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Hereditary Angioedema:     more detail

A Selection of Important Papers in Hereditary Angioedema: A Compilation of Key Peer-Reviewed Papers From Leading Journals Urticaria and Angioedema: Anaphylaxis, Solar Urticaria, Angioedema, Cold Urticaria, Dermatographic Urticaria, Hereditary Angioedema Study finds C1-inhibitor concentrate is safe and effective in treating skin swelling in patients with hereditary angioedema.(GENERAL DERMATOLOGY)(use of ... An article from: Dermatology Nursing by Daniel B. Burfeind, 2007-06-01 Erythema marginatum and hereditary angioedema.(Original Article): An article from: Southern Medical Journal by John C. Starr, George W. Brasher, et all 2004-10-01 Clinical Review of Hereditary Angioedema (Postgraduate Medicine) by MD Mark Weis, 2010-05-16 Icatibant promising for hereditary angioedema.(News): An article from: Skin & Allergy News by Nancy Walsh, 2007-07-01 Hereditary angioedema: a case study.(Case study)(Clinical report): An article from: Journal of Neuroscience Nursing by Vincent M., Jr. Vacca, 2007-10-01 Hereditary angioedema.: An article from: Journal of Drugs in Dermatology by Michael M. Sachse, Amor Khachemoune, et all 2006-10-01

lists with details

41. Health, Conditions And Diseases, Genetic Disorders: Hereditary Angioedema
    hereditary angioedema (HAE) is an autosomal dominant disorder manifested by painless, nonpruritic swelling of the skin.  
    http://www.combose.com/Health/Conditions_and_Diseases/Genetic_Disorders/Heredita
    
    Extractions: Hereditary angioedema (HAE) is an autosomal dominant disorder manifested by painless, nonpruritic swelling of the skin. Help build the largest human-edited directory on the web. Submit a Site Open Directory Project Become an Editor The combose.com directory is based on the Open Directory and has been modified and enhanced using our own technology. About ComboSE Download Combose Toolbar

42. Hereditary Angioedema
    hereditary angioedema A Personal Case History. I hope you find it helpful. What is hereditary angioedema? (taken from www.hereditaryangioedema.com).  
    http://www.btinternet.com/~third.bass/hae.html
    
    Extractions: As a sufferer of Hereditary Angioedema (HAE) I am posting this page, detailing my own case history, as a resource for other sufferers. I hope you find it helpful. Hereditary Angioedema (HAE) is a rare and serious genetic condition occurring in about 1/10,000 to 1/50,000 individuals. The disease is characterised by episodes of edema (swelling) in body parts, most notably the hands, feet, face, and airway passages. In addition, patients often have bouts of excruciating abdominal pain, nausea, and vomiting that is caused by swelling in the intestinal wall. HAE patients have a defect in the gene that controls a blood protein called C1-inhibitor. The genetic defect results in production of either inadequate or nonfunctioning C1-inhibitor protein. Normal C1-inhibitor helps to regulate the complex biochemical interactions of blood based systems involved in disease fighting, inflammatory response, and coagulation. Because defective C1-inhibitor does not adequately perform its regulatory function, a biochemical imbalance can occur and produce unwanted peptides that induce the capillaries to release fluids into surrounding tissues, thereby causing edema. (continued at www.hereditaryangioedema.com

43. Angioedema, Hereditary
    hereditary angioedema is a rare inherited disorder characterized by an accumulation of fluids outside of the blood vessels, blocking the normal flow of blood  
    http://www.bchealthguide.org/kbase/nord/nord98.htm
    
    Extractions: It is possible that the main title of the report is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Information on the following diseases can be found in the Related Disorders section of this report: Hereditary Angioedema is a rare inherited disorder characterized by an accumulation of fluids outside of the blood vessels, blocking the normal flow of blood or lymphatic fluid and causing rapid swelling of tissues in the hands, feet, limbs, face, intestinal tract, or airway. Usually, this swelling is not accompanied by itching, as it might be with an allergic reaction. Swelling of the gastrointestinal tract leads to cramping. Swelling of the airway may lead to obstruction, a potentially very serious complication. These symptoms develop as the result of deficiency or improper functioning of certain proteins that help to maintain the normal flow of fluids through very small blood vessels (capillaries). In some cases, fluid may accumulate in other internal organs. The severity of the disease varies greatly among affected individuals.

