61. Clinical Trial: C1-Esteraseremmer-N For The Treatment Of Hereditary (and Acquire Inclusion criteria for hereditary angioedema patients. Established diagnosis of hereditary angioedema type I or II markedly decreased C1 inhibitor http://www.clinicaltrials.gov/ct/show/NCT00125541

Home Search Browse Resources ... About C1-Esteraseremmer-N for the Treatment of Hereditary (and Acquired) Angioedema This study is not yet open for patient recruitment. Verified by Sanquin July 2005 Sponsored by: Sanquin Information provided by: Sanquin ClinicalTrials.gov Identifier: Purpose In part C of the study, a number of HAE patients will be treated prophylactically with open-label C1-esteraseremmer-N for a period of 16 weeks. The number of attacks occurring will be compared with historical data. If possible, also some patients treated prophylactically with C1 inhibitor for acquired angioedema will be included. Condition Intervention Phase Angioedema Drug: C1 inhibitor concentrate (C1-esteraseremmer-N) Phase II Phase III MedlinePlus related topics: Hives Vascular Diseases Study Type: Interventional Study Design: Prevention, Non-Randomized, Open Label, Historical Control, Single Group Assignment, Safety/Efficacy Study Official Title: Pharmacokinetics, Clinical Efficacy and Safety of C1 Inhibitor Concentrate (C1-Esteraseremmer-N) for the Treatment of Hereditary (and Acquired) Angioedema Further Study Details: Primary Outcomes: The primary objective in this clinical study is to evaluate the prevention of angioedema attacks in patients prophylactically treated with C1-esteraseremmer-N.

62. Clinical Trial: Subcutaneous Treatment With Icatibant For Acute Attacks Of Hered cutaneous and/or abdominal attacks in patients with hereditary angioedema (HAE). Formulation of Icatibant for the Treatment of hereditary angioedema http://www.clinicaltrials.gov/ct/gui/show/NCT00097695

Home Search Browse Resources ... About Subcutaneous Treatment with Icatibant for Acute Attacks of Hereditary Angioedema This study is currently recruiting patients. Verified by Jerini AG September 2005 Sponsored by: Jerini AG Information provided by: Jerini AG ClinicalTrials.gov Identifier: Purpose The purpose of this study is to assess the efficacy and safety of Icatibant, a bradykinin antagonist in the treatment of acute cutaneous and/or abdominal attacks in patients with hereditary angioedema (HAE). Condition Intervention Phase Angioedema Drug: Icatibant Phase II Phase III MedlinePlus related topics: Hives Vascular Diseases Study Type: Interventional Study Design: Treatment, Randomized, Double-Blind, Placebo Control, Parallel Assignment, Safety/Efficacy Study Official Title: Randomized, Double Blind, Placebo-Controlled, Multicenter Study of a Subcutaneous Formulation of Icatibant for the Treatment of Hereditary Angioedema Further Study Details: Primary Outcomes: Symptom relief (patient) Secondary Outcomes: Safety and tolerability; Additional efficacy assessments; Pharmacoeconomics Expected Total Enrollment: 56 Study start: August 2004 Eligibility Ages Eligible for Study: 18 Years and above, Genders Eligible for Study: Both

63. Current Clinical Trial Studies Diagnosed with hereditary angioedema. Exclusion Criteria, Pregnant, breastfeeding, abnormal liver or kidney function. Length of Study, 28 days http://www.hmc.psu.edu/clinicaltrials/studies/weblistings/2003-255.htm

64. Recombinant Human C1 Inhibitor Trial Shows Positive Results In Treating Heredita hereditary angioedema is a genetic disorder caused by a deficiency of the Pharming s lead product (rhC1INH) for hereditary angioedema is in Phase III of http://www.rxpgnews.com/research/pharmacology/article_1331.shtml

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65. DX-88 : A Recombinant Small Protein Shows Significant Benefit In Hereditary Angi The prevalence of hereditary angioedema is believed to be between 1/10000 and 1/50000 people worldwide. The condition is caused by a genetic deficiency of http://www.rxpgnews.com/research/pharmacology/article_800.shtml

