Micromolar: Abbreviated as μM, it is used to designate the amounts of alpha-1 antitrypsin protein when serum levels are tested. A person is considered deficient in AAT protein when their serum level is 11 μM or below.
Panniculitis: Panniculitis is an inflammation within the layers of fat beneath the skin which causes the skin to may harden and form extremely painful lumps, patches, or lesions. It is likely that the damage is initiated by destructive action of unrestrained neutrophils. In some patients, damage from panniculitis occur after an incident of trauma to the affected area. It occurs in children as well as adults, and has been linked to the ZZ and MZ phenotypes and possibly other alleles as well.
Phenotype: The specific characteristic or type of ATT protein circulating in your blood; it is genetically determined by the alpha-1 genes received from your mother and father at birth. Other environmental factors may affect these characteristics.
Phlegm: Thick, sticky, stringy mucus secreted by the mucous membrane of the respiratory tract, as during a cold or other respiratory infection.
Pneumonia: An acute or chronic disease marked by inflammation of the lungs and caused by viruses, bacteria, or other microorganisms and sometimes by physical and chemical irritants.
Portal Hypertension: Blood flows from veins in the stomach, intestines, spleen and pancreas and goes into the liver through the portal vein. When the liver is diseased and unable to function properly, this blood flow is impaired, and pressure builds in the portal vein, which can cause a number of problems. This condition is known as portal hypertension.
Pruritus: Medical term for itching.
Sclerotherapy: A procedure that may be used in the treatment of bleeding from varices in the oesophagus. Intravenous medication is injected directly into the enlarged veins to stop the bleeding.
Spleen: An organ that is a part of the lymphatic system in the human body. It functions as the body’s defense mechanism, is involved in the formation and destruction of certain blood cells, and acts as a blood reservoir. Blood from the spleen goes into the liver.
Splenomegaly: Splenomegaly, an enlarged spleen, occurs when the spleen has a disease or when portal hypertension develops due to liver disease.
Sputum: Matter coughed up and usually expelled from the mouth, especially mucus or pus that is expectorated (ejected or spit) in diseases of the air passages.
TPN: Total Parenteral Nutrition (TPN) is the administration of nutritionally-adequate solution intravenously; TPN may become necessary to provide nutrition to individuals with severe liver damage.
Vitamins A, D, E, K: Fat-soluble vitamins that are necessary for proper nutrition and are frequently prescribed as dietary supplements when severe liver disease prevents their absorption into the blood stream.
Alpha-1 antitrypsin is a vital protein produced by the liver to protect the lungs. It provides protection from the harmful effects of infections and inhaled irritants, particularly tobacco smoke. It can be easily measured by a simple blood test.
WhatisAlpha-1AntitrypsinDeficiency?
Alpha-1 antitrypsin deficiency (Alpha-1) is a genetic condition which, after cystic fibrosis, is the commonest genetic disorder in Ireland. It severely affects more than 15,000 people, with another 250,000 carriers also at risk of lung and liver disease on the island of Ireland. It is a proven genetic risk factor for chronic obstructive pulmonary disease (COPD).
HowDoIGetTested?
The Alpha-1 Foundation Ireland provides free testing for Alpha-1 as part of a national screening programme which is funded by the HSE. It is a simple blood test. For more details ring 01-8093871 or email alpha1@rcsi.ie
Glossary of Terms
M – V
Micromolar: Abbreviated as μM, it is used to designate the amounts of alpha-1 antitrypsin protein when serum levels are tested. A person is considered deficient in AAT protein when their serum level is 11 μM or below.
Panniculitis: Panniculitis is an inflammation within the layers of fat beneath the skin which causes the skin to may harden and form extremely painful lumps, patches, or lesions. It is likely that the damage is initiated by destructive action of unrestrained neutrophils. In some patients, damage from panniculitis occur after an incident of trauma to the affected area. It occurs in children as well as adults, and has been linked to the ZZ and MZ phenotypes and possibly other alleles as well.
Phenotype: The specific characteristic or type of ATT protein circulating in your blood; it is genetically determined by the alpha-1 genes received from your mother and father at birth. Other environmental factors may affect these characteristics.
Phlegm: Thick, sticky, stringy mucus secreted by the mucous membrane of the respiratory tract, as during a cold or other respiratory infection.
Pneumonia: An acute or chronic disease marked by inflammation of the lungs and caused by viruses, bacteria, or other microorganisms and sometimes by physical and chemical irritants.
Portal Hypertension: Blood flows from veins in the stomach, intestines, spleen and pancreas and goes into the liver through the portal vein. When the liver is diseased and unable to function properly, this blood flow is impaired, and pressure builds in the portal vein, which can cause a number of problems. This condition is known as portal hypertension.
Pruritus: Medical term for itching.
Sclerotherapy: A procedure that may be used in the treatment of bleeding from varices in the oesophagus. Intravenous medication is injected directly into the enlarged veins to stop the bleeding.
Spleen: An organ that is a part of the lymphatic system in the human body. It functions as the body’s defense mechanism, is involved in the formation and destruction of certain blood cells, and acts as a blood reservoir. Blood from the spleen goes into the liver.
Splenomegaly: Splenomegaly, an enlarged spleen, occurs when the spleen has a disease or when portal hypertension develops due to liver disease.
Sputum: Matter coughed up and usually expelled from the mouth, especially mucus or pus that is expectorated (ejected or spit) in diseases of the air passages.
TPN: Total Parenteral Nutrition (TPN) is the administration of nutritionally-adequate solution intravenously; TPN may become necessary to provide nutrition to individuals with severe liver damage.
Vitamins A, D, E, K: Fat-soluble vitamins that are necessary for proper nutrition and are frequently prescribed as dietary supplements when severe liver disease prevents their absorption into the blood stream.
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Info Centre
What is Alpha-1 Antitrypsin?
Alpha-1 antitrypsin is a vital protein produced by the liver to protect the lungs. It provides protection from the harmful effects of infections and inhaled irritants, particularly tobacco smoke. It can be easily measured by a simple blood test.
What is Alpha-1 Antitrypsin Deficiency?
Alpha-1 antitrypsin deficiency (Alpha-1) is a genetic condition which, after cystic fibrosis, is the commonest genetic disorder in Ireland. It severely affects more than 15,000 people, with another 250,000 carriers also at risk of lung and liver disease on the island of Ireland. It is a proven genetic risk factor for chronic obstructive pulmonary disease (COPD).
How Do I Get Tested?
The Alpha-1 Foundation Ireland provides free testing for Alpha-1 as part of a national screening programme which is funded by the HSE. It is a simple blood test. For more details ring 01-8093871 or email alpha1@rcsi.ie