What is secondary or tertiary adrenal insufficiency (central adrenal insufficiency)?

The pituitary gland and hypothalamus send hormones from the brain to different parts of the body to monitor and regulate their functions. The pituitary hormone is called adrenocorticotropin (ACTH), while the hypothalamus hormone is corticotropin releasing hormone (CRH). Together, these hormones tell our adrenal glands to make cortisol. 

Cortisol helps the body:

• regulate stress
• control metabolism
• suppress inflammation
• regulate blood pressure and blood sugar
• regulate sleep cycle

Secondary or Tertiary adrenal insufficiency is a condition where the pituitary gland can’t produce ACTH and/or the hypothalamus can’t produce CRH, and so the adrenal glands don’t make enough cortisol. Without treatment, the adrenal glands can shrink and stop working over time.

The good news is that with treatment, most people with secondary or tertiary adrenal insufficiency can live happy and normal lives.

Signs and symptoms of secondary or tertiary adrenal insufficiency

Children with secondary or tertiary adrenal insufficiency may experience:

• Fatigue
• Weakness
• Slow recovery from illness

Severe or lengthy illness or infection can result in vomiting, which may quickly progress to a medical emergency. Children suffering from “adrenal crisis” will experience:

• Low blood pressure
• Low blood sugar
• Lethargy
• Possible loss of consciousness

Causes of secondary or tertiary adrenal insufficiency

Central adrenal insufficiency causes include:

• Congenital disorders (medical conditions that occur at or before birth)
• An underdeveloped or absent pituitary gland
• An underdeveloped hypothalamus
• Certain genetic conditions
• Loss of blood flow to the pituitary gland or hypothalamus
• Certain bacterial or viral infections
• Inflammation of or nearby the pituitary gland
• Tumors within or nearby the pituitary gland or hypothalamus
• Surgeries done on or nearby the pituitary gland or hypothalamus
• Radiation to the pituitary gland, hypothalamus or nearby areas

Testing and diagnosis

Secondary or Tertiary adrenal insufficiency is diagnosed with a special blood test that measures the amount of cortisol in the body.

Additional tests, such as a Cortrosyn stimulation test, may be done to better understand the ability of the body to produce cortisol or ACTH.

Your child’s doctor may also order imaging tests, such as an MRI of the brain.

Treatment for secondary or tertiary adrenal insufficiency

Children with secondary or tertiary adrenal insufficiency will need to take a hydrocortisone tablet once to twice daily. Patients must talk to their doctor prior to stopping their medication or lowering the dose. Any sudden changes to their daily dosage may result in adrenal crisis, which is quickly reversible with a high dose, or “stress dose,” of hydrocortisone given through the blood or by mouth. Stress doses of cortisol replacement are also needed during illness, injury or procedures such as surgery.

In some emergency situations, when your child is sick and unable to tolerate taking the medicine by mouth (i.e. unable to swallow or keep the pills down due to vomiting), you may need to give cortisol as an injection. Families should always have an emergency hydrocortisone injection with them when traveling. Wearing a medical information card or a medic-alert bracelet or tag will also ensure proper emergency treatment for your child.

Follow-up care

Children with secondary or tertiary adrenal insufficiency need regular follow up and blood testing. Monitoring is essential to avoid symptoms of secondary or tertiary adrenal insufficiency and ensure proper growth and development.

Outlook 

With proper diagnosis and treatment, the symptoms of secondary or tertiary adrenal insufficiency can be improved. Children with this chronic condition can live normal lives and participate fully in school and other activities.

Why choose CHOP?

CHOP’s Neuroendocrine Center gives your child access to coordinated care by a team of pediatric specialists who will collaborate on the evaluation and treatment of secondary or tertiary adrenal insufficiency.

To ensure children are closely monitored, whether in our care or at home, we have established programs to educate you on caring for a child at risk of adrenal crisis and more.