21. Arch Intern Med -- Page Not Found 162;2142, October 14, 2002, hereditary angioedema and Hormones, Ajit Singh Kashyap,MD; Surekha Kashyap, MD Ugochukwu C. Nzeako, MD, MPH; William J. Tremaine http://archinte.ama-assn.org/issues/v162n18/ffull/ilt101402-4.html

Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery MSJAMA Science News Updates Meetings Peer Review Congress The page you requested was not found. The JAMA Archives Journals Web site has been redesigned to provide you with improved layout, features, and functionality. The location of the page you requested may have changed. To find the page you requested, click here HOME CURRENT ISSUE PAST ISSUES ... HELP Error 404 - "Not Found"

22. Arch Intern Med -- Page Not Found hereditary angioedema A Broad Review for Clinicians Author Information UgochukwuC. Nzeako, MD, MPH; Evangelo Frigas, MD; William J. Tremaine, MD http://archinte.ama-assn.org/issues/v161n20/abs/ira10011.html

Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery MSJAMA Science News Updates Meetings Peer Review Congress The page you requested was not found. The JAMA Archives Journals Web site has been redesigned to provide you with improved layout, features, and functionality. The location of the page you requested may have changed. To find the page you requested, click here HOME CURRENT ISSUE PAST ISSUES ... HELP Error 404 - "Not Found"

23. Health Ency.: Disease: Hereditary Angioedema hereditary angioedema. Also, see angioedema. Causes and Risks. Hereditaryangioedema is an inherited disorder. It is caused by the http://www.austin360.com/shared/health/adam/ency/article/001456.html

SEARCH: The Web Yellow Pages HOME Illustrated Health Encyclopedia Important notice Ency. home Disease H 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 and Risks 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. Ency. home

24. Health Ency.: Disease: Hereditary Angioedema hereditary angioedema. http://www.austin360.com/shared/health/adam/ency/article/001456trt.html

SEARCH: The Web Yellow Pages HOME Illustrated Health Encyclopedia Important notice Ency. home Disease H Hereditary angioedema Overview Symptoms Treatment Prevention Treatment Try to prevent attacks by avoiding trauma and precipitating factors. In contrast to other causes of angioedema (e.g., from allergies), antihistamines and other related treatments are of limited benefit in hereditary angioedema. Attenuated androgens, which are derivatives of normal sex hormones, can reduce the frequncy and severity of attacks. Once an attack occurs, treatment includes pain relief and intravenous fluids. A new treatment using C1-inhibitor concentrate is being tested. Prognosis Because this disorder can be life-threatening and treatment options are limited, avoidance of triggers is crucial. There are no definitive studies on the outlook, as individuals differ in their patterns and severity of symptoms. Complications Swelling of the airways may be fatal. Call Your Health Care Provider If: Call your health care provider if you are considering having children and would like some additional information about this disorder, or if you are experiencing a group of the above symptoms. Swelling of the airway is a life-threatening emergency. If you are having

25. HAE Danmark - Hereditary Angioedema Translate this page Wir beschäftigen uns auf freiwilliger basis mit Patienten und Angehörigen zu Patientendie unter HAE hereditary angioedema / Hereditäres AngioÖdem leiden. http://www.hae.dk/de.htm

Willkommen bei www.hae.dk Sie haben die Internetseite der dänischen HAE Patienten Vereinigung "HAE Danmark" erreicht. Wir beschäftigen uns auf freiwilliger basis mit Patienten und Angehörigen zu Patienten die unter HAE Hereditary AngioEdema / Hereditäres AngioÖdem leiden. Durch dieses Link werden Sie bald eine aufsummierung der dänischen Webseite finden! Haben Sie Fragen bezüglich HAE in Dänemark sind Sie willkommen uns zu kontakten: Henrik Boysen Lindeparken 33 DK-6230 Rothenkrug Dänemark Phone: +45 73 693 693 Mobile: +45 40 632 632 Fax: +45 73 694 695 E-mail: henrik.boysen@hae.dk Danke für Ihren Besuch! Bitte kommen Sie bald wieder zurück!

