Amyloid Flashcards
What is mechanism behind amyloidosis?
Extra-cellular insoluble protein deposition
What is diagnostic?
A positive reaction to congo red staining leading to green birefringement under polarized light
How many different types of protein have been identified?
27
AL is what form of amyloid?
Light chain- leads to proliferation of plasma cells and production of kappa/lambda immunglobulins
What drug can be used to treat heart failure in cardiac AL amyloid
Midodrine- alpha-1-agonist properties
What is the worst prognostic factor of amyloidosis AL?
Heart involvement
What is the outlook when heart involvement is found?
1 year, 9 months from symptomatic heart failure
Name a highly characteristic feature of AL amyloid?
Macroglossia
what anaesthetic consideration in AL amyloid?
Potential for difficult intubation due to laryngeal deposition and macroglossia
trache-bronchial involvement may predispose to?
atelectasis and recurrent pneumonia
what is the classical echo finding in AL amyloid?
Restrictive cardiomyopathy
What can lead to diastolic dysfunction in this setting?
Rapid, brief ventricular filling and abnormal relaxation lead to significant diastolic dysfunction
Characeteristic features of AL cardiac amyloid?
Congestive heart failure, reduced ejection fraction, bi-atrial enlargement, and septal and valvular thickening are characteristic features.
In AL disease, pathogenic mechanisms for bleeding tendency include …
In AL disease, pathogenic mechanisms for bleeding tendency include microvascular fragility, platelet dysfunction, and impaired fibrin formation, in addition to qualitative and quantitative clotting factor deficiencies.
What is the most common acquired factor deficincy
Acquired factor X deficiency is the most common, occurring in approximately a third of patients.
If autonomic neuropathy is present- what may occur in bradycardia?
If autonomic neuropathy is present, the heart will not respond predictably to atropine. Bradycardia may require treatment with β1-agonists or pacing.
In heritable neuropathies, depolarizing neuromuscular blocking agent administration may result?
In heritable neuropathies, depolarizing neuromuscular blocking agent administration may result in a dangerous increase in plasma potassium concentrations, predisposing to cardiac arrhythmias.
Lack of autonomic stimuli can make what difficult to assess?
Lack of autonomic stimuli can make depth of anaesthesia difficult to assess. Electroencephalography or evoked-potential monitoring may be used to reduce the risk of awareness.
in AL amyloid Renal involvement presents with 3
Renal involvement presents with non-specific proteinuria, nephrotic syndrome, or renal insufficiency.
The ECG in amyloidosis is associated with 3
The ECG in amyloidosis is associated with low-voltage QRS complexes, conduction abnormalities, and pseudo-infarction patterns
