Familial amyloidosis natural history, complications and prognosis: Difference between revisions
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===Natural History=== | ===Natural History=== | ||
*The symptoms of | *The symptoms of familial amyloidosis usually develop: | ||
* | ** [[Transthyretin|Transthyretin (TTR)]]: After 50 years of age | ||
* | ** [[Apolipoprotein AI]]: Third decade and older | ||
** [[Transthyretin|Apolipoprotein AII]]: Early adulthood | |||
** [[Fibrinogen A alpha-chain associated amyloidosis|Fibrinogen Aa]]: Fourth to fifth decade | |||
** [[Lysozyme]]: Third to fourth decade | |||
** [[Gelsolin]]: Late adulthood | |||
** [[Cystatin C]]: Third to fourth decade | |||
*In amyloidosis, insoluble fibrils of [[amyloid]] are deposited in the organs, causing organ dysfunction and eventually death.<ref name="pmid232272782">{{cite journal |vauthors=Baker KR, Rice L |title=The amyloidoses: clinical features, diagnosis and treatment |journal=Methodist Debakey Cardiovasc J |volume=8 |issue=3 |pages=3–7 |date=2012 |pmid=23227278 |pmc=3487569 |doi= |url=}}</ref> | |||
===Complications=== | ===Complications=== | ||
* | *In patients with amyloidosis, the most frequent complications include:<ref name="pmid26155101">{{cite journal |vauthors=Jerzykowska S, Cymerys M, Gil LA, Balcerzak A, Pupek-Musialik D, Komarnicki MA |title=Primary systemic amyloidosis as a real diagnostic challenge - case study |journal=Cent Eur J Immunol |volume=39 |issue=1 |pages=61–6 |date=2014 |pmid=26155101 |pmc=4439975 |doi=10.5114/ceji.2014.42126 |url=}}</ref> | ||
**[ | **[[Heart failure]] | ||
**[ | **[[Nephrotic syndrome]] | ||
**[ | **[[Hepatomegaly]] | ||
**[[Peripheral neuropathy]] | |||
===Prognosis=== | ===Prognosis=== |
Revision as of 04:52, 21 November 2019
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
OR
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
OR
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
Natural History, Complications, and Prognosis
Natural History
- The symptoms of familial amyloidosis usually develop:
- Transthyretin (TTR): After 50 years of age
- Apolipoprotein AI: Third decade and older
- Apolipoprotein AII: Early adulthood
- Fibrinogen Aa: Fourth to fifth decade
- Lysozyme: Third to fourth decade
- Gelsolin: Late adulthood
- Cystatin C: Third to fourth decade
- In amyloidosis, insoluble fibrils of amyloid are deposited in the organs, causing organ dysfunction and eventually death.[1]
Complications
- In patients with amyloidosis, the most frequent complications include:[2]
Prognosis
- Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [--]%.
- Depending on the extent of the [tumor/disease progression] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent.
- The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
- [Subtype of disease/malignancy] is associated with the most favorable prognosis.
- The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.
References
- ↑ Baker KR, Rice L (2012). "The amyloidoses: clinical features, diagnosis and treatment". Methodist Debakey Cardiovasc J. 8 (3): 3–7. PMC 3487569. PMID 23227278.
- ↑ Jerzykowska S, Cymerys M, Gil LA, Balcerzak A, Pupek-Musialik D, Komarnicki MA (2014). "Primary systemic amyloidosis as a real diagnostic challenge - case study". Cent Eur J Immunol. 39 (1): 61–6. doi:10.5114/ceji.2014.42126. PMC 4439975. PMID 26155101.