41. ► Hereditary Angioedema A medical encycopedia article on the topic hereditary angioedema. http://www.umm.edu/ency/article/001456.htm

var MenuLinkedBy='AllWebMenus [2]', awmBN='530'; awmAltUrl=''; Disease Nutrition Surgery Symptoms Injury ... Encyclopedia (English) Toggle English Spanish 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.

42. Hives: Definition And Much More From Answers.com This form is called hereditary angioedema (HAE) or hereditary Treatment of hereditary angioedema with a vaporheated C1 inhibitor concentrate. http://www.answers.com/topic/angioedema

showHide_TellMeAbout2('false'); Business Entertainment Games Health ... More... On this page: Dictionary Diagnosis Encyclopedia Health WordNet Wikipedia Mentioned In Or search: - The Web - Images - News - Blogs - Shopping hives Dictionary hives hīvz pl.n. (used with a sing. or pl. verb) A skin condition characterized by intensely itching welts and caused by an allergic reaction to internal or external agents, an infection, or a nervous condition. Also called nettle rash urticaria [Origin unknown.] Diagnosis Hives (Urticaria) And Angioedema What are hives (urticaria)? "Hives" and "welts" are red, itchy, and raised areas of the skin of varying shapes and sizes. Medically, hives are also known as urticaria. Hives are the result of histamine and other compounds that are released from cells called mast cells. Histamine causes serum to leak from the local blood vessels, which causes swelling in the skin. Hives can occur anywhere on the body, but are most commonly found on the trunk. Hives tend to erupt in clusters. Clusters of hives typically fade away and clear up within 2-48 hours as new clusters appear on other areas of the skin. Urticaria is a common skin condition that will affect one out of every five persons in the population. The cause of urticaria is often unknown. Fortunately, urticaria usually resolves by itself, and is rarely life-threatening.

43. Canadian Hereditary Angioedema Society hereditary angioedema (HAE) and Acquired Angioedema (AAE) from C1 inhibitor deficiency By fall 2001, the Canadian hereditary angioedema Society/Société http://www.calgaryallergy.ca/Articles/HereditaryAngioedema.htm

44. Angioedema, Hereditary hereditary angioedema is a rare inherited disorder characterized by recurrent episodes of the accumulation of fluids outside of the blood vessels, http://www.bchealthguide.org/kbase/nord/nord98.htm

var hwPrint=1;var hwDocHWID="nord98";var hwDocTitle="Angioedema, Hereditary";var hwRank="1";var hwSectionHWID="nord98-Header";var hwSource="en-caQ2_05";var hwDocType="Nord"; National Organization for Rare Disorders, Inc. Angioedema, Hereditary Important It is possible that the main title of the report Angioedema, Hereditary 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. Synonyms 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 General Discussion Hereditary angioedema is a rare inherited disorder characterized by recurrent episodes of the 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.

45. Patients With Hereditary Angioedema - St. Joseph Mercy, Ann Arbor Michigan Patients with hereditary angioedema St. Joseph Mercy Health System Hospitals serving Ann Arbor, SE Michigan, Washtenaw County, Livingston County, http://www.sjmercyhealth.org/1718.cfm

@import url(default.css); Clinical Trials Treatment Allergy and Immunology Trial Title Patients with Hereditary Angioedema Description Drug Study for Patients with Hereditary Angioedema. Study�Number HSR-04-0372 Principal�Name Leflein MD, Jeffrey Phone Back Clinical Research Report Job Openings RN Recruiting Events ... Physicians Assistant Residency Program

46. Angioedema - Wikipedia, The Free Encyclopedia This form is called hereditary angioedema (HAE) or hereditary angioneurotic In hereditary angioedema, there is often no direct identifiable cause, http://en.wikipedia.org/wiki/Angioedema

