41. HEREDITARY ANGIOEDEMA Pediatric Database (PEDBASE); Discipline H O; Last Updated 8/03/93hereditary angioedema. DEFINITION A disorder of complement control http://www.icondata.com/health/pedbase/files/HEREDITA.HTM

Pediatric Database (PEDBASE) Last Updated: 8/03/93 HEREDITARY ANGIOEDEMA DEFINITION: A disorder of complement control proteins due to a deficiency of C1 inhibitor resulting in attacks characterized by circumscribed areas of swelling with gastrointestinal and respiratory complaints. EPIDEMIOLOGY: incidence: ? age of onset: first 2 years of life risk factors: familial - autosomal dominant (10% spontaneous mutation rate) chrom.#: 11q13.1 gene: complement component-1 inhibitor M = F PATHOGENESIS: 1. Background the C1 inhibitor gene has been cloned and contains 7 exons with intervening introns containing many Alu repeats the C1 inhibitor protein is composed of 478 amino acids which is cleaved between positions 444-445 and it is the amino terminal (444 amino acids) which is the active protein involved in the classsical pathway 2. Genetic Defects localized areas intestinal wall - abdominal cramps, diarrhea, vomiting laryngeal wall - upper airway obstruction it appears that C1 activity is stimulated under certain stressful situations which can precipitate an attack: emotional stress menses trauma vigorous exercise temperature extremes a C1 inhibitor deficiency may also be acquired with certain conditions: autoimmune (autoantibodies to C1 inhibitor) lymphoid cancer oral birth control pill pregnancy SLE 3. Types

42. Forward To The Herditary Angioedema Home Page The hereditary angioedema Support And Discussion Group Is Now Located Athttp//www.hereditaryangioedema.com. Please Update Your Bookmarks. http://home.sprintmail.com/~mccoyfs/

The Hereditary Angioedema Support And Discussion Group Is Now Located At http://www.hereditaryangioedema.com . Please Update Your Bookmarks. If Your Browser Does Not Automatically Forward You, Please Click On The Link Above To Go To The New Site.

43. Baxter News - Baxter And Pharming Start Clinical Study With Recombinant Human C1 BAXTER AND PHARMING START CLINICAL STUDY WITH RECOMBINANT HUMAN C1 INHIBITOR FORhereditary angioedema DEERFIELD, Ill., USA, and LEIDEN, the Netherlands, March http://www.baxter.com/utilities/news/releases/2001/03-09pharming.html

BAXTER AND PHARMING START CLINICAL STUDY WITH RECOMBINANT HUMAN C1 INHIBITOR FOR HEREDITARY ANGIOEDEMA DEERFIELD, Ill., USA, and LEIDEN, the Netherlands, March 9, 2001 - Baxter Healthcare Corporation and Pharming Group N.V. announced the start of a Phase I clinical study with recombinant human C1 inhibitor in asymptomatic patients with hereditary angioedema. This study will be the first clinical study with any form of recombinant human C1 inhibitor. In this study the safety, tolerability, clearance and activity of the product will be investigated. "We are very excited with the start of this clinical study", said Philippe van Holle, Pharming's executive vice president Biotherapeutics. He continued, "Genetic disorders receive special attention within Pharming. Recombinant human C1 inhibitor has been developed through our breakthrough transgenic technology and we hope it will prove to be a highly effective therapeutic modality in hereditary angioedema. This disorder has a strong impact on quality of life, due to its unpredictable nature, the potential severity of the attacks, which can be life-threatening, and the possible side effects of some currently available therapies." "Advancing our recombinant human C1 inhibitor into the clinic is an important milestone for people impacted by the rare but serious disease of hereditary angioedema", said Norbert Riedel, Ph.D., president of Baxter BioScience's recombinant business unit. "In addition, it is a key milestone for Baxter as we continue to build an innovative research pipeline to accelerate future business growth. We are particularly excited to be developing a transgenic product that complements our portfolio of plasma-derived and recombinant therapeutic proteins."

