Autoimmune polyendocrinopathy syndrome, type I

Autoimmune polyendocrinopathy syndrome, type I Complications

Autoantibodies Immune deficiency Adrenal cortex insufficiency Malabsorption syndrome

Autoimmune polyendocrinopathy syndrome, type I Definition

Autoimmune polyendocrinopathy syndrome, type I is a very unusual autoimmune disorder distinguished by autoimmune polyendocrinopathy (APE), candidiasis (C) and ectodermal dysplasia (ED). Theh disorder is also called as candidiasis-hypoparathyroidism-Addison's disease-syndrome following its major features: * Mild immune deficiency that leads to mucosal and cutaneous infections alog with candida yeasts. The function of the spleen is also reduced or referred to as asplenism. * Parathyroid gland's autoimmune dysfunction that leads to hypocalcemia and the adrenal gland. Other ailments that are related: hypothyroidism hypogonadism and infertility vitiligo (depigmentation of the skin) alopecia (baldness) malabsorption pernicious anemia chronic active (autoimmune) hepatitis In contrast to type 2, this type takes over an autosomal recesive style and it is because of a defect in AIRE or autoimmune regulator a gene that is seen on the 21st chromosomes. A transcription factor, normal function AIRE exhibits to display immune lenience for antigens from endocrine organs.