You did it! Over US$240,000 was donated in the 21 day fund drive. Thank you for your generosity! You are still welcome to make a donation or purchase Wikimedia merchandise Angioedema From Wikipedia, the free encyclopedia. Angioedema BE angiooedema ), also known by its eponym Quincke's edema and the older term angioneurotic edema , is the rapid swelling ( edema ) of the skin mucosa and submucosal tissues. Apart from the common form, mediated by allergy , it has been reported as a side effect of some medications , specifically ACE inhibitors . Additionally, there is an inherited form, due to deficiency of the blood protein C1-inhibitor . This form is called hereditary angioedema HAE ) or hereditary angio-neurotic edema (HANE), which is due to C1-esterase inhibitor deficiency. Cases where angioedema progresses rapidly should be treated as a medical emergency as airway obstruction and suffocation can occur. Rapid treatment with epinephrine , often with an epi-pen , can be life-saving. Contents Signs and symptoms Diagnosis Pathophysiology Therapy ... edit Signs and symptoms The skin of the face (around the mouth) and the mucosa of the mouth, as well as the

47. HAEA Frequently Asked Questions hereditary angioedema (HAE) is a rare and serious genetic condition occurring in about 1/10000 to At what age do attacks of hereditary angioedema start? http://haea.org/pamphlet.htm

var MenuLinkedBy='AllWebMenus [2]', awmBN='530'; awmAltUrl=''; United States Hereditary AngioEdema Association C1 Inhibitor Deficiency Disorder Presents Frequently Asked Questions What is Hereditary Angioedema? Hereditary Angioedema (HAE) is a rare and serious genetic condition occurring in about 1/10,000 to 1/50,000 individuals. The disease is characterized 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. HAE is called hereditary because the genetic defect is passed on in families. A child has a 50 percent chance of inheriting this disease if one of his or her parents has it. The absence of family history does not rule out the HAE diagnosis, however. Scientists report that as many as 20 percent of HAE cases result from patients who had a spontaneous mutation of the C1-inhibitor gene at conception. These patients can pass the defective gene to their offspring.

48. United States Hereditary Angioedema Association Information Concerning A Rare Blood Disease Called hereditary angioedema. http://haea.org/database.html

Be A Part Of Our Clinical Trial Database Dear HAE Friend: 5 TRIALS, 60 PATIENTS PER TRIAL, 300 WILLING PARTICPANTS NEEDED! A new clinical trial database has been created for those of you who would like to participate in a clinical trial. After submission of the clinical trial form, HAE clinical trial coordinator Donna Davis will contact you with information on clinical trials in your area. These trials are for patients that have a confirmed diagnosis of Hereditary Angioedema. “This is a pivotal moment for all HAE patients in America. The HAE Association is dedicated to ensuring that each of the five drug companies (Dyax/Genzyme, Jerini, Pharming, Lev Pharma, and ZLB Behring) conducting US clinical trials have enough patients to complete testing on their promising new therapies. It is very unusual to have this level of interest in a rare disease. We need to seize the moment and help get all of these drugs approved Having options is what it is all about, we now have the chance to make this a reality” Anthony Castaldo, HAEA President. Now is the time for all HAE suffers to do their part. Please consider this opportunity to have access to promising new acute attack therapies while seizing the moment to bring the same, long awaited standard of care to the US that other counties have benefited from for many years.

49. Hereditary Angioedema - Washington DC hereditary angioedema Washington Hospital Center is located in Washington DC. http://www.whcenter.org/15243.cfm

cddcodebase = "navtools/topmenu/";cddcodebase367050 = "navtools/topmenu/"; Article Manager Home Back to Health Library Print This Page ... Antibodies 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 low levels or improper function of a protein called C1 inhibitor . This, in turn, affects 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. There is usually a family history of the condition, but this may 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

50. Hereditary Angioedema hereditary angioedema (HAE) is an autosomal dominant disorder hereditary angioedema is caused by mutation in the C1 inhibitor gene (C1NH) (MIM.606860). http://www.humpath.com/article.php3?id_article=4216

51. Hereditary Angioedema And Lupus Erythematosus hereditary angioedema and Lupus Erythematosus Theresa Pacheco MD1 Dermatology Online Journal 7(1)24B. 1.Department of Dermatology, University of Colorado http://dermatology.cdlib.org/DOJvol7num1/medderm/abstracts/pacheco.html

DOJ Contents English Portuguese Hereditary Angioedema and Lupus Erythematosus Theresa Pacheco MD Dermatology Online Journal 7(1):24B 1.Department of Dermatology, University of Colorado Health Sciences Center 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.

