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User:Mr. Ibrahem/Amyloidosis

From Wikipedia, the free encyclopedia
Amyloidosis
Classic facial features of AL amyloidosis with bleeding under the skin (bruising) around the eyes[1]
SpecialtyEndocrinology, rheumatology, cardiology
SymptomsFeeling tired, weight loss, swelling of the legs, shortness of breath, bleeding, feeling light headed with standing[2]
Usual onset55–65 years old[2]
TypesLight chain (AL), inflammation (AA), dialysis (Aβ2M), hereditary and old age (ATTR)[2]
CausesGenetic or acquired[3]
Diagnostic methodTissue biopsy[2]
TreatmentSupportive care, directed at the underlying cause, dialysis, organ transplantation[3]
PrognosisImproved with treatment[3]
Frequency3–13 per million per year (AL amyloidosis)[2]
Deaths1 per 1,000 people (developed world)[3]

Amyloidosis is a group of diseases in which abnormal proteins, known as amyloid fibrils, build up in tissue.[4] Symptoms depend on the type and are often variable.[2] They may include diarrhea, weight loss, feeling tired, enlargement of the tongue, bleeding, numbness, feeling faint with standing, swelling of the legs, or enlargement of the spleen.[2]

There are about 30 different types of amyloidosis, each due to a specific protein misfolding.[5] Some are genetic while others are acquired.[3] They are grouped into localized and systemic forms.[2] The four most common types of systemic disease are light chain (AL), inflammation (AA), dialysis (Aβ2M), and hereditary and old age (ATTR).[2]

Diagnosis may be suspected when protein is found in the urine, organ enlargement is present, or problems are found with multiple peripheral nerves and it is unclear why.[2] Diagnosis is confirmed by tissue biopsy.[2] Due to the variable presentation, a diagnosis can often take some time to reach.[3]

Treatment is geared towards decreasing the amount of the involved protein.[2] This may sometimes be achieved by determining and treating the underlying cause.[2] AL amyloidosis occurs in about 3–13 per million people per year and AA amyloidosis in about 2 per million people per year.[2] The usual age of onset of these two types is 55 to 60 years old.[2] Without treatment, life expectancy is between six months and four years.[2] In the developed world about 1 per 1,000 people die from amyloidosis.[3] Amyloidosis has been described since at least 1639.[2]

References[edit]

  1. ^ Hawkins, P (29 April 2015). "AL amyloidosis". Wikilite.com. Archived from the original on 22 December 2015. Retrieved 19 December 2015.
  2. ^ a b c d e f g h i j k l m n o p q Hazenberg, BP (May 2013). "Amyloidosis: a clinical overview" (PDF). Rheumatic Diseases Clinics of North America. 39 (2): 323–45. doi:10.1016/j.rdc.2013.02.012. PMID 23597967. Archived (PDF) from the original on 2021-04-17. Retrieved 2019-04-17.
  3. ^ a b c d e f g Pepys, MB (2006). "Amyloidosis". Annual Review of Medicine. 57: 223–41. doi:10.1146/annurev.med.57.121304.131243. PMID 16409147.
  4. ^ "AL amyloidosis". rarediseases.info.nih.gov. Genetic and Rare Diseases Information Center (GARD). Archived from the original on 24 April 2017. Retrieved 22 April 2017.
  5. ^ Sipe, Jean D.; Benson, Merrill D.; Buxbaum, Joel N.; Ikeda, Shu-ichi; Merlini, Giampaolo; Saraiva, Maria J. M.; Westermark, Per (2014-12-01). "Nomenclature 2014: Amyloid fibril proteins and clinical classification of the amyloidosis". Amyloid. 21 (4): 221–224. doi:10.3109/13506129.2014.964858. ISSN 1744-2818. PMID 25263598.