81. IDR Factfile For Hereditary Angioedema hereditary angioedema is episodic and asymptomatic in most patients. Study of Heparin Prophylaxis of hereditary angioedema Exacerbations, http://dna.uta.fi/xml/idr/FF97.xml

General information Disease Hereditary angioedema Alternative names: HANE; HAE; Angioneurotic edema, hereditary; C1 esterase inhibitor deficiency; C1 inhibitor deficiency; Hereditary angioedema type I and type II; Quinke's disease Description Hereditary angioedema is episodic and asymptomatic in most patients. There are 2 types of the disorder. In type I, representing 85% of patients, serum levels of C1NH are less than 35%. In type II, the levels are normal or elevated, but the protein is nonfunctional. The two types are clinically similar. Classification Defects of Complement Regulatory Proteins Inheritance Autosomal dominant OMIM Angioedema, hereditary; HAE Angioedema, hereditary; HAE Cross references Phenotype related immunodeficiencies Incidence Clinical information Description Patients can develop rapid swelling of the hands, feet, limbs, face, intestinal tract, or airway (larynx or trachea). HANE is 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 may frequently mimic an acute abdomen. Diagnosis Diagnostic recommendations: ESID/PAGID recommendations by IDR Additional Information: C1 inhibitor deficiency: consensus document Angioedema, Atlas of Dermatology, Health Portal

82. Hives And Angioedema A more serious condition — hereditary angioedema (HAE) — is an uncommon, hereditary angioedema is an inherited form of angioedema and is related to low http://www.cnn.com/HEALTH/library/DS/00313.html

International Edition MEMBER SERVICES The Web CNN.com Home Page World U.S. Weather ... Autos SERVICES Video E-mail Newsletters Your E-mail Alerts RSS ... Contact Us SEARCH Web CNN.com In association with: WOMEN'S HEALTH Hair/Nails/Skin Corns and calluses Nail fungus Ingrown toenails Moles ... Vitiligo INFORMATION CENTERS: Pick a category Health Centers Family Health Men's Health Women's Health Children's Health Seniors' Health Working Life Pain Management Condition Centers Immune System Allergy Alzheimer's Arthritis Respiratory System Cancer Endocrine System Digestive System Heart and Blood Infectious Disease Mental Health Note: All links within content go to MayoClinic.com Diseases and Conditions Hives and angioedema From MayoClinic.com Special to CNN.com Overview As many as one in five people experiences acute hives or angioedema at one time or another. HAE affects only about 6,000 people in the United States. In most cases, hives and angioedema are harmless and leave no lasting marks. The common treatment is medications. Serious angioedema can be life-threatening if swelling causes your throat or tongue to block your airway and leads to loss of consciousness. Allergy Signs and symptoms Hives are raised, red bumps of various sizes that appear and disappear on your skin. They're often itchy and may look similar to mosquito bites. Wheals tend to occur in batches.

83. BioMed Central | Full Text | Hereditary Angioedema: The Rewards Of Studying A Ra To obtain access to Current Allergy and Asthma Reports through your institution use the options below. If you would like information about a personal http://www.biomedcentral.com/1529-7322/4/251

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84. BioMed Central | Abstract | Hereditary Angioedema: The Rewards Of Studying A Rar hereditary angioedema The Rewards of Studying a Rare Disease There is currently widespread recognition of hereditary angioedema as an episodic disease, http://www.biomedcentral.com/1529-7322/4/251/abstract

home journals A-Z subject areas advanced search ... Issue 4 Viewing options Abstract Full text PDF Related literature: Other articles by authors Frank MM Tools: E-mail to a friend Download references Invited Commentary Hereditary Angioedema: The Rewards of Studying a Rare Disease Michael M Frank MD Children's Health Center, Box 3352 Room T901, Duke University Medical Center, Durham, NC, 27710, USA Current Allergy and Asthma Reports Published First paragraph (this article has no abstract) Terms and Conditions Privacy statement Information for advertisers Contact us

85. Successful Use Of Oxandrolone In The Prophylaxis Of Hereditary Angioedema: A Cas Originally described by Osler in 1888, hereditary angioedema (HAE) is a rare autosomal hereditary angioedema the clinical syndrome and its management. http://www.gfmer.ch/International_activities_En/Oxandrolone.htm

