Diferencia entre revisiones de «Amyloidosis»
(Created page with "==Background== *accumulation of proteins (amyloid fibrils) in tissues * heart and kidneys most commonly affected organs ==Clinical Features== *Nephrotic syndrome with ki...") |
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==Background== | ==Background== | ||
* | *Accumulation of proteins (amyloid fibrils) in tissues | ||
* | *Heart and kidneys most commonly affected organs | ||
*Causes: | |||
**Primary amyloidosis, hereditary amyloidosis | |||
**Secondary (due to systemic inflammatory condition) | |||
**[[ESRD]]-associated (caused by β2-microglobulin amyloids, not filtered out by dialysis membranes)) | |||
**Senile amyloidosis (causes cardiac amyloid) | |||
==Clinical Features== | ==Clinical Features== | ||
[[File:PMC3495844 1750-1172-7-54-1.png|thumb|Systemic light change amyloidosis: A. Macroglossia with lateral scalloping of the tongue. B. Bilateral periorbital purpura. C. Pseudo athletic appearance secondary to diffuse muscular infiltration. D. Voluminous hepatomegaly due to primary hepatic amyloidosis. E. Diffuse bilateral interstitial lung disease. F. Submandibular gland enlargement. G-H. Localized AL amyloidosis. G. Nodular conjunctival amyloidosis. H. Laryngeal supraglottic amyloid lump.]] | |||
*[[Nephrotic syndrome]] with kidney involvement | *[[Nephrotic syndrome]] with kidney involvement | ||
*Restrictive [[cardiomyopathy]] | *Restrictive [[cardiomyopathy]] | ||
*Sensory and autonomic neuropathies (no CNS involvement) | *Sensory and autonomic neuropathies (no CNS involvement) | ||
** Sensory neuropathy: symmetrical pattern, progresses distal to proximal | **[[numbness|Sensory neuropathy]]: symmetrical pattern, progresses distal to proximal | ||
**Autonomic neuropathy: orthostatic hypotension or nonspecific GI | **Autonomic neuropathy: orthostatic [[hypotension]] or nonspecific GI symptoms | ||
*Hepatomegaly, elevated serum AST and AlkPhos | *[[Hepatomegaly]], elevated serum AST and AlkPhos | ||
*[[Diabetes]] secondary to pancreatic infiltration | *[[Diabetes]] secondary to pancreatic infiltration | ||
*[[Macroglossia]] | *[[Macroglossia]] | ||
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==Evaluation== | ==Evaluation== | ||
* | *Evaluate for other causes of symptoms | ||
*Diagnosis via fat pad biopsy: apple-green birefringence with Congo red stain | |||
==Management== | ==Management== | ||
*Varies depending on underlying etiology and organ systems involved | |||
==Disposition== | ==Disposition== | ||
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==References== | ==References== | ||
<references/> | <references/> | ||
[[Category:Neurology]] | |||
[[Category:Cardiology]] | |||
[[Category:Renal]] | |||
Revisión actual - 09:44 24 oct 2020
Background
- Accumulation of proteins (amyloid fibrils) in tissues
- Heart and kidneys most commonly affected organs
- Causes:
- Primary amyloidosis, hereditary amyloidosis
- Secondary (due to systemic inflammatory condition)
- ESRD-associated (caused by β2-microglobulin amyloids, not filtered out by dialysis membranes))
- Senile amyloidosis (causes cardiac amyloid)
Clinical Features
Systemic light change amyloidosis: A. Macroglossia with lateral scalloping of the tongue. B. Bilateral periorbital purpura. C. Pseudo athletic appearance secondary to diffuse muscular infiltration. D. Voluminous hepatomegaly due to primary hepatic amyloidosis. E. Diffuse bilateral interstitial lung disease. F. Submandibular gland enlargement. G-H. Localized AL amyloidosis. G. Nodular conjunctival amyloidosis. H. Laryngeal supraglottic amyloid lump.
- Nephrotic syndrome with kidney involvement
- Restrictive cardiomyopathy
- Sensory and autonomic neuropathies (no CNS involvement)
- Sensory neuropathy: symmetrical pattern, progresses distal to proximal
- Autonomic neuropathy: orthostatic hypotension or nonspecific GI symptoms
- Hepatomegaly, elevated serum AST and AlkPhos
- Diabetes secondary to pancreatic infiltration
- Macroglossia
Differential Diagnosis
Evaluation
- Evaluate for other causes of symptoms
- Diagnosis via fat pad biopsy: apple-green birefringence with Congo red stain
Management
- Varies depending on underlying etiology and organ systems involved