Hypopituitarism

[David Baxter PhD]

Hypopituitarism
By Mayo Clinic Staff
Oct. 2, 2008

Hypopituitarism is a disorder in which your pituitary gland fails to produce one or more of its hormones, or doesn't produce enough of them.

The pituitary is a small bean-shaped gland located at the base of your brain, somewhat behind your nose and between your ears. Despite its size, this gland secretes hormones that influence nearly every part of your body.

In hypopituitarism, you have a short supply of one or more of these pituitary hormones. This deficiency can affect any number of your body's routine functions, such as growth, blood pressure and reproduction.

Hypopituitarism is rare. You'll likely need medications to treat it for the rest of your life, but your symptoms of hypopituitarism can be controlled.

Symptoms
Hypopituitarism is often progressive. Although the signs and symptoms can occur suddenly, usually they tend to develop gradually. They are sometimes vague and subtle and may be overlooked for many months or even years.

Signs and symptoms of hypopituitarism vary, depending on which pituitary hormones are deficient. The signs and symptoms may include:

• Fatigue
• Headaches
• Low tolerance for stress
• Muscle weakness
• Nausea
• Constipation
• Weight loss or gain
• A decline in appetite
• Abdominal discomfort
• Sensitivity to cold or difficulty staying warm
• Visual disturbances
• Loss of underarm and pubic hair
• Joint stiffness
• Hoarseness
• Facial puffiness
• Thirst and excess urination
• Low blood pressure
• Lightheadedness when standing

If you're a man, you may also have signs and symptoms such as:

Loss of interest in sexual activity
Erectile dysfunction
Decrease in facial or body hair
If you're a woman, you may develop:

Irregular or no menstrual periods
Infertility
Inability to produce milk for breast-feeding
Children may experience:

Stunted growth
Short stature
Slowed sexual development
When to see a doctor
If you develop signs and symptoms associated with hypopituitarism, see your doctor to determine the cause.

Also, if certain signs or symptoms of hypopituitarism develop suddenly ? a severe headache or visual disturbances, confusion, or a drop in blood pressure ? contact your doctor immediately. Such symptoms could represent sudden bleeding into the pituitary (pituitary apoplexy), which requires prompt medical attention.

Causes
The pituitary gland is part of your endocrine system, which consists of glands that produce hormones that regulate processes throughout your body. The hormones secreted by the pituitary gland help regulate important functions, such as growth, blood pressure and reproduction. For example, the pituitary secretes:

• Growth hormone (GH). This hormone controls bone and tissue growth and maintains the appropriate balance of muscle and fat tissue.
• Anti-diuretic hormone (ADH). By regulating urine production, this hormone manages water balance in your body. A deficiency of ADH results in a condition called diabetes insipidus, causing excess urination and thirst.
• Thyroid-stimulating hormone (TSH). This hormone stimulates your thyroid gland to produce key hormones that regulate your metabolism. Shortage of TSH results in an underactive thyroid gland (hypothyroidism).
• Luteinizing hormone (LH). In men, LH regulates testosterone production. In women, it fosters production of estrogen.
• Follicle-stimulating hormone (FSH). Working in tandem with LH, FSH helps stimulate sperm production in men, and egg development and ovulation in women.
• Adrenocorticotropic hormone (ACTH). This hormone stimulates your adrenal glands to produce cortisol and other hormones. Cortisol helps your body deal with stress and influences many body functions, affecting blood pressure, heart function and your immune system.
• Prolactin. This hormone regulates the development of female breasts, as well as the production of breast milk.

Hypopituitarism is frequently triggered by a tumor of the pituitary gland. As pituitary tumors increase in size, they can compress and damage pituitary tissue, interfering with hormone production. A tumor can also compress the optic nerves, causing visual disturbances.

The cause of hypopituitarism can also be other diseases and events that damage the pituitary, such as:

• Head injuries
• Brain tumor
• Brain surgery
• Radiation treatment
• Autoimmune inflammation (hypophysitis)
• Stroke
• Infections of the brain, such as meningitis
• Tuberculosis
• Infiltrative diseases, such as sarcoidosis, which is an inflammatory disease occurring in various organs; histiocytosis X, in which abnormal cells cause scarring in numerous parts of the body, such as the lungs and bones; and hemochromatosis, which causes excess iron deposition in the liver and other tissues
• Severe loss of blood during childbirth, which may cause damage to the front part of the pituitary gland (Sheehan syndrome, or postpartum hypopituitarism)
• Genetic mutations resulting in impaired pituitary hormone production

Diseases of the hypothalamus, a portion of the brain located just above the pituitary, also can cause hypopituitarism. The hypothalamus produces hormones of its own that directly affect the activity of the pituitary.

In some cases, the cause of hypopituitarism is unknown.

Preparing for your appointment
You're likely to start by first seeing your family doctor or a general practitioner. However, in some cases when you call to set up an appointment you may be referred immediately to a specialist called an endocrinologist.

Here's some information to help you prepare for your appointment and what to expect from your doctor.

• What you can do
• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance to prepare for common diagnostic tests.
• Write down all symptoms and changes you're experiencing, even if they seem unrelated to each other.
• Write down key personal information, including any recent life changes or a noticeable difference in your ability to tolerate stress.
• Make a list of your key medical information, including recent surgical procedures, the names of all medications you're taking and any other conditions for which you've been treated. Your doctor will also want to know about any recent injuries to your head.
• Take a family member or friend along, if possible. Sometimes it can be difficult to soak up all the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions to ask your doctor.