Amin J. Barakat - Pediatrics/Pediatric nephrology Successful Use of Oxandrolone in the Prophylaxis of Hereditary Angioedema: A Case Report Amin J. Barakat, M.D, Anthony J. Castaldo, M.P.A. Pediatric Asthma, Allergy and Immunology, 1999, 13 (4) 189-193. ABSTRACT Hereditary angioedema (HAE) is a rare autosomal dominant disorder typified by a deficiency or dysfunction of C1-esterase inhibitor (C1-INH), and characterized clinically by swelling of the extremities, severe episodic abdominal pain, and sometimes upper airway obstruction. This paper reports for the first time the successful use of oxandrolone in the prophylaxis of HAE in a 14-year-old girl. Oxandrolone has comparatively milder side effects and less potential for hepatotoxicity and virilization than other attenuated androgens used in the prophylaxis of this disease. We believe oxandrolone should be considered as an alternative androgen therapy for children and adults with HAE, particularly females experiencing untoward side effects from danazol or stanozolol, and patients who are not adequately controlled on maximum doses of androgens currently prescribed for HAE. INTRODUCTION Hereditary angioedema (HAE) is a rare autosomal dominant disorder typified by a deficiency or dysfunction of C1-esterase inhibitor (C1-INH), an alpha-2-globulin synthesized in the liver. The disease is characterized by episodic swelling of the extremities, face, bowel wall and upper airway. Long-term prophylaxis for symptoms of the disease has been successfully achieved by the use of the 17 alpha alkylated androgens, danazol and stanozolol. There is currently no approved treatment for children with HAE. Additionally, many women experience untoward side effects from the androgens currently being used for HAE prophylaxis. The purpose of this paper is to report the successful use of oxandrolone (17B-hydroxy-17a-methyl-2-oxa-5a-androstan-3-one) for HAE prophylaxis in a 14-year-old girl with severe HAE symptoms, and to review treatment options in this disease.

86. Baxter News - Baxter And Pharming Start Clinical Study With Recombinant Human C1 hereditary angioedema is characterized by acute attacks of localized swelling of soft tissues, which can become extremely painful or even lifethreatening, http://www.baxter.com/about_baxter/news_room/news_releases/2001/03-09pharming.ht

Conditions Therapies Products Services ... Baxter Worldwide > United States Training and Education Sustainability Contact Us < Back to News Releases ... 2001 Archives News Release Home About Baxter News Room News Releases 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."

87. Hereditary Angioedema And Pregnancy -- Stiller Et Al. 64 (1): 133 -- Obstetrics hereditary angioedema is a relatively uncommon disease that may affect women during A case report of a pregnancy complicated by hereditary angioedema, http://www.greenjournal.org/cgi/content/abstract/64/1/133

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: This Article Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager PubMed PubMed Citation Articles by Stiller, R. Articles by Andreoli JW, , Jr Articles Hereditary angioedema and pregnancy RJ Stiller, BM Kaplan, and Andreoli JW Jr Hereditary angioedema is a relatively uncommon disease that may affect women during their reproductive years. A case report of a pregnancy complicated by hereditary angioedema, followed by a review of the literature, is presented. Although the disease usually follows a benign course, maternal mortality has been noted. Features unique to pregnancy are discussed. Knowledge of the pathophysiology and possible complications is essential for the proper management of the pregnancy. HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS

88. Hereditary Angioedema And Immunoglobulin A Deficiency In Pregnancy -- Peters Et hereditary angioedema is a rare immunologic disorder, and immunoglobulin A deficiency is the most common isolated immunodeficiency. http://www.greenjournal.org/cgi/content/abstract/72/3/454

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: This Article Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager PubMed PubMed Citation Articles by Peters, M Articles by Lockwood, C Articles Hereditary angioedema and immunoglobulin A deficiency in pregnancy M Peters, D Ryley, and C Lockwood Hereditary angioedema is a rare immunologic disorder, and immunoglobulin A deficiency is the most common isolated immunodeficiency. These rarely coexisting conditions may affect pregnancy. We report a gravida with the combination who developed severe acute laryngeal edema. HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS

89. From The Grand Rounds Archive At Baylor hereditary angioedema is rare, but knowledge of its mechanism allows diagnosis and hereditary angioedema is inherited in an autosomal dominant fashion, http://www.bcm.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.

90. C1 Inhibitor Infusion Modifies Platelet Activity In Hereditary Angioedema Patien Patients with hereditary angioedema (HAE) lack a sufficient plasma level of C1 Replacement therapy in hereditary angioedema successful treatment of http://arpa.allenpress.com/arpaonline/?request=get-document&doi=10.1043/0003-998

91. Hereditary Angioedema - SWMC Serving Vancouver Washington Portland Oregon hereditary angioedema Online Medical Encyclopedia courtesy of Southwest Washington Medical Center, a Top 100 Hosptital award winner locacted in Vancouver, http://www.swmedicalcenter.com/118130.cfm

@import url(default.css); Health Information Manager Back to Health Library Print This Page Email to a Friend 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

92. Hereditary Angioedema hereditary angioedema is an inherited abnormality of the immune system that causes swelling, particularly of the face, and abdominal cramping. http://www.pennhealth.com/ency/article/001456.htm

Appointments Medical Services Health Information Find a Doctor Search: Search Encyclopedia: List of Topics Print This Page  General Dermatology 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, 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

93. Disease - Hereditary Angioedema - Detroit, Michigan Disease hereditary angioedema - courtesy of Henry Ford Health System of Detroit, Michigan. http://www.henryfordhealth.org/12506.cfm

3D Tour of the Vattikuti Institute About the Institute The Vattikuti Institute Prostatectomy Prostate Cancer ... Health Encyclopedia Health Encyclopedia 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.