52. Disease Category Listing (653): Hereditary Angioedema CenterWatch Listing of Clinical Research Trials for hereditary angioedema. http://www.centerwatch.com/patient/studies/cat653.html

Clinical Trials: Hereditary Angioedema Alabama Various Cities; Research Center Human C1 Esterase Inhibitor (C1-INH) in Subjects with Congenital C1-INH Deficiency and Acute Abdominal or Facial HAE Attacks. Alaska Various Cities; Research Center Human C1 Esterase Inhibitor (C1-INH) in Subjects with Congenital C1-INH Deficiency and Acute Abdominal or Facial HAE Attacks. Arizona Various Cities; Research Center Human C1 Esterase Inhibitor (C1-INH) in Subjects with Congenital C1-INH Deficiency and Acute Abdominal or Facial HAE Attacks. Arkansas Various Cities; Research Center Human C1 Esterase Inhibitor (C1-INH) in Subjects with Congenital C1-INH Deficiency and Acute Abdominal or Facial HAE Attacks. California Various Cities; Research Center Human C1 Esterase Inhibitor (C1-INH) in Subjects with Congenital C1-INH Deficiency and Acute Abdominal or Facial HAE Attacks. Colorado Various Cities; Research Center Human C1 Esterase Inhibitor (C1-INH) in Subjects with Congenital C1-INH Deficiency and Acute Abdominal or Facial HAE Attacks. Connecticut Various Cities; Research Center

53. Study Posting (2337) -- Trial #64031, Hereditary Angioedema, Mineola, NY CenterWatch Clinical Research Trial Posting for Hereditary AngiodemaEDEMA2Evaluation of DX-88 s Effects in Mitigating Angiodema. http://www.centerwatch.com/patient/studies/stu64031.html

Trial Information Summary: Hereditary Angiodema-EDEMA2:Evaluation of DX-88's Effects in Mitigating Angiodema. An Open-label study to assess the efficacy and Tolerability of Repeated Doses of DX-88 (recombinant plasma kallikrein inhibitor) in patients with Hereditary Angioedema (HAE). Inclusion Criteria: Patient must be 10 years of age or older Patient must have a confirmed diagnosis of HAE determined by clinical and laboratory criteria Exclusion Criteria: Patients must not have serious intercurrent illness or active infection For more information, Contact: Kira Lupeke, RN, Nurse Coordinator Winthrop South Nassau University Health System Clinical Trials Network 222 Station Plaza North, Suite 300 Mineola, NY 11501 Telephone: 516-663-9582 Fax: 516-663-9587 Email: Profile Page: Winthrop South Nassau University Health System Clinical Trials Network, Mineola, NY Provided by MapQuest If you would like to learn more about participating in this study, please send an e-mail message using the form below. Name: Address: City: State: Zip: Phone: Email address: Message: Please note: A number of commercial online services display an error message after you click on Send. Please be assured that your message has been received.

54. Arch Intern Med Hereditary Angioedema And Hormones, October 14 We read with interest the scholarly review on hereditary angioedema by Nzeako et hereditary angioedema a broad review for clinicians. Arch Intern Med. http://archinte.ama-assn.org/cgi/content/extract/162/18/2142

55. Arch Intern Med -- Abstract: Hereditary Angioedema: A Broad Review For Clinician hereditary angioedema (HAE) is an autosomal dominant disease that afflicts 1 in Immunol. 2003;10982989. FULL TEXT. hereditary angioedema and Hormones http://archinte.ama-assn.org/cgi/content/abstract/161/20/2417

Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress Vol. 161 No. 20, November 12, 2001 Featured Link E-mail Alerts Review Article Article Options Full text PDF Send to a Friend Related articles in this issue ... Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager PubMed citation Articles in PubMed by Nzeako UC Tremaine WJ Articles that cite this article ISI Web of Science (32) ... Contact me when this article is cited Topic Collections Review Topic Collection Alerts Hereditary Angioedema A Broad Review for Clinicians Ugochukwu C. Nzeako, MD, MPH Evangelo Frigas, MD William J. Tremaine, MD Arch Intern Med. Hereditary angioedema (HAE) is an autosomal dominant disease HAE has been reported in all races, and no sex predominance has been found. It manifests as recurrent attacks of intense, massive, localized edema without concomitant pruritus, often

56. 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. Click here for more information...

