1. Alpha-1 Association - Home Information about this non profit organization as well as the disorder, research, membership and contact Category Health Conditions and Diseases...... To identify those affected by alpha1 antitrypsin deficiency and to improve thequality of their lives through support, education, advocacy, and research. . http://www.alpha1.org/

Alpha-1 Association 815 Connecticut Ave., NW Suite 1200 Washington, DC 20006-4004 Phone: (202) 887-1900 Toll-Free: (800) 521-3025 Fax (202) 887-1964 E-mail info@alpha1.org Alpha-1 Association Mission "To identify those affected by Alpha-1 Antitrypsin Deficiency and to improve the quality of their lives through support, education, advocacy, and research." /* You may give each page an identifying name, server, and channel on the next lines. */var pageName = "";/**** DO NOT ALTER ANYTHING BELOW THIS LINE! ****/var code = ' '; document.write(' '); document.write('>');

2. Understanding Alpha-1 Antitrypsin Deficiency Information on signs and symptoms of this disorder, as well as risk factors and treatment. http://www.nhlbi.nih.gov/health/public/lung/other/antitryp.htm

Understanding Alpha-1 Antitrypsin Deficiency Table of Contents: Introduction What is alpha-1 antitrypsin deficiency? How do normal lungs work? What happens if there isn't enough alpha-1 antitrypsin? ... Can the disorder be treated? Home Page Lung Information for the General Public Introduction Your health care team has made this web page to answer your questions about alpha-1 antitrypsin deficiency. It contains information on signs and symptoms of the disorder as well as risk factors and treatment. When you understand more about alpha-1 antitrypsin deficiency, you will be able to play a more active role in your care. [Return to the Table of Contents] What is alpha-1 antitrypsin deficiency? Alpha-1 antitrypsin is a protein that is made in the liver. The liver releases this protein into the bloodstream. Alpha-1 antitrypsin protects the lungs so they can work normally. Without enough alpha-1 antitrypsin, the lungs can be damaged, and this damage may make breathing difficult. Alpha-1 antitrypsin deficiency is an inherited (passed down from parents) disorder that causes low levels of, or no alpha-1 antitrypsin in the blood. [Return to the Table of Contents] How do normal lungs work?

3. Alpha-1 Antitrypsin Deficiency alpha1 antitrypsin deficiency. 30 years since recognition that it could cause liver disease in children http://www.childliverdisease.org/aatd.html

Alpha-1 Antitrypsin Deficiency 30 years since recognition that it could cause liver disease in children The link between alpha-1 antitrypsin deficiency (AATD) and liver disease in children was first described 30 years ago by Dr E F Freier. Alpha-1 antitrypsin deficiency is the most common genetic cause of liver disease children and of emphysema in adults. It is also the most common genetic disease for which liver transplantation is undertaken in children. What is it? Alpha-1 antitrypsin is a protease (a digestive enzyme that causes the breakdown of protein) inhibitor (PI), genotype MM, which protects tissues from the effects of neutrophil (a type of white blood cell which can ingest and kill bacteria. It is mainly produced in the liver. Work carried out at University College London Medical School and funded by CLDF found that alpha-1 antitrypsin played a role in tissue repair by stimulating fibroblast proliferation and procollagen production. Following tissue injury and inflammation levels of alpha-1 antitrypsin rise sharply three or four fold. (Dickson and Alper 1994) In AATD an abnormal protein is made by the body, genotype ZZ. Studies have suggested that accumulation of this abnormal protein in the liver cell causes inflammation and damage. This is associated with polymerisation, ie where a large number of abnormal alpha-1 antitrypsin molecules become linked together.

