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Hereditary angioedema

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https://www.readbyqxmd.com/read/28905884/clinical-utility-gene-card-for-hereditary-angioedema-with-normal-c1-inhibitor-haenc1
#1
Christiane Stieber, Sven Cichon, Markus Magerl, