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PubMed1977

[Idiopathic adrenal cortex dystrophy with the clinical picture of Addison's disease].

Clinical and autopsy findings are reported of three cases of Addisons disease due to idiopathic dystrophy of the adrenal cortex as seen in two women, 20 and 37 years of age respectively as well as a boy of 14 years. Idiopathic dystrophy of the adrenal cortex as a cause of Addisons disease has become aware of more and more frequently for some years. Its classification as an auto-aggression disease has been made a subject for discussion. Morphological findings and immunological investigations with three own cases confirm this conception. This disease should be reminded of with regard to the problem of clinically diagnosing it and the fatal prognosis of untreated cases.

Addison Disease↗
PubMed1977

An evaluation of laboratory tests for the detection and differential diagnosis of Cushing's syndrome.

1. Results of tests for the diagnosis of Cushings syndrome of varoius aetiologies are discussed for twenty-five patients in whom the pathology was established by operation or autopsy. 2. Control values for the urinary excretion of free cortisol, 17-OHCS, Porter-Silber chromogens (P-SC) and 17-OS and plasma levels of P-SC are compared with those for normal subjects. 3. The results indicated that urinary values are within the normal range for some patients with Cushing's syndrome. 4. Plasma levels of P-SC in the morning were within the normal range for the majority and elevated for the rest. 5. Some patients showed day-night variation of plasma P-SC but evening values were above the normal range. 6. The expected response for low dosage dexamethasone was found in all patients tested but unexpected responses followed high dosage in some. 7. Plasma 11-OHCS in the five patients tested failed to respond to insulin induced hypoglycaemia. 8. Metyrapone administration and corticotrophin infusion tests had limited usefulness in establishing the aetiology of the disease. The 17-OHCS excretion became raised in the response to corticotrophin and the evaluation was prolonged beyond normal responsiveness.

17-Hydroxycorticosteroids↗
PubMedDate not supplied

[Functional and morphologic investigations of the human adrenal cortex in various diseases].

The paper is devoted to investigations of the human adrenal cortex on autopsy material obtained 1 h after death. The purpose was to study the features of 3 cortical zones in some diseases with relation to sex and age. Quantitative histoenzymological analysis was used, metabolic indices of corticocytes and the cortical microvascular wall in 3 functionally differentiated zones (glomerular, fascicular and reticular) were estimated. Histoenzymological reactions were subjected to cytospectrophotometric analysis with subsequent statistical processing of data array. Age-related features of the adrenal cortex differed considerably among men and women, and were associated with corticocytes as well as with the cortical microvascular wall. There was no age-related decrease in enzymatic activity in all the adrenocortical structures but there was change in the interrelationship among the 3 differentiated zones. In some diseases change of enzymatic activity proper was noted in all the zones however a histotopogram of the cortex typical of a given age, was preserved. Functional and morphological investigations permitted assessment of the rearrangement of intraadrenal homeostasis, reflecting, probably, shifts in the synthesis of the main 3 classes of corticosteroid hormones.

Adrenal Cortex↗
PubMed2001

Autoimmunity in isolated Addison's disease and in polyglandular autoimmune diseases type 1, 2 and 4.

Sera from 300 Italian patients with Addison's disease were collected over a 30 year period. Among these patients, 82% had autoimmune disease, 13% had tuberculosis and 5% had another causal condition. In 59% of the cases, autoimmune disease was associated with the autoimmune manifestations contributing to the description of polyglandular autoimmune disease (PGAD). In PGAD type 1, the disease was associated with chronic candidiasis and/or chronic hypoparathyroidism. In PGAD type 2, the patients had autoimmune thyroid disease and/or diabetes mellitus type 1, and in PGAD type 4, they presented a combination with other autoimmune diseases excluding those previously mentioned. Finally, the autoimmune disease was apparently isolated in 41% of the cases. In addition, patients with these four forms of disease exhibited a different genetic pattern, sex distribution, and age at presentation in addition to minor frequency of autoimmune diseases. Adrenal cortex autoantibodies directed against 21-hydroxylase were common serological markers for these four main clinical forms, showing a very high frequency at clinical onset of adrenal insufficiency. In some patients, steroid-producing cell autoantibodies were also present and correlated with gonadal failure and they recognize of 17alpha-hydroxylase or P450 side chain cleavage enzymes as target antigens.

Addison Disease↗