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Hereditary angioedema

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https://www.readbyqxmd.com/read/28202404/recombinant-human-c1-esterase-inhibitor-to-treat-acute-hereditary-angioedema-attacks-in-adolescents
#1
James W Baker, Avner Reshef, Dumitru Moldovan, Joseph R Harper, Anurag Relan, Marc A Riedl
BACKGROUND: Recombinant human C1-esterase inhibitor (rhC1-INH) is efficacious and well tolerated for managing hereditary angioedema (HAE) attacks in adults. However, there are insufficient data on its efficacy and safety in adolescents. OBJECTIVE: To evaluate the efficacy and safety profiles of rhC1-INH for acute HAE attacks in adolescents. METHODS: Adolescents (aged 12-18 y) with HAE enrolled in 2 randomized controlled trials and 2 open-label extension trials were included and received intravenous rhC1-INH for acute attacks...
February 12, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28194776/mutational-spectrum-of-the-serping1-gene-in-swiss-patients-with-hereditary-angioedema
#2
Urs C Steiner, Melanie Keller, Pirmin Schmid, Sven Cichon, Walter A Wuillemin
Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is a rare autosomal dominant disease caused by mutations in the C1 inhibitor gene SERPING1. Phenotype and clinical features of the disease are very heterogeneous, varying even in the same family. Compared to HAE cohorts in other countries the genetic background of the Swiss HAE patients has not been elucidated yet. In the present study we investigated the mutational spectrum of the SERPING1 gene in nineteen patients of nine unrelated Swiss families...
February 14, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28178173/screening-for-hereditary-angioedema-hae-at-13-emergency-centers-in-osaka-japan-a-prospective-observational-study
#3
Tomoya Hirose, Futoshi Kimbara, Masahiro Shinozaki, Yasuaki Mizushima, Hidehiko Yamamoto, Masashi Kishi, Takeyuki Kiguchi, Shigeru Shiono, Mitsuhiro Noborio, Akihiro Fuke, Hiroshi Akimoto, Takaaki Kimura, Shinichiro Kaga, Takahiko Horiuchi, Takeshi Shimazu
Hereditary angioedema (HAE) with deficiency of C1 inhibitor (C1-INH) is an autosomal-dominant disease characterized by recurrent episodes of potentially life-threatening angioedema. The objective is to study the incidence of HAE among patients who visit the emergency department.This was a 3-year prospective observational screening study involving 13 urban tertiary emergency centers in Osaka prefecture, Japan. Patients were included if they met the following criteria: unexplained edema of the body, upper airway obstruction accompanied by edema, anaphylaxis, acute abdomen with intestinal edema (including ileus and acute pancreatitis), or asthma attack...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28159372/effect-of-c1-esterase-inhibitor-in-hereditary-angioedema-treatment
#4
Ekim Saglam Gurmen, Serkan Dogan, Eren Sert, Cesareddin Dikmetas, Sennaz Hussein
No abstract text is available yet for this article.
January 24, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28158879/hereditary-angioedema-implications-of-management
#5
Mohini Pathria, Guha Krishnaswamy, Juan C Guarderas
Hereditary angioedema (HAE) is a genetic condition that is characterized by frequent episodes of localized angioedema. It is a rare disorder that a primary care provider, otolaryngologist, dermatologist, or rheumatologist may encounter only occasionally. This disease is being reviewed because of the significant advances in further understanding the genetics, biology, and therapeutic management surrounding the condition. Histamine-mediated angioedema responds to steroids, antihistamines, and epinephrine, whereas bradykinin-mediated angioedema is resistant to those interventions and requires specialized therapy...
February 2017: Southern Medical Journal
https://www.readbyqxmd.com/read/28151501/-angioedema-and-the-role-of-bradykinins-new-treatments-and-implications-in-patients-with-heart-failure
#6
Marta Mansi, Maddalena Alessandra Wu, Andrea Zanichelli, Marco Cicardi
The definition of angioedema is an edema of subcutaneous and submucosal tissues due to increased vascular permeability and fluid extravasation. It can affect different areas, including extremities, genitals, upper airways and intestinal mucosa. The symptoms are disabling and this condition can be fatal if it involves the larynx. We can distinguish different forms of angioedema (hereditary and acquired) with different pathogenetic mechanisms, therefore responding to different treatments. Bradykinin-mediated angioedema (such as hereditary angioedema due to C1-inhibitor deficiency) does not respond to the standard therapy used for histamine-mediated angioedema...
