Addison's Disease in Children

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Addison's disease is a disorder in which the adrenal glands do not produce enough of the steroid hormones cortisol or aldosterone. Together, these hormones control how the body uses fats, proteins, carbohydrates, sodium and potassium. They also help control how the immune system functions.

 Children diagnosed with Addison’s disease in childhood will have to manage it throughout their lives. This disease is rare, but it shares symptoms with many other medical conditions. If not treated, Addison's disease may lead to:

  • Severe abdominal pain
  • Weakness
  • Low blood pressure
  • Kidney failure
  • Hypovolemic shock

Causes of Addison's Disease

Addison's disease is the result of damage to the outer layer of the adrenal gland. The most common cause of this damage is when the body’s immune system attacks the adrenal gland. Other causes in children include:

  • Genetic factors, such as enzyme defects and familial glucocorticoid insufficiency
  • Medications, such as ketoconazole or etomidate
  • Radiation treatment

Factors that increase your child’s chance of getting Addison's disease include:

  • Having the following autoimmune diseases:
    • Type I diabetes
    • Pernicious anemia
    • Hypoparathyroidism
    • Hypopituitarism
    • Hyperthyroidism or hypothyroidism
    • Myasthenia gravis
  • Family members with autoimmune-caused Addison's disease
  • Long-term steroid medication treatment
  • Hereditary disorders, such as Prader-Willi syndrome and congenital adrenal hyperplasia

Symptoms

The more mild symptoms may appear only when your child is under stress, but common symptoms include:

  • Extreme weakness, fatigue
  • Weight loss
  • Nausea or vomiting
  • Chronic diarrhea
  • Muscle weakness or pain
  • Darkening of freckles, nipples, scars, skin creases, gums, mouth, nail beds, and vaginal lining
  • Emotional changes, especially depression
  • Cognitive impairment or confusion
  • Craving salty foods
  • Abdominal pain
  • Anorexia
  • Amenorrhea (absence of menstruation)

Addisonian crisis, also called adrenal crisis, is a life-threatening complication of the disease. Its symptoms include:

  • Severe abdominal, back or leg pain
  • Fainting
  • Severe low blood pressure
  • Severe dehydration
  • Severe nausea, vomiting and diarrhea
  • Low blood sugar
  • Generalized muscle weakness

Diagnosis & Treatment at UVA

Your child’s doctor will conduct these tests to determine if your child has Addison’s disease:

  • Blood test
  • Urine tests
  • ACTH stimulation test
  • MRI
  • CT scan

Children who have Addison’s disease must take medication that replaces the missing hormones. Your child will need regular blood tests to make sure that the medicine works.  In times of stress, your child may need higher doses. 

Immediate treatment of adrenal crisis includes:

  • Self-injection of dexamethasone, corticosteroid, similar to a natural hormone your adrenal gland produces 
  • Hydrocortisone by IV
  • Normal saline by IV

Your child may need surgery for adrenal tumors or brain tumors causing the disease.