Autoimmune polyendocrine syndrome classification: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Autoimmune polyendocrine syndrome}} | {{Autoimmune polyendocrine syndrome}} | ||
{{CMG}} | {{CMG}}; {{AE}} | ||
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==Overview== | ==Overview== | ||
==Classification== | ==Classification== | ||
===Type 1=== | |||
=== Type 1 === | |||
{{Seealso|Autoimmune polyendocrine syndrome type 1}} | {{Seealso|Autoimmune polyendocrine syndrome type 1}} | ||
''Autoimmune polyendocrine syndrome, type 1'' is also known as the [[candidiasis]]-[[hypoparathyroidism]]-[[Addison's disease]]-syndrome after its main features: | ''Autoimmune polyendocrine syndrome, type 1'' is also known as the [[candidiasis]]-[[hypoparathyroidism]]-[[Addison's disease]]-syndrome after its main features: | ||
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A EU-funded consortium is currently doing translational research on this condition and has establised w webpage at [http://www.apeced.net EurAPS]. | A EU-funded consortium is currently doing translational research on this condition and has establised w webpage at [http://www.apeced.net EurAPS]. | ||
=== Type 2 === | ===Type 2=== | ||
''Autoimmune polyendocrine syndrome, type 2'' also known as "Schmidt's syndrome". | ''Autoimmune polyendocrine syndrome, type 2'' also known as "Schmidt's syndrome". | ||
Features of this syndrome are: | Features of this syndrome are: | ||
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Some researchers favour splitting this syndrome into three distinct syndromes (numbering 2, 3 and 4), but research evidence for these distinct combinations is not convincing. | Some researchers favour splitting this syndrome into three distinct syndromes (numbering 2, 3 and 4), but research evidence for these distinct combinations is not convincing. | ||
=== XPID === | ===XPID=== | ||
The most serious but rarest form is the ''X-linked polyendocrinopathy, immunodeficiency and [[diarrhea]]''-syndrome, also called [[IPEX]]. Most patients develop diabetes and diarrhea as neonates and many die due to autoimmune activity against many organs. Boys are affected, while girls are carriers and might suffer mild disease. | The most serious but rarest form is the ''X-linked polyendocrinopathy, immunodeficiency and [[diarrhea]]''-syndrome, also called [[IPEX]]. Most patients develop diabetes and diarrhea as neonates and many die due to autoimmune activity against many organs. Boys are affected, while girls are carriers and might suffer mild disease. | ||
=== Other diseases === | ===Other diseases=== | ||
Other diseases featuring polyendocrine autoimmunity: | Other diseases featuring polyendocrine autoimmunity: | ||
* Chromosomal abnormalities ([[Down's syndrome]]) increase the risk of endocrine autoimmunity | * Chromosomal abnormalities ([[Down's syndrome]]) increase the risk of endocrine autoimmunity | ||
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==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
[[Category:Disease]] | [[Category:Disease]] | ||
[[Category:Endocrinology]] | [[Category:Endocrinology]] | ||
[[Category: | [[Category:Endocrinology]] | ||
{{WikiDoc Help Menu}} | {{WikiDoc Help Menu}} | ||
{{WikiDoc Sources}} | {{WikiDoc Sources}} | ||
Revision as of 17:02, 22 July 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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Overview
Classification
Type 1
Template:Seealso Autoimmune polyendocrine syndrome, type 1 is also known as the candidiasis-hypoparathyroidism-Addison's disease-syndrome after its main features:
- A mild immune deficiency, leading to persistent mucosal and cutaneous infections with candida yeasts. There is also decreased function of the spleen (asplenism).
- Autoimmune dysfunction of the parathyroid gland (leading to hypocalcemia) and the adrenal gland (Addison's disease: hypoglycemia, hypotension and severe reactions in disease).
- Other disease associations are:
- hypothyroidism
- hypogonadism and infertility
- diabetes mellitus (type 1)
- vitiligo (depigmentation of the skin)
- alopecia (baldness)
- malabsorption
- pernicious anemia
- chronic active (autoimmune) hepatitis
A EU-funded consortium is currently doing translational research on this condition and has establised w webpage at EurAPS.
Type 2
Autoimmune polyendocrine syndrome, type 2 also known as "Schmidt's syndrome". Features of this syndrome are:
- Addison's disease
- hypothyroidism
- diabetes mellitus (type 1)
- less common associations:
- hypogonadism
- vitiligo
Some researchers favour splitting this syndrome into three distinct syndromes (numbering 2, 3 and 4), but research evidence for these distinct combinations is not convincing.
XPID
The most serious but rarest form is the X-linked polyendocrinopathy, immunodeficiency and diarrhea-syndrome, also called IPEX. Most patients develop diabetes and diarrhea as neonates and many die due to autoimmune activity against many organs. Boys are affected, while girls are carriers and might suffer mild disease.
Other diseases
Other diseases featuring polyendocrine autoimmunity:
- Chromosomal abnormalities (Down's syndrome) increase the risk of endocrine autoimmunity
- POEMS syndrome - the E is for endocrinopathy; the cause is a paraprotein excreted by a plasmacytoma or multiple myeloma; other features are polyneuropathy, organomegaly (hepatomegaly and splenomegaly), M-protein (paraprotein) and skin changes.
- Several very rare diseases.