44. Dyax's Product Pipeline: DX88 Activated kallikrein is thought to play a role in a number of inflammatory and autoimmunediseases or conditions including hereditary angioedema, blood loss http://www.dyax.com/pipeline/dx88.asp

Home Careers Press Contact ... Product Pipeline Quick Find Working with Dyax Phage Display Dyax The Company Investor Relations Product Pipeline Careers Press Contact Us Home Page DX-890 DX-88 Links DX-88 Using phage display, Dyax discovered DX-88, a small protein that inhibits an enzyme in the blood called kallikrein. Kallikrein is a key component responsible for the regulation of inflammatory and blood clotting processes. Activated kallikrein is thought to play a role in a number of inflammatory and autoimmune diseases or conditions including hereditary angioedema, blood loss following major surgical procedures (such as cardiac bypass grafting and hip replacement), and rheumatoid arthritis. Due to its high specificity to kallikrein, DX-88 may have fewer side effects and/or greater effectiveness in the treatment of inflammation than naturally occurring inhibitors. Indication 1 - Hereditary Angioedema Hereditary angioedema (HAE) is a rare genetic disorder (autosomal dominant) that leads to acute attacks of localized swelling and inflammation. The prevalence of hereditary angioedema is believed to be between 1/10,000 and 1/50,000 people worldwide. HAE is caused by the deficiency of C1 esterase inhibitor (C1-INH), a molecule that inhibits kallikrein and other serine proteases in the blood. HAE is characterized by acute episodic attacks of edema (swelling) in body parts, most notably the hands, feet, face, and abdomen. In the case of an attack that effects the airway passages, HAE can be life-threatening. Abdominal attacks are often associated with bouts of severe pain, nausea, and vomiting caused by swelling in the intestinal wall.

45. Pharming Baxter and Pharming start clinical study with recombinant human C1 inhibitor forhereditary angioedema. Backgrounder on C1 inhibitor and hereditary angioedema. http://www.pharming.com/News/press_2001_09_04_TXT.html

March 9, 2001 back to the index of 2001 Baxter and Pharming start clinical study with recombinant human C1 inhibitor for Hereditary Angioedema Deerfield, IL, USA, and Leiden, the Netherlands, March 9, 2001 - Baxter Healthcare Corporation and Pharming Group N.V. announced the start of a Phase I clinical study with recombinant human C1 inhibitor in asymptomatic patients with hereditary angioedema. This study will be the first clinical study with any form of recombinant human C1 inhibitor. In this study the safety, tolerability, clearance and activity of the product will be investigated. "We are very excited with the start of this clinical study", said Philippe van Holle, Pharming's executive vice president Biotherapeutics. He continued, "Genetic disorders receive special attention within Pharming. Recombinant human C1 inhibitor has been developed through our breakthrough transgenic technology and we hope it will prove to be a highly effective therapeutic modality in hereditary angioedema. This disorder has a strong impact on quality of life, due to its unpredictable nature, the potential severity of the attacks, which can be life-threatening, and the possible side effects of some currently available therapies." "Advancing our recombinant human C1 inhibitor into the clinic is an important milestone for people impacted by the rare but serious disease of hereditary angioedema", said Norbert Riedel, Ph.D., president of Baxter BioScience's recombinant business unit. "In addition, it is a key milestone for Baxter as we continue to build an innovative research pipeline to accelerate future business growth. We are particularly excited to be developing a transgenic product that complements our portfolio of plasma-derived and recombinant therapeutic proteins."

