Diferencia entre revisiones de «Amyloidosis»
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Sin resumen de edición |
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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== | ||
*[[Nephrotic syndrome]] with kidney involvement | *[[Nephrotic syndrome]] with kidney involvement | ||
| Línea 7: | Línea 13: | ||
*Sensory and autonomic neuropathies (no CNS involvement) | *Sensory and autonomic neuropathies (no CNS involvement) | ||
** Sensory neuropathy: symmetrical pattern, progresses distal to proximal | ** 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 | ||
| Línea 15: | Línea 21: | ||
==Evaluation== | ==Evaluation== | ||
* | *Evaluate for other causes of symptoms | ||
*Diagnosis via fat pad biopsy: apple-green birefringence with Congo red stain | |||
==Management== | ==Management== | ||
Revisión del 16:16 3 abr 2017
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
- 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