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At least 757 records · Page 42Linked to original sources
PubMed2006

Akinetic mutism followed by a manic reaction on introduction of steroid replacement for Addison's disease.

Neuropsychiatric changes during exogenous corticosteroid administration are well-recognized. However, reports of neuropsychiatric reactions to corticosteroid replacement for Addison's disease are distinctively rare. We report on a patient with primary adrenocortical insufficiency, initially presenting with depressive symptoms, who developed akinetic mutism followed by acute manic illness shortly after the initiation of steroid replacement. Both disorders occurred with physiological doses of hydrocortisone and resolved spontaneously. The pathogenesis of the above neuropsychiatric reactions is discussed in the context of glucocorticoid receptor-related brain effects of glucocorticoids. In addition, this report points to the need for accurate psychiatric assessment of patients with Addison's disease upon introduction of replacement therapy.

Addison Disease↗
PubMed1995

[A case of Addison's disease complicated by lung carcinoma treated with surgical resection--a case report].

Surgery was successful in a 72-year-old man with Addison's disease complicated by right lower lobe carcinoma of the lung. The patient had been diagnosed Addison's disease 25 years earlier and had been treated with cortisone acetate 25 mg every morning. A tumor shadow was found in the right lower lobe of the lung on chest X-ray. Squamous cell carcinoma was diagnosed by cytological examination. Right lower lobectomy with partial resection of the right diaphragma and lymph-node dissection were performed. The bronchial stump was closed using Sweet's method and covered with a pericardial fat pad. The intra- and post-operative course was uneventful with intravenous administration of adequate doses of hydrocortisone. The patient has been free from the carcinoma for two years.

Addison Disease↗
PubMed1989

Recurrent deep vein thrombosis and Addison's disease in "primary" antiphospholipid syndrome.

We describe a 43-year-old Caucasian man who, after a 10 year history of recurrent deep vein thromboses and pulmonary emboli, was found to have a "lupus anticoagulant" and marked elevation of antibodies to cardiolipin. He subsequently developed skin and buccal pigmentation and biochemical investigations revealed the presence of Addison's disease. The relationship of the Addison's disease to the recurrent thrombotic events in the presence of antibodies to phospholipids is discussed.

Addison Disease↗
PubMed2000

Low dose (1 microg) ACTH test in the evaluation of adrenal dysfunction in pre-clinical Addison's disease.

OBJECTIVE: The presence of 21-hydroxylase autoantibodies (21OHAb) is a marker of adrenal autoimmunity and can be used to identify subjects with pre-clinical Addison's disease. The low-dose (1 microg) ACTH test (LDT) is more sensitive than the high-dose (250 microg) test (HDT) for the diagnosis of pituitary adrenal insufficiency, but no information is available on the use of a LDT in subjects with autoimmune adrenalitis and primary adrenal insufficiency. The aim of our study was to evaluate the clinical use of the LDT in the diagnosis of early adrenocortical dysfunction in patients with adrenal autoantibodies. DESIGN AND METHODS: Firstly, we evaluated the cortisol responses to both a LDT and a HDT in a group of 12 healthy volunteers. We then performed a LDT in 11 subjects positive for 21OHAb, but without clinical signs of Addison's disease identified by screening 920 patients with one or more organ-specific autoimmune diseases. In all cases, the LDT was followed by a sequential HDT which was used as a control test of the sensitivity and specificity of the LDT. RESULTS: In healthy subjects, the peak cortisol levels after the LDT were similar to those after the classical HDT. In 21OHAb-positive subjects, the LDT showed a pathological response in five out of 11 (45%) cases and the diagnostic concordance between the results of the LDT and those of the HDT was 100%. All the five cases with pathological LDT were also positive for adrenal cortex autoantibodies (ACA) and 4/5 had high levels of basal ACTH. One subject with pathological LDT developed clinical Addison's disease 4 months after the test had been performed. CONCLUSIONS: Our study demonstrates that the low dose ACTH test has a high diagnostic sensitivity and specificity for primary adrenal insufficiency and suggests that it can accurately identify subjects with pre-clinical adrenal dysfunction.

Addison Disease↗
PubMed1986

Diurnal rhythms of proopiomelanocortin-derived N-terminal peptide, beta-lipotropin, beta-endorphin and adrenocorticotropin in normal subjects and in patients with Addison's disease and Cushing's disease.