46. Pharming PHARMING AND BAXTER TO CODEVELOP HUMAN C1 INHIBITOR TO TREAT hereditary angioedema.Glendale, CA, USA and Leiden, the Netherlands, March 21, 2000. http://www.pharming.com/News/press_2000_21_03_TXT.html

March 21, 2000 back to the index of 2000 PHARMING AND BAXTER TO CO-DEVELOP HUMAN C1 INHIBITOR TO TREAT HEREDITARY ANGIOEDEMA Glendale, CA, USA and Leiden, the Netherlands, March 21, 2000. Baxter Healthcare Corporation's Hyland Immuno division and Pharming Group N.V. today announced an agreement to collaborate on the development of recombinant human C1 Inhibitor to treat Hereditary Angioedema. This potentially fatal disease results in the swelling of tissues and organs and affects approximately 50,000 people in Europe and North America. Pharming is world renowned for its proprietary transgenic technology platform, which is integral to the production of recombinant human C1 Inhibitor. In 1999, the US Food and Drug Administration granted Pharming Orphan Drug Designations for human C1 Inhibitor for both acute and prophylactic treatment of Hereditary Angioedema. The drug is currently in pre-clinical studies and is expected to enter Phase I clinical development in the course of this year. "Baxter has extensive experience in commercializing plasma and recombinant proteins," said George J.M. Hersbach, President and Chief Executive Officer of Pharming, "Our strong technology position, combined with Baxter's clinical and marketing expertise, provides a significant opportunity to make this critical therapy available to patients who desperately need it."

47. IDR Factfile For Hereditary Angioedema ,hereditary angioedema has been known to exist for over a century.......General information. Disease, hereditary angioedema. http://bioinf.uta.fi/xml/idr/FF97.xml

General information Disease Hereditary angioedema Alternative names: Angioneurotic edema, hereditary; HANE C1 esterase inhibitor, deficiency of Angioedema, hereditary, type I Angioedema, hereditary, type II, included Description Hereditary angioedema has been known to exist for over a century. The disease has been attributed to a C1 esterase inhibitor deficiency, transmitted as an autosomal dominant condition. Hereditary angioedema is episodic and asymptomatic in most patients. People with hereditary angioedema can develop rapid swelling of the hands, feet, limbs, face, intestinal tract, or airway (larynx or trachea). Classification Defects of Complement Regulatory Proteins OMIM  Angioedema, hereditary; HAE Cross references Clinical information Description People with hereditary angioedema can develop rapid swelling of the hands, feet, limbs, face, intestinal tract, or airway (larynx or trachea). Hereditary and acquired angioedema are characterized by recurrent episodes of angioedema involving any part of the body. Laryngeal edema is common, and it is the major cause of death. Angioedema of the gastrointestinal tract frequently may mimic an acute abdomen, resulting in unnecessary surgery. The emergency physician should be aware of these conditions and should initiate prompt treatment in order to avoid unnecessary morbidity and death.

48. Hereditary Angioedema - General Practice Notebook hereditary angioedema. Hereditary angioneurotic oedema is an autosomal dominantlyinherited 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. Click here for more information...

49. Thesis INSTITUTE OF MEDICAL BIOLOGY, DEPARTMENT OF BIOCHEMISTRY UNIVERSITY OFTROMSØ. hereditary angioedema. by Erik Waage Nielsen. Du var vinden. http://www.nss.nl.no/utdanning/thesis/content.htm

Meny: STARTSIDE AVDELINGER PASIENTINFO HELSEPERSONELLINFO ... Klipp fra presse/media Se motivet fra vårt web-kamera på sykehustaket Web-ansvar: I nformasjonsleder Randi Angelsen og spesialsykepleier Knut Dybwik FROM THE DEPARTMENT OF ANESTHESIOLOGY AND THE DEPARTMENT OF IMMUNOLOGY AND TRANSFUSION MEDICINE, NORDLAND CENTRAL HOSPITAL, BODØ. INSTITUTE OF MEDICAL BIOLOGY, DEPARTMENT OF BIOCHEMISTRY UNIVERSITY OF TROMSØ HEREDITARY ANGIOEDEMA by Erik Waage Nielsen Du var vinden Eg er ein båt utan vind. Du var vinden. Var det den leidi eg skulde? Kven spør etter leidi når en har slik vind! Olav H. Hauge Tilegnet Frank Henry Waage Contents Acknowledgements Summaries of Findings: List of Papers Paper I Abbreviations Paper II Introduction: Paper III Hereditary angioedema Paper IV Plasma cascade systems with emphasis on the role of C1-inhibitor ... · Kallikrein-kinin system Discussion: · Coagulation Clinical aspects · Fibrinolysis Functional assay of C1-inhibitor ... Cascade activation Purpose of the study: Applicability of HAE-studies to edema in non-HAE patients Background Conclusions Aim ... Papers I - VI (Citations from Medline with Abstracts) Download as word 97-file thesis.zip