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66. AADMC: Selected Articles 2000: Laryngeal Obstruction In Hereditary Angioedema Laryngeal obstruction in hereditary angioedema. Summary Airway obstruction, a major cause of mortality in hereditary angioedema (HAE) is frequently not http://www.aaaai.org/aadmc/currentliterature/selectedarticles/2000archive/laryng

SELECTED ARTICLES FROM THE RECENT LITERATURE 2000 Laryngeal obstruction in hereditary angioedema Summary 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 Mayo Clinic Proc 2000'75:349-54 Editor's Comments 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

67. Icatibant - A New Approach For Hereditary Angioedema Icatibant a new approach for hereditary angioedema. Summary potentially fatal episodes of hereditary angioedema (HAE) has not been very satisfactory. http://www.aaaai.org/aadmc/currentliterature/selectedarticles/2005archive/icatib

SELECTED ARTICLES FROM THE RECENT LITERATURE 2005 Icatibant - a new approach for hereditary angioedema Summary Background - The treatment of acute, potentially fatal episodes of hereditary angioedema (HAE) has not been very satisfactory. It is generally agreed that bradykinin (Bk) is the major mediator of angioedema in such episodes. Findings - Icatibant (Icat) is a potent, selective peptidomimetric antagonist of the bradykinin beta 2-receptor. As reviewed in this report, Icat appears to be the first such an antagonist that does not also stimulate this Bk receptor. Preliminary studies of Icat use by either IV or subcutaneous formulations showed very encouraging results with no evidence of adverse effects. Therefore, Jenini AG, a German pharmaceutical company has initiated controlled Phase IIb studies of Icat treatment for acute HAE episodes. Reference Drugs RD 2004;5:343-8 Editor's Comments I have briefly reviewed the preliminary findings described above because of the potential value of this treatment. The frequency and/or severity of acute HAE episodes is often (nut not always) reduced by maintenance therapy with altered androgens (danazol, stanazolol). However, there is still a potential risk for a possibly fatal acute HAE reaction. Treatment with plasmin inhibitors (e.g. - EACA) has yielded inconsistent benefit and/or adverse side-effects. A plasma fraction containing the C1 esterase inhibitor is available in several European countries (but not the USA ) for treatment of acute HAE episodes. Icat looks sufficiently promising so that the FDA has awarded a "fast track" review of the results of Icat trials in HAE as well as an "orphan drug" status.

68. Sally's World: Hereditary Angioedema hereditary angioedema is a rare inherited form that differs from other types of Check out the United States hereditary angioedema Association and the http://www.sallyalexander.com/2005/06/hereditary_angi.html

hostName = '.sallyalexander.com'; Sally's World Photo Albums Best of 2003 Best of 2004 Glacier National Park July 2005 New Mexico Memorial Day 2005 Trip ... Yellowstone Trip Roomba Roomba Review - Robotic Reviews, tips and news. iRobot - Robots for the Real World : Home Page RoombaHelp.Com News Links Michael Totten www.AndrewSullivan.com - Daily Dish DRUDGE REPORT 2003® South African News Sunday Times Iraq links Iraq The Model THE MESOPOTAMIAN Healing Iraq Food Blogs The Amateur Gourmet Cook Sister! Archives September 2005 August 2005 ... December 2004 Powered by TypePad Member since 12/2003 Main Hereditary Angioedema Hereditary Angioedema is " a rare inherited form that differs from other types of angiodema. It is not triggered by an allergic reaction and involves deep tissue swelling and is never associated with hives. Swelling in the gastrointestinal tract can led to severe pain in the abdomen and can be mistaken for appendicitis. Hereditary angiodema can be detected through blood tests and can be effectively treated with special hormone medications." Reference here.

69. 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 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).

70. PiA - Primary Immunodeficiency Association - Common Variable Immunodeficiency CV What Is hereditary angioedema? (taken from www.hereditaryangioedema.com). hereditary angioedema (HAE) is a rare and serious genetic condition occurring in http://www.pia.org.uk/hae/

Home HAE Publications Services ... Helpline Welcome to the Hereditary Angioedema (HAE) Pages What Is Hereditary Angioedema? (taken from www.hereditaryangioedema.com 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.

