81. THE MERCK MANUAL, Sec. 8, Ch. 94, Eyelid Disorders hereditary angioedema due to C1 esterase inhibitor deficiency (see HereditaryAngioedema in Ch. 148) can also cause acute lid edema. Treatment. http://www.merck.com/pubs/mmanual/section8/chapter94/94a.htm

82. Indian Pediatrics - Case Reports hereditary angioedema ‘Type I’ Report of a Family with a Fatal Case. Treatmentof hereditary angioedema with a vapour treated C1 inhibitor concentrate. 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.

83. Listado De Publicaciones Fabiani JE, Squiquera HL, Leoni J, Simkin G, and Mathov E hereditary angioedemaFibrinogen and C4 Findings in skin biopsies in one family. http://www.centromedico.netfirms.com/publicaciones LS.htm

This site is hosted by Netfirms Web Hosting CONSULTORIOS DE ESPECIALIDADES MEDICAS Servicio Especializado en Inmunología y Dermatología Clínica Un emprendimiento de AAIP Asociación Civil . Registro Nº 1636571. Res. Nº 950 INJ Incorporada al registro de entidades de accion comunitaria. disposicion 120-dgdcom-2000. Sitio seleccionado por: Dr. Luis Squiquera PUBLICACIONES NACIONALES E INTERNACIONALES Casala A, and Squiquera, HL .: Celulas de Langerhans Epidérmicas. in Greiding L. et al editors. Update in Pediatric Allergy and Immunology. 1st ed, 1984. Squiquera HL , Cannavo AB, Gonzales-Alonso G, and Casala A: Abrikossoff Tumor (Granular cell Shwannoma): immunohistochemical study of cellular origin. Arch Arg Dermatol 35:123_129, 1985. Greiding L, Reisefeld F, and Squiquera HL : Patch tests with histamine in patients with Atopic Dermatitis. Ann Allergy 52(2):350, 1985. Reisefeld F, Greiding L, Mathov E, and Squiquera HL : Patch tests with Dermatophagoides pteronissinus and House dust mite in Atopic Dermatitis Patients. Ann Allergy 35(2)350, 1985. Fabiani JE

84. Immune Complement Angioedema, hereditary (C1 inhibitor deficiency) Angio (neurotic) edema (allergic)(any site). C1 inhibitor deficiency is associated with hereditary angioedema. http://www.jcaai.org/Param/Immune/Comp.HTM

Diagnosis and Management of Hereditary and Acquired Deficiencies of complement Components Summary Statements CLASSIFICATION: ICD-9 CODE NO. Angioedema, acquired with malignancy Angioedema, hereditary (C1 inhibitor deficiency) Angio (neurotic) edema (allergic) (any site) (with Urticaria) Complement factor deficiency NEC Complement factor deficiency specified: classical pathway components alternative pathway components factor D, properdin C3 and its regulatory proteins C3, factor I, factor H Terminal complement pathway components I. Diagnosis and Evaluation A. Clinical Evaluation of Complement Component Deficiency It should be recognized that: Primary deficiencies of complement components, aside from homozygous null C2 (1:10,000), are quite rare. Deficiencies of all complement components have been described. Deficiencies of the early components of complement (C1q,r,s C4, C2) are associated with systemic lupus erythematosus-like symptoms, glomerulonephritis and, less frequently, with pyogenic infections. C3 deficiency is associated with severe pyogenic infections, glomerulonephritis, and systemic lupus erythematosus. Deficiencies of the terminal components (C5, C6, C7, C8, C9) are associated with recurrent disseminated neisserial infections.

85. Hepe - HTL hereditary angioedema. http//webmd.lycos.com/adam/asset/adam_disease_articles/a_to_z/h.hereditary angioedema. http//www.icondata.com/health/pedbase/pedlynx.htm. http://www.healthmednet.com/Hepe - HTL.htm

Hepe - HTL Hepertension http://drkoop.com/conditions Herbal (Alternative Medicine) Herbal Health Products: What You Should Know http://familydoctor.org/cgi-bin/list.pl?element=healthtopic Herbal Health Products: What You Should Know http://www.familydoctor.org/cgi-bin/list.pl?element=handout Herbal Medicine http://www.health.gov/NHIC/NHICScripts/AlphaKey.cfm?Alpha Herbal Medicine http://www.nlm.nih.gov/medlineplus/all_healthtopics.html Herbal Medicines http://www.healthcentral.com/library/library.cfm Herbals Herbs http://www.health.gov/NHIC/NHICScripts/AlphaKey.cfm?Alpha Herbs http://www.healthcentral.com/library/library.cfm Herbs and Supplements http://www.healthcare.ucla.edu/pls/index.htm Herbs for Health http://home.about.com/health/index.htm?PM=59_0208_T Herbs for Health http://www.about.com/health/index.htm?PM=59_208_T http://thunderstone.go2net.com/texis/websearch/?c=Health hereditary amyloidosis http://webmd.lycos.com/adam/asset/adam_disease_articles/a_to_z/h hereditary angioedema http://webmd.lycos.com/adam/asset/adam_disease_articles/a_to_z/h HEREDITARY ANGIOEDEMA http://www.icondata.com/health/pedbase/pedlynx.htm

