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lüll Update on autoimmune polyendocrine syndromes (APS) Betterle C; Zanchetta RActa Biomed 2003[Apr]; 74 (1): 9-33Autoimmune Polyendocrine Syndromes (APS) were initially defined as a multiple endocrine gland insufficiency associated to an autoimmune disease in a patient. Neufeld & Blizzard (1980) suggested a classification of APS, based on clinical criteria only, describing four main types. APS-1 is characterized by presence of chronic candidiasis, chronic hypoparathyroidism, Addison's disease. It is a very rare syndrome interesting young subjects correlating to different mutations of AIRE (AutoImmuneRegulator) gene on chromosome 21. APS-2 is characterized by presence of Addison's disease (always present), autoimmune thyroid diseases and/or type 1 diabetes mellitus. It is a rare syndrome interesting particularly adult females and associated to a genetic pattern of HLA DR3/DR4. Autoimmune thyroid diseases associated to other autoimmune diseases (excluding Addison's disease and/or hypoparathyroidism), are the main characteristics of APS-3. The different clinical combinations of autoimmune diseases not included in the previous groups are characteristics of APS-4. In this paper criteria for defining a disease as autoimmune are presented. Furthermore, the classification, epidemiology, pathogenesis, genetic, animal models, clinical features, laboratory's tests, imaging, therapy, recent progresses in understanding the APS and a detailed analysis of large group of our patients affected by different types of APS are proposed and discussed.|*Autoimmune Diseases/classification/diagnosis/epidemiology/genetics/immunology/therapy[MESH]|*Endocrine System Diseases/classification/diagnosis/epidemiology/genetics/immunology/therapy[MESH]|Addison Disease/diagnosis/genetics/immunology/therapy[MESH]|Adult[MESH]|Animals[MESH]|Autoantibodies/analysis[MESH]|Candidiasis/etiology[MESH]|Child[MESH]|Chromosomes, Human, Pair 21/genetics[MESH]|Chronic Disease[MESH]|Diabetes Mellitus, Type 1/diagnosis/genetics/immunology/therapy[MESH]|Disease Models, Animal[MESH]|Female[MESH]|Genetic Predisposition to Disease[MESH]|HLA Antigens/genetics[MESH]|Hepatitis, Autoimmune[MESH]|Humans[MESH]|Hypogonadism/etiology[MESH]|Hypoparathyroidism/diagnosis/genetics/immunology/therapy[MESH]|Male[MESH]|Prevalence[MESH]|Syndrome[MESH]|Thyroiditis, Autoimmune/diagnosis/genetics/immunology/therapy[MESH] |