Hypopituitarism is loss of function in an endocrine gland due to failure of the pituitary gland to secrete hormones which stimulate that gland's function. The pituitary gland is located at the base of the brain. Patients diagnosed with hypopituitarism may be deficient in one single hormone, several hormones, or have complete pituitary failure.
Description
The pituitary is a pea-sized gland located at the base of the brain, and surrounded by bone. The hypothalamus, another endocrine organ in the brain, controls the function of the pituitary gland by providing "hormonal orders." In turn, the pituitary gland regulates the many hormones that control various functions and organs within the body. The posterior pituitary acts as a sort of storage area for the hypothalamus and passes on hormones that control function of the muscles and kidneys. The anterior pituitary produces its own hormones which help to regulate several endocrine functions.
In hypopituitarism, something interferes with the production and release of these hormones, thus affecting the function of the target gland. Commonly affected hormones may include:
Gonadotropin deficiency
Replacement of gonadal steroids is common treatment for LH and FSH deficiency. Estrogen for women and testosterone for men will be prescribed in the lowest effective dosage possible, since there can be complications to this therapy. To correct women's loss of libido, small doses of androgens may be prescribed. To restore fertility in men, regular hormone injections may be required. Male and female patients whose hypopituitarism results from hypothalamic disease may be successfully treated with a hypothalamic releasing hormone (GnRH), which can restore gonadal function and fertility.
Thyroid stimulating hormone deficiency
In patients who have hypothyroidism, the function of the adrenal glands will be tested and treated with steroids before administering thyroid hormone replacement.
Adrenocorticotopic hormone deficiency
Hydrocortisone or cortisone in divided doses may be given to replace this hormone deficiency. Most patients require 20 mg or less of hydrocortisone per day.
Growth hormone deficiency
It is essential to treat children suffering from growth hormone deficiency. The effectiveness of growth hormone therapy in adults, particularly elderly adults, is not as well documented. It is thought to help restore normal muscle to fat ratios. Growth hormone is an expensive and cautiously prescribed treatment.
Other hormone deficiencies
If a test calculates normal levels of prolactin, deficiency of the hormone is eliminated as a diagnosis. A TRH (thyrotropin-releasing hormone) simulation test can determine prolactin levels. A number of tests are available to detect ADH levels and to determine diagnosis of diabetes insipidus.
Multiple hormone deficiencies
Patients with multiple hormone deficiencies will show symptoms of one or more specific hormone deficiencies or some of the generalized symptoms listed above.
Panhypopituitarism
The insulin-induced hypoglycemia, or insulin tolerance test, which is used to determine specific hormone deficiencies, is an excellent test to diagnose panhypopituitarism. This test can reveal levels of growth hormone, ACTH (cortisol) and prolactin deficiency. The presence of insufficient levels of all of these hormones is a good indication of complete pituitary failure. Imaging studies and clinical history are also important.
Causes and symptoms
There are three major mechanisms which lead to the development of hypopituitarism. The first involves decreased release of hypothalamic hormones that stimulate pituitary function. The cause of decreased hypothalamic function may be congenital or acquired through interference such as tumors, inflammation, infection, mass lesions or interruption of blood supply. A second category of causes is any event or mass which interrupts the delivery of hormones from the hypothalamus. These may include particular tumors and aneurysms. Damage to the pituitary stalk from injury or surgery can also lead to hypopituitarism.
The third cause of hypopituitarism is damage to the pituitary gland cells. Destroyed cells can not produce the pituitary hormones that would normally be secreted by the gland. Cells may be destroyed by a number of tumors and diseases. Hypopituitarism is often caused by tumors, the most common of which is pituitary adenoma.
Symptoms of hypopituitarism vary with the affected hormones and severity of deficiency. Frequently, patients have had years of symptoms that were nonspecific until a major illness or stress occurred. Overall symptoms may include fatigue, sensitivity to cold, weakness, decreased appetite, weight loss and abdominal pain. Low blood pressure, headache and visual disturbances are other associated symptoms.
Diagnosis
Once the diagnosis of a single hormone deficiency is made, it is strongly recommended that tests for other hormone deficiencies be conducted.
Multiple and general hypopituitarism tests
Physicians should be aware that nonspecific symptoms can indicate deficiency of one or more hormones and should conduct a thorough clinical history. In general, diagnosis of hypopituitarism can be accomplished with a combination of dynamic tests and simple blood tests, as well as imaging exams. Most of these tests can be conducted in an outpatient lab or radiology facility. Magnetic resonance imaging (MRI) exams with gadolinium contrast enhancement are preferred imaging exams to study the region of the hypothalamus and pituitary gland. When MRI is not available, a properly conducted computed tomography scan (CT scan) exam can take its place. These exams can demonstrate a tumor or other mass, which may be interfering with pituitary function.
Treatment
Treatment differs widely, depending on the age and sex of the patient, severity of the deficiency, the number of hormones involved, and even the underlying cause of the hypopituitarism. Immediate hormone replacement is generally administered to replace the specific deficient hormone. Patient education is encouraged to help patients manage the impact of their hormone deficiency on daily life. For instance, certain illnesses, accidents or surgical procedures may have adverse complications due to hypopituitarism.
Treatment of multiple deficiencies and panhypopituitarism
The treatment of hypopituitarism is usually very straightforward, but must normally continue for the remainder of the patient's life. Some patients may receive treatment with GnRH, the hypothalamic hormone. In most cases, treatment will be based on the specific deficiency demonstrated. Patients with hypopituitarism should be followed regularly to measure treatment effectiveness and to avoid overtreatment with hormone therapy. If the cause of the disorder is a tumor or lesion, radiation or surgical removal are treatment options. Successful removal may reverse the hypopituitarism. However, even after removal of the mass, hormone replacement therapy may still be necessary.
Prognosis
The prognosis for most patients with hypopituitarism is excellent. As long as therapy is continued, many experience normal life spans. However, hypopituitarism is usually a permanent condition and prognosis depends on the primary cause of the disorder. It can be potentially life threatening, particularly when acute hypopituitarism occurs as a result of a large pituitary tumor. Morbidity from the disease has increased, although the cause is not known. It is possible that increased morbidity and death are due to overtreatment with hormones. Any time that recovery of pituitary function can occur is preferred to lifelong hormone therapy.
Prevention
There is no known prevention of hypopituitarism, except for prevention of damage to the pituitary/hypothalamic area from injury.
BOOKS
Current Diagnosis. Vol. 9. Ed. Rex B. Conn, et al. Philadelphia: W. B. Saunders Co., 1997.