86. Aventis Behring AB Society for Immunodeficiencies IPOPI International Patient Organisation for Patientswith Primary Immunodeficiencies hereditary angioedema HAE Danmark The http://www.aventisbehring.se/en/links.asp

Please click on one of the subjects below to view links to other web sites. Governmental bodies Product info Treatment Centres Aventis ... Other sites Links to sites outside of Aventis are provided as a resource to the user. Aventis accepts no responsibility for the content of linked sites. If you think there are other sites that would be useful to show here, please e-mail us: info@aventisbehring.se Läkemedelsverket Medical products agency in Sweden Lægemiddelstyrelsen Danish medicines agency Statens legemiddelverk Norwegian medicines agency Lääkelaitos National agency for medicines in Finland Lyfjastofnun Icelandic Medicines Control Agency EMEA The European Agency for the Evaluation of Medicinal Products Riksförsäkringsverket The Swedish National Social Insurance Board-divison for pharmaceutical questions Information on swedish medical products for the general public and professionals Lægemiddelkataloget online Lægemiddelkataloget and Medicinhåndbogen with information of medical products in Denmark for professionals and general public, respectively Felleskatalogen Information on Norwegian medical products for health care professionals Medisinhåndboken Information on Norwegian medical products for the general public Lääkeopas 2000 Information on Finnish medical products for the general public

87. Ziele The GSAR is mainly dealing with hereditary angioedema due to C1 esterase inhibitordeficiency and other types of hereditary angioedema, but also with http://www.uni-mainz.de/Organisationen/dga/englisch/ziele.htm

German Society for Angioedema Research The German Society for Angioedema Research (GSAR) is an association of medical doctors and scientists who are engaged in angioedema research. The society was established in Mainz in 1996. The GSAR is mainly dealing with hereditary angioedema due to C1 esterase inhibitor deficiency and other types of hereditary angioedema, but also with angioedema that occurs as an adverse effect of drugs; it also focuses on so-called idiopathic angioedema and other immunological and non-immunological types of the disease. The aims of the society are: · Advancement of clinical and scientific research into angioedema. · Communication of information to medical doctors and the public on the various types of angioedema, especially hereditary angioedema, in order to prevent fatalities due to asphyxiation as a result of laryngeal edema. · Setting-up of guidelines for diagnosis, monitoring and treatment of patients and the compilation of data on the epidemiology of angioedema in conjunction with corresponding specialist societies in other countries. · Representation of the interests of afflicted patients and their physicians to health organizations and authorities, insurance companies, as well as to public social institutions.

88. Angioedema the blood. hereditary angioedema is a very rare kind of angioedema, whichruns in families. A website on hereditary angioedema. The Hereditary http://www.users.globalnet.co.uk/~aair/angioedema.htm

89. PROFESSIONAL REFERENCE Allergy Immunlogy Cardiology Dermatology Detailed recommendations in the Report, Ask about family history of autoimmunedisorders, thyroiditis, and hereditary angioedema. more. Physical Examination http://merck.praxis.md/index.asp?page=bpm_brief&chapter=BPM01AL08

90. C1Esterase - Austrian Anesthesia       Keywords C1Esterase Inhibitor, Septic shock, Vascular leak syndrome, Complementactivation, Contact system activation, hereditary angioedema. http://www.anesthesia.at/aventis/C1INH.HTML

