Difficulties in diagnosis of addisonian crisis in primary adrenal insufficiency

The observation of a rare case of Addisonian crisis, which developed in 28-year old patient with asymptomatic hypocorticoidism in a stressful situation and was fatal, is described. In patient with left-sided interrupted tubal pregnancy after recovery from anesthesia after a successful laparoscopic tubectomy, shock blood pressure lowering happened, an acute left ventricular heart failure occurred. Addisonian crisis developed under stress conditions (pregnancy, rupture of the fallopian tube, culdocentesis, anesthesia, performed surgery) amid the previously undiagnosed primary adrenal insufficiency due to congenital hypoplasia of the adrenal cortex. During the autopsy there was found hypoplasia of fasciculate and reticular zones of adrenal cortex, which formed only about 40%, while normally form 85% of the adrenal cortex. Under stress (in the presence of normal adrenal glands) fasciculate zone must significantly increase in size (and account for more than 75%), while in patient who was under the severe stress they accounted for only 10-20% of the adrenal cortex. Adrenal insufficiency was not recognized. The patient has no hyperpigmentation and hyperkalemia, which confirms the conclusion of primary adrenal insufficiency. Therapeutic measures were reduced to the administration of low-dose prednisone, sympathomimetic drugs and liquid. Due to the possible existence of forms of the adrenal insufficiency with mild manifestations, there should be an awareness in terms of possible hypocorticoidism in case of development of hemodynamic instability in response to stress, despite intensive infusion therapy and administration of adrenergic drugs. In such cases, it is necessary to administer, perhaps ex juvantibus, high doses of glucocorticoids, which at the correct diagnosis match «as the key to the lock», and at the same time are safe for use. In the treatment of shock of various etiologies, there is a need for wider use of glucocorticoid therapy using high doses of hormones.

congenital adrenal hypoplasia, latent chronic adrenal insufficiency, Addisonian crisis, acute heart failure