In order to clarify the diurnal pattern of secretion of plasma immunoreactive (IR) proopiomelanocortin (POMC)-derived peptides, IR-N-terminal peptide (Nt), IR-beta-endorphin (Ep), IR-beta-lipotropin (LPH), and IR-ACTH (ACTH) in normal subjects and in patients with Addison's disease and Cushing's disease, we measured these 4 peptides in the same plasma obtained at 0900 h and then every three hours until 0600 h at the next day. All four peptides showed diurnal rhythms with the peaks at 0600 h, and the nadirs of ACTH, LPH, Ep and Nt were at 0000 h, 0000 h, 1800 h and 0300, respectively in normal subjects. In patients with Addison's disease, these four peptides also showed diurnal rhythms with the peaks at 0600 h for ACTH and Ep and at 0900 h for LPH and Nt, and the nadirs at 2100 h for ACTH and Ep and at 0000 h for LPH and Nt. The molar ratios of Ep/ACTH, LPH/ACTH and Nt/ACTH in plasma also presented diurnal variations in normal subjects and in patients with Addison's disease. On the other hand, in patients with Cushing's disease, ACTH, LPH and Nt showed no rhythmicity or change in molar ratios of Ep/ACTH, LPH/ACTH or Nt/ACTH. Only Ep showed diurnal variation. The molar ratios of Ep/ACTH, LPH/ACTH and Nt/ACTH in patients with Cushing's disease were significantly higher than those in normal subjects and in patients with Addison's disease at 0000 h.(ABSTRACT TRUNCATED AT 250 WORDS)

Addison Disease↗
PubMed1991

The HLA-DQ beta non-Asp-57 allele: a predictor of future insulin-dependent diabetes mellitus in patients with autoimmune Addison's disease.

HLA-DR specificities in 72 Addison's (AD) patients and 808 local controls were compared. We confirmed earlier reports that the HLA-DR3 specificity is significantly increased in AD patients. In our study a relative risk of 3.4 chi 2 = 22.5; pc = 0.01) for the disease was calculated. Analysis of HLA-DQB1 alleles in DR4+ Addison's patients with diabetes mellitus (N = 6) and without IDDM (14 of 18 individuals tested) revealed that the HLA-DQw8 allele (DQB1*0302) was significantly increased in AD patients with IDDM (chi 2 = 13.5; p = 0.001); conversely, a clustering of the HLA-DQw7 allele was detected in DR4+ Addison's patients without IDDM. We thus conclude that particular polymorphic alleles corresponding to non-charged amino acids at position 57 of the HLA-DQ beta-chain [non-Asp-57 alleles] are associated with IDDM also in Addison's patients.

Addison Disease↗
PubMed2004

Autoantibodies against 21-hydroxylase and side-chain cleavage enzyme in autoimmune Addison's disease are mainly immunoglobulin G1.

OBJECTIVE: Immunoglobulin G (IgG) antibodies to the steroidogenic enzymes 21-hydroxylase (21OH) and side-chain cleavage enzyme (SCC) are important diagnostic markers for autoimmune Addison's disease and autoimmune polyendocrine syndromes (APS) types I and II. The characterization of autoantibody (IgG) subclasses may reveal information on how tIssue destruction takes place; therefore, IgG subtypes of anti-21OH and anti-SCC antibodies from sera of patients with Addison's disease, APS I and APS II were determined using recombinant 21OH and SCC. METHODS: SCC(51-521) and his-SCC(51-521) were expressed by pET-scc in the Escherichia coli strain BL21 Star (DE3) and inclusion bodies were purified. Full-length, human 21OH fused to an N-terminal 6x histidine affinity tag was expressed in insect cells by using the baculovirus expression system bac-to-bac. Western blots were used to investigate the IgG subtype(s) of the autoantibodies against 21OH and SCC in patients and healthy blood donors. RESULTS: All anti-SCC positive sera (n=10) contained autoantibodies of the IgG1 subclass, while four out of ten also contained IgG3. All anti-21OH positive sera (n=16) had autoantibodies exclusively against IgG1. Sera from 20 healthy subjects did not show any reactivity against 21OH or SCC. CONCLUSIONS: The finding of a predominating IgG1 response against 21OH and SCC may suggest that T helper (Th) cells of the Th1 subclass are involved in destruction of the adrenal cortex in patients with autoimmune Addison's disease.