26. HAE Danmark - Hereditary Angioedema Der er skabt et internationalt netværk mellem læger og patientforeninger, hvorman kan hente et hav af informationer om HAE (hereditary angioedema). http://www.hae.dk/main1.htm

Velkommen til www.hae.dk Velkommen til Danmarks første og eneste webside tilegnet patienter der lider af sygdommen Hereditært Angioødem (HAE) samt deres pårørende. D er er skabt et internationalt netværk mellem læger og patientforeninger, hvor man kan hente et hav af informationer om HAE (Hereditary Angioedema). Se under "mere information" for at finde en række spændende links. EFTERLYSNING Hjælp os!! Vi mangler både fotos og personlige oplevelser, historier, kommentarer til InfoBrev, informationsmateriale til patienter og pårørende samt artikler i bl.a. Ugeskrift for læger. Jeres bidrag er afgørende for den korrekte og bedste formidling af sygdomsbilledet overfor personer som ikke har et forudgående kendskab til HAE. Kun gennem denne formidling kan vi på sigt sikre en interesse for sygdommen samt en opsporing af sygdomstilfælde og dermed en rettidig og korrekt behandling. Historier, oplevelser og billeder kan sendes til patientforeningen: Patientforeningen HAE Danmark, Lindeparken 33, 6230 Rødekro. Patientforeningen ønsker alle en rigtig glædelig jul og et godt og lykkebringende nytår!

27. NEJM -- Treatment Of Hereditary Angioedema With A Vapor-Heated C1 Inhibitor Conc Original Article from The New England Journal of Medicine Treatment ofhereditary angioedema with a VaporHeated C1 Inhibitor Concentrate. http://content.nejm.org/cgi/content/abstract/334/25/1630

HOME SEARCH CURRENT ISSUE PAST ISSUES ... HELP 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. Table of Contents Full Text of this article PDF of this article Find Similar Articles in the Journal ... Related Articles in Medline Articles in Medline by Author: Waytes, A. T. Frank, M. M. Medline Citation Child Health ... Related Chapters at Harrison's Online 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.

28. NEJM -- Replacement Therapy In Hereditary Angioedema: Successful Treatment Of Ac Original Article from The New England Journal of Medicine Replacement therapyin hereditary angioedema successful treatment of acute episodes of angioedema http://content.nejm.org/cgi/content/short/302/10/542

HOME SEARCH CURRENT ISSUE PAST ISSUES ... HELP Previous Volume 302:542-546 March 6, 1980 Number 10 Next Replacement therapy in hereditary angioedema: successful treatment of acute episodes of angioedema with partly purified C1 inhibitor JE Gadek, SW Hosea, JA Gelfand, M Santaella, M Wickerhauser, DC Triantaphyllopoulos, and MM Frank Table of Contents Find Similar Articles in the Journal Notify a friend about this article Add to Personal Archive ... Related Articles in Medline Articles in Medline by Author: Gadek, J. E. Frank, M. M. Medline Citation Abstract This article has been cited by other articles: Shah, U. K., Jacobs, I. N. (1999). Pediatric Angioedema: Ten Years' Experience. Arch Otolaryngol Head Neck Surg [Abstract] [Full Text] Bork, K., Barnstedt, S.-E. (2001). Treatment of 193 Episodes of Laryngeal Edema With C1 Inhibitor Concentrate in Patients With Hereditary Angioedema. Arch Intern Med [Abstract] [Full Text] Fay, A, Abinun, M (2002). Current management of hereditary angio-oedema (C'1 esterase inhibitor deficiency). J Clin Pathol [Abstract] [Full Text] Caliezi, C., Wuillemin, W. A., Zeerleder, S., Redondo, M., Eisele, B., Hack, C. E. (2000). C1-Esterase Inhibitor: An Anti-Inflammatory Agent and Its Potential Use in the Treatment of Diseases Other Than Hereditary Angioedema.

29. JRM Jul96: Fresh Frozen Plasma Prophylaxis For Hereditary Angioedema During Preg Title Fresh Frozen Plasma Prophylaxis for hereditary angioedema During PregnancyA Case Report Authors Henry L. Galan, MD, Mark B. Reedy, MD, John Starr, MD http://www.jreprodmed.com/abs/jrm123.htm

Title: Fresh Frozen Plasma Prophylaxis for Hereditary Angioedema During Pregnancy A Case Report Authors: Henry L. Galan, M.D., Mark B. Reedy, M.D., John Starr, M.D., and Alfred B. Knight, M.D. Background : Hereditary angioedema (HAE) is an uncommon disorder of the complement system due to a deficiency or dysfunction of the inhibitor of the first component of complement (C1 INH). Clinically, HAE is characterized by episodic abdominal pain or edema of the extremities, face, larynx and vulva. Laryngeal edema is potentially lethal and accounts for mortality rates as high as 30%. Therapy is divided into short-term (fresh frozen plasma) and long-term (antifibrinolytic agents, hormonal therapy) prophylaxis, or acute treatment (epinephrine, steroids, antihistamines). Case: An 18 year-old, primiparous woman presented with a history of multiple episodes of abdominal pain, swelling of abdomen and extremities. A diagnosis of hereditary angioedema was made based on the patient's and family's history and on decreased levels of C1 INH and complement component 4. Standard prophylactic methods were contraindicated due to potential teratogenic effects. Fresh frozen plasma given twice weekly over 2.5 months resulted in reduction in the number and severity of attacks and allowed successful completion of pregnancy. Conclusion: This is the first report of fresh frozen plasma therapy as a means of long-term prophylaxis in a pregnant woman. Fresh frozen plasma may serve as an alternate mode of long-term prophylaxis if the standard agents (antifibrinolytic, hormone) are unsuccessful or contraindicated. (J Reprod Med 1996;41:541#544)

