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Pediatric Adrenal Disorders

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Summary

The adrenal glands are located at the top of both kidneys. They release hormones that are important to your child’s health and growth.  Adrenal gland hormones manage blood sugar levels, they regulate the balance of salt, potassium and water in the body; they control the body’s response to stress, and they control sexual maturation during childhood and puberty.

Health-related problems fall into one of two major categories. Some are related to too much of one or more hormones being produced. Others are seen when too little of a hormone is being released.

Symptoms

Symptoms of adrenal dysfunction can vary depending on a child’s age.

Newborns and infants:

  • Dry skin and lips
  • Feeding problems
  • Vomiting
  • Weakness

Older children may complain of:

  • Dizziness
  • Fatigue
  • Stomach or muscle pain
  • Trouble thinking clearly
  • Weakness

They also may have a decreased appetite or weight loss along with a craving for salty food, and they might notice that skin color changes in sun-exposed areas. There also may be a decrease or absence of pubic hair following puberty.

Types

Adrenal carcinomas - cancers of the adrenal gland, are rare in children, especially after the age of 5. The symptoms your child has may change depending on exactly what is happening. Generally there will be a lump, pain or a feeling of fullness in the abdomen. Your child may also complain about pain in his back. 

Symptoms often seen with increased cortisol or other hormone production include:

  • A fatty hump on the back just below the neck, also called a “buffalo hump”
  • A flushed, round face and big cheeks
  • Obesity
  • Short stature and stunted growth
  • The appearance of male characteristics in girls, such as increased body hair, pubic hair, acne, a deep voice and an enlarged clitoris.

If there is increased production of the steroid hormone aldosterone, you will often see symptoms of low potassium in the blood, such as muscle cramps and weakness.

Cushing’s syndrome - occurs when the adrenal glands produce too much cortisol, which is the hormone that is connected to stress. It can be caused by taking certain medications. Less common causes include tumors that may either be cancerous or benign. Symptoms may include:

  • Obesity in the upper body along with a round face and neck, yet thin arms and legs
  • Skin problems including acne or reddish-blue streaks on the abdomen or in the area under their arms
  • High blood pressure
  • Weakness in the muscles and bones
  • Moodiness or depression
  • Increased blood sugar levels
  • Slowing growth rates

Hyperaldosteronism - is when the body makes too much aldosterone. Aldosterone is important in keeping sodium and potassium levels in balance, which controls blood pressure and fluids in the blood.

The main symptom that will probably bring you to your child’s doctor is high blood pressure. Others include muscle cramping and frequent urination, especially during the night, as well as headaches and generalized weakness.

Congenital adrenal hyperplasia (CAH) - a common genetic disorder that is usually seen at birth. It prevents the body from making enough cortisol. Those with CAH may have other hormone imbalances.

CAH has a wide variety of responses. For many, the disease may never be diagnosed because it doesn’t cause notable symptoms. Milder forms may be diagnosed in either children or adults. The more severe forms usually are found during childhood.

Symptoms of the milder form include shorter than average height, early puberty, acne, irregular menstrual periods after puberty, and extra facial hair in women. The more severe form has symptoms that include dehydration, low blood pressure and blood sugars, shorter than average height, early signs of puberty, irregular periods after puberty and excess facial hair in women.
In girls, there may be differences in external genitals. This is usually seen at birth and may need to be corrected through surgery.

Pheochromocytoma - a rare tumor of adrenal gland tissue that causes too much adrenaline to be excreted.  Most of the time these tumors are not cancerous.
The most common symptom is high blood pressure. Others include:

  • Anxiety and/or panic attacks
  • Blurry vision
  • Constipation
  • Headaches
  • High blood sugar
  • Increased heart rate
  • Pain in the abdomen
  • Pale skin
  • Sweating
  • Shaking hands
  • Unplanned or unexplained weight loss

Addison’s disease is a relatively rare disorder that happens when the adrenal glands don’t make cortisol. A lack of aldosterone is also often evident. This is an autoimmune disease in which the immune system mistakenly sees the adrenal glands as an invader and attacks them. This results in damage to the glands and too little cortisol.

There can be some variation in the symptoms depending on the exact cause of the disease. These can include weight loss, weakness, fatigue, nausea and vomiting. Other possible symptoms are a decrease in blood pressure, patches of darkened skin, a craving for salt, dizziness when the child stands up, and depression. Although not directly a disorder of the adrenal glands, some medications can stop (or suppress) their function. This is seen when children are taking steroid medications such as prednisone, hydrocortisone or dexamethasone for asthma or other health problems.

Usually the dose of steroids is tapered off slowly over several weeks and then stopped. If the dosing stops suddenly, the adrenal glands may not be able to get back to full hormone production for several weeks or even months.

