21. Alpha-1 Antitrypsin Deficiency alpha1 antitrypsin deficiency (A 1 AD) is a common genetic disorder that primarilyaffects the lungs and liver and can lead to emphysema and cirrhosis. http://www.continuingeducation.com/nursing/alpha/intro.html

INTRODUCTION Alpha-1 antitrypsin deficiency (A AD) is a common genetic disorder that primarily affects the lungs and liver and can lead to emphysema and cirrhosis. Laurell and Erikkson first identified the condition in 1963 in Sweden. A AD is shown to be highly prevalent in Caucasians of Northern European descent, especially Scandinavians. A AD occurs in one of every 2,000 live births, yet less than 100,000 Americans are diagnosed with the disease at any given time. A1AD can be difficult to diagnose, especially in adults, and is significantly underrecognized. Age appears to be a factor in the type of manifestations caused by the deficiency, but symptoms may vary greatly from person to person. In infants, A AD is most likely to cause cirrhosis. If no symptoms are exhibited in childhood, emphysema is the most common adult manifestation, with age of onset occurring between 30 and 50 years of age. Between 2-18% of all emphysema patients are Alpha-1 deficient. This disease causes a significant number of emphysema cases. Alpha-1 antitrypsin deficiency accounts for 2 to 3% of COPD patients. Alpha-1 antitrypsin deficiency has been given many names over the years. The following are some well-known synonyms:

22. Alpha-1 AntiTrypsin Deficiency degradation of aberrant protein. There are two clinical problems asa result of alpha1 antitrypsin deficiency Panacinar emphysema http://www.mcevoy.demon.co.uk/Medicine/Pathology/Genetics/a1AntiTDef.html

Alpha-1 Anti-Trypsin Deficiency Incidence Homozygotes about 1 in 3600. Aetiology Autosomal recessive enzyme deficiency. Alpha-1 anti-trypsin normally inhibits the action of collagenase, elastase and other proteases (including trypsin Heterozygotes have 60% of the normal enzyme levels. Homozygotes have 10% of the normal enzyme levels. Pathogenesis Alpha-1 antitrypsin is a protein synthesised in the liver and immediately secreted into the blood where it acts as an antiprotease (protease inhibitor Pi). There are several phenotypes : MM normal (90% population) MZ and MS are heterozygotes SS is a homozygote which reduces alpha-1 antitrypsin levels without clinical manifestations ZZ is a homozygote which produces a clinical deficiency a glu is changed to a lys decreased ability for normal protein folding accumulation of abnormal protein in hepatocytes increased lysosomal degradation of aberrant protein There are two clinical problems as a result of alpha-1 antitrypsin deficiency : Panacinar emphysema occurs earlier than in those without the deficiency both males and females are affected Hepatitis and Cirrhosis the unusual phenotypes of alpha-1 antitrypsin are not released readily from the liver and the unreleased protein then accumulates in the cytoplasm of periportal hepatocytes in the from of hyaline intracellular globules NB all PiZZ people accumulate abnormal protein but only up to 20% develop liver disease neonatal hepatitis with cholestatic jaundice adolescents get attacks of acute hepatitis

23. Alpha-1 Antitrypsin Deficiency Respiratory. alpha1 antitrypsin deficiency. alpha-1 antitrypsin deficiencyis an inherited disorder which can lead to emphysema at a young age. http://jhhs.client.web-health.com/web-health/topics/GeneralHealth/generalhealths

24. A1AD Symptoms, Diagnosis And Treatment alpha1 antitrypsin deficiency Symptoms and Treatment What are the symptoms ofalpha-1 antitrypsin deficiency? How is alpha-1 antitrypsin deficiency treated? http://jhhs.client.web-health.com/web-health/topics/GeneralHealth/generalhealths

25. 1Up Health > Alpha-1 Antitrypsin Deficiency > Causes, Incidence, And Risk Factor 1Up Health Diseases Conditions Alpha1 antitrypsindeficiency Causes, Incidence, and Risk Factors. http://www.1uphealth.com/health/alpha_1_antitrypsin_deficiency_info.html

