Amyloid

Question 1

What is mechanism behind amyloidosis?

Answer 1

Extra-cellular insoluble protein deposition

Question 2

What is diagnostic?

Answer 2

A positive reaction to congo red staining leading to green birefringement under polarized light

Question 3

How many different types of protein have been identified?

Answer 3

27

Question 4

AL is what form of amyloid?

Answer 4

Light chain- leads to proliferation of plasma cells and production of kappa/lambda immunglobulins

Question 5

What drug can be used to treat heart failure in cardiac AL amyloid

Answer 5

Midodrine- alpha-1-agonist properties

Question 6

What is the worst prognostic factor of amyloidosis AL?

Answer 6

Heart involvement

Question 7

What is the outlook when heart involvement is found?

Answer 7

1 year, 9 months from symptomatic heart failure

Question 8

Name a highly characteristic feature of AL amyloid?

Answer 8

Macroglossia

Question 9

what anaesthetic consideration in AL amyloid?

Answer 9

Potential for difficult intubation due to laryngeal deposition and macroglossia

Question 10

trache-bronchial involvement may predispose to?

Answer 10

atelectasis and recurrent pneumonia

Question 11

what is the classical echo finding in AL amyloid?

Answer 11

Restrictive cardiomyopathy

Question 12

What can lead to diastolic dysfunction in this setting?

Answer 12

Rapid, brief ventricular filling and abnormal relaxation lead to significant diastolic dysfunction

Question 13

Characeteristic features of AL cardiac amyloid?

Answer 13

Congestive heart failure, reduced ejection fraction, bi-atrial enlargement, and septal and valvular thickening are characteristic features.

Question 14

In AL disease, pathogenic mechanisms for bleeding tendency include …

Answer 14

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.

Question 15

What is the most common acquired factor deficincy

Answer 15

Acquired factor X deficiency is the most common, occurring in approximately a third of patients.

Question 16

If autonomic neuropathy is present- what may occur in bradycardia?

Answer 16

If autonomic neuropathy is present, the heart will not respond predictably to atropine. Bradycardia may require treatment with β1-agonists or pacing.

Question 17

In heritable neuropathies, depolarizing neuromuscular blocking agent administration may result?

Answer 17

In heritable neuropathies, depolarizing neuromuscular blocking agent administration may result in a dangerous increase in plasma potassium concentrations, predisposing to cardiac arrhythmias.

Question 18

Lack of autonomic stimuli can make what difficult to assess?

Answer 18

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.

Question 19

in AL amyloid Renal involvement presents with 3

Answer 19

Renal involvement presents with non-specific proteinuria, nephrotic syndrome, or renal insufficiency.

Question 20

The ECG in amyloidosis is associated with 3

Answer 20

The ECG in amyloidosis is associated with low-voltage QRS complexes, conduction abnormalities, and pseudo-infarction patterns