Polyglandular Deficiency Syndromes

Polyglandular Deficiency Syndromes 

Definition

Polyglandular deficiency syndromes are disorders characterized by the failure of more than one endocrine gland to make hormones in sufficient quantities for the body to function normally.

Description

the endocrine system is a diverse group of glands located all over the body that work together to regulate the body's metabolic activities. It includes:

There are over a dozen different syndromes that involve failure of more than one endocrine gland.

Causes and symptoms

The cause of polyglandular deficiency syndromes is usually an autoimmune response—a condition in which the body generates antibodies to its own tissues. The immune system may attack one or more glands; however, because of their inter dependence, the destruction of one gland can often lead to the impairment of another. Other causes may include infectious disease; insufficient blood flow to the glands due to an obstruction such as a blood clot; or the presence of a tumor.

Doctors usually group polyglandular deficiency syndromes into three types:

Not all symptoms of any syndrome appear at once or in the same patient.

Diagnosis

Because these diseases evolve over time, the final diagnosis may not appear for years. A family history is very helpful in knowing what to expect. Any single endocrine abnormality should heighten suspicion that there are others, since they so often occur together, both as underproduction and overproduction of hormones. Most hormone levels can be monitored through blood tests. Many of the antibodies that characterize these conditions can also be found by blood testing.

Treatment

Fortunately there are replacements available for all the missing hormones. Careful balancing of them all can provide a reasonably comfortable quality of life for these patients.

Resources

Books

Sherman, Steven I., and Robert F. Gagel. "Disorders Affecting Multiple Endocrine Systems." In Harrison's Principles of Internal Medicine, edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1997.

Key terms

Antibody — A weapon in the body's immune defense arsenal that attacks a specific antigen.

Congenital — Present at birth.

Myasthenia gravis — A disease that causes muscle weakness.

Rubella — German measles.

Syndrome — A collection of abnormalities that occur often enough to suggest they have a common cause.