4. Alpha-1 Antitrypsin Deficiency alpha1 antitrypsin deficiency Who to Contact alpha-1 antitrypsin deficiency National Association 4220 Old Shakopee Road, Suite 101 Minneapolis, MN 55437-2974 http://www.familyvillage.wisc.edu/lib_alph.htm

Alpha-1 Antitrypsin Deficiency Who to Contact Where to Go to Chat with Others Learn More About It Web Sites ... Search AltaVista for "Alpha-1 Antitrypsin Deficiency" Who to Contact Alpha-1 Antitrypsin Deficiency National Association 4220 Old Shakopee Road, Suite 101 Minneapolis, MN 55437-2974 E-mail: brandley@Alpha1.org Website: http://www.alpha1.org The mission of this association is to improve, through support, education, and research, the quality of life of those affected by alpha1-antitrypsin deficiency. Available services include: offering support and education to patients with A1AD, the general public, and the medical community; acting as a clearinghouse of information to assist healthcare professionals and individuals with A1AD; acting as an advocate for people with A1AD; and aiding in support of research of A1AD. Alpha-1-Antitrypsin Deficiency Registry Cleveland Clinic Foundation Department of Pulmonary Diseases 9500 Euclid Avenue Cleveland, OH 44195 Where to Go to Chat with Others AlphaLink Alpha1 Antitrypsin Deficiency Online Support through mailing lists, web boards, chat rooms, etc.

5. MEDLINEplus: Alpha-1 Antitrypsin Deficiency Topics. alpha1 antitrypsin deficiency. Contents Organizations. Search MEDLINEfor recent research articles on • alpha-1 antitrypsin deficiency. You http://www.nlm.nih.gov/medlineplus/alpha1antitrypsindeficiency.html

Skip navigation Other health topics: A B C D ... List of All Topics Alpha-1 Antitrypsin Deficiency Contents of this page: News From the NIH General/Overviews Coping ... Organizations Search MEDLINE for recent research articles on Alpha-1 Antitrypsin Deficiency You may also be interested in these MEDLINEplus related pages: COPD (Chronic Obstructive Pulmonary Disease) Genetics/Birth Defects Lungs and Breathing Latest News State Begins Screening for Hereditary Disorder (03/19/2003, New York Times Syndicate) From the National Institutes of Health Understanding Alpha-1 Antitrypsin Deficiency (National Heart, Lung, and Blood Institute) General/Overviews Alpha-1 Antitrypsin Deficiency: Frequently Asked Questions (Alpha 1 Association) Guide for the Recently Diagnosed Individual: Alpha-1 Antitrypsin Deficiency (Alpha One Foundation) Clinical Trials ClinicalTrials.gov: alpha 1-Antitrypsin Deficiency (National Institutes of Health) Coping Work Simplification and Energy Conservation (Alpha 1 Association) Diagnosis/Symptoms Understanding PFT's (Pulmonary Function Testing) (Alpha 1 Association) Nutrition Eating Better (Alpha 1 Association) Specific Conditions/Aspects Alpha-1 Related Emphysema (American Lung Association) Liver Disease Information (in Alpha-1 Antitrypsin Deficiency) (Alpha 1 Association) Organizations Alpha 1 Association National Heart, Lung, and Blood Institute

6. Health And Medical Information: Diseases And Conditions, Medical Dictionary, Pro An alpha1 antitrypsin deficiency article with details such as what it is, normal lungs and how they work, smoking, risk factors, how it is inherited, signs, symptoms and treatment. http://www.medicinenet.com/Script/Main/Art.asp?li=MNI&ArticleKey=278

7. OMIM ENTRY 107400 International Lay Advocacy Groups. Understanding alpha1 antitrypsin deficiency, National Heart, Lung, and Blood http://www3.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?107400

8. MEDLINEplus Medical Encyclopedia: Alpha-1 Antitrypsin Deficiency alpha1 antitrypsin deficiency. Causes, incidence, and risk factors Returnto top alpha-1 antitrypsin deficiency results from a genetic defect. http://www.nlm.nih.gov/medlineplus/ency/article/000120.htm

