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  1. A Selection of Important Papers in Hereditary Angioedema: A Compilation of Key Peer-Reviewed Papers From Leading Journals
  2. Urticaria and Angioedema: Anaphylaxis, Solar Urticaria, Angioedema, Cold Urticaria, Dermatographic Urticaria, Hereditary Angioedema
  3. 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
  4. 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
  5. Clinical Review of Hereditary Angioedema (Postgraduate Medicine) by MD Mark Weis, 2010-05-16
  6. Icatibant promising for hereditary angioedema.(News): An article from: Skin & Allergy News by Nancy Walsh, 2007-07-01
  7. Hereditary angioedema: a case study.(Case study)(Clinical report): An article from: Journal of Neuroscience Nursing by Vincent M., Jr. Vacca, 2007-10-01
  8. Hereditary angioedema.: An article from: Journal of Drugs in Dermatology by Michael M. Sachse, Amor Khachemoune, et all 2006-10-01

21. Hereditary Angioedema - Medical Dictionary Definitions Of Popular Medical Terms
MedicineNet Home MedTerms medical dictionary AZ List hereditary angioedema. Advanced Search. hereditary angioedema A genetic form of angioedema.
http://www.medterms.com/script/main/art.asp?articlekey=3721

22. NEJM -- Hereditary Angioedema
hereditary angioedema. Clinical Studies of Sudden Upper Airway Obstruction in Patients With hereditary angioedema Due to C1 Esterase Inhibitor Deficiency.
http://content.nejm.org/cgi/content/short/334/25/1666
HOME SEARCH CURRENT ISSUE PAST ISSUES ... HELP Please sign in for full text and personal services Previous Volume 334:1666-1667 June 20, 1996 Number 25 Next Hereditary Angioedema
Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.
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"So soon as ever she came into the room I recognized the affection, for there lay, across the face from temple to temple, an oblong tumor almost closing both eyes." Thus, J.L. Milton in 1876 described one of his patients with angioedema, six years before Quincke introduced the term angioneurotic edema to describe this disease. As Dr. Milton pointed out, the clinical syndrome can be identified quickly, but its cause is not so easy to discern. Because of the severity of the symptoms and the need for a specific therapeutic approach, angioedema due to deficiency of C1 inhibitor must Full Text of this Article References
This article has been cited by other articles:
  • Bork, K., Hardt, J., Schicketanz, K.-H., Ressel, N. (2003). Clinical Studies of Sudden Upper Airway Obstruction in Patients With Hereditary Angioedema Due to C1 Esterase Inhibitor Deficiency.

23. NEJM -- Treatment Of Hereditary Angioedema With A Vapor-Heated C1 Inhibitor Conc
Original Article from The New England Journal of Medicine Treatment of hereditary angioedema with a VaporHeated C1 Inhibitor Concentrate.
http://content.nejm.org/cgi/content/short/334/25/1630
HOME SEARCH CURRENT ISSUE PAST ISSUES ... HELP Please sign in for full text and personal services Previous Volume 334:1630-1634 June 20, 1996 Number 25 Next Treatment of Hereditary Angioedema with a Vapor-Heated C1 Inhibitor Concentrate
A. Thomas Waytes, M.D., Ph.D., Fred S. Rosen, M.D., and Michael M. Frank, M.D.
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ABSTRACT Background Hereditary angioedema results from a congenital deficiency of functional C1 inhibitor and is characterized by episodic bouts of edema, which may be life-threatening when they involve the larynx. We evaluated the effectiveness of a C1 inhibitor concentrate in the prevention and treatment of attacks of hereditary angioedema. The concentrate was vapor-heated to inactivate hepatitis and human immunodeficiency viruses. Methods We conducted two double-blind, placebo-controlled studies. The first was a crossover study consisting of two 17-day trials in which prophylactic infusions of either C1 inhibitor (25 plasma units per kilogram of body weight) or placebo were given intravenously every third day to six patients with hereditary angioedema.

24. NORD - National Organization For Rare Disorders, Inc.
Research. hereditary angioedema Baxter Healthcare manufactured a C1 inhibitor protein, extracted from human blood and heat-sterilized, for HAE in Europe.
http://www.rarediseases.org/nord/research/hereditary_angioedema

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Read about events on Capitol Hill, funding for rare-disease research, and other topics of interest from NORD's office in Washington, DC. Research Hereditary Angioedema Baxter Healthcare manufactured a C-1 inhibitor protein, extracted from human blood and heat-sterilized, for HAE in Europe. However, this treatment, which was administered intravenously, is no longer available. Aventis now distributes a plasma-based product in Europe and is making it available on a limited basis in the United States. For information about obtaining the Aventis product, patients and physicians should contact the Immune Deficiency Foundation in Maryland: Immune Deficiency Foundation 40 West Chesapeake Avenue Suite 308 Towson, MD 21230

25. NORD - National Organization For Rare Disorders, Inc.
General Discussion hereditary angioedema is a rare inherited disorder characterized by an accumulation of fluids outside of the blood vessels, blocking the
http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Angioedema, H

