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Polyglandular Deficiency Syndromes

(Autoimmune Polyglandular Syndromes; Polyendocrine Deficiency Syndrome)

By

Jennifer M. Barker

, MD, Children's Hospital Colorado, Division of Pediatric Endocrinology

Last full review/revision Mar 2021| Content last modified Mar 2021
CLICK HERE FOR THE PROFESSONAL VERSION

Polyglandular deficiency syndromes are hereditary disorders in which several endocrine glands malfunction simultaneously or sequentially.

Endocrine glands Endocrine Glands The endocrine system consists of a group of glands and organs that regulate and control various body functions by producing and secreting hormones. Hormones are chemical substances that affect... read more are organs that secrete one or more specific hormones. The actual cause of the endocrine gland malfunctioning may be related to an autoimmune reaction Autoimmune Disorders An autoimmune disorder is a malfunction of the body's immune system that causes the body to attack its own tissues. What triggers autoimmune disorders is not known. Symptoms vary depending on... read more in which the body's immune defenses mistakenly attack the body’s own cells. Genetic factors and environmental triggers (such as viral infections, dietary factors, or taking certain drugs) may be involved. Polyglandular deficiency syndromes are classified into three types.

In people with polyglandular deficiency syndromes, symptoms depend on which endocrine organs are affected. The hormone deficiencies do not always appear at the same time and may require a period of years to develop. The deficiencies do not occur in a particular sequence, and not every person will have all of the deficiencies associated with each type.

Symptoms

In people with polyglandular deficiency syndromes, symptoms depend on which endocrine organs are affected.

Diagnosis

  • Blood tests to measure hormone levels

Doctors suspect a polyglandular deficiency syndrome because of the specific symptoms. The diagnosis is confirmed by detecting deficient hormone levels in a sample of blood. Sometimes doctors also measure specific antibodies to look for an autoimmune reaction to the affected gland.

Because other endocrine organs may not malfunction for many years, doctors usually do blood tests at regular intervals in people with hormone deficiencies to ensure that any new deficiency is identified as soon as possible.

Because these syndromes are often inherited, genetic testing of the affected person's relatives may be done.

Treatment

  • Hormone replacement

Treatment is replacement of any hormones that are deficient. Treatment of multiple hormone deficiencies may be more complex than treating deficiency of a single hormone. People who are prone to yeast infections may need long-term treatment with an antifungal drug.

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