Alpha-1-antitrypsin: A Simple Overview of a Complex Protein

Dr. Michell Shiffman of Bon Secours' Liver Institute of Virginia

Dr. Michell Shiffman of Bon Secours' Liver Institute of Virginia

Alpha-1-antitrypsin is a protein made within the liver and secreted into the blood. In the blood this protein is important to inactive certain enzymes which could damage other organs such as the lung. Patients with alpha-1-antitrypsin deficiency have low levels of this protein in the blood because this protein remains trapped within the liver. This causes increasing amounts of this protein to accumulate within the liver and this leads to liver damage and cirrhosis.

Alpha-1-antitrypsin deficiency is a genetic disease. It is not contagious. Two forms of the disease exit.  Total deficiency where very little alpha-1-antitrypsin is in the blood and a partial deficiency where the level of alpha-1-antitrypsin within the blood is about half the normal amount. Patients with total deficiency are at risk to develop either lung disease or cirrhosis. For some reason that is not understood, it is very uncommon to develop both liver and lung disease from total deficiency. Why some patients develop lung disease and others develop liver disease is also not understood.

Patients with partial deficiency do not develop lung disease. Until recently it was also thought that patients with partial deficiency did not develop liver disease either. However, it is now believed that patients with the partial deficiency are at increased risk to develop cirrhosis because their liver is more susceptible to injury from other causes. It is therefore recommended that patients with partial deficiency should not consume alcohol on a regular basis and should avoid medications which could harm the liver.

There is no treatment for alpha-1-antitrypsin deficiency. Patients who develop cirrhosis and liver failure can undergo liver transplant as long as they do not also have significant lung disease.

Dr. Shiffman and April Long, NP at the Liver Institute of Virginia have a vast experience monitoring and caring for patients with alpha-1-antitrypsin deficiency and could educate patients and their families regarding the implications of this disease and how this should be monitored.

+ Find a liver specialist
+ Learn more about the Liver Institute of Virginia

This entry was posted in Bon Secours News, Bon Secours Specialties, Featured Health News, General News, Liver Health, Uncategorized and tagged , , , , , , on by .

About Dr.MLShiffman

Dr. Mitchell Shiffman is the Bon Secours expert on liver disease and liver treatments for Virginia. Prior to joining Bon Secours, Dr. Shiffman was a professor of Medicine at Virginia Commonwealth University (VCU) in Richmond, VA. He was the Chief of the Hepatology Section and Medical Director of the Liver Transplant Program at the VCU Medical Center from 1989 until 2009. During his 20 years at the VCU Medical Center Dr. Shiffman built and directed one of the most respected and productive hepatology programs in the United States. Dr. Shiffman is recognized as one of the world’s leaders in the field of Hepatology. His particular area of research is viral hepatitis. He has been involved with and/or directed numerous clinical trials to develop new and better treatments for viral hepatitis B and C. Dr. Shiffman is a regular speaker on the treatment of viral hepatitis, the management of various liver disorders, and issues related to liver transplantation at regional, national and international society meetings. He has published over 200 articles in medical journals related to the treatment of liver diseases and has edited two books on this topic. In addition, he has contributed to various HCV educational resources including an HCV educational kit. Dr. Shiffman is also a widely recognized expert consultant to pharmaceutical companies on the treatment of liver disease. Dr. Shiffman holds a Bachelor of Arts from State University of New York at Buffalo, a Master of Science in physiology from the University of New Mexico School of Medicine in Albuquerque, N.M. and his Doctor of Medicine from State University of New York Upstate Medical Center in Syracuse, N.Y. He completed his internship and residency in internal medicine and fellowship training in gastroenterology and hepatology at the Medical College of Virginia Hospitals in Richmond, VA. Dr. Shiffman is a member of many professional organizations including: the American College of Gastroenterology, American College of Physicians, the American Gastroenterological Association, American Society for the Study of Liver Diseases, American Society of Transplantation, European Association for the Study of the Liver, International Liver Transplantation Society, Richmond Academy of Medicine, Virginia Medical Society and the Virginia Gastrointestinal Society. He was a member of the Board of Trustees with the American College of Gastroenterology from 2003-2009.

Speak Your Mind