Autoimmune polyglandular syndrome background information

Treatment, Depends on type. Autoimmune polyendocrine syndromes (APSs), also called polyglandular autoimmune journal requires |journal= (help); ^ " Autoimmune polyglandular syndrome type 2 | Genetic and Rare Diseases Information.
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Case of autoimmune polyglandular syndrome type 2: how we uncovered the diagnosis. Statistics from Altmetric.


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    autoimmune polyglandular syndrome type 1

    Published by BMJ. Read the full text or download the PDF:. Log in. Autoimmune polyglandular syndrome type 1 APS1 is an autoimmune condition that results in insufficiencies of multiple endocrine glands.

    Polyglandular Autoimmune Syndrome Type 3 (PAS-3)

    It is also known as autoimmune polyendocrine syndrome type 1, polyendocrinopathy-candidiasis— ectodermal dystrophy APECED , Whitaker syndrome, and candidiasis-hypoparathyroidism—Addison disease syndrome, among its many other names. APS1 is inherited, with women and girls being slightly more likely than men and boys to develop the syndrome. It most often occurs in particular ethnic populations due to consanguinity or the clustering of descendants from a common family founder.

    It is most prevalent in:. It is inherited in an autosomal recessive pattern two copies of an abnormal gene must be present for the syndrome to develop.

    Epidemiology

    Symptoms most often appear in children aged 3—5 years, most cases of APS1 have appeared by early adolescence, and all cases by the time an individual is in their early 30s. If an individual presents with evidence of more than one endocrine deficiency, further testing can be done to confirm autoimmune polyglandular syndrome type 1 APS1 , including:.

    Who gets autoimmune polyglandular syndrome type 1?

    Additional possible blood tests can include testing of testosterone , oestradiol, follicle -stimulating hormone FSH , luteinising hormone LH , prolactin, adrenocorticotropic hormone ACTH , plasma renin activity, electrolyte levels, and a complete blood count. The prognosis can be threatened by: oral or esophageal squamous cell carcinoma, sepsis, fulminant hepatitis, renal failure due to interstitial nephritis or bronchial involvement.

    HISTORICAL BACKGROUND OF TYPE 2 AUTOIMMUNE POLYGLANDULAR SYNDROME

    On average patients live into their forties, but this varies considerably between patients depending on the severity of disorders. Other search option s Alphabetical list. Suggest an update. Summary and related texts.

    Autoimmune Polyendocrine Syndrome, Type II - American Family Physician

    Related genes. Clinical signs. Check this box if you wish to receive a copy of your message. Disease definition A rare, genetic, disease that manifests in childhood or early adolescence with a combination of chronic mucocutaneous candidiasis, hypoparathyroidism and autoimmune adrenal failure.

    https://biosanguisoftkend.tk Clinical description The first manifestation of the disease usually candidiasis occurs in childhood with other manifestations appearing progressively. Diagnostic methods Diagnosis is based on the presence of at least two of the three following pathologies: mucocutaneous candidiasis, hypoparathyroidism and adrenal failure. Differential diagnosis Differential diagnoses include IPEX syndrome and, principally, autoimmune polyendocrinopathy type 2 see these terms.


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    Genetic counseling Transmission is autosomal recessive. Management and treatment Management is essentially symptomatic.