Skip navigation Medical Encyclopedia Other encyclopedia topics: A-Ag Ah-Ap Aq-Az B-Bk ... Z Alpha-1 antitrypsin deficiency Contents of this page: Illustrations Alternative names Definition Causes, incidence, and risk factors ... Prevention Illustrations Lungs Liver anatomy Alternative names Return to top AAT deficiency Definition Return to top A deficiency of a protein produced in the liver that blocks the destructive effects of certain enzymes. The condition may lead to emphysema and liver disease Causes, incidence, and risk factors Return to top Alpha-1 antitrypsin deficiency results from a genetic defect. The mechanism that causes associated liver disease and emphysema in some people with this deficiency is not known, however some evidence suggests that it may be related to inflammation. Approximately 75% of adults with severe deficiency will develop emphysema, which often begins before 40 years of age. Smoking can increase risk. Symptoms Return to top shortness of breath shortness of breath during mild exertion unintentional weight loss wheezing Additional symptoms that may be associated with emphysema or cirrhosis thirst, excessive

9. Health Information At Your Fingertips - Patient.co.uk A list of resources and support for persons living in the UK who are interested in alpha1 antitrypsin deficiency. http://www.patient.co.uk/showdoc.asp?doc=90

10. Alpha-1 Antitrypsin Deficiency What is alpha1 antitrypsin deficiency? alpha-1 antitrypsin deficiencyis an inherited disorder that may cause lung or liver disease. http://www.lung.ca/diseases/alph1.html

What is Alpha-1 Antitrypsin Deficiency? Alpha-1 Antitrypsin deficiency is an inherited disorder that may cause lung or liver disease. Normally, the protein alpha-1 antitrysin, is released into the bloodstream and travels to the lung where it protects the lungs from the destructive actions of common illnesses and exposures, particularly tobacco smoke. People with a deficiency of this protective protein often suffer from progressive lung damage known as emphysema. Unlike the common form of emphysema seen in otherwise healthy individuals who have smoked for many years, this alpha antitrypsin deficiency form of emphysema may occur at an unusually young age and after minimal exposure to tobacco smoke. Visit the Alpha 1 Association web site. They are a non-profit, membership organization, dedicated to improving the lives of individuals and their families affected by alpha-1 antitrypsin deficiency. Alpha1 Canadian Registry This is the Canadian site for the International Alpha1 Registry. The International Registry has been set up with approximately 3500 participants. There are several goals of this registry. Initially, the registry will gather existing medical information about people who are deficient in the protein to understand better why some people remain healthy and free of lung disease and why some people develop emphysema. In the longer term, people who are participants in the registry may be asked if they are willing to participate in research trials that might improve our diagnoses, assessment or management of the disease. By coordinating research efforts at this international level, the registry scientists hope to make more rapid progress than individual scientists could achieve working in isolation.

11. HealthlinkUSA Alpha-1 Antitrypsin Deficiency Links Try it, you'll love it! AhHa. Click here for page 1 of alpha-1 antitrypsin deficiencyinformation from the HealthlinkUSA directory. Save on Drugs Here. http://www.healthlinkusa.com/10ent.htm

12. Alpha-1 Antitrypsin Deficiency; Treatment, Prevention, Cure alpha1 antitrypsin deficiency Search here for information which may include treatment,diagnosis, prevention, support groups, email lists, messageboards http://www.healthlinkusa.com/content/10.html

Latest Health News Find Drug Information Health Calculators All Words Any Words A B C D ... Z Sunday, March 30, 2003 Alternative therapy struggles to bridge East-West divide Click here to read more Doctors are not following guidelines recommending flu and pneumonia vaccinations for hospitalized adults, leaving millions of elderly patients vulnerable to potentially deadly ailment Click here to read more A helping hand for uninsured; Agencies find ways to provide health care for area children Click here to read more The government has approved the first generic version of the acne drug, Accutane Click here to read more Gates to give India $100m for AIDS; Microsoft CEO begins controversy-laced trip to fight virus Click here to read more Allergies and Asthma Alternative Medicine Arthritis and Your Health ... Women's Health Sunday March 30, 2003 Spina Bifida: Spina Bifida is a rare birth disorder, affecting approximately 1 in 12-1400 live births. Click here to learn more Craniosynostosis: Sagittal Synostosis...males are affected about three times as often as females. Click here to learn more Spinal Cord Injury: Acts of violence have now overtaken falls as the second most common source of spinal cord injury.

