Hemodynamic Instability due to Adrenal Insufficiency after Open Heart Surgery / 대한흉부외과학회지

Unexplained hypotension during a stay in the ICU is not uncommon in patients who underwent major surgery such as open heart surgery. When the cardiac output of patients is low, the heart may be the origin of this problem. If the heart function is normal, then we have to consider adrenal insufficiency as a possible cause of this hypotension. Adrenal insufficiency is a rare condition in the general population, yet patients who are under a stressful condition might experience adrenal insufficiency more frequently. We report here on a case of a patient who was in an unstable postoperative state with adrenal insufficiency after surgery and the patient dramatically recovered after the replacement of hydrocortisone.

Síndrome de Allgrove: reporte de un caso / Allgrove's syndrome: a case report

Introducción. El síndrome de Allgrove, también conocido como síndrome de triple A, es un trastorno autosómico recesivo caracterizado por alacrima, acalasia e insuficiencia adrenal. Allgrove describió la entidad en hermanos con insuficiencia adrenal, acalasia y deficiente producción de lágrima. Actualmente se han agregado al cuadro clínico diversas alteraciones neurológicas y existe una gran variabilidad fenotípica en los casos reportados. El gen responsable ha sido identificado por Tullio-Pullet y col., y es responsable de la producción de una proteína denominada ALADIN. Caso clínico. Se presenta el caso de un paciente de sexo masculino de 7 años de edad con deficiencia de lágrima, atrofia óptica y antecedente de acalasia. Hasta el momento no se ha demostrado insuficiencia adrenal. Conclusión. Esta entidad es rara, algunos síntomas, principalmente los neurológicos y la insuficiencia adrenal, pueden ser tardíos, por lo que el oftalmólogo y el pediatra deben considerarla en pacientes con deficiente producción de lágrima o acalasia, además de datos sistémicos sugestivos de un síndrome.

Introduction. Allgrove syndrome, also known as Triple A syndrome, is an autosomal recessive disorder characterized by adrenocorticotropin hormone (ACTH)-resistant adrenal insufficiency, achalasia and alacrima. Allgrove described siblings with isolated glucocorticoid failure, achalasia and defective tear production. It has also been associated with some neurologic abnormalities and there is a variable clinical presentation. The Triple A gene was identified by Tullio-Pullet et al and it is responsible for the production of a protein called ALADIN. Case report. We report a 7 years old patient with alacrima, optic atrophy and achalasia. Until now he has not had adrenal insufficiency. Conclusion. This entity is rare, some of the symptoms appear later in the development of the disease, including neurologic abnormalities and adrenal insufficiency. The ophtalmologist and the pediatrician must consider it in patients with alacrima or acalasia.

Adrenal Crisis after Off-pump Coronary Artery Bypass Surgery / 대한흉부외과학회지

Addisonian crisis, also commonly referred to as adrenal crisis, occurs when the cortisol produced by the adrenal gland is insufficient to meet the body's needs. Pituitary apoplexy usually occurs as hemorrhagic and ischemic necrosis in the presence of a pre-existing pituitary adenoma, and is a rare sequela of cardiovascular surgery. Most pituitary apoplexy that happens in cardiovascular surgery has been known to be related to harmful effects of the cardiopulmonary bypass. The case presented herein illustrates occult pituitary apoplexy that occurred after off-pump coronary artery bypass grafting. In this patient, the initial signs of addisonian crisis was similar to those of septic shock, and were overlooked. However, once recognized, they were reduced dramatically with standard stress-dose cortisone.

The Development and Study of the Glucocorticoid Receptor Blockade Animal Model / 第二军医大学学报

In order to study the physiological action of glucocorticoid receptor (GR) in intact animal, an animal model of GR depletion was developed in the rat by mifepristone (RU486), an antagonist of GR. Mifepristone 50 mg/kg body weight mixed in polyvinyl alcohol (PVA) was injected intramuscularly every 12 h in order to maintain the plasma mifepristone at the concentration of about 10-6mol/L for 72 h. In the meanwhile, the plasma corticosterone (B) was stabilized at about 15 ?g/dl by unilateral adrenalectomy 3 d before. In this model mifepristone occupied 88.1 ?10.7% of the total GR in hepatic, brain cytosol and thymocytes as measured by the exchange assay with [3H]dexamethasone (Dex) as ligand. Some indexes of glucocorticoid response were measured in the control, model and bilateral adrenalectomized (AdxT) rats. The polymorphonuclear eosinophil leukocyte (PME) count in blood and phospholipase A2(PLA2) activity in serum were decreased and tyrosine aminotransferase (TAT) activity of the liver was elevated in the control rats after stress provoked by epinephrine, but these changes were reversed in the model rats as well as in the AdxT rats. Thus it may be concluded that decrease of GR to about 12% may result in adrenoglucocorticoid insufficiency in spite of the normal functioning adrenal gland and high plasma level of B. So far as we know this is the first success to produce experimental endocrine insufficiency in a receptor depletion animal model and to quantitate the occupational threshold of about 12% of the total GR in rats.