PATHOPHYSIOLOGY OF THE ADRENAL GLANDS
There are two main forms of the adrenal dysfunction: hyperfunction and hypofunction (fig. 38).
Causes of the adrenal cortex hypofunction.
• Primary - inability of the adrenals to elaborate sufficient quantities of hormones.
• Secondary - low production of adrenal hormones due to the inadequate ACTH formation or release.
Primary total chronic adrenocortical deficiency - Addison's disease
Etiology.
• Idiopathic atrophy (autoimmune).
• Chronic granulomatous diseases:
— tuberculosis, histoplasmosis, coccidioidomycosis, cryptococcosis.
• Bilateral hemorrhage.
• Tumor metastases.
• Amyloidosis.
• Adrenomyeloneuropathy.
• Familial adrenal insufficiency.
• Sarcoidosis.
Clinical signs and symptoms of the chronic total adrenocortical insufficiency (Addison's disease):
• insidious onset of fatigability;
• weakness, anorexia;
Fig. 38
• nausea and vomiting;
• diarrhea, abdominal pains;
• weight loss;
• cutaneous and mucosal pigmentation;
• hypotension;
• occasionally, hypoglycemia;
• personality changes: excessive irritability, restlessness.
Laboratory findings in Addison's disease:
• serum levels of [Na], [Cl], [HCO3] are reduced;
• serum level of [K] is elevated;
• plasma ACTH and associated peptides (beta-lipotropin) are elevated;
• basal levels of cortisol and aldosterone are subnormal and fail to increase following ACTH administration.
Secondary total adrenocortical insufficiency
Etiology of the acute form:
• rapid withdrawal of steroids from patients with adrenal atrophy owing to chronic steroid administration;
• intensification of chronic adrenal insufficiency (e.g. due to sepsis or surgical stress);
• acute hemorrhagic destruction of both adrenal glands:
— anticoagulant therapy or a coagulation disorder;
— septicemia with Pseudomonas or meningococcemia (in children - the Waterhouse-Friderichsen's syndrome).