Clinical manifestations of adrenocorticotropic hormone deficiency are similar to those of primary adrenal insufficiency, except for cutaneous hyperpigmentation and electrolyte disturbances.
Secondary adrenal insufficiency is not associated with a lack of aldosterone, so symptoms and signs of mineralocorticoid deficiency, such as salt craving, postural hypotension, and electrolyte abnormalities, are typically absent.
This life-threatening illness is characterized by extreme fatigue, acute abdominal pain, nausea, vomiting, diarrhea, fever, severe hypotension, and hypoglycemia, and may be irreversible if not treated promptly.
[2] The most common causes of adrenocorticotropic hormone deficiency appear to be related to the pituitary gland and include autoimmune processes, congenital etiologies, and physical trauma.
[6] Patients with lymphocytic hypophysitis or a deficiency in Adrenocorticotropic hormone have been found to have antibodies against a 22 kDalton pituitary protein, which lends support to this theory.
Adrenocorticotropic hormone deficiency can also be part of an atypical Sheehan's syndrome, be linked to an empty sella, and appear after brain tumor radiation therapy.
[12] Blood chemistry tests may reveal mild hypoglycemia, hyponatremia, normal-to-high potassium levels, slight anemia, lymphocytosis, and eosinophilia.
[2] Hypercalcemia can occur in rare cases resulting from increased intestinal absorption as well as decreased renal excretion of calcium caused by glucocorticoid deficiency.
[2] Glucocorticoid replacement dose must be increased when supervising stressful events such as illnesses, trauma, fever, and major surgical or diagnostic procedures.
[17] Cognitive impairment is one of the most serious outcomes of undiagnosed adrenal crises and inadequately adjusted hydrocortisone treatment in adrenocorticotropic hormone deficiency caused by TBX19 mutations.
To avoid brain damage, early diagnosis, close clinical monitoring in specialized centers, and multiple therapeutic education sessions for parents are critical.