1Up Health Alpha-1 antitrypsin deficiency Alternative Medicine Clinical Trials ... Health Topics A-Z Search 1Up Health Alpha-1 antitrypsin deficiency Information Alpha-1 antitrypsin deficiency Causes, Incidence, and Risk Factors Alternative names : AAT deficiency Definition : 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 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. Previous Next Jump to Another Section of this Guide Definition Causes, Incidence, and Risk Factors Symptoms Prevention Diagnosis, Signs, and Tests Treatment ... Calling your Health Care Provider Related Tools and Utilities Search Books on Amazon: Read Articles on eLibrary: Health Products on drugstore.com:

26. Alpha-1 Antitrypsin Deficiency - Bayer Biologicals alpha1 antitrypsin deficiency. alpha-1 antitrypsin deficiency, also knownas Alpha-1, is an inherited disorder that causes a significant http://www.bayerbiologicals.com/Products/Therapeutic/Proteinase/Alpha-1.asp

Alpha-1 Antitrypsin Deficiency Alpha-1 antitrypsin deficiency, also known as Alpha-1, is an inherited disorder that causes a significant reduction in the naturally occurring protein alpha-1 antitrypsin (AAT). Reduced levels of AAT result in the body being less able to protect itself from elastase, an enzyme in the white blood cells that normally helps to fight bacteria and that can cause damage to the air sacs in the lungs if not neutralized by AAT. AAT deficiency may predispose an individual to several serious illnesses, most commonly emphysema beginning in the third or fourth decade of life. Less frequently, AAT deficiency can result in liver disease, or a skin condition called panniculitis. It is believed that AAT deficiency affects as many as 200,000 people in the North America and Europe. AAT deficiency is most common among Caucasians of Northern European descent. It is estimated that as many as 95% of people with Alpha-1 have not been identified. Bayer Biological Products (BP) has worked over the past 15 years to provide the only available augmentation therapy for people with this deficiency. Our commitment to this community is demonstrated in our continued support of a number of organizations that serve the Alpha 1 community, and our support of patient identification programs. This commitment extends to research as well. In August 2000, Bayer BP entered into a partnership with

27. University Of Miami School Of Medicine - Glossary - Alpha-1 Antitrypsin Deficien alpha1 antitrypsin deficiency. alpha-1 antitrypsin deficiency (A-1ATD). Smokerswith alpha-1 antitrypsin deficiency have a faster rate of lung damage. http://www.med.miami.edu/patients/glossary/art.asp?articlekey=278

28. Alpha 2 Alpha - Alpha1 Antitrypsin Deficiency Support And Resources DISCLAIMER This website contains general information about our Lung/Liver/Skin GeneticDisease, alpha1 antitrypsin deficiency, for Alpha Patients, Caregivers http://www.alpha2alpha.net/

Welcome to the Alpha to Alpha web site ! United We Stand! Alphas Helping Alphas (Liver List Mbrs) Note: The Alpha Atlas's Patient Stories is and has always been open to all Alphas to add their stories in dealing with Alpha-1. Alpha's Web Sites Alpha Atlas Alpha Lung Volume Reduction Info Alpha Doctor's List ... Hospice Care A ny of these early warning signs may signal his presence. This guide may help you to identify a possible oncoming infection and afford you the opportunity to seek out the appropriate treatment. Peach Ribbon Symbolizes Alpha-1 O ur mission is to provide peer-to-peer support and information to people having emphysema and other health disorders related to Alpha-1 , (alpha-1 antitrypsin deficiency , also called A1AD.) A lpha-1 is a genetic disease of the liver, lungs and a skin disease, panniculitis. It results from a lack of the Alpha-1 Antitrypsin (AAT) protein in a person's blood, which protects the lungs from germs, dust, air pollution, cigarette smoke and other inhaled irritants. Without an adequate supply of AAT, substances in the white blood cells can damage the tissues of the body. I n adults, alpha-1 often leads to early onset emphysema that affects the lower lobes of the lung: but can also cause liver disease. In infants, the disease causes neonatal cirrhosis.

