram of body weight (0.43 +/- 0.08 mg/kg; mean +/- SD) was significantly (P = 0.032) higher than in men with normal bone mineral density (0.35 +/- 0.10 mg/kg). After correction for possible confounding variables, a significant linear correlation was found between hydrocortisone dose per kilogram of body weight and bone mineral density of the lumbar spine in the men (regression coefficient, -0.86; Cl, -1.60 to -0.13; P = 0.029) but not in the women. CONCLUSIONS: Long-term treatment with standard replacement doses of glucocorticoids may induce bone loss in men with Addison disease. Adjustment of glucocorticoid therapy to the lowest acceptable dose is mandatory in Addison disease, and regular measurement of bone mineral density may be helpful in identifying men at risk for the development of osteoporosis.
Addison Disease↗
Addison disease in patients treated with glucocorticoid therapy.
Acute adrenal crisis in patients with unrecognized chronic adrenocortical failure is difficult to diagnose and potentially fatal. We describe 2 patients with acute adrenal crisis whose diagnoses were hindered because of concomitant glucocorticoid treatment. Acute adrenal insufficiency is primarily a state of mineralocorticoid deficiency. Prednisolone and prednisone, the most frequently prescribed anti-inflammatory corticosteroid agents, have minimal mineralocorticoid activity. Several conditions that may be treated with pharmacological glucocorticoids are associated with an increased risk of Addison disease. An acute adrenal crisis, against which concurrent glucocorticoid therapy does not confer adequate protection, may develop in such patients.
Acute Disease↗
Delayed diagnosis of Addison's disease.
Addison's disease may be difficult to diagnose because of the non-specificity of the most frequently occurring symptoms. Hyponatraemia, hyperkalaemia, and uraemia are commonly detected in sera from patients with Addison's disease. We report two patients in whom the diagnosis was delayed, despite typical biochemical abnormalities in their sera during initial presentation. A computer search of 4 weeks of biochemical data (9862 requests for electrolytes) indicated that these typical biochemical abnormalities occurred commonly in other patients, increasing the difficulty of the clinical biochemist in detecting Addison's disease. Awareness of this problem, in combination with adequate clinical information, should enable the clinical biochemist to assist in the earlier diagnosis of Addison's disease.
Addison Disease↗
Mitochondrial DNA deletion with Kearns Sayre syndrome in a child with Addison disease.
UNLABELLED: Kearns Sayre syndrome (KSS) is a multisystem disorder with a confounding variety of clinical manifestations, including ocular myopathy, pigmentary retinopathy, heart block and ataxia. Endocrinopathies are common in KSS, including growth hormone deficiency, hypogonadism, diabetes mellitus and hypoparathyroidism. A variety of deletions of mitochondrial DNA (mtDNA) are found in most cases. We report on a 5-year-old boy with Addison disease in whom further investigation revealed a 4.9 kilobase mtDNA deletion and KSS. Later he developed severe lactic acidosis and expired. CONCLUSION: The degree of mutant mtDNA heteroplasmy in various tissues on autopsy did not correlate well with the clinical manifestations, although this may be due at least in part to replacement with other tissue types. Our report is the first of non-autoimmune Addison disease in KSS and patients with KSS should be evaluated for adrenal insufficiency. Early recognition of adrenal insufficiency is crucial to prevent mortality from this cause.
Acidosis, Lactic↗
Addison's disease.
Addison's disease is an uncommon endocrine condition manifested by a variety of nonspecific symptoms, such as malaise, anorexia and nausea. Symptoms usually do not occur until most of the adrenal gland has been destroyed. Autoimmune disease has surpassed tuberculosis as the primary cause of Addison's disease. Nevertheless, tuberculosis still accounts for a significant proportion of cases. The rapid adrenocorticotropic hormone (ACTH) stimulation test is useful for identifying adrenal insufficiency. Maintenance therapy consists of hydrocortisone and fludrocortisone.
Addison Disease↗
The autoimmune basis of adrenocortical destruction in Addison's disease.
Addison's disease is mainly caused by autoimmune destruction of the steroid-producing cells in the adrenal glands. Key enzymes in steroid biosynthesis have been implicated as the major targets of autoantibodies in this disease. The study of B-cell-dependent autoimmune responses reveals two distinguishable forms of Addison's disease. This should permit very accurate diagnosis of the disease and increase future prospects of disease prevention.
Addison Disease↗
Fibromyalgia, chronic fatigue syndrome, and Addison disease.
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Addison Disease↗
Tuberculous Addison's disease.
Addison's disease is a possible diagnosis in any patient who presents with weakness, weight loss, hyperpigmentation, hyponatremia, and hypotension. Laboratory findings, including depressed levels of cortisol and aldosterone, help to confirm the diagnosis. Computed tomography may reveal adrenal calcification and abnormal-sized adrenal glands. In most cases, autoimmune destruction of the adrenal cortex is the cause of Addison's disease; however, as in the patient described here, tuberculosis is a possible cause.
Addison Disease↗
[Primary adrenal insufficiency. Addison's disease].
Addison's disease is due to an insufficiency of corticosuprarrenal hormones to maintain the peripheral needs and its clinical sign is a constitutional syndrome with cutaneous hyperpigmentation and low blood pre-assure. The ethiology has drastically variated in the last century; being the origin almost exclusively tuberculous at the beginning of 1900 and mainly autoimmune actually. Nowadays it is difficult to understand Addison's disease out of the context of autoimmune polyglandular syndromes in view of the frequent association to other endocrinopathies. The present article pretend to realize a vision of the global disease related to these multiple endocrine deficits.
Addison Disease↗
Computed tomographic follow-up in a case of Addison's disease.
Addison's disease of the adrenal glands presents with different pathological findings depending on the stage. In the acute phase the adrenal glands are bilaterally enlarged while in the chronic phase the glands are small and calcified. A case of Addison's disease with follow-up over a period of a year by CT is reported. CT showed the evolution of the adrenal glands from bilateral masses to small calcified glands. The differential diagnostic problems in the acute phase are also discussed.
Addison Disease↗
[Pregnancy in Addison disease and diabetes mellitus].
Pregnancies in patients with Addison's disease and Diabetes mellitus are rarely observed. A case of pregnancy in such patient is reported. The course of pregnancy and the fetal development show, that pregnancy can be finished without great problems, if there is therapeutical full compensation of metabolic derangement by hydrocortisone and insulin. There are less complications due to Addison's disease than to Diabetes mellitus. Thus the management of delivery should be the same as in isolated Diabetes mellitus.
Addison Disease↗