Adrenal insufficiency in childhood: two cases of a rare condition

Adrenal insufficiency is a rare condition in children, usually presenting as general and gastrointestinal symptoms and skin hyperpigmentation.Symptoms can appear gradually or suddenly as in Addisonian crisis, a life-threatening condition, with hemodynamic and electrolyte disturbances.Autoimmune Polyglandular Syndromes are rarer conditions characterized by two or more autoimmune endocrinopathies.We aimed to evidence the different presentations of adrenal insufficiency and draw attention for its severity Two adolescents were referred to pediatric emergency department with asthenia, anorexia, abdominal pain and vomiting.The first patient was hemodynamically unstable, discolored, dehydrated mucous membranes and cutaneous hyperpigmentation.Analytically showed hyponatremia, hyperkalemia, hypochloremia, hypoglycemia and mild metabolic acidosis.Fluid resuscitation and intravenous glucocorticoids were started, with favorable evolution.The second one presented reasonable general condition, sunken eyes, cutaneous hyperpigmentation, maintaining hemodynamic stability.Analytically showed hyponatremia, hyperkaliemia, hypochloremia, hypoglycemia, decreased cortisol and aldosterone and increased corticotropin and renin, along with adrenal antibodies.Investigation showed increased TSH and normal free T4 and antiperoxidase antibodies.The association of Addison's disease with Hashimoto's Thyroiditis allowed the diagnosis of Autoimmune Polyglandular Syndrome.These cases highlight the rarity of Addison's disease in pediatric age, whose diagnosis shouldn't be delayed as it can be fatal.Clinical suspicion of this uncommon pathology is essential.Despite the same diagnosis, there were different presentations, including an equally rare Polyglandular Syndrome.