30. Canadian Hereditary Angioedema Society CAN logo www.calgaryallergy.ca. The Canadian HereditaryAngioedema 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.

31. Hereditary Angioedema In Europe Main Frame hereditary angioedema (HAE ) IN EUROPE. Associations, organizations,patients groups or people involved with hereditary angioedema. http://www.angioedemaereditario.org/angioedema Europe GB 1.htm

H.A.E. - HEREDITARY ANGIOEDEMA - INTERTIONAL NET For each nation indicated in the index, we listed all the information available to us and useful to patients with Hereditary Angioedema, regarding : Associations, organizations, patients groups or people involved with Hereditary Angioedema. Representatives who are capable to communicate in english or other languages. Hospital centers who are able to correctly treat the patients with Hereditary Angioedema. Doctors who are capable to communicate in english or other languages. Name and address of the distributing company of C-1 Hum an Inhibitor.

32. Hereditary Angioedema In The World Main Frame hereditary angioedema (HAE) IN THE WORLD Associations, organizations,patients groups or people involved with hereditary angioedema. http://www.angioedemaereditario.org/angioedema world 1.htm

33. 1998 Archives - June C1 Esterase Inhibitor Transfusions in Patients with hereditary angioedema. C1EsteraseInhibitor Transfusions in Patients with hereditary angioedema. http://allergy.edoc.com/1998_archives/98_jun.html

June 1998 GUEST EDITORIAL C1 Esterase Inhibitor Transfusions in Patients with Hereditary Angioedema by M Michael Glovsky , MD (no abstract) Ann Allergy Asthma Immunol VIEW OR PRINT THIS EDITORIAL CLINICAL ALLERGYIMMUNOLOGY ROUNDS Immunodeficiency with Elevated IgM, Lymphoid Hyperplasia, Autoimmune Anemia, and Thrombocytopenia by Bettina C Hilman , MD; Rubina Wahid , MD; Ronald Negrich , MD; Prem Menon , MD; Fleurette Abreo , MD; and Diana Veillon , MD (no abstract) Ann Allergy Asthma Immunol VIEW OR PRINT THIS ARTICLE ADVICE FROM YOUR ALLERGIST Insect Stings (no abstract) Ann Allergy Asthma Immunol VIEW OR PRINT THIS ARTICLE ORIGINAL ARTICLES C1-Esterase Inhibitor Transfusions in Patients with Hereditary Angioedema by D E Visentin W H Yang , and J Karsh Background: Hereditary angioedema results from the deficiency of C1-esterase inhibitor (C1-INH), and C1-INH replacement would represent definitive treatment for angioedema attacks. In Canada, C1-INH is available only on a compassionate basis at select medical facilities. Our objective is to assess the efficacy of C1-INH transfusions during angioedema attacks at a single Canadian institution. Methods: A restrospective chart review of transfusion data between January 1, 1995 and June 30, 1996 was performed. Phone interviews with patients elicited their opinions of the treatment. Data collected included the number and duration of angioedema attacks, dose of transfused C1-INH, and side effects of treatment.

34. Hereditary Angioedema In A Chinese Family About the HKMJ Subscriptions Reprints Search past issues hereditary angioedema in a Chinese family. D Choy, A Ho, JKW Chan http://www.hkmj.org.hk/hkmj/abstracts/v3n4/436.htm

About the HKMJ Subscriptions Reprints Search past issues Hereditary angioedema in a Chinese family D Choy, A Ho, JKW Chan, CKW Lai, E Li, R Leung Department of Medicine, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, Hong Kong Hereditary angioedema is characterised by recurrent episodes of peri-orbital and peri-oral swelling which can cause an upper airway obstruction, abdominal pain, vomiting, diarrhoea, and even hypotensive collapse. This potentially fatal condition is frequently misdiagnosed; its early recognition and appropriate treatment are thus important. We report a familial cluster of hereditary angioedema in a Chinese family and describe the clinical course of two patients. HKMJ 1997;3(4):436-8 Key words: Angioneurotic edema; Complement 1 inactivators; Immunologic deficiency syndromes Full-text article Previous abstract Next abstract The issue's contents ... Volume index