December 2016: Giornale Italiano di Cardiologia
https://www.readbyqxmd.com/read/28115964/hereditary-angioedema-health-related-quality-of-life-in-canadian-patients-as-measured-by-the-sf-36
#7
Nina Lakhani Jindal, Elaine Harniman, Nieves Prior, Elia Perez-Fernandez, Teresa Caballero, Stephen Betschel
BACKGROUND: Hereditary angioedema (HAE) is a rare but serious condition characterized by recurrent spontaneous attacks of angioedema affecting superficial tissues of upper respiratory and gastrointestinal tracts. The potentially fatal and disfiguring nature of HAE impacts the health-related quality of life (HRQoL) of patients with this condition. OBJECTIVES: To assess the health-related quality of life of Canadian patients with HAE using the 36-item Short-Form Health Survey (SF-36v2)...
2017: Allergy, Asthma, and Clinical Immunology
https://www.readbyqxmd.com/read/28093999/health-related-quality-of-life-with-hereditary-angioedema-following-prophylaxis-with-subcutaneous-c1-inhibitor-with-recombinant-hyaluronidase
#8
K Weller, M Maurer, M Fridman, D Supina, M Magerl, J Schranz
BACKGROUND: To estimate health-related quality-of-life changes in patients with hereditary angioedema due to C1-inhibitor (C1-INH) deficiency who received subcutaneous C1-INH with recombinant hyaluronidase (rHuPH20) for attack prophylaxis in a randomized, double-blind, dose-ranging, cross-over study. METHODS: Patients with type I/II hereditary angioedema received 1000 U of C1-INH with 24,000 U of rHuPH20 or 2000 U of C1-INH with 48,000 U of rHuPH20 every 3-4 days for 8 weeks and then crossed over for another 8-week period...
January 16, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28092989/therapeutic-complement-inhibition-a-promising-approach-for-treatment-of-neuroimmunological-diseases
#9
Kjara S Pilch, Peter Spaeth, Yuki Nobuhiro, Benjamin R Wakerley
Autoimmunity is an important cause of disease both in the central and peripheral nervous systems. Aetiologies and clinical manifestations are complex and heterogeneous. Inappropriate control of complement activation at inappropriate sites has been recognized as a major determinant in several neurological conditions, including Guillain-Barré syndrome and neuromyelitis optica. In each case pathogenesis is thought to be associated with generation of autoantibodies which upon binding guide activation of the complement system to self-tissue...
January 17, 2017: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/28069032/glucocorticoid-receptor-gene-polymorphisms-in-hereditary-angioedema-with-c1-inhibitor-deficiency
#10
Zsuzsanna Zotter, Zsolt Nagy, Attila Patócs, Dorottya Csuka, Nóra Veszeli, Kinga Viktória Kőhalmi, Henriette Farkas
BACKGROUND: Hereditary angioedema caused by C1-inhibitor deficiency (C1-INH-HAE) is a rare, autosomal dominant disorder. C1-INH-HAE is characterized by edema-formation, which may occur in response to stress. The individual's response to stress stimuli is partly genetically determined. Activation of the hypothalamic-pituitary-adrenal axis results in the release of cortisol. In turn, the secreted gluco- and mineralocorticoids affect the metabolism, as well as the cardiovascular and immune systems...
January 10, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28051822/novel-usage-of-fresh-frozen-plasma-in-hereditary-angioedema
#11
N Hanizah, C A Affirul, N A Farah, M A Shamila, M I Ridzuan
Hereditary angioedema (HAE) is a rare and potentially life threatening autosomal dominant disease characterized by recurrent episodes of cutaneous and mucosal oedema. It results from reduced expression or loss of function of CI-esterase inhibitors (C1-INH). As opposed to the more common histamine-mediated angioedema, HAE does not respond well to conventional treatments with anti-histamines, steroids and adrenaline. Early recognition and timely intervention with the correct treatment are crucial particularly preventing airway obstruction...
November 2016: La Clinica Terapeutica
https://www.readbyqxmd.com/read/28045547/peptide-macrocycle-inhibitor-of-coagulation-factor-xii-with-subnanomolar-affinity-and-high-target-selectivity
#12
Simon J Middendorp, Jonas Wilbs, Claudia Quarroz, Sara Calzavarini, Anne Angelillo-Scherrer, Christian Heinis
Factor XII (FXII) is a plasma protease that has emerged in recent years as a potential target to treat or prevent pathological thrombosis, to inhibit contact activation in extracorporeal circulation, and to treat the swelling disorder hereditary angioedema. While several protein based inhibitors with high affinity for activated FXII (FXIIa) were developed, the generation of small molecule inhibitors has been challenging. In this work, we have generated a potent and selective FXIIa inhibitor by optimizing a peptide macrocycle that was recently evolved by phage display (Ki = 0...