26. Genzyme Research: Hereditary Angioedema
Pathogenesis. Characterised by recurrent and mucosal swelling, including potentially lifethreatening angioedema in the narrow passages of the larynx.
http://www.genzyme.com.au/practitioners/hereditary_angioedema.htm
- Introduction
Etiology

Pathogenesis

Clinical Description

Symptoms
...
Management,

Hereditary Angioedema
Genetic Disease Profile Introduction In the classic complement pathway of the humoral immune response
Etiology
Caused by functional deficiency of C1-esterase inhibitor. Loss of regulatory control results in over expression of the complement-mediated inflammatory response. Top of Page
Pathogenesis
Characterised by recurrent and mucosal swelling, including potentially life-threatening angioedema in the narrow passages of the larynx. Top of Page Clinical Description
  • May present during early childhood. Episodic localized tissue swelling resulting in large, firm welts. These welts may occur on any part of the body, including the face and limbs and internal organs.
Top of Page Symptoms
  • Localized swelling (edema) may appear without warning on any part of the body. The symptoms will differ depending on where the edema occurs. Most dangerous is swelling of the airways, which may interfere with breathing.

27. Genetic Disorders, Hereditary Angioedema
Submit Your Site to the hereditary angioedema category. Sponsored hereditary angioedema Sites. Submit Your Site to the hereditary angioedema category.
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  • Adam.com: An Overview - A definition of hereditary angioedema along with a look at the causes, incidence and risk factors.
  • E-Medicine: HAE - An in dept look at hereditary angioedema beginning with an introduction, clinical features, differentials, work up, treatment, medication and follow up.

28. Genetic Disorders, Hereditary Angioedema
Category Home Health Conditions and Diseases Genetic Disorders hereditary angioedema. * Site Title ยท The name of the site.
http://www.iseekhealth.com/directory/index.php?method=show_link_exchange&directo

29. Hereditary Angioedema,Angioneurotic Edema Hereditary,C1-INH,C1NH,Complement Comp
hereditary angioedema,Angioneurotic Edema Hereditary,C1INH,C1NH,Complement Component 1 Inhibitor Deficiciency,Complement Component C1 Regulatory Component
http://www.icomm.ca/geneinfo/herang.htm
Hereditary Angioedema,Angioneurotic Edema Hereditary,C1-INH,C1NH,Complement Component 1 Inhibitor Deficiciency,Complement Component C1 Regulatory Component Deficiency,Esterase Inhibitor Deficiency,HAE,HANE,C1 Esterase Inhibitor Deficiency Type I Angioedema,C1 Esterase Inhibitor Dysfunction Type II Angioedema For Information on Workshops and Seminars for Special Needs Children click here The GAPS INDEX
to Information on the Internet about Genetic Disorders and Birth Defects

Genetic Information and Patient Services, Inc. (GAPS)
HOME
DISORDERS GLOSSARY Hereditary Angioedema
also known as:
Angioneurotic Edema, Hereditary
C1-INH
Complement Component 1 Inhibitor Deficiciency
Complement Component C1, Regulatory Component Deficiency
Esterase Inhibitor Deficiency
HAE HANE Disorder Subdivisions C1 Esterase Inhibitor Deficiency, Type I, Angioedema C1 Esterase Inhibitor Dysfunction, Type II, Angioedema (as defined by the National Organization for Rare Disorders
    Hereditary Angioedema is a rare inherited vascular disorder characterized by an excessive accumulation of body fluids that results in areas of obstruction in lymphatic vessels or veins. The blockage of the normal flow of blood or lymphatic fluid results in temporary swelling of the skin and mucous membranes.

30. Hereditary Angioedema Association
hereditary angioedema Association provides information and support for sufferers of HAE which is a rare genetic disorder (autosomal dominant) that leads to
http://www.wecaretoo.com/Organizations/FL/haea.html
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31. Hereditary Angioedema- Medcohealth.com
hereditary angioedema is an inherited abnormality of the immune system that causes swelling, particularly of the face, and abdominal cramping.
http://www.medcohealth.com/medco/consumer/ehealth/ehsarticle.jsp?ltSess=y&articl

32. Canadian Hereditary Angioedema Society
CAN logo www.calgaryallergy.ca. The Canadian hereditary angioedema Society (www.haecanada.com).
http://www.calgaryallergy.ca/Articles/HereditaryAngioedema.htm
www.calgaryallergy.ca
The Canadian Hereditary Angioedema Society
www.haecanada.com
SYMPTOMS
DIAGNOSIS

ABOUT THE SOCIETY
SYMPTOMS
Hereditary Angioedema (HAE) and Acquired Angioedema (AAE) from C1 inhibitor deficiency are generally characterized by the occurrence of episodic attacks of swelling. This swelling may affect the face, extremities, genitals, gastrointestinal tract and upper airways. Some patients exhibit a distinctive rash. Swelling of the intestinal mucosa may lead to vomiting and painful, colic-like intestinal spasms which may mimic intestinal obstruction. Airway edema may be life-threatening. Episodes may be triggered by trauma, surgery, dental work, menstruation, some medications, viral illness and stress, however this is not always readily determined. DIAGNOSIS
The incidence of this rare disease is uncertain, but 1 in 10,000 to 1 in 50,000 is often quoted. HAE is an autosomal dominant trait: a child has a 50-percent chance of having HAE if one parent has it. Spontaneous mutation may account for HAE in the absence of family history. Patients with the genetic defect produce either insufficient or dysfunctional C1 inhibitor protein. C1 inhibitor is a complement protein that regulates various pathways within the body important in the maintenance of health, for example - fighting infections or healing inflammation. Sometimes when the demand for C1 inhibitor within the body isn't met, swelling occurs.

