Amyloidosis
Background
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Refers to the extracellular tissue deposition of fibrils
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Primary amyloidosis: is a plasma cell disorder marked by the accumulation of immunoglobulin light chains.
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Secondary amyloidosis: is observed in association with several other conditions, including multiple myeloma, rheumatoid arthritis, & a prolonged antigenic challenge, such as may be produced by chronic infection
Considerations
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Difficult airway: macroglossia, laryngo-tracheo-bronchial tree involvement, stridor
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Aspiration risk
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Multisystem infiltrative disease:
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Cardiovascular:
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Restrictive cardiomyopathy
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Diastolic dysfunction
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Complete heart block
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Risk of sudden death
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Coronary heart disease
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Respiratory: interstitial lung disease
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Renal: nephrotic syndrome & renal failure
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GI: dysphagia, risk of aspiration secondary to autonomic/peripheral neuropathy
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Nervous system: autonomic neuropathy, mixed sensory/motor neuropathy
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Heme: bleeding risk → factor X deficiency, ↓ coagulation factors, platelet dysfunction
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Potential co-existing secondary causes: e.g. multiple myeloma & rheumatoid arthritis
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Medications: steroids/chemotherapy
Goals & Conflicts
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RSI for possible gastroparesis, aspiration risk vs. potential difficult airway
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Prior to neuraxial/regional technique:
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Document pre-existing neurodeficits
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Confirm coagulation profile & clinical assessment of coagulopathy
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Mindful of restrictive cardiomyopathy, hemodynamic instability due to autonomic neuropathy