anesthesiology These pages are maintained by Univ.Prof. Nikolaus Mayer, AKH Vienna, Austria C1 Esterase Inhibitor Review article Keywords : C1-Esterase Inhibitor, Septic shock, Vascular leak syndrome, Complement activation, Contact system activation, hereditary angioedema. C1-Esterase-Inhibitor (C1-INH) is the major plasma inhibitor of the complement and contact systems. Functional levels of C1-INH tend to be normal in septic patients although paradoxically this inhibitor is an acute phase protein . The similarity between vascular leak syndrome (VLS) and sepsis encompasses more than complement and contact activation since a number of other inflammmatory mediators considered to play a role in the pathogenesis of septic shock , are also involved in the developement of VLS. A further syndrom is characterized by reduced levels of C1-INH, because of a decreased synthetic rate - the Hereditary Angioedema C1-Esterase Inhibitor (C1-INH) C1-Inhibitor (C1-INH) is a serin protease inhibitor. It is a major inhibitor of both factor XIIa and kallikrein of the contact system and also the only known inhibitor of actiivated C1s and C1r from the pathway of complement. C1-INH regulates the activity of two plasma cascade systems that during activation form biologically active peptides, i.e. the anaphylatoxins and bradykinin, which is known for its vasodilatating and vasopermeability enhancing effects. Vascular leak syndrome (VLS) In the late 1980s, a syndrom of multiple organ dysfunction was seen in patients with sepsis, polytrauma, severe burns, pancreatitis and other forms of critical illness. It was characterized by pulmonary, cardiovascular, gastrointestinal, renal hepatic and central nervous system dysfunction and associated with high mortality. In autopsy increased lungs, heart, kidney, liver, accumulation of leucocytes and interstitial edema with no signs of circulatory overload was found. So it was hypotheSIZEd that multiple organ failure was the result of a generalized inflamatory reaction with a specific response consisting of vasodilatation, increased permeability, edema and leucocyte emigration and activation.

91. European Orphan Drug Status Granted To Recombinant Human C1 This European designation is essential to reach commercial success in an importantniche market like hereditary angioedema, said Philippe van Holle http://www.pslgroup.com/dg/1fe506.htm

92. Asociación Argentina De AngioEdema Hereditario http://www.angioedemahereditario.com/

Asociación Argentina de AngioEdema Hereditario (entidad en formación) Deficiencia de C1 inhibidor Hereditary AngioEdema

93. AMSAO - Liens Translate this page Allemagne - HAE Vereinigung eV Belgique - HAE Belgium Canada - Canadian Hereditaryangioedema Society Croatie Danemark - HAE Danmark Espagne - angioedema http://amsao.free.fr/liens.html

Association des Malades Souffrant d'Angio-Oédèmes par déficit en C1 inhibiteur LIENS INTERNET Allemagne - HAE Vereinigung e.V. Belgique - HAE Belgium Canada - Canadian Hereditary Angioedema Society Croatie ... Pologne En Europe, l' association italienne Angioedema table (anglais) ORPHANET - base de données sur les maladies rares CHU Grenoble Online Mendelian Inheritance in Man - Hereditary Angioedema (HAE) (anglais) MEDLINE - Hereditary angioedema (anglais) Université de LYON - Le complement Online Mendelian Inheritance in Man - COMPLEMENT (anglais) International Complement Society (anglais) XIX International Complement Workshop (anglais) CHU Rennes - Adrenaline Syndicat National des Gynecologues et Obstetriciens de France (en anglais) Hépato-Gastro - Crises douloureuses abdominales récidivantes chez une jeune fille 2.6 Autres associations Alliance Maladies Rares

94. Katalog - Wirtualna Polska Serwis Katalog w Wirtualna Polska S.A. pierwszy portal w Polsce. http://katalog.wp.pl/DMOZ/Health/Conditions_and_Diseases/Genetic_Disorders/Hered

Poczta Czat SMS Pomoc Szukaj.wp.pl: -Katalog -Polskie www -¦wiatowe www -Wirtualna Polska -FTP/Pliki -Grupy dyskusyjne -Encyklopedia -Produkty wp.pl Katalog Katalog ¦wiatowy DMOZ ... Conditions and Diseases > Genetic Disorders Fakty o Katalogu Pomoc Regulamin Serwis szukaj ... Ostatnio dodane NAWIGACJA Fakty o katalogu Pomoc Regulamin Serwis Szukaj ... FAQ Dodaj stronê Katalog WP Polskie Strony WWW Oferta dla firm WP-HIT ... Wirtualna Polska

95. Baxter News - PHARMING AND BAXTER TO CO-DEVELOP HUMAN C1 INHIBITOR TO TREAT HERE PHARMING AND BAXTER TO CODEVELOP HUMAN C1 INHIBITOR TO TREAT HEREDITARYANGIOEDEMA GLENDALE, Calif., USA and LEIDEN, THE NETHERLANDS http://www.baxter.com/utilities/news/releases/2000/03-21pharming.html

PHARMING AND BAXTER TO CO-DEVELOP HUMAN C1 INHIBITOR TO TREAT HEREDITARY ANGIOEDEMA GLENDALE, Calif., 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 U.S. 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."

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