Hiperplasia suprarrenal macronodular bilateral independiente de ACTH: caso clínico / ACTH independent bilateral macronodular adrenal hyperplasia: report of one case

ACTH Independent cushing syndrome, involving both adrenal glands, represents a minority of endogenous hyperadrenocorticisms and is caused by infrequent diseases. ACTH independent macronodular adrenal hyperplasia (AIMAH) is one of them and is caused by aberrant cortical adrenal receptors expression that are able to stimulate cortisol secretion. We report a 62 years old male, presenting with hypertension, weight gain, urinary lithiasis and osteopenia. The diagnosis of Cushing's syndrome was made with a morning cortisol level after 1 mg nocturnal dexamethasone of 8.2 ug/dL. Urinary free 24 hour cortisol levels were normal, circadian rhythm of cortisol was maintained and ACTH was <5 pg/mL. Abdominal magnetic resonance imaging showed bilateral nodular adrenal enlargement. The functional study, looking for abnormal receptors, showed a potent cortisol secretory response after stimulation with vasopressin.

Doenças benignas e malignas das supra-renais / Benign and malignant diseases of the adrenal

The purpose of this article is to provide the reader with a comprehensive, simplified and didactic vision of the adrenal diseases. The main characteristics of adrenal cortical tumors (aldosterona producing tumors and adrenal cortical tumors), medullary tumors (pheochromocytoma, neuroblastoma, ganglioneuroma and ganglioneuroblastoma), stromal tumors, metastatic tumors, as well as benign lesion (adrenal cortical hyperplasia, cysts, psudocysts, granulomas and abcesses) are described. A minimal biochemical assessment which may be more cost-effective is suggested. The role of ultrasonography, computed tomography and magnetic resonance in each of the above situations is emphasized. The surgical removal of the lesion by laparoscopy, lumbotomy or thoraco-laparotomy is the best treatment in almost all patients. The surgical procedures are safe when adequate pre-operative preparation in undertaken. The surgical exploration provides the pathological diagnosis avoiding the need for life-term follow-up with expensive exams and offers the best chance of definitive cure

Adreno-cortical insufficiency

Adrenocortical insufficiency is not an uncommon disorder. It results from decreased corticosteroid hormones in the circulation, either due to complete or partial destruction of adrenal cortex or diseases of the hypothalamus pituitary axis. A variety of causes including infections, autoimmune disorders, tumourous infiltration and iatrogenic factors have been identified. The presentation of adrenocortical insufficiency may be insidious but Addisonian crisis is a potentially lethal condition which should be dealt with properly with rewarding results. A number of laboratory tests, simple and sophisticated have been used and applied to assess the adrenal functions are described. The management of Addisonian crisis and steroid replacement therapy, including their brand and generic names alongwith their relative potencies have also be discussed. It also includes the current concepts, controversies and the management of patients with adrenocortical insufficiency who either become pregnant or undergo major or minor surgery

Insuficiencia supra-renal na infancia associada a severas alteracoes do sistema nervoso central: relato de um caso de adrenoleucodistrofia / Supra-renal failure in infancy associated with severe alterations of central nervous system: a adrenoleukodystrophy case report

E apresentado um caso de adrenoleucodistrofia (ALD), doenca metabolica hereditaria ligada ao sexo e caracterizada por alteracoes no metabolismo de acidos graxos de cadeia muito longa (Very Long-Chain Fatty Acids - VLCFA), manifestando-se clinicamente por insuficiencia adrenal e anormalidades neurologicas progressivas. Sao discutidas a forma de apresentacao da doenca e o seu diagnostico, sendo enfatizada a importancia de ter-se presente a possibilidade de ALD em criancas portadoras de doenca de Addison, porque trata-se de molestia sujeita a intervencao terapeutica, com prevencao e ate regressao dos disturbios neurologicos