Tests and Diagnosis

For Cushing’s syndrome, the diagnosis is based on a review of your child’s medical history, a complete physical examination and laboratory tests. Usually the doctor will order one or more of a group of three screening tests. One looks for cortisol levels in saliva, while another measures the level of cortisol found in urine over a 24-hour period. The third one uses a synthetic steroid known as dexamethasone to see if it stops or lessens cortisol production in the body. X-rays of glands may be used to find or rule out tumors.

In those children where hyperaldosteronism is being considered, your child’s doctor may order blood or urine tests to check for too much aldosterone and too little potassium. The physician will also perhaps order a CT scan to look for noncancerous tumors or other abnormal growths on the adrenal glands.

Congenital adrenal hyperplasia (CAH) is most often diagnosed through a screening test of a newborn, by measuring 17 –hydroxyprogesterone from dried blood spots on filter paper cards. If the first one indicates the possibility of CAH, your child’s doctor usually orders another test using blood serum as confirmation.

A complete medical history and physical examination is often the first step in diagnosing pheochromocytoma. Tests include blood and urine tests measuring adrenaline, noradrenaline and their breakdown products; a CT scan to look for tumors or other abnormal growths and a scan using low-level radioactive tagged substances to see how well the adrenal gland is working. The doctor also may suggest genetic testing.

Testing for Addison’s disease may require a blood test that measures cortisol and adrenocorticotropic (ACTH) levels. Depending on the results, the next step is often ACTH stimulation, in which your child will be given a man-made form of ACTH with blood levels taken both before and after administration. A CT scan may be used to look for any tumors or other abnormal growths on the glands.

Laboratory studies that often are ordered to test for adrenocortical carcinoma include blood tests for glucose, cortisol and other hormones. This helps determine which tumors are functional (meaning they produce hormones) or nonfunctional.

Not every child needs all these tests. Your physician will tell you exactly what the next steps are. Each year, the endocrinology specialists at Children’s Medical Center see more than 12,000 pediatric patients for adrenal disorders and other endocrine-related conditions. We have the skill and resources necessary to provide comprehensive and compassionate care for your child too.

Treatment

The primary treatment for adrenocortical carcinoma is surgery to remove the tumor. Depending on the results, chemotherapy often follows.

Cushing’s syndrome treatment will vary based on the cause. If a prescribed medication is creating the release of extra cortisol, your child’s doctor may change either the dosage of the current medication or try a different medicine. If it is caused by the body making too much of the hormone, then oral medication, surgery or radiation may be used.

Congenital Adrenal Hyperplasia (CAH) is a disease that can’t be cured, but can be controlled. Usually this requires taking medication to replace the hormones the body isn’t making. Your child’s doctor will decide if medicine needs to be taken routinely or only when symptoms occur.

Pheochromocytomas are usually treated by surgically removing the tumor. Most of the time, this improves blood pressure levels.

Replacement of cortisol and/or aldosterone is the preferred treatment for Addison’s disease. Your child may also need to add salt to her diet.

Medications that block the effect of aldosterone are the main treatment for hyperaldosteronism. If there is a noncancerous tumor on only one adrenal gland, surgery to remove that tumor is often effective.

Children’s Medical Center uses a multidisciplinary approach to caring for your child. This allows us to offer our patients care from multiple specialists and experts, in a single appointment, at one location. If your child has symptoms of an adrenal disorder, contact us. We provide the comprehensive and individualized care necessary to put your child back on the path to a healthy life.

FAQs

What are the adrenal glands?

These are glands located at the top of each kidney. They are made up of two sections. The adrenal cortex is responsible for secreting several steroid hormones, including cortisol and aldosterone. The medulla secretes epinephrine.

What is the importance of these hormones?

Among their functions are several that are very important to how well the body works. They help maintain a pregnancy, for example, and they assist your body in handling stress. They keep water, potassium and salt in balance. They also control sexual maturity during childhood and puberty.

What are some of the symptoms of adrenal gland disorders?

The symptoms are largely related to what is happening to the glands. In some instances there may be too little of a hormone being made; in others, too much.  Symptoms of too much cortisol include weight gain, slow growth, acne, bruising, constant and severe fatigue, and upper body obesity. When the gland doesn’t make enough cortisol, it can have an impact on growth, on the normal development of the genitals, and on early puberty.
 
One symptom that will probably bring you to your child’s doctor for an aldosterone   disorder is high blood pressure that is hard to control. Others include muscle cramping, frequent urination, especially during the night; headaches and generalized weakness.

How are adrenal gland disorders diagnosed?

Exactly what your child’s doctor does to diagnose your child depends on which disorder is suspected. Generally, blood or other fluid samples will be taken to look for abnormal levels of hormones. Depending on the results, the next step may be getting CT scans or Magnetic Resonance Imaging (MRI) studies to see if there are any growths on the glands.

How are adrenal gland disorders treated?

As with diagnosis, treatment will depend on the problem’s cause. For many children with low hormone levels, replacement medication may be all that is needed. At high levels, your child’s doctor may prescribe medications to block production of the hormone. Surgery to remove growths on one or both of the glands may also be recommended.

Resources

 

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