50. Thesis hereditary angioedema Quincke first described and named angioneurotic edema (1),the striking influence of mental stress on the disorder led to the term http://www.nss.nl.no/utdanning/thesis/intro1.htm

Meny: STARTSIDE AVDELINGER PASIENTINFO HELSEPERSONELLINFO ... Klipp fra presse/media Se motivet fra vårt web-kamera på sykehustaket Web-ansvar: I nformasjonsleder Randi Angelsen og spesialsykepleier Knut Dybwik Previous: Abbreviations Introduction Hereditary angioedema Quincke first described and named angioneurotic edema (1), the striking influence of mental stress on the disorder led to the term neurotic which now is abandoned as the genetic cause is known. Nathaniel Hawthorne was apparently familiar with this disorder for in his 'House of the Seven Gables' (2), he describes the Pyncheon family with members who gurgled in the throat and chest when excited and who would sometimes die this way, ever since a curse to choke on blood had been placed on one of their ancestors. The following passages could indicate that Hawthorne recognised that a hereditary disease, not a curse, was responsible for the deaths: "This mode of death has been an idiosyncrasy with his family, for generations past" and "the curse,.. had become a part of the Pyncheon inheritance" (3). Persons with hereditary angiodema are victims of recurrent bouts of edema which may occur in nearly every part of the body (Fig. 1 - 3). Attacks typically last for 2 to 5 days. The disease has been reviewed in several papers (4-11).

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

52. Dorlands Medical Dictionary hereditary angioedema, inherited C1 inhibitor (C1 INH) deficiency, an autosomal dominantdisorder manifested as recurrent episodes of edema of the skin, upper http://www.mercksource.com/pp/us/cns/cns_hl_dorlands.jspzQzpgzEzzSzppdocszSzuszS

53. Health Library Find Information On Hereditary Angioedema At Find information on hereditary angioedema at MerckSource. Learn moreabout hereditary angioedema. Definition hereditary angioedema is http://www.mercksource.com/pp/us/cns/cns_hl_adam.jspzQzpgzEzzSzppdocszSzuszSzcns

54. From The Grand Rounds Archive At Baylor hereditary angioedema is rare, but knowledge of its mechanism allowsdiagnosis and initiation of potentially lifesaving therapy. http://www.bcm.tmc.edu/oto/grand/52694.html

Grand Rounds Archives The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at The Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. ANGIOEDEMA May 26, 1994 Thomas A. Salzer, M.D. Angioedema is a problem that relates to the specialty of otolaryngology because its symptoms manifest most often in the head and neck, often in dramatic form. The term angioedema is used to describe several closely related diseases that manifest by recurrent acute edema of the skin and mucosa. The most serious manifestation of the disease is laryngeal edema which can be fatal. Hereditary angioedema is rare, but knowledge of its mechanism allows diagnosis and initiation of potentially lifesaving therapy. Angiotensin converting enzyme inhibitors account for an increasing proportion of angioedema cases as the popularity of the drug class grows. Angioedema is characterized by subcutaneous edema resulting from increased vascular permeability, dilation of venules and capillaries and separation of collagen bundles. This is in distinction to urticaria which is limited to the superficial dermis. A history of atopy can be elicited in 15% patients.