44. Hereditary Angioedema
    hereditary angioedema. Definition Also, see angioedema. Causes, incidence, and risk factors hereditary angioedema is an inherited disorder.  
    http://www.healthscout.com/ency/article/001456.htm
    
    Extractions: Causes, incidence, and risk factors: Hereditary angioedema is an inherited disorder. It is caused by the absence of a protein called C1 inhibitor (or a defect in this protein), which in turn effects blood vessels. People with hereditary angioedema can develop rapid swelling of the hands, feet, limbs, face, intestinal tract, or airway (larynx or trachea). Unlike an allergic reaction , there is usually no itching or hives . Swelling of the gut can cause intestinal cramping. If swelling closes the airway, it can be fatal. Attacks of swelling can become more severe in late childhood and adolescence. A family history is usually noted, at times only reported to descendants as an unexpected, sudden, and premature death of a parent, aunt/uncle, or grandparent.

45. Angioedema, Hereditary
    hereditary angioedema is a rare inherited disorder characterized by an accumulation of fluids outside of the blood vessels, blocking the normal flow of blood  
    http://my.webmd.com/hw/health_guide_atoz/nord98.asp
    
    Extractions: Hereditary Angioedema is a rare inherited disorder characterized by an accumulation of fluids outside of the blood vessels, blocking the normal flow of blood or lymphatic fluid and causing rapid swelling of tissues in the hands, feet, limbs, face, intestinal tract, or airway. Usually, this swelling is not accompanied by itching, as it might be with an allergic reaction. Swelling of the gastrointestinal tract leads to cramping. Swelling of the airway may lead to obstruction, a potentially very serious complication. These symptoms develop as the result of deficiency or improper functioning of certain proteins that help to maintain the normal flow of fluids through very small blood vessels (capillaries). In some cases, fluid may accumulate in other internal organs. The severity of the disease varies greatly among affected individuals.

46. Primary Immunodeficiency Association - Understanding Hereditary Angioedema And A
    CVID, Hypogammaglobulinaemia, Agammaglobulinaemia, Severe Combined Immunodeficiency SCID and Xlinked SCID, Neutropenia, hereditary angioedema HAE, X-linked  
    http://www.pia.org.uk/publications/general_publications/hereditary_angioedema.ht
    
    Extractions: Home Publications Services Events ... Helpline Hereditary angioedema (HAE) and acquired C1-inhibitor deficiency are uncommon complement deficiencies which are caused by the deficiency of a plasma protein known as C1-inhibitor. They can lead to attacks of abdominal pain and/or severe swelling in the tissues. This booklet describes what it means to have hereditary angioedema and outlines the medical facts behind both conditions. Living with Hereditary Angioedema Ann Price recalls her family’s experience of hereditary angioedema over four generations: The story of hereditary angioedema in our family is a very positive one. Although it started with much ignorance, fear, pain and mismanagement, we now understand the disorder and have confidence and enough control over the situation to live full and active lives. We hope that in the future, with the advances in gene therapy, the prospects will be still brighter. I have told our story to encourage those who have had to struggle — and perhaps continue to struggle — with many years of misdiagnosis and inadequate treatment. Let me start by introducing our family as we are today. I am a 52-year-old part-time nurse. My husband John, also 52, is a health and safety specialist. We have three children: Helen, who is 24 and has just qualified as a doctor, David who is 23 and studying medicine, and Edward, aged 13.

47. Associations Self-Help - H.A.E. Belgium - HEREDITARY ANGIOEDEMA
    Translate this page HAE Belgium - hereditary angioedema 87 bte 77 rue des Floralies - 1200Bruxelles Tél 02/762.91.83,  
    http://www.associations-self-help.org/fr/groupe.cfm?Group_ID=1125193783

48. Associations Self-Help - Résultats De Votre Recherche
    Translate this page N°, Nom du groupe, Problèmes traités, Activités. 1, HAE Belgium - hereditary angioedema, hereditary angioedema, entraide mutuelle, information.  
    http://www.associations-self-help.org/fr/liste.cfm?criteria=probleme&company=&KW

49. Hereditary Angioedema
    hereditary angioedema Support Group A non profit Organization dedicated to those families that are touched by a rare blood disease known as Hereditary  
    http://www.thenewhealthfind.com/Health/ConditionsandDiseases/GeneticDisorders/He

50. Hereditary Angioedema And Lupus Erythematosus
    hereditary angioedema and Lupus Erythematosus Theresa Pacheco MD 1 Dermatology Online Journal 7(1)24B. 1.Department of Dermatology  
    http://dermatology.cdlib.org/DOJvol7num1/medderm/abstracts/pacheco.html
    
    Extractions: The association between hereditary angioedema and lupus erythematosus in a family with twin boys was reported in the medical literature in 1974. Subsequent reports have confirmed this association. The daughter of one of the twins from the original report has hereditary angioedema and systemic lupus erythematosus (SLE). We present her case and discuss the affected family.