71. OB-GYN-L Messages For June, 1996: Hereditary Angioedema hereditary angioedema. From STEWART PRINGLE (100724.2333@CompuServe.COM) Fri Jun 14 163134 1996. Messages sorted by date thread subject http://forums.obgyn.net/ob-gyn-l/OBGYNL.9606/0285.html

-VISIT OUR OTHER FORUMS- OB-GYN-L Ultrasound Ultrasound History Physicians-in-Training Tech Talk OBSTET-L (portuguese) OBGIN-L (spanish) Nursing hereditary angioedema From: STEWART PRINGLE 100724.2333@CompuServe.COM Fri Jun 14 16:31:34 1996 Messages sorted by: [ date ] [ thread ] [ subject ] [ author ] Next message: OBDocRick@aol.com: "Re: epidurals for version" Previous message: Michael S. Wolkomir: "Epidural for version" Next in thread: Malcolm Griffiths: "Re: hereditary angioedema" Reply: Malcolm Griffiths: "Re: hereditary angioedema" Maybe reply: Paula R. Dhanda: "Re: hereditary angioedema" I have a 21 yr old with angioedema ( C1 esterase inhibitor deficiency). She's never had any airway problems but has attacks at other sites quite often. She has a Bartholin's cyst which I propose to marsupialise. Firstly does anyone know about the anaesthetic implcations? Next she has some dysmen not helped by an NSAID and says the combined OC pill makes her angioedema worse. She needs contraception too having already had 1 unwanted pregnancy and I suggested DepoProvera so for bonus marks is that OK with her condition? Stewart Pringle Glasgow, UK

72. OB-GYN-L Messages For June, 1996: Re: Hereditary Angioedema Online Free service ; In reply to Malcolm Griffiths Re hereditary angioedema ; Next in thread Paula R. Dhanda Re hereditary angioedema http://forums.obgyn.net/ob-gyn-l/OBGYNL.9606/0298.html

-VISIT OUR OTHER FORUMS- OB-GYN-L Ultrasound Ultrasound History Physicians-in-Training Tech Talk OBSTET-L (portuguese) OBGIN-L (spanish) Nursing Re: hereditary angioedema From: Malcolm Griffiths Malcolm@mgriff22.demon.co.uk Sat Jun 15 17:53:14 1996 Messages sorted by: [ date ] [ thread ] [ subject ] [ author ] Next message: Peter V. Weston, M.D.: "Re: Physicians' Online: Free service" Previous message: rossmann@MEM.po.com: "Physicians' Online: Free service" In reply to: Malcolm Griffiths: "Re: hereditary angioedema" Next in thread: Paula R. Dhanda: "Re: hereditary angioedema" Malcolm@mgriff22.demon.co.uk 100724.2333@CompuServe.COM Sorry - doesn't necessarily answer her contraception problems, but ... Malcolm Malcolm@mgriff22.demon.co.uk "It is dangerous to be right on a subject on which the established authorities are wrong." (Voltaire) Next message: Peter V. Weston, M.D.: "Re: Physicians' Online: Free service" Previous message: rossmann@MEM.po.com: "Physicians' Online: Free service" In reply to: Malcolm Griffiths: "Re: hereditary angioedema" Next in thread: Paula R. Dhanda: "Re: hereditary angioedema"

73. Dorlands Medical Dictionary hereditary angioedema, inherited C1 inhibitor (C1 INH) deficiency, an autosomal dominant disorder manifested as recurrent episodes of edema of the skin, http://www.merckmedicus.com/pp/us/hcp/thcp_dorlands_content.jsp?pg=/ppdocs/us/co

74. ALLSA - Urticaria And Angioedema Cholinergic Urticaria; Cold Urticaria; hereditary angioedema (HAO) hereditary angioedema (HAO) Index. HAO is caused by a deficiency of the C1 inhibitor http://www.allergysa.org/urticariahdbook.htm