Addison Disease↗
PubMed1999

Clinical presentation of thyroid dysfunction and Addison's disease in young adults with type 1 diabetes.

In a clinic population of 509 type 1 diabetic patients aged 16-45 years, 5.5% had received treatment for thyroid disorders (20 hypothyroid, three males; eight thyrotoxicosis, four males), and Addison's disease was present in four patients (0.8%, one male). In all patients, type 1 diabetes preceded the diagnosis of the other autoimmune disorder. The clinical presentation of hypothyroidism was usually insidious with few symptoms, although an increased frequency of hypoglycaemic symptoms and/or raised serum cholesterol levels often prompted thyroid function testing. In contrast, the patients with thyrotoxicosis had florid symptoms, weight loss (mean 8.12 kg), palpable goitres, increasing insulin requirements, and low cholesterol levels. Six patients did not achieve remission or had recurrent thyrotoxicosis after oral antithyroid treatment and required 131I or thyroid surgery. A family history of autoimmune disease was present in 25% of patients with thyroid disorders (seven thyrotoxic and one hypothyroid) and in three of the four patients with Addison's disease. In this population of young adult type 1 diabetic patients, appropriate tests for thyroid dysfunction and Addison's disease should be carried out if there is clinical suspicion and/or unexplained changes in diabetic metabolic control or serum cholesterol. Careful follow-up of patients with a family history of these conditions is recommended.

Addison Disease↗
PubMed2006

Addison's disease presenting as male infertility.

OBJECTIVE: To present a case of primary male infertility with severe oligospermia and hyperpigmented skin as the main presenting clinical signs. DESIGN: Case report. SETTING: University-affiliated teaching hospital. PATIENT(S): A 32-year-old male with severe oligospermia and his 31-year-old female partner with normal cycles. INTERVENTION(S): Hydrocortisone, fludrocortisone, methimazole, and eventual IVF. MAIN OUTCOME MEASURE(S): Improved semen analysis, correction of Addison's disease, correction of hyperthyroidism, and ART pregnancy. RESULT(S): Severe oligospermia of 5 million per milliliter with 15% motility and ACTH level of 2,800 pg/mL on presentation. Endocrinology evaluation revealed cortisol of <0.1 microg/dL, and Cortrosyn test showed no response (Addison's disease). The patient was incidentally found to have a thyroid stimulating hormone level of <0.1 microIU/mL, T4 of 9.5 microg/dL, Free T4 Index calculation of 11.6 microg/dL, and quantitative triiodothyronine of 273 ng/dL on presentation. His Addison's disease was managed with hydrocortisone and fludrocortisone, and his hyperthyroidism, with methimazole. Semen parameters increased to 34 million per milliliter with 45% motility and 5% Kruger morphology 1 year after initial presentation. Hormone parameters normalized. Rather than allowing for more time for natural conception or IUI, the couple decided to proceed with IVF because of insurance coverage before semen parameters normalized. CONCLUSION(S): This is the first reported case of Addison's disease presenting as male infertility with hyperpigmentation of the skin being the only other presenting sign. The underlying etiology of the Addison's in this case remains uncertain, but it is presumed because of autoimmunity. Addison's disease may be associated with hyperthyroidism, and hyperthyroidism may have contributed to the oligospermia. This case highlights why men with abnormal semen parameters should be thoroughly evaluated before proceeding with assisted reproduction.

Addison Disease↗
PubMed1984

Addison's disease with adrenal enlargement on computed tomographic scanning. Report on two cases of tuberculosis and review of the literature.

Two patients with Addison's disease related to urogenital tuberculosis had enlargement of one or both adrenal glands detected with computed tomographic scanning. Review of reports of adrenal size on computed tomographic examination suggests that adrenal enlargement in the presence of Addison's disease demands further investigation about the cause of the adrenal insufficiency.

Addison Disease↗
PubMed2004

[Addison's diseases caused by adrenal tuberculosis--case report].

A case of adrenal tuberculosis with acute Addison's disease is described. The disease manifested acute as suprarenal crisis. Metastatic and autoimmunological (antisuprarenal antibodies were not found) etiology was excluded. Computed tomography demonstrated calcifications in both, enlarged adrenals. After antituberculosis treatment and corticosteroid therapy we observed significant improvement in our patient's condition.

Addison Disease↗