Adrenal insufficiency is a disorder in which the adrenal glands, endocrine organs that sit atop each kidney, fail to produce adequate amounts of hormone. The adrenal glands produce a number of hormones that help in regulating levels of sodium and glucose in the blood, moderate the "fight or flight" response to stress, and affect the development and function of the reproductive system. Both adults and children can suffer from adrenal insufficiency
Causes
Any condition that causes a decrease in adrenal hormones can result in adrenal insufficiency. The adrenal glands can also be affected by disorders originating in the hypothalamus or the pituitary gland, endocrine organs that secrete hormones that regulate adrenal function.
Genetic factors can influence adrenal gland function. Type 1 polyendocrine deficiency is a genetically inherited disorder involving numerous endocrine glands that can result in adrenal insufficiency in children.
Autoimmune disorders, in which the body's own immune system mistakenly attacks the adrenal gland, can damage adrenal tissue and reduce the production of adrenal hormones. The National Endocrine and Metabolic Diseases Information Service states that Addison's disease is a disorder in which up to 90 percent of the adrenal gland has been destroyed by an autoimmune response, thus resulting in adrenal insufficiency.
Effects
Genetically inherited forms of adrenal insufficiency result in symptoms that become apparent in infancy. Hypotension and hypoglycemia can occur. Female children with adrenal insufficiency may show signs of masculinization.
In addition to adrenal effects, children with inherited type 1 polyendocrine deficiency may experience slow sexual development, pernicious anemia, chronic fungal infections and chronic liver disease.
Dr. Jose L. Gonzalez of the University of Hawaii's John A. Burns School of Medicine states that the symptoms of acquired adrenal insufficiency are difficult to diagnose, due to their lack of a specific pattern. Symptoms such as lethargy, dizziness and hypoglycemia may be so subtle as to go unnoticed by a doctor.
The effects of adrenal insufficiency can sometimes manifest during childhood and adolescence as changes in skin color and thickening of nails.
Diagnosis
The diagnosis of adrenal insufficiency is first addressed by taking a thorough medical and family history of the patient. A physical exam is conducted, and if adrenal insufficiency is suspected, laboratory tests will be taken in order to compare the levels of various adrenal hormones in the blood. Additional information can be gained through the use of computerized tomography, or CT scanning, to gain a series of cross sectional views of the adrenal gland itself.
Treatment
Some children with adrenal insufficiency may require fluid replacement and intravenous salt solutions to correct the loss of sodium levels in their blood.
Adrenal insufficiency is treated by replacing the deficient hormones. Synthetic hydrocortisone and fludrocortisones acetate are prescription hormone substitutes that are used when cortisol and aldosterone levels are not being appropriate supplied by the adrenal gland. These hormones may be administered orally or via injection, depending on the severity of the adrenal crisis.
Prognosis
Episodes of acute adrenal insufficiency respond to therapy within 48 hours, according to Gonzales, but those with permanent adrenal damage may have to continue to take hormone replacements throughout their lifetime.
Researchers from All Children's Hospital in The University of South Florida College of Medicine warn that undiagnosed adrenal insufficiency can result in potentially fatal cardiovascular collapse.
References
- Pediatrics; Adrenal insufficiency: Still a Cause of Morbidity and Death in Childhood; Shulman DI; 2007
- National Endocrine and Metabolic Diseases Information Service: Adrenal Insufficiency and Addison's Disease
- University of Hawaii: Case Based Pediatrics For Medical Students and Residents: Adrenal Disorders
