What is polyglandular failure syndrome?

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Polyglandular failure appears to be an HLA-B8-associated syndrome with a high prevalence of disease in relatives. Immunologic dysfunction resulting from a gene(s) on chromosome 6, in linkage dysequilibrium with the HLA-B8 allele, may be a factor in the pathogenesis of polyglandular failure illnesses. PMID: 314765 [Indexed for MEDLINE]

What is the pathophysiology of polyglandular failure?

Polyglandular failure appears to be an HLA-B8-associated syndrome with a high prevalence of disease in relatives. Immunologic dysfunction resulting from a gene (s) on chromosome 6, in linkage dysequilibrium with the HLA-B8 allele, may be a factor in the pathogenesis of polyglandular failure illnesses. Research Support, U.S. Gov’t, Non-P.H.S.

What are polyglandular deficiency syndromes?

Key Points Polyglandular deficiency syndromes (PDS) involve deficiencies in the function of several endocrine glands, which may occur simultaneously or sequentially. Nonendocrine organs also may be affected. Most cases are autoimmune; triggers are often unknown but may involve viruses or dietary substances.

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What is polyglandular syndrome type 1?

Summary Summary. Autoimmune polyglandular syndrome type 1 is an inherited autoimmune condition that affects many of the body’s organs. Symptoms often begin in childhood or adolescence and may include mucocutaneous candidiasis, hypoparathyroidism, and Addison disease.


More about What is polyglandular failure syndrome?


1. The polyglandular failure syndrome: disease inheritance, HLA type, …

Polyglandular failure appears to be an HLA-B8-associated syndrome with a high prevalence of disease in relatives. Immunologic dysfunction resulting from a gene (s) on chromosome 6, in linkage dysequilibrium with the HLA-B8 allele, may be a factor in the pathogenesis of polyglandular failure illnesses.

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2. Polyglandular Deficiency Syndromes – Endocrine and Metabolic …

Polyglandular deficiency syndromes (PDS) are characterized by sequential or simultaneous deficiencies in the function of several endocrine glands that have a common cause. Etiology is most often autoimmune. Categorization depends on the combination of deficiencies, which fall within 1 of 3 types.

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3. Autoimmune polyglandular syndrome type 2 | Genetic and Rare …

Dec 28, 2015 · Autoimmune polyglandular syndrome type 2 is an autoimmune disorder that affects many hormone-producing glands. It is characterized by the presence of Addison’s disease along with autoimmune thyroid disease and/or type 1 diabetes . [1]

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4. Autoimmune polyglandular syndrome type 1 | Genetic and Rare …

50 rows · Apr 04, 2018 · Autoimmune polyglandular syndrome type 1 is an inherited …

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6. Polyglandular Deficiency Syndromes – MSD Manuals

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.

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7. Polyglandular syndromes type I, II, III – Autoimmune Association

Autoimmune polyglandular syndrome (APS) type 3 is an autoimmune condition that affects the body’s endocrine glands. The syndrome, which typically affects women during middle age, results from failure of the glands to produce their hormones.

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8. Autoimmune Polyglandular Syndromes – PubMed

Apr 10, 2021 · The autoimmune polyglandular syndromes (APS) are clusters of endocrine abnormalities that occur in discreet patterns in subjects with immune dysregulation and that permit treatment and anticipation of associated systemic or other hormonal deficiencies. Three major entities are recognized, APS1, APS2 …

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9. Autoimmune Polyglandular Syndrome Type 1 – NORD (National …

General Discussion. Autoimmune polyglandular syndrome type 1 (APS-1) is a rare and complex recessively inherited disorder of immune-cell dysfunction with multiple autoimmunities. It presents as a group of symptoms including potentially life-threatening endocrine gland and gastrointestinal dysfunctions. Autoimmune disorders occur when antibodies and immune cells …

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