Create a list of questions ahead of your appointment so that you can make the most of your time with your doctor. For hypopituitarism, some basic questions to ask your doctor include:

• What is likely causing my symptoms or condition?
• Other than the most likely cause, what are possible causes for my symptoms or condition?
• What kinds of tests do I need now?
• Is my condition likely temporary or chronic?
• What treatment approach do you recommend?
• If you recommend surgery, what will my recovery be like?
• How long will I need to take medications?
• How will you monitor whether my treatment is working?
• Under what circumstances might my medications need to be adjusted?
• I have these other health conditions. How can I best manage them together?
• Are there any restrictions that I need to follow?
• Should I see a specialist? What will that cost, and will my insurance cover seeing a specialist?
• Is there a generic alternative to the medicine you're prescribing me?
• Are there any brochures or other printed material that I can take home with me? What Web sites do you recommend visiting?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment at any time that you don't understand something.

What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over any points you want to spend more time on. Your doctor may ask:

• What are your symptoms, and when did you first notice them?
• How have your symptoms changed over time?
• Have you noticed any changes in your vision?
• Do you experience severe headaches?
• Has your appearance changed, including your weight or the amount of your body hair?
• Have you lost interest in sex? If you're a woman, has your menstrual cycle changed?
• Are you currently being treated or have you recently been treated for any other medical conditions?
• Have you recently had a baby?
• Have you had any recent head injuries or have you had neurosurgery?
• Have any of your family members been diagnosed with pituitary or hormonal conditions?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to worsen your symptoms?

Tests and diagnosis
If your doctor suspects a pituitary disorder, he or she will likely order several tests to check levels of various hormones in your body. Your doctor may also want to check for hypopituitarism if you've had a recent head injury that might have put you at risk of damage to your pituitary gland.

Blood tests can help detect deficits in hormones as a result of pituitary failure. For example, these tests can identify low levels of thyroid, adrenal or sex hormones, and determine that these low levels are associated with inadequate pituitary hormone production.

Your doctor may also suggest that you go to a specialized endocrine clinic to undergo stimulation or dynamic testing. These tests check your body's secretion of hormones after you've taken certain medications that can stimulate hormone production.

Your doctor may also recommend:

• Brain imaging. A computerized tomography (CT) or magnetic resonance imaging (MRI) scan of your brain can detect a pituitary tumor or other structural abnormality.
• Vision tests. These tests can determine if growth of a pituitary tumor has impaired your sight or visual fields.
• X-ray. In children, an X-ray of the hand and wrist can measure whether the bones are growing normally.

Treatments and drugs
Successful treatment of the underlying condition causing hypopituitarism may lead to a complete or partial recovery of your body's normal production of pituitary hormones. The usual treatment for pituitary tumors is surgery to remove the growth. In some instances, doctors also recommend radiation treatment.

If hormone deficiencies persist after treatment, then you'll need prescriptions of one or more hormone replacement medications. These drugs are considered as "replacement" rather than treatment, because the dosages are set to match the amounts that your body would normally manufacture if it didn't have a pituitary problem. Treatment is usually lifelong.

Hormone replacement medications may include:

• Corticosteroids. These drugs, such as hydrocortisone or prednisone, replace the adrenal hormones that aren't being produced because of an adrenocorticotropic hormone (ACTH) deficiency. You take them by mouth.
• Levothyroxine (Levoxyl, Synthroid, others). This medication replaces deficient thyroid hormone levels caused by low or deficient TSH production.
• Sex hormones. These include testosterone in men and estrogen or a combination of estrogen and progesterone in women. Testosterone is administered through the skin with either a patch, a gel or by injection. Female hormone replacement can be administered with either pills or patches.
• Desmopressin (DDAVP). You take these hormones to replace ADH and to reduce your body's loss of water through frequent urination. Desmopressin is taken as a nasal spray, oral tablet or by injection.
• Growth hormone. Also called somatropin, growth hormone is taken through an injection beneath your skin. It promotes growth, thus producing more normal height in children. Adults with a growth hormone deficiency also may benefit from growth hormone replacement, but they won't become taller.

If you've become infertile, preparations containing LH and FSH, also called gonadotropins, can be administered by injection to stimulate ovulation in women and sperm production in men.

Monitoring and adjusting
A doctor who specializes in endocrine disorders (endocrinologist) may regularly monitor the levels of these hormones in your blood to ensure you're getting adequate ? but not excessive ? amounts of these hormones.

Your doctor will advise you to adjust your dosage of corticosteroids if you become seriously ill or experience major physical stress. During these times, your body would ordinarily produce extra cortisol hormone. The same kind of fine-tuning of dosage may be necessary when you have the flu, experience diarrhea or vomiting, or have surgery or dental procedures. Adjustments in dosage may also be necessary during pregnancy or with marked changes in weight. You may need periodic CT or MRI scans as well to monitor a pituitary tumor or other diseases causing the hypopituitarism.

In case of emergency
Wear a medical alert bracelet or pendant, and carry a special card, notifying others ? in emergency situations, for example ? that you're taking corticosteroids and other medications.