29. Health Ency.: Disease: Alpha-1 Antitrypsin Deficiency home Disease A alpha1 antitrypsin deficiency. alpha-1 antitrypsin deficiencySee images. alpha-1 antitrypsin deficiency results from a genetic defect. http://www.accessatlanta.com/shared/health/adam/ency/article/000120.html

SEARCH: The Web Yellow Pages HOME AJC.COM Illustrated Health Encyclopedia Important notice Ency. home Disease A Alpha-1 antitrypsin deficiency See images Overview Symptoms Treatment ... Prevention Alternative names: AAT deficiency Definition: 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 and Risks 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. Ency. home Disease A Please read this Important notice Also Check Out Subscribe to The Atlanta Journal-Constitution Home Autos Classifieds ... Our Sponsors By using AccessAtlanta.com you accept the terms of our Visitor Agreement . Please read it. Privacy statement Registered site users: edit your profile

30. What Is The Longterm Outcome Of Alpha-1 After Liver Transplant? Answer Jeff Punch, MD A search on Medline reveals no reports of recurrenceof alpha1 antitrypsin deficiency in the scientific literature. http://www.transweb.org/qa/asktw/answers/answers9509/longtermoutcomeofalpha1.htm

What is the longterm outcome of Alpha-1 after liver transplant? Question was submitted by: Peggy Dreker on 10/2/95. Question: My son had a liver txp in 1984 and re-tpx in 10/87. He is now 10 1/2 years old. He suffered from both biliary atresia and alpha 1 antitrypsin deficiency. My question is has there been any research done on the re-occurence of the alpha 1 after transplant. I realize that no long term studies can be done due to the relative short time that liver transplants have been done on kids; but is any one doing any genetic research on alpha 1? Thank you. Answer: Jeff Punch, MD: A search on Medline reveals no reports of recurrence of alpha-1 antitrypsin deficiency in the scientific literature. If our current understanding of alpha-1 antitrypsin deficiency is correct, it should not be possible for alpha-1 antitrypsin deficiency to recur in the transplnted liver. The new liver has a normal gene that produces alpha-1 antitrypsin. Current areas of research include gene therapy to treat the disease in asymptotic carriers. Alpha 1 antitrypsin deficiency would be an ideal disease to treat with gene therapy. The genetics of alpha 1 antitrypsin deficiency are pretty well worked out, but it is still not possible to predict the rate the disease will progress with absolute the certainty. Bob Merion, MD:

31. ALPHA-1 ANTITRYPSIN DEFICIENCY (Pediatric Oncall) Find out more about Alpha 1 Antitrypsin Deficiency in children and its consequencesat www.pediatriconcall.com. ALPHA1 – ANTITRYPSIN DEFICIENCY http://www.pediatriconcall.com/fordoctor/DiseasesandCondition/alpha1deficiency.a

Question of day Alternative Medicine Journals Queries ... Shopping Mall ALPHA-1 – ANTITRYPSIN DEFICIENCY UserName Password New Register Dr Ira Shah M.D, DCH(GoldMedalist), FCPS , DNB Q: What is alpha – l-antitrypsin ? A: Alpha –1- antitrypsin is a glycoprotein produced by the liver. Its primary function is to protect the lungs from neutrophil elastase, which is an enzyme that digests the phagocyte cells and bacteria to promote healing. However, it requires alpha-l – antitrypsin to prevent it from damaging the lung tissue. Alpha-l- antitrypsin is an acute- phase reactant. Its level increases to 3 or 4 times the normal amount at times of infection, pregnancy or any conditions that cause an increase in neutrophil elastase. Q: What happens where there is deficiency of alpha-l- antitrypsin? A: In alpha –l deficient persons there is insufficient amount of alpha-1 antitrypsin circulating in the blood, hence the destructive effect of neutrophil elastase goes unchecked and lung tissue is destroyed causing emphysema