57. HEREDITARY ANGIOEDEMA hereditary angioedema. DYAX’ DX88. We love to see new drugs treat diseases that have yet to find specific treatments. One of the treatments that are are http://www.prohostonline.com/ImpactingNews/IN 4 29 05.htm

Prohost Biotechnology Online The Newsletter of Biotechnology Research and Investment HEREDITARY ANGIOEDEMA DYAX’ DX-88 We love to see new drugs treat diseases that have yet to find specific treatments . One of the treatments that are are promising is Dyax’ (DYAX) DX-88 (ecallantide) for the treatment of hereditary angioedema (HAE). Why important? Because the attacks can be life-threatening if laryngeal edema occurs and obstructs patients airways. Acute swelling and inflammation can peripherally affect the extremities (hands, feet, face). When they occur, peripheral attacks are the most physically disfiguring. All types of HAE attacks are debilitating. Severe facial edema can also progress to the larynx. Abdominal obstruction caused by HAE is often associated with bouts of severe pain, nausea, and vomiting caused by swelling in the intestinal wall. On average, patients have 12 HAE attacks per year which, if left untreated, endure for two to five days. The inconsistent nature of HAE attacks contributes to the patients' sense of uncertainty and the fear of having a laryngeal attack that can rapidly close the airways The condition is caused by a genetic deficiency of C1 esterase inhibitor (C1-INH), a naturally occurring molecule that inhibits kallikrein and other serine proteases in the blood. There is

58. Disease - Hereditary Angioedema - Hartford, Connecticut 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

@import url(main.css); About Us Health Information Health Focus Todays Breast Health News ... Ways To Help Adam Article Manager Back Back to main Health Information page 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.

59. Angioedema hereditary angioedema (HAE), type I Recurrent episodes involving both skin and 277.6 Other deficiencies of circulating enzymes (hereditary angioedema) http://www.5mcc.com/Assets/SUMMARY/TP0060.html

Angioedema DESCRIPTION: Rapid, localized edema of subcutaneous tissues up to several centimeters in diameter. Life threatening if larynx, pharynx is affected. Resolves in hours to days. Hereditary angioedema (HAE), type I: Recurrent episodes involving both skin and mucous membranes, without urticaria. 25% mortality. Many have abdominal pain from edema of intestinal mucosa. Due to hereditary deficiency of C1-esterase inhibitor (C1-INH). HAE, type II: Like type I, but has normal levels of non-functional C1-INH Acquired angioedema: Rare, in some patients with lymphoproliferative malignancies. Treatment of malignancy causes resolution of edema. Autoantibody inactivation or consumption of C1-INH. Vibratory angioedema: Rare. Local swelling in response to vibration. May be hereditary. Drug-induced angioedema: Immunologic hypersensitivity, as in penicillin reaction, or non-immunologic, as in aspirin or NSAID Angiotensin converting enzyme (ACE) inhibitor-induced angioedema has become the most common form of AE. Nonimmune; tendency to involve tongue, with life-threatening airway edema. Can't predict. May occur immediately, or many months after starting drug. Idiopathic angioedema: Acute or chronic System(s) affected: Skin/Exocrine Genetics: 2% of cases of angioedema are hereditary autosomal dominant 85% have CI-INH deficiency 15% have normal levels of defective CI-INH Incidence/Prevalence in USA: Approximately 1 in 5,000. Accompanies urticaria 50% of time.

60. Hereditary Angioedema Have a diagnosis of hereditary C1INH deficiency; Have a documented history of facial or abdominal attacks; Are 6 years of age or older http://www.haetrial.com/

Study Title Human C1 Esterase Inhibitor (C1-INH) in Subjects With Congenital C1-INH Deficiency and Acute Abdominal or Facial HAE Attacks. Study Purpose The purpose of this study is to show that C1-INH concentrate (an investigational product) shortens the time to onset of relief of symptoms of abdominal or facial HAE attacks compared to placebo. What is HAE? HAE is a rare disorder that results from a genetic deficiency of the plasma protein, C1 inhibitor (C1-INH). HAE is characterized by attacks of edema (swelling) in the hands, feet, face, abdomen, and/or throat (airway). What is C1-INH? C1-INH concentrate is an investigational plasma-derived intravenous therapy, containing the protein that is missing or defective in HAE patients. This study will test whether replacing the missing C1 protein in acute HAE attacks will help relieve the pain and swelling associated with this disease. Who can participate?

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