Categories: Gastrointestinal

Why does Alpha-1 Antitrypsin Deficiency cause Cirrhosis?

Alpha-1 antitrypsin is an anti protease that protects the lungs from endogenous elastases, collagenases, and protease. These enzymes are responsible for the break down of proteins.

In smoking patients with early onset liver disease or panlobular emphysema, the diagnosis of  alpha-1 antitrypsin deficiency should be considered.

Why does it cause cirrhosis?

The cirrhosis results from the inability to release the mutated alpha-1 antitrypsin protein which is made in the liver. It accumulates in the liver and results in cellular death and necrosis.

REFERENCES

  1. Laboratory Medicine: The Diagnosis of Disease in the Clinical Laboratory. William E. Winter. Chapter 16: The Liver and Biliary Tract.
  2. Stoller JK, Aboussouan LS. A review of α1-antitrypsin deficiency. Am J Respir Crit Care Med. 2012 Feb 1;185(3):246-59. doi: 10.1164/rccm.201108-1428CI.
Get Medical Pearls directly to your inbox every week!
Weekly posts with high yield medical knowledge, directly to your mailbox!
Dr. C Humphreys

Internal Medicine

Share
Published by
Dr. C Humphreys

Recent Posts

Nocturia and OSA: Mechanism

Nocturia is a common symptom associated with obstructive sleep apnea. [1] Img Cred: Am J…

6 months ago

ASD vs. VSD Murmur Difference

ASD (Atrial Septal Defect) Wide, Fixed split S2 (in contrast to the normal variation in…

6 months ago

Mechanism of a Mixed Apnea

Mixed apneas are characterized by absent respiratory effort and airflow in the first section of…

1 year ago

How Does Hypothyroidism Cause Hypoventilation?

Although rare, the differential diagnosis of hypoventilation and hypercapnia respiratory failure includes hypothyroidism. It is…

1 year ago

Why is Pro-BNP/ BNP lower in Obesity?

B-type natriuretic peptide (BNP) is a hormone created in response to cardiac wall stretch due…

2 years ago

What is Peribronchovascular Distribution on CT imaging?

A common finding described on computed tomography (CT) imaging. A disease with a peribronchovascular distribution…

3 years ago

This website uses cookies.