55. Health Library - Angioedema, Hereditary hereditary angioedema is a rare inherited vascular disorder characterized by anexcessive accumulation of body fluids that results in areas of obstruction in http://health_info.nmh.org/Library/HealthGuide/IllnessConditions/topic.asp?hwid=

56. Health Ency.: Disease: Hereditary Angioedema hereditary angioedema. Prevention. Genetic counseling may be beneficial forprospective parents with a family history of hereditary angioedema. Ency. http://www.accessatlanta.com/shared/health/adam/ency/article/001456prv.html

SEARCH: The Web Yellow Pages HOME AJC.COM Illustrated Health Encyclopedia Important notice Ency. home Disease H Hereditary angioedema Overview Symptoms Treatment Prevention Prevention Genetic counseling may be beneficial for prospective parents with a family history of hereditary angioedema. Ency. home Disease H 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

57. ThirdAge - Adam - Hereditary Angioedema hereditary angioedema is an inherited abnormality of the immune system thatcauses swelling, particularly of the face, and abdominal cramping http://www.thirdage.com/health/adam/ency/article/001456.htm

document.write(''); document.write(''); document.write('<'); document.write('/SCRIPT>'); document.write(''); document.write(''); document.write('<'); document.write('/A>'); document.write('<'); document.write('/NOSCRIPT>'); document.write('<'); document.write('/IFRAME>'); Activities Computers Family Tree Health ... Prevention Hereditary angioedema 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.

58. Dyax Initiates EDEMA1 Phase II Trial Of DX-88 For Hereditary Angioedema Dyax Initiates EDEMA1 Phase II Trial of DX88 for hereditary angioedema.Wednesday, 29-Jan hereditary angioedema. The prevalence of http://quickstart.clari.net/qs_se/webnews/wed/cl/Bma-dyax-corp.Rce7_DJT.html

Get the depth on this story! Dyax Initiates EDEMA1 Phase II Trial of DX-88 for Hereditary Angioedema Wednesday, 29-Jan-2003 5:20AM PST Story from Dyax via BizWire Dyax Initiates EDEMA1 Phase II Trial of DX-88 for Hereditary Angioedema; Provides Update of Clinical Program Clari News Two hours to dynamic, compelling News on YOUR site! The primary endpoints of the EDEMA1 study are efficacy and safety. Drug safety is being monitored during the study by an independent committee. To the Company's knowledge, DX-88 is the only investigational treatment for HAE in clinical trials in the United States, where no marketed therapy is available. Evaluation of DX-88 in the HAE indication is being conducted by Dyax under its collaboration with Genzyme Corporation. The EDEMA1 study complements the Company's ongoing open-label phase II study of DX-88 in angioedema being conducted in Europe. In that study, eight patients have been treated, all of whom had relief of symptoms within four hours. The duration of a typical angioedema attack would be expected to last from two to five days without treatment. An anaphylactoid reaction occurred in one patient, yet the reaction was controlled quickly and DX-88 treatment was completed per protocol. "Acute attacks of angioedema are both distressing and potentially fatal to the patient. Currently, there is little specific therapy that can be offered, and EDEMA1 will prospectively study the effect of kallikrein inhibition in this acute and hereditary condition. We look forward to continuing this clinical development program in accordance with U.S. Food and Drug Administration requirements and in close collaboration with our EDEMA1 investigators and patient advocacy groups such as the Hereditary Angioedema Association," commented Tony Williams, M.D., Senior Vice President of Medical Affairs and Clinical Operations for Dyax.

59. Welcome To ENH.org - Health Encyclopedia: Hereditary Angioedema hereditary angioedema. Also, see angioedema. Causes, incidence, andrisk factors hereditary angioedema is an inherited disorder. http://www.enh.org/Encyclopedia/ency/article/001456.asp

Disease Reference Injury Reference Test Reference Nutrition Reference ... Symptoms Reference Hereditary angioedema Disease Injury Nutrition Poison ... Z 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. Symptoms: Swelling in the arms, legs, lips, eyes, tongue, or throat

60. Searchalot Directory For Hereditary Angioedema hereditary angioedema Support Group A non profit Organization dedicated to thosefamilies that are touched by a rare blood disease known as Hereditary http://www.searchalot.com/Top/Health/ConditionsandDiseases/GeneticDisorders/Here

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