33. Genetic Disorders: Hereditary Angioedema
Genetic Disorders hereditary angioedema. A definition of hereditary angioedema along with a look at the causes, incidence and risk factors. EMedicine HAE.
http://www.puredirectory.com/Health/Conditions-and-Diseases/Genetic-Disorders/He
Genetic Disorders: Hereditary Angioedema
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A definition of hereditary angioedema along with a look at the causes, incidence and risk factors.
E-Medicine: HAE
An in dept look at hereditary angioedema beginning with an introduction, clinical features, differentials, work up, treatment, medication and foll...
Hereditary Angioedema Support Group
A non profit Organization dedicated to those families that are touched by a rare blood disease known as Hereditary Angioedema
MedicineNet.com - Medical Dictionary
A brief article about hereditary angioedema.
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Hereditary Angioedema, a general discussion, alternate names and resources.
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34. Pharming Group N.V.
Patients hereditary angioedema. Medical Need hereditary angioedema (HAE) is a genetic disorder which causes shortage of a protein called C1 inhibitor.
http://www.pharming.com/index.php?act=medi&pg=1

35. Conditions And Diseases - Hereditary Angioedema Top Links
hereditary angioedema Web Site Links. hereditary angioedema Support Group A non profit Organization dedicated to those families
http://www.disease-resources.com/Top_Health_Conditions_and_Diseases_Genetic_Diso
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Hereditary Angioedema Web Site Links 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.
MedicineNet.com - Medical Dictionary
A brief article about hereditary angioedema.
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Hereditary Angioedema, a general discussion, alternate names and resources.
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A definition of hereditary angioedema along with a look at the causes, incidence and risk factors.
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36. ► Hereditary Angioedema
Home Medical Reference Encyclopedia (English) Toggle English / Spanish hereditary angioedema. hereditary angioedema is an inherited disorder.
http://www.umm.edu/ency/article/001456.htm
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Hereditary angioedema
Overview Symptoms Treatment Prevention Definition: Hereditary angioedema is an inherited abnormality of the immune system that causes swelling , particularly of the face, and abdominal cramping. Also, see angioedema
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.
Antibodies
Review Date: 1/17/2002
Reviewed By: Frederic F. Little, M.D., Department of Allergy and Pulmonary/Critical Care Medicine, Boston University School of Medicine, Boston, MA and Julie A. Miller, M.D., Department of Surgery, Royal Melbourne Hospital, Melbourne, Australia. Review provided by VeriMed Healthcare Network.

37. Hereditary Angioedema - A Medical Reference Article
hereditary angioedema. A Medical Medical Center. A resource with information on over 10,000 medical topics including hereditary angioedema.
http://www.umm.edu/dwp/003273.htm
Hereditary angioedema
A Medical Article provided by University of Maryland Medical Center A resource with information on over 10,000 medical topics including: Hereditary angioedema Previous Next

38. Hereditary Angioedema - General Practice Notebook
hereditary angioedema. Hereditary angioneurotic oedema is an autosomal dominantly inherited condition caused by a deficiency of C1 esterase inhibitor.
http://www.gpnotebook.co.uk/cache/328859677.htm
hereditary angioedema Hereditary angioneurotic oedema is an autosomal dominantly inherited condition caused by a deficiency of C1 esterase inhibitor. The deficiency in C1 esterase inhibitor results in intermittent episodes of spontaneous complement activation. Clinically the patient suffers oedema of the skin and mucosal surfaces. Fatalities may occur if the airway is compromised.
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39. Disease - Hereditary Angioedema - Hartford, Connecticut , Saint Francis Care
Disease hereditary angioedema - courtesy of Saint Francis Care of Hartford, Connecticut, contemporary medicine with major clinical concentrations in heart
http://www.saintfranciscare.com/12357.cfm
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Disease - Hereditary angioedema
Antibodies Definition: Hereditary angioedema is an inherited abnormality of the immune system that causes swelling , particularly of the face, and abdominal cramping. Also, see angioedema Causes And Risk: 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. Prevention: Genetic counseling may be beneficial for prospective parents with a family history of hereditary angioedema.

40. THE MERCK MANUALSECOND HOME EDITION, Hereditary Angioedema Not
YearRound Allergies Food Allergy Mastocytosis Physical Allergy Exercise-Induced Allergic Reactions Hives and Angioedema Anaphylactic Reactions.
http://www.merck.com/mrkshared/mmanual_home2/sb/sb185_2.jsp

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