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Polyglandular Deficiency Syndromes

By Jennifer M. Barker, MD, Children's Hospital Colorado, Division of Pediatric Endocrinology

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Polyglandular deficiency syndromes (also called autoimmune polyglandular syndromes, or polyendocrine deficiency syndrome) are hereditary disorders in which several endocrine glands malfunction simultaneously or sequentially.

Endocrine glands are organs that secrete one or more specific hormones (see Endocrine Glands). The actual cause of the endocrine gland malfunctioning may be related to an autoimmune reaction in which the body's immune defenses mistakenly attack the body’s own cells. Genetic factors and environmental triggers may be involved. Polyglandular deficiency syndromes are classified into three types:

  • Type 1: In this type, which develops in children, the parathyroid and adrenal glands are underactive, which can lead to diabetes, hepatitis, malabsorption of nutrients and weight loss, and hair loss. Affected children are prone to chronic yeast infections as well. Yeast infections may be the first sign of a problem, particularly in children younger than 5 years.

  • Type 2 (also called Schmidt syndrome): In this type, which develops in adults (particularly women), the adrenal and thyroid glands are underactive, although the thyroid gland sometimes becomes overactive. People with type 2 polyglandular deficiency also develop diabetes.

  • Type 3: This type is very similar to type 2, except that the adrenal glands remain normal.

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.

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. Because other endocrine organs may not malfunction for many years, doctors usually do blood tests at regular internals 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 is replacement of any hormones that are deficient. People who are prone to yeast infections may need long-term treatment with an antifungal drug.

* This is the Consumer Version. *