35. Hereditary Angioedema Association Translate this page hereditary angioedema Association Preguntas Mas Frecuentes Sobre AngioedemaHereditario. ¿Que es el Angioedema Hereditario? El Angioedema http://www.angioedemahereditario.com/Preguntas Mas Frecuentes.html

Hereditary Angioedema Association Preguntas Mas Frecuentes Sobre Angioedema Hereditario ¿Que es el Angioedema Hereditario? El Angioedema Hereditario es una rara y seria afección genética que se manifiesta en aproximadamente en 1/10.000 a 1/50.000 individuos. Esta enfermedad se caracteriza por episodios de edema (hinchazón) en diferentes partes del cuerpo, particularmente en las manos, pies, cara y vías respiratorias. Además, la mayoría de los pacientes experimenta episodios de intenso dolor abdominal, nauseas, y vómitos que son causados por edema de la pared intestinal. Los pacientes de Angioedema tienen una deficiencia en el gen que controla una proteína sanguínea llamada C1 Inhibidor. Este defecto genético trae como consecuencia la producción insuficiente o el mal funcionamiento de esa proteína. El C1 Inhibidor ayuda a regular las complejas interacciones bioquímicas de nuestro sistema inmunológico o sea aquellas que están involucradas en la defensa contra enfermedades, el control de las inflamaciones y la coagulación. Hay mas de 20 proteínas que interactúan dentro del sistema del complemento, el C1 Inhibidor, es precisamente la proteína que ayuda a mantener la estabilidad de este sistema. La producción insuficiente o el mal funcionamiento del C1 Inhibidor impide que éste realice adecuadamente su función reguladora, provocando un desequilibrio bioquímico que trae como consecuencia la producción innecesaria de peptidos. Estos péptidos inducen a los capilares a liberar líquidos en los tejidos de alrededor causando de esta manera, el edema.

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

HOME Hereditary Angioedema ANGIOEDEMA Angioneurotic edema hereditary due to C1 esterase inhibitor deficiency Hereditary Angioedema Hereditary Angioedema ... HOME

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

Hereditary angioedema Hereditary angioedema See C1 inhibitor deficiency (congenital).

38. LeftField:The Paranormal And The Unexplained:sixth Sense:hereditary Angioedema i went to my doctor and they thought i was having a allergic reaction but when iwent to the allergist they diagnosed me with hereditary angioedema, im afraid http://bboard.scifi.com/bboard/browse.cgi/7/6/1566/11

Attention SCIFI.COM members. We are currently working on a new BBoard system. This change will be more than the upgrade of the past. We are building a whole new system from the ground up. If you have any suggestions or features you would like to see in the new BBoard, please e-mail them to, bboard@www.scifi.com hereditary angioedema Date: 12/14/2002 From: hae Reply Title Created by Beginning Text There are currently no replies to this article. Children seeing ghosts Post Reply for: hereditary angioedema

39. Hereditary Angioedema hereditary angioedema. Definition Also, see angioedema. Causes, incidence,and risk factors hereditary angioedema is an inherited disorder. http://www.pennhealth.com/ency/article/001456.htm

Disease Injury Nutrition Poison ... 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. 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.

40. Hereditary Angioedema : Meddie Health Search ITEMS LINKS Adam.com An Overview A definition of hereditary angioedemaalong with a look at the causes, incidence and risk factors. http://www.meddie.com/search/Health/Conditions_and_Diseases/Genetic_Disorders/He

HOME ADD A LINK MODIFY A LINK NEW LINKS ... TOP RATED Search Meddie: the entire directory only this category More search options Home Health Conditions and Diseases ... Genetic Disorders : Hereditary Angioedema ITEMS: LINKS: Adam.com: An Overview A definition of hereditary angioedema along with a look at the causes, incidence and risk factors. (Rating: 0.00 Votes: 0) Rate It E-Medicine: HAE An in dept look at hereditary angioedema beginning with an introduction, clinical features, differentials, work up, treatment, medication and follow up. (Rating: 0.00 Votes: 0) Rate It Hereditary Angioedema Support Group A non profit Organization dedicated to those families that are touched by a rare blood disease known as Hereditary Angioedema (Rating: 0.00 Votes: 0) Rate It MedicineNet.com - Medical Dictionary A brief article about hereditary angioedema. (Rating: 0.00 Votes: 0) Rate It Reader Digest Health Hereditary Angioedema, a general discussion, alternate names and resources. (Rating: 0.00 Votes: 0) Rate It HOME ADD A LINK MODIFY A LINK ... Design © ISC Enterprises Inc.

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