32. Alpha-1-antitrypsin Deficiency Understanding alpha1 antitrypsin deficiency - Understanding Alpha-1 AntitrypsinDeficiency Table of Contents alpha-1 antitrypsin deficiency. http://www.health-nexus.com/alpha-1-antitrypsin deficiency.htm

Health-Nexus.Net Health-Nexus.Org The #1 Health information site Home ... Up Search Health-Nexus for: Match ALL words Match ANY word Email this page to a friend ! Post a question or comment on our Message Board Home Page Health Specialties Health News ... Alternative Health Options Substance Abuse Animal Health Search: Books Magazines Video Keywords: Find it Here Alpha-1-Antitrypsin Deficiency Alpha 1 Association Organization which attempts to identify those affected by alpha-1-antitrypsin deficiency and to improve their quality of their lives through support, education, advocacy and research. Understanding Alpha-1 Antitrypsin Deficiency ... Understanding Alpha-1 Antitrypsin Deficiency Table of Contents ... Introduction What is alpha-1 antitrypsin deficiency? How do normal ... symptoms of alpha-1 antitrypsin deficiency? Can the disorder ... questions about alpha-1 antitrypsin deficiency. It contains ... Alpha-1 Antitrypsin Deficiency Home page. Normal protein folding. Cystic fibrosis. Alpha-1-antitrypsin. Nephrogenic diabetes insipidous.

33. Understanding Alpha-1 Antitrypsin Deficiency Understanding alpha1 antitrypsin deficiency. What is alpha-1 antitrypsin deficiency? Whatare the signs and symptoms of the alpha-1 antitrypsin deficiency? http://www.clevelandclinic.org/health/health-info/docs/1100/1138.asp?index=5731

34. Spiderspun; An Alpha-1 Antitrypsin Deficiency Haven Translate this page Español Deutsche Français. http://www.spiderspun.net/

Español Deutsche Français Español Deutsche Français

35. Alpha-1 Antitrypsin Deficiency Alpha1-Antitrypsin Deficiency (A1AD) is one of the most common inherited disordersamong whites. Its primary manifestation is early-onset panacinar emphysema. http://www.spiderspun.net/faqs.htm

Alpha-1 Facts The Basics The History The Genetic Path The Symptoms ... The Testing Alpha-1-Antitrypsin Deficiency i s one of the most common inherited disorders among Caucasians. Its primary manifestation is early-onset panacinar emphysema. A minority of patients develops hepatic cirrhosis. Slow progressive dyspnea is the primary symptom, although many patients initially have symptoms of cough, sputum production, or wheezing.(7) Alpha-1-antitrysin deficiency, called A1AD, for short, is a genetic disorder characterized by a marked reduction in serum levels of a naturally occurring protein called Alpha-1-antitrypsin (also known as AAT) that causes emphysema cirrhosis , and/or panniculitus . AAT, produced by the liver, shields the body from damage caused by neutrophil elastase, an enzyme released by a type of white blood cell known as neutrophil. Imagine neutrophil elastase as like being a heavy tile cleaner that you spray on mildew. Under normal conditions, neutrophil elastase helps fight bacteria that cause infection because it destroys it. However, if not neutralized by AAT, neutrophil elastase can slowly damage lung tissue, or related tissues where elasticity is needed. In other words, AAT is like the water-filled sponge that wipes away the cleaner before it destroys the floor. Over time this process can result in emphysema. Therefore, people with A1AD are at risk of developing emphysema (1,2). By following their doctor's advice, people with A1AD can learn how to manage their condition and maintain the best possible health.