February 9, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28042430/experimental-protocol-of-dental-procedures-in-patients-with-hereditary-angioedema-the-role-of-anxiety-and-the-use-of-nitrogen-oxide
#13
A Rosa, M Miranda, R Franco, M G Guarino, A Barlattani, P Bollero
Hereditary angioedema (HAE) is a rare disease, little known to the medical and dental community, but with a growing rate of hospitalization over the years. HAE is due to a deficit/dysfunction of C1 esterase inhibitor which leads to an increase in vascular permeability and the appearance of edemas widespread in all body areas. The airways are the most affected and laryngeal swelling, which can occur, it is dangerous for the patient's life, is also a sensitive spot in our daily practice, therefore, it is also important to be aware of all the signs of this disease...
April 2016: Oral & Implantology
https://www.readbyqxmd.com/read/28025012/catabolism-of-c1-inhibitor-influences-the-response-to-replacement-therapy-in-hereditary-angioedema
#14
Marco Cicardi, Andrea Zanichelli, Chiara Suffritti, Maddalena A Wu, Thomas Machnig, Annalisa De Silvestri, Mario Regazzi, Carmine Tinelli
No abstract text is available yet for this article.
December 23, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28007084/manifestations-of-hereditary-angioedema
#15
Alessandro Testori, Isaac Melamed
No abstract text is available yet for this article.
January 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/27996949/triggers-and-prodromal-symptoms-of-angioedema-attacks-in-patients-with-hereditary-angioedema
#16
T Caballero, M Maurer, H J Longhurst, W Aberer, L Bouillet, V Fabien
No abstract text is available yet for this article.
2016: Journal of Investigational Allergology & Clinical Immunology
https://www.readbyqxmd.com/read/27965672/hereditary-angioedema-as-a-metabolic-liver-disorder-novel-therapeutic-options-and-prospects-for-cure
#17
Rohan Ameratunga, Adam Bartlett, John McCall, Richard Steele, See-Tarn Woon, Constance H Katelaris
Hereditary angioedema (HAE) is a rare autosomal dominant disorder caused by mutations of the SERPING1 or the Factor 12 genes. It is potentially fatal, particularly if not identified at an early stage. Apart from androgens, which are contraindicated in children and in pregnant women, a range of effective, albeit very expensive treatments have recently become available for HAE patients. The cost of these new treatments is beyond the reach of most developing countries. At this time, there is no cure for the disorder...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27940765/management-of-children-with-hereditary-angioedema-due-to-c1-inhibitor-deficiency
#18
Michael M Frank, Bruce Zuraw, Aleena Banerji, Jonathan A Bernstein, Timothy Craig, Paula Busse, Sandra Christiansen, Marc Davis-Lorton, H Henry Li, William R Lumry, Marc Riedl
Hereditary angioedema (HAE) is a potentially life-threatening inherited disease characterized by attacks of skin swelling, severe abdominal pain, and upper airway swelling. Attacks typically begin in childhood, but the appropriate diagnosis is often missed. Attacks do not respond to epinephrine, antihistamines, or glucocorticoids. Recently, many effective drugs have been approved for treatment of adults with HAE, and the Medical Advisory Board of the HAE Patient's Association has developed and reported treatment recommendations for adults...
November 2016: Pediatrics
https://www.readbyqxmd.com/read/27936514/comparing-acquired-angioedema-with-hereditary-angioedema-types-i-ii-findings-from-the-icatibant-outcome-survey
#19
H J Longhurst, A Zanichelli, T Caballero, L Bouillet, W Aberer, M Maurer, O Fain, V Fabien, I Andresen
Icatibant is used to treat acute hereditary angioedema with C1 inhibitor deficiency types I/II (C1-INH-HAE types I/II) and has shown promise in angioedema due to acquired C1 inhibitor deficiency (C1-INH-AAE). Data from the Icatibant Outcome Survey (IOS) were analysed to evaluate the effectiveness of icatibant in the treatment of patients with C1-INH-AAE and compare disease characteristics with those with C1-INH-HAE types I/II. Key medical history (including prior occurrence of attacks) was recorded upon IOS enrolment...
December 9, 2016: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27931305/subcutaneous-administration-of-human-c1-inhibitor-with-recombinant-human-hyaluronidase-in-patients-with-hereditary-angioedema
#20
Marc A Riedl, William R Lumry, H Henry Li, Aleena Banerji, Jonathan A Bernstein, Murat Ba, Janne Bjrkander, Markus Magerl, Marcus Maurer, Kevin Rockich, Hongzi Chen, Jennifer Schranz
BACKGROUND: The currently approved method of C1 inhibitor (C1 INH) administration for patients with hereditary angioedema with C1 INH deficiency (HAE) is by intravenous injection. A C1 INH subcutaneous formulation may provide an attractive mode of administration for some patients. OBJECTIVE: To evaluate efficacy and safety of two doses of subcutaneous, plasma-derived C1 INH with the dispersing agent, recombinant human hyaluronidase (rHuPH20) to prevent angioedema attacks in patients with HAE...
November 2016: Allergy and Asthma Proceedings:
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