51. :: Ez2Find :: Hereditary Angioedema
    Guide hereditary angioedema, Global Metasearch Any Language Guides, hereditary angioedema. ez2Find Home Directory Health Conditions  
    http://ez2find.com/cgi-bin/directory/meta/search.pl/Health/Conditions_and_Diseas
    
    Extractions: Any Language English Afrikaans Arabic Bahasa Melayu Belarusian Bulgarian Catala Chinese Simplified Chinese Traditional Cymraeg Czech Dansk Deutsch Eesti Espanol Euskara Faroese Francais Frysk Galego Greek Hebrew Hrvatski Indonesia Islenska Italiano Japanese Korean Latvian Lietuviu Lingua Latina Magyar Netherlands Norsk Polska Portugues Romana Russian Shqip Slovensko Slovensky Srpski Suomi Svenska Thai Turkce Ukrainian Vietnamese Mode Guides Hereditary Angioedema Web Sites Adam.com: An Overview [Site Info] [Translate] [Open New Window] E-Medicine: HAE [Site Info] [Translate] [Open New Window] An in dept look at hereditary angioedema beginning with an introduction, clinical features, differentials, work up, treatment, medication and follow up. URL: http://www.emedicine.com/derm/topic24.htm

52. Hereditary Angioedema
    HOME. hereditary angioedema. hereditary angioedema; hereditary angioedema; ANGIONEUROTIC EDEMA, HEREDITARY; hereditary angioedema (HAE) United Kingdom Report. HOME.  
    http://www.bdid.com/heredangio.htm

53. Conditions And Diseases: Genetic Disorders: Hereditary Angioedema
    Health and Home. hereditary angioedema. http//www.rd.com/common/nav/index.jhtml?articleId=8612968. hereditary angioedema. Last Update Mon May 10 2004.  
    http://www.health-home.net/Conditions_and_Diseases/Genetic_Disorders/Hereditary_

54. Blackwell Synergy - Cookie Absent
    Newvariant hereditary angioedema in three brothers with normal C1 esterase inhibitor level and function. hereditary angioedema a board review for clinicians.  
    http://www.blackwell-synergy.com/links/doi/10.1111/j.1398-9995.2004.00428.x/abs/
    
    Extractions:  Home An Error Occurred Setting Your User Cookie A cookie is a small amount of information that a web site copies onto your hard drive. Synergy uses cookies to improve performance by remembering that you are logged in when you go from page to page. If the cookie cannot be set correctly, then Synergy cannot determine whether you are logged in and a new session will be created for each page you visit. This slows the system down. Therefore, you must accept the Synergy cookie to use the system. What Gets Stored in a Cookie? Synergy only stores a session ID in the cookie, no other information is captured. In general, only the information that you provide, or the choices you make while visiting a web site, can be stored in a cookie. For example, the site cannot determine your email name unless you choose to type it. Allowing a web site to create a cookie does not give that or any other site access to the rest of your computer, and only the site that created the cookie can read it. Please read our for more information about data collected on this site.

55. AADMC: Selected Articles 2000: Laryngeal Obstruction In Hereditary Angioedema
    SELECTED ARTICLES FROM THE RECENT LITERATURE 2000. 6/10/00. Laryngeal obstruction in hereditary angioedema. Summary Airway obstruction  
    http://www.aaaai.org/aadmc/currentliterature/selectedarticles/2000archive/laryng
    
    Extractions: Airway obstruction, a major cause of mortality in hereditary angioedema (HAE) is frequently not recognized promptly, sometimes with disastrous results. Bork et al of the University of Mainz in Germany reviewed details of 6 HAE patients who died because of laryngeal edema in Germany over a 10 year period. The interval between first symptoms and asphyxiation was 1-14 hours. No patient had an urgent cricothyrotomy or replacement C1 inhibitor concentrate. A retrospective review of 58 HAE cases in Germany, revealed 23 deaths by asphyxiation with an average age at death of 39 years. Reference Although HAE is not a common disorder, I have reviewed this report because it emphasizes: 1) that laryngeal obstruction is common in HAE and is a frequent cause of death at a relatively young age; 2) the first episode of laryngeal edema can be fatal; 3) although the disorder is congenital, most airway fatalities occur in young adults/middle age; 4) most important, such tragic outcomes can be prevented by appropriate chronic and emergency treatment. American Academy of Allergy, Asthma and Immunology

