This site is intended for healthcare professionals
Light micrograph of a human liver stained with hematoxylin and eosin. The hepatocytes are arranged in cords separated by clear areas where hepatic sinusoids showing red blood cells are located.
Alpha-1 Antitrypsin Deficiency: Bridging Care

Assessment

Declaration of sponsorship: Takeda
AATD: Bridging Care – Overview

Please complete the final knowledge assessment and evaluation to earn your CME credit.

1. Patients with which of the following alpha-1 antitrypsin deficiency genotypes have the highest likelihood of developing cirrhosis?

The correct answer is C. Pi*ZZ.

People with the Pi*ZZ genotype have a markedly higher risk of fibrosis, cirrhosis, and primary liver cancer than those with other genetic modifiers.

2. Which of the following is a strong predictor of advanced liver disease in alpha-1 antitrypsin deficiency?

The correct answer is B. Low platelet count.

Low platelet count, in addition to elevated ALT, elevated international normalized ratio (INR), and high BMI, is a predictor of advanced liver disease in patients with alpha-1 antitrypsin deficiency.

3. What should hepatologists consider when monitoring asymptomatic patients with alpha-1 antitrypsin deficiency?

The correct answer is A. Annual lung function tests.

Asymptomatic patients should ideally receive annual full lung function tests, while symptomatic patients should also receive HRCT, basic treatment for lung disease, and be considered for referral for augmentation therapy.

4. Which investigational treatment for liver disease in patients with alpha-1 antitrypsin deficiency has demonstrated improvements in liver health in a phase 2 study?

The correct answer is B. Fazirsiran.

Liver transplantation is currently the only approved treatment for liver disease in alpha-1 antitrypsin deficiency; however, phase 2 study results showed fazirsiran to reduce median liver Z-AAT concentrations and the hepatic PAS+D globule burden compared with placebo and baseline.

5. How likely is it that you will adjust the way in which you screen for liver disease in your patients with alpha-1 antitrypsin deficiency after this symposium?
6. How likely is it that you will adjust the way in which you initiate diagnostic tests for liver disease in your patients with alpha-1 antitrypsin deficiency after this symposium?
7. How likely is it that you will adjust the way in which you collaborate with other specialties when caring for your patients with alpha-1 antitrypsin deficiency after this symposium?
8. Would you be willing to give us further input on your perceptions and experience of this accredited activity in a follow-up 15–20-minute interview?