36. Mayo Clinic - Defect In Alpha-1 Antitrypsin Gene Found To Increase Colorectal Ca The genetic defect, alpha1 antitrypsin deficiency, presents an inadequate levelof the protease inhibitor alpha-1 antitrypsin, which protects the surfaces of http://www.mayoclinic.org/news2001-rst/789.html

search: Mayo Clinic Locations: Arizona Florida Minnesota Mayo Clinic in Rochester Wednesday, January 03, 2001 Defect in Alpha-1 Antitrypsin Gene Found to Increase Colorectal Cancer Risk Risk Greatly Magnified by Cigarette Smoking ROCHESTER MINN. Mayo Clinic scientists have discovered that carriers of a genetic defect previously linked to emphysema have a threefold increased risk of developing a type of sporadic colorectal cancer. Carriers who currently smoke have a 20-fold increased risk. "This study provides important new insight into the controversy surrounding the link between cigarette smoking and colorectal cancer, confirming a strong association in a subgroup of colorectal cancers," says Dr. Ping Yang, Mayo Clinic clinical epidemiologist and principal investigator of the study. The genetic defect, alpha-1 antitrypsin deficiency, presents an inadequate level of the protease inhibitor alpha-1 antitrypsin, which protects the surfaces of multiple organs. Alpha-1 antitrypsin is synthesized primarily in the liver and in a small amount of the gastrourinary systems. In individuals who lack this inhibitor, the protease destroys the membrane system, leaving the colon and rectum vulnerable to colorectal cancer development. The investigators believe that cigarette smoking causes a similar effect, further exacerbating tissue destruction for carriers of alpha-1 antitrypsin deficiency, since smoking is a strong inactivator of the protease inhibitor alpha-1 antitrypsin.

37. ORPHANET® : Alpha-1 Antitrypsin Deficiency Translate this page ORPHANET. ORPHANET database access. Alpha-1 antitrypsindeficiency. Direct access to details Alias Home Page. http://www.orpha.net/static/GB/alpha_1_antitrypsin.html

ORPHANET database access Alpha-1 antitrypsin deficiency Direct access to details Alias : Home Page

38. ALF - LEARN ABOUT LUNG HEALTH alpha1 antitrypsin deficiency LUNG DISEASE Alpha-1-antitrypsin deficiencyis an inherited disorder which usually affects the lungs. http://www.lungnet.org.au/Fact Sheets/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.

39. Alpha 1 Antitrypsin Deficiency HOME Alpha 1 Antitrypsin Deficiency (Cholestasis, Neonatal). Inherited Emphysema;alpha1 antitrypsin deficiency; DEFICIT ALFA-1-ANTITRIPSINA; Support Groups. HOME http://www.bdid.com/alpha1anti.htm

HOME Alpha 1 Antitrypsin Deficiency (Cholestasis, Neonatal) Alpha1 National Association Alpha-1 Support UK A1AD Related Emphysema PROTEASE INHIBITOR 1; PI ... HOME

40. Swiss Registry For Alpha-1 Antitrypsin Deficiency Swiss Registry for alpha1 antitrypsin deficiency. Several years ago weestablished a National Registry for alpha-1 antitrypsin deficiency http://www.aatregistry.org/Switzusa.html

Swiss Registry for alpha-1 Antitrypsin Deficiency Several years ago we established a National Registry for alpha-1 antitrypsin deficiency at the Pulmonary Division of the University Hospital of Zurich (http://www.unispital.ch/lungenthorax). The purpose is to collect information on patients with this inherited condition within our country. Data are reported by the patients physicians, provided the patient has given his consent. Our registry comprises about 70 patients, mostly with a Pi ZZ or Pi SZ phenotype. Prof. E.W. Russi, head of the Pulmonary Division is a member of the Task Force AIR, which is a multinational group of experts on alpha 1-antitrypsin deficiency. A working group of the Swiss Pulmonary Society made statements several years ago on intravenous augmentation therapy with the human plasma derived product Prolastinâ. Due to a lack of controlled prospective, randomised clinical trials convincingly showing a positive effect on the progression of pulmonary emphysema, this type of treatment is not covered by health insurance in Switzerland. Patients with alpha-1 antitrypsin deficiency get advice on their disease and the current treatment options by our group. For patients suffering from an advanced stage of their disease we recommend VAT lung volume reduction surgery or eventually lung transplantation.

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