Polyendocrine deficiency syndrome

Polyendocrine deficiency syndrome

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Overview

The polyendocrine deficiency syndrome is classified into two separate forms, referred to as type I and type II.

Type I occurs in children, and adrenal insufficiency may be accompanied by:

• underactive parathyroid glands
• slow sexual development
• pernicious anemia
• chronic candida infections
• chronic active hepatitis
• hair loss (in very rare cases)

Type II, often called Schmidt's syndrome, usually afflicts young adults. Features of type II may include:

• an underactive thyroid gland
• slow sexual development
• diabetes mellitus
• vitiligo
• loss of pigment on areas of the skin

Scientists think that the polyendocrine deficiency syndrome is inherited because frequently more than one family member tends to have one or more endocrine deficiencies.

References

• Stewart PM. The adrenal cortex. In: Larsen P, ed. Williams Textbook of Endocrinology. 10th ed. Philadelphia: Saunders; 2003: 491–551.
• Chrousos GP. Glucocorticoid therapy. In: Felig P, Frohman L, eds. Endocrinology and Metabolism. 4th ed. New York: McGraw-Hill; 2001: 609–632.
• Miller W, Chrousos GP. The adrenal cortex. In: Felig P, Frohman L, eds. Endocrinology and Metabolism. 4th ed. New York: McGraw-Hill; 2001: 387–524.
• Ten S, New M, Maclaren N. Clinical Review 130: Addison's disease 2001. Journal of Clinical Endocrinology & Metabolism. 2001;86(7):2909–2922.
• Williams GH, Dluhy, RC. Disorders of the adrenal cortex. In: Braunwald E, ed. Harrison's Principles of Internal Medicine. 15th ed. New York: McGraw-Hill Professional; 2001: 2084–2105.