Alpha-1 Antitrypsin Flashcards Preview

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Flashcards in Alpha-1 Antitrypsin Deck (14):
1

What is A1AT

An inhibitor for trypsin and neutrophil elastase, both of which are proteases

2

What is a protease

A protein that breaks down other proteins

3

What is A1ATs function in the lungs

Block neutrophil elastase

4

What does neutrophil elastase do

Breaks down elastin

5

What does elastin do

Gives elasticity and strength

6

What is the overall cause of A1AT deficiency in the lungs

COPD (emphysema and chronic bronchitis)

7

Where in the lungs does A1ATD affect

Lower lobes

8

What is the gene that has the code for A1AT

Serpine 1 gene

9

How does A1ATD affect the liver

Misfolded A1AT trapped in endoplasmic reticulum and cause cell death

10

Is it a recessive or dominant disease

Recessive

11

What are symptoms of A1AT in the lungs

SOB, wheeze, mucus and chronic cough

12

What are the liver problems caused

Cirrhosis, build up of toxins, cannot make some coagulation factors, hepatocellular carcinomas and jaundice.

13

Diagnosis

CXR (hyperinflation and damaged tissue), CT, pulmonary function, blood test A1AT deficiency, liver biopsy

14

Treatment

Augmentation therapy (replace A1AT), COPD treatment and standard cirrhosis treatment