13. Alpha-1-Antitrypsin Deficiency & Lung Disease alpha1 antitrypsin deficiency LUNG DISEASE. Alpha-1-antitrypsin deficiency Whatis alpha-1-antitrypsin deficiency? Alpha-1-antitrypsin deficiency http://www.nevdgp.org.au/geninf/lung_f/alpha-1-health.html

Alpha-1-antitrypsin deficiency is an inherited disorder which usually affects the lungs. The information presented here is intended to answer your questions and provide you with a better understanding of this rare condition. What is alpha-1-antitrypsin? Alpha-1-antitrypsin is a protein which is produced by the liver and enters the blood stream. Its main role is to protect the lungs from destruction by other proteins called enzymes. Enzymes are found in all parts of the body and are needed for digestion to ensure that chemical reactions in the body proceed normally. Enzymes are also involved in areas of inflammation and tissue injury where they occur as a result of cell damage. What is alpha-1-antitrypsin deficiency? Alpha-1-antitrypsin deficiency is present when there is less than the normal amount of this protein in the blood. It becomes important only when the concentration in the blood is less than 20% to 30% of what we would normally expect. When this deficiency occurs, the lung is poorly protected from destructive enzymes and loss of lung tissue occurs, leading to a condition called emphysema. Once in every 2500 people in Australia has severe alpha-1-antitrypsin deficiency (levels below 20% of normal). This translates to nearly 7000 Australians with alpha-1-antitrypsin deficiency.

14. Alpha-1 Antitrypsin Deficiency alpha1 antitrypsin deficiency. Written by Dr David Maxton, gastroenterologistWhat is alpha-1 antitrypsin deficiency? http://www.netdoctor.co.uk/diseases/facts/alpha1def.htm

The UK's leading independent health website Search NetDoctor NetDoctor.co.uk Home News and features News Newsletter Features Encyclopaedia Diseases Medicines Examinations Health centres Cancer Children's health Depression Erectile dysfunction ... All health centres Discussion and support Discussion forums Support groups Services Ask the doctor Find a hospital NetDoctor on call Search Medline ... Text message services Information About NetDoctor Commercial opportunities NetDoctor.com Alpha-1 antitrypsin deficiency Written by Dr David Maxton , gastroenterologist What is alpha-1 antitrypsin deficiency? Alpha-1 antitrypsin deficiency is a disease caused by reduced or abnormal production in the body of the enzyme inhibitor alpha-1 antitrypsin. Body tissues and blood normally contain powerful enzymes - known as proteases - that can attack foreign substances within the body that may be harmful, such as tobacco smoke. However, these protease enzymes must be carefully regulated as they could attack and damage normal tissues rather than the intended target resulting in local tissue damage. Blood and tissues have a protease inhibitor that binds the enzyme to prevent unrestricted and potentially harmful protease activity. The commonest protease inhibitor in the blood is alpha-1 antitrypsin and its role is to protect the tissues from protease attack.