56. Protease Inhibitor Therapy In Hereditary Angioedema
    SELECTED ARTICLES FROM THE RECENT LITERATURE 2003. 12/5/03. Protease inhibitor therapy in hereditary angioedema. Summary Although hereditary  
    http://www.aaaai.org/aadmc/currentliterature/selectedarticles/2003archive/protea
    
    Extractions: Although hereditary angioedema (HAE) is a very uncommon disorder, it can cause considerable morbidity and even morta;lity. HAR has also taught us much about the interaction of the complement, coagulation, and bradykin generating systems. The basic defect in congenital HAE is in either deficient production of the C1 inhibitor (85% of cases) or production of a dysfunctional C1 inhibitor (C1 INH) molecule (15% of cases). There are also unusual acquired variants of HAE The major therapeutic approach in HAE has been to either stimulate production of C1 INH or replace it with a C1 INH concentrate (not yet FDA approved for use in the USA); see Arch Intern Med 2001;161:714-18).

57. Hereditary Angioedema (HAE) - United Kingdom Report
    September / October 1999. Newsletter. hereditary angioedema (HAE) United Kingdom Report. I have a very rare condition hereditary angioedema.  
    http://www.pidsnz.co.nz/news/1999/sepoct/hae.htm
    
    Extractions: September / October 1999 Newsletter Hereditary Angioedema (HAE) - United Kingdom Report (Our thanks to the PiA/UK for their kind permission to reprint this article) Ann Price, who knows all about HAE, having had a family history of the disorder, has done a lot of work recently in preparation for setting up a national network for people with HAE. She sends us this report. People with HAE commonly have the disorder for many years before the correct diagnosis is made. We are now in the process of setting up, under the umbrella of the PiA, an HAE Network. The network will offer support for people with HAE and help them access effective medical treatment. This condition can be very frightening and isolating before a diagnosis is made, but there are now effective treatments available which can transform the picture for those of us with HAE. Hereditary Angiodema (HAE) PiA/UK Member Ann Blake describes a set of circumstances leading up to her diagnosis that hopefully are not too typical, but we suspect they probably are! "I have a very rare condition: Hereditary Angioedema. This was finally diagnosed in November 1994 by accident, after 14 years of misdiagnosis!!

58.  Hereditary Angioedema 
    hereditary angioedema hereditary angioedema, See C1 inhibitor deficiency (congenital).  
    http://www.rcpa.edu.au/pathman/heredita.htm

59. ScienceDaily -- Browse Topics: Health/Conditions_and_Diseases/Genetic_Disorders/
    Top Health Conditions and Diseases Genetic Disorders hereditary angioedema (5 links). News about hereditary angioedema Books about hereditary angioedema  
    http://www.sciencedaily.com/directory/Health/Conditions_and_Diseases/Genetic_Dis
    
    Extractions: Front Page Today's Digest Week in Review Email Updates ... Genetic Disorders Hereditary Angioedema (5 links) News about Hereditary Angioedema Scientists Confirm New Breast Cancer Gene (May 17, 2004) full story Researchers Confirm Genetic Link Between Hereditary Breast And Prostate Cancer (May 4, 2004) full story Researchers Discover New Clinical Syndrome Leading To Severe Osteoarthritis (May 4, 2004) full story Study Of Growth Hormone Treatment And Creutzfeldt-Jakob Disease Underscores Need For Prevention Of Adrenal Crises (April 7, 2004) full story Genetic Basis Of Hereditary Nerve Disorder Revealed (April 6, 2004) full story [ More news about Hereditary Angioedema Links about Hereditary Angioedema Hereditary Angioedema Support Group - A non profit Organization dedicated to those families that are touched by a rare blood disease known as Hereditary Angioedema E-Medicine: HAE - An in dept look at hereditary angioedema beginning with an introduction, clinical features, differentials, work up, treatment, medication and follow up.

60. Medical Encyclopedia
    BACK, Back to main Health Information page Disease hereditary angioedema. Causes And Risk hereditary angioedema is an inherited disorder.  
    http://www.medstarhealth.org/body.cfm?id=124&action=display&articlenum=1456

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