94. Hereditary Angioedema hereditary angioedemahereditary angioedema is an inherited abnormality of the immune system that causes , particularly of the face, and abdominal cramping. Also, see . http://healthcentral.com/ency/408/001456.html

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95. Genzyme Research: Hereditary Angioedema Genetic Disease profile of Hereditary Angiodema including etiology, pathogenesis, clinical description, symptoms, genetics, management, evaluation and http://www.genzyme.com.au/practitioners/hereditary_angioedema.htm

- Introduction Etiology Pathogenesis Clinical Description Symptoms ... Management, Hereditary Angioedema Genetic Disease Profile Introduction In the classic complement pathway of the humoral immune response Etiology Caused by functional deficiency of C1-esterase inhibitor. Loss of regulatory control results in over expression of the complement-mediated inflammatory response. Top of Page Pathogenesis Characterised by recurrent and mucosal swelling, including potentially life-threatening angioedema in the narrow passages of the larynx. Top of Page Clinical Description May present during early childhood. Episodic localized tissue swelling resulting in large, firm welts. These welts may occur on any part of the body, including the face and limbs and internal organs. Top of Page Symptoms Localized swelling (edema) may appear without warning on any part of the body. The symptoms will differ depending on where the edema occurs. Most dangerous is swelling of the airways, which may interfere with breathing.

96. WebMD - HEREDITARY ANGIOEDEMA Message Boards Clinical Trials J. Joseph Giffels hereditary angioedema. Joseph Giffels. New hereditary angioedema (by JAR1956 on Jun27-05) http://boards.webmd.com/webx?THDX@845.hmy5a69Cpp6.0@.59abb805!thdchild=.59abb805

97. Hereditary Angioedema In Europe Main Frame HAE hereditary angioedema - INTERTIONAL NET. For each nation indicated in the index, we listed all the information available to us and useful to patients 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.

98. Resource Library Find Information On Hereditary Angioedema At Find information on hereditary angioedema at MerckSource. Learn more about hereditary angioedema, hereditary angioedema is an inherited abnormality of the http://www.mercksource.com/pp/us/cns/cns_hl_adam.jspzQzpgzEzzSzppdocszSzuszSzcns

99. New Prevention, Treatment For Hereditary Angioedema Attacks, September 1996 New Prevention, Treatment for hereditary angioedema Attacks. Infusions of an immune system protein known as C1 inhibitor can safely prevent and reduce acute http://www.niaid.nih.gov/publications/dateline/0996/page8.htm

New Prevention, Treatment for Hereditary Angioedema Attacks Infusions of an immune system protein known as C1 inhibitor can safely prevent and reduce acute swelling caused by hereditary angioedema (HAE), according to an NIAID study. HAE is caused by a defect in the gene that encodes the C1 inhibitor protein, an important regulator of a group of immune system enzymes known collectively as complement. Affected individuals produce either insufficient or dysfunctional C1 inhibitor. The resulting disregulation of complement in these individuals leads to episodes of swelling, typically in the extremities, face, larynx, and abdomen. Patients with severe HAE have at least one attack per month, with symptoms lasting from one to four days. Attacks affecting the abdomen often cause nausea, vomiting, and severe pain, while those involving the larynx and other upper airway structures can lead to asphyxiation, a potentially fatal complication. "We wanted to show that C1 inhibitor prepared this way would be safe and efficacious, particularly in patients for whom other therapies were not effective," says Dr. Frank, who now chairs the Department of Pediatrics at Duke University Medical Center. The researchers tested the heat-sterilized C1 inhibitor in placebo-controlled prevention and treatment studies. For the prevention study, they gave multiple infusions of either C1 inhibitor or placebo over a 17-day period to HAE patients whose symptoms could not be controlled with standard therapy. Three weeks later, the patients received the alternate treatment over a second 17-day period. Swelling attacks were significantly less frequent and less severe during treatment with C1 inhibitor than with placebo. In addition, no patients experienced swelling of the larynx while receiving C1 inhibitor. Four of the six patients had swelling in this area while receiving placebo.

100. Revista Da Associação Médica Brasileira - Translate this page PURPOSE hereditary angioedema was first described by William Osler in 1888 and it is hereditary angioedema as a cause of transient abdominal pain. http://www.scielo.br/scielo.php?pid=S0104-42302004000300041&script=sci_arttext&t

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