Handbook Urticaria and Angioedema Written by Dr Cassim Motala A. Incidence B. Aetiology C. Clinical Features D. Diagnostic Tests E. Treatment F. Forms of urticaria and angioedema: (requiring special mention) Cholinergic Urticaria Cold Urticaria Hereditary Angioedema (HAO) Papular Angioedema (HAO) Urticaria may be acute ( 6 weeks duration). Angioedema is frequently associated with urticaria but the two may occur independently. A. Incidence Index Acute urticaria is said to affect 10%-20% of the population at some time during life. It is not uncommon in childhood, but the greatest incidence appears to be in young adults (15%). Chronic urticaria occurs more frequently in mid-life, especially in women. B. Aetiology Index A clinical classification is given in Table 1. In chronic urticaria a causative agent or precipitating cause may be established in only about 10% to 30% of cases. Therefore, most chronic urticaria is idiopathic. In acute urticaria the chances of determining the cause are higher. For example as many cases of acute urticaria in children may be associated with a viral, bacterial or parasitic infection (not always clinically apparent). Acute urticaria and angiooedema may also be caused by allergen exposure including insect stings or food or drug ingestion. Some of the more common drugs include penicillin, cephalosporins and sulphonamides. Opiates may act directly on mast cells to cause histamine release. Aspirin and other non-steroidal anti-inflammatory agents can cause urticaria or angioedema in the same way. Angiotensin converting enzyme inhibitors are a common cause of drug induced angioedema.

75. Health Encyclopedia hereditary angioedema is an inherited abnormality of the immune system that causes swelling , particularly of the face, and abdominal cramping. http://healthcontent.baptisteast.com/adamcontent/ency/article/001456.asp

Member Sign In Visitors/Patients Physicians/Clinicians Employees Search: About Baptist Services Education Health Information ... Contact Us Printer Friendly Send to a Friend Search Health Encyclopedia Click here Disease Injury ... Test Hereditary angioedema Antibodies 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 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.

76. Hereditary Angioedema In A Chinese Family hereditary angioedema is characterised by recurrent episodes of We report a familial cluster of hereditary angioedema in a Chinese family and describe 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

77. Hereditary Angioedema With Recurrent Abdominal Pain. Seth Avnish K, Nair V, Sing Indian J Gastroenterol, The official publication of the Indian Society of Gastroenterology (ISG) http://www.indianjgastro.com/article.asp?issn=0254-8860;year=2002;volume=21;issu

78. Indian Pediatrics - Case Reports Case Reports. Indian Pediatrics 2000;37 431432. hereditary angioedema ‘Type I’ Report of a Family with a Fatal Case http://www.indianpediatrics.net/april2000/april-431-432.htm

Home Past Issue About IP About IAP ... Subscription Case Reports Indian Pediatrics 2000;37: 431-432 Hereditary Angioedema ‘Type I’: Report of a Family with a Fatal Case Gurvinder P . Thami Amrinder J. Kanwar From the Department of Dermatology and Venereo-logy, Government Medical College Hospital, Sector 32, Chandigarh 160 047, India. Reprint requests: Dr. A.J. Kanwar, Professor and Head, Department of Dermatology and Venereology, Government Medical College Hospital, Sector 32, Chandigarh 160 047, India. Manuscript Received: August 10, 1999; Initial review completed: September 28, 1999; Revision Accepted: October 25, 1999 Angioedema, characterized by non-pitting, erythematous swelling of soft tissues, can be hereditary or acquired. Hereditary angioedema (HAE) is an autosomal dominant disease due to mutations at C1 inhibitor gene. The defective gene does not produce sufficient levels of C1 inhibitor in plasma which leads to auto-activation of C1 and consumption of C2 and C4. It is further classified into Type I (lower production of C1 inhibitor proteins) and Type II (functional defect of C1 inhibitor with normal plasma levels)(1,2). Acquired angioedema may be a manifestation of urticaria; it has recently been described with drugs like angiotensin converting enzyme (ACE) inhibitors.

79. DI&DMH Research : Hereditary Angioedema Genotypephenotype correlation in patients with hereditary angioedema hereditary angioedema (HAE) is a autosomal dominant disorder that results from an http://www.molecular-haemostasis.de/research/hereditary.html

Institute of Transfusion Medicine and Immunohaematology Department of Immunohaematology and Department of Molecular Haemostasis Head: Johannes Oldenburg (MD, PhD) Immunohaematology Molecular Haemostasis Education / Training ... Links Research : Hereditary Angioedema Genotype-phenotype correlation in patients with Hereditary Angioedema home contact print up

80. BioSpace News: Angioedema (DYAX) Release DX88 Trials In hereditary angioedema To Be Featured At 4th (DYAX) Provides Update On DX-88 Development For hereditary angioedema; http://www.biospace.com/news_rxtarget.cfm?RxTargetID=62620

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