15. Alpha-1 Antitrypsin Deficiency Research On Cutting Edge At National Jewish Medic njc.org. alpha1 antitrypsin deficiency Research on Cutting Edgeat National Jewish Medical and Research Center. DENVER-A genetic http://www.njc.org/news/antitrypsin.html

January 2001 Contact: William Allstetter allstetterw@njc.org Alpha-1 Antitrypsin Deficiency Research on Cutting Edge at National Jewish Medical and Research Center DENVER-A genetic lung disease affecting tens of thousands of mostly younger Americans is the subject of a study at National Jewish Medical and Research Center. Alpha1-Antitrypsin (AAT) is a protein protecting the body against an enzyme in the body’s own white blood cells that can damage the lungs. The deficiency or absence of this protein is known as Alpha-1 Antitrypsin Deficiency (Alpha-1). “Alpha-1 is a genetic disease that usually affects people in their thirties,” says Dr. Robert Sandhaus , M.D., Ph D., director of the Alpha-1 Clinic at National Jewish, and medical director and executive vice president of the Alpha One Foundation and AlphaNet. “They exhibit symptoms of older people who suffer from chronic obstructive pulmonary disease (COPD) and associated diseases like chronic bronchitis, emphysema and asthma.” Dr. Sandhaus explains that previous medical studies, performed on blood bank samples, had predicted an estimated 100,000 cases of Alpha-1 in the U.S. alone, yet only six percent have actually been identified. The difficulty in diagnosing Alpha-1 is that patients exhibit symptoms of respiratory diseases like COPD. “From the onset of the initial symptoms, anyone suffering from Alpha-1 will see an average of five doctors over a period of about seven years before receiving a correct diagnosis,” says Dr. Sandhaus. Individuals identified with Alpha-1 may be predisposed to any of the diseases listed above as well as chronic liver disease.

16. Alpha-1 alpha1 antitrypsin deficiency. About alpha-1 antitrypsin deficiency.alpha-1 antitrypsin deficiency, also known as A1AD, is a genetic http://www.alpha1.org.uk/Alpha1/

Alpha-1 Antitrypsin Deficiency Contact Webmaster Official Website of Alpha-1 Support UK The intention of this site is to provide support for people living in the UK, who suffer from the genetic condition Alpha-1 Antitrypsin Deficiency Alpha-1 Support UK was formed in 1997. The intentions of this organisation are to: Provide support for UK based people who have been diagnosed as being Alpha-1 Antitrypsin Deficient. Provide a mechanism for addressing all issues associated with this condition. Increase awareness of this common genetic defect, particularly within the medical profession. Help with research into this condition. The bias of this site is unashamedly British. Any information given about the National Health Service, attitudes to illness, the facilities available to the sick and the support available for carers are all specific to the United Kingdom. About alpha-1 antitrypsin deficiency Alpha-1 antitrypsin deficiency, also known as A1AD, is a genetic condition that can lead to serious liver problems , mainly in children, and emphysema in adults. There are also several reported effects of this condition, unfortunately alpha-1 antitrypsin deficiency is not as well known as other conditions and

17. Virtual Hospital: The International Thoracic Teaching Resource: Chronic Obstruct Alpha1-antitrypsin deficiency. Rosen R, Dalinka M, Gralino B, Goldenberg D, WalshR The roentgenographic findings alpha-1 antitrypsin deficiency (AAD). http://www.vh.org/adult/provider/radiology/ITTR/Alpha1/Alpha1.html

For Providers The International Thoracic Teaching Resource: Chronic Obstructive Lung Disease Alpha-1-antitrypsin deficiency Jeffrey R. Galvin, M.D.; Michael P. D'Alessandro, M.D.; Yasayuki Kurihara, M.D. Peer Review Status: Internally Peer Reviewed Clinical Sx: Alpha-1-antitrypsin deficiency causes panacinar emphysema is responsible for 2% of all cases of emphysema in the US. Typically the patients are middle age. Other clinical symptoms are neonatal cholestasis, hepatitis, liver cirrhosis, and ulcerative panniculitis. Etiology: Mendelian inheritance consistent with deficiency gene. The gene for Alpha-1-antitrypsin is present on chromosome 14 and expressed in the hepatocyte and in the mononuclear phagocyte. Two Parental alleles, Z type and S type, are codominantly expressed. Therefore phenotypes, null-null, ZZ, and SZ, are responsible for emphysema. Pathophysiology: Inhibited proteases of leukocytic origin probably are partly responsible for the damage to the lung in the absence of the major protease inhibitor. Pathology: Pan lobular emphysema Miscellaneous: Imaging: Radiographically, severe emphysematous changes are present predominantly in the lower lobes. There may be increased vascularity of the upper lobes. Evidence of pulmonary hypertension and right ventricular enlargement is often present. The plain chest x-ray may not be particularly abnormal in the early stages.

18. Virtual Hospital : Health Topics A-Z : Alpha-1 Antitrypsin Deficiency Health Topics AZ alpha1 antitrypsin deficiency. For Adult Providers. The InternationalThoracic Teaching Resource Alpha-1-antitrypsin deficiency http://www.vh.org/navigation/vh/topics/alpha-1_antitrypsin_deficiency.html

Health Topics A-Z Alpha-1 Antitrypsin Deficiency All Topics Adult Patient Topics Adult Provider Topics Pediatric Patient Topics ... Pediatric Provider Topics For Adult Patients Health Prose: AAT Deficiency: A Serious Disorder Largely Unknown For Adult Providers The International Thoracic Teaching Resource: Alpha-1 Antitrypsin Deficiency Virtual Hospital Home Virtual Children's Hospital Home Site Map ... UI Health Care Home http://www.vh.org/navigation/vh/topics/alpha-1_antitrypsin_deficiency.html

19. Hep C Vets, Understanding Alpha-1 Antitrypsin Deficiency Understanding alpha1 antitrypsin deficiency Introduction. This webpage is toanswer your questions about alpha-1 antitrypsin deficiency. http://hepcvets.com/labs/alpha1.html

What's New? Our Latest Additions Hepatitis C Books On Liver Disease (Highly Recommended) Clinical Trials Depression Drugs Used In Treatment Fibrosis/Cirrhosis Genotypes Hepatitis B HEPC INFO Only List Sign Up HEPC INFO Only Full Text Medical Articles Herbs/Alternative Medicine Insurance Issues Liver Disease Glossary Miscellaneous Vaccine Information Veterans Table Of Contents Viral Loads What The Heck Is....VERY INFORMATIVE 2002 NIH Hepatitis C Consensus Hep C Vets Bulletin Board Hepatitis C Bulletin Board (for Non Vets) Our Favorite Links Poetry/Short Stories Understanding Alpha-1 Antitrypsin Deficiency Introduction This webpage is to answer your questions about alpha-1 antitrypsin deficiency. It contains information on signs and symptoms of the disorder as well as risk factors and treatment. When you understand more about alpha-1 antitrypsin deficiency, you will be able to play a more active role in your care. What is alpha-1 antitrypsin deficiency? Alpha-1 antitrypsin is a protein that is made in the liver. The liver releases this protein into the bloodstream. Alpha-1 antitrypsin protects the lungs so they can work normally. Without enough alpha-1 antitrypsin, the lungs can be damaged, and this damage may make breathing difficult.

20. Alpha-1 Antitrypsin Deficiency Author. Jann Washington CRTT, LRCP. Accreditation. Medway, a divisionof SSB, Inc. is approved by the state of Florida, provider number http://www.continuingeducation.com/nursing/alpha/

Author Jann Washington CRTT, LRCP Accreditation Medway, a division of SSB, Inc. is approved by the state of Florida, provider number FBN 3236 and by the state of California, provider number CEP 11921. AARC Approval # 230102000 Last Updated: January 2003 This program is acceptable for 1.0 hour of Continuing Education Credit (0.1 CEU) The price of this course is: $15.00 This course is also available in PDF format so you can print it out and return later to take the test at your convenience. To view the course in PDF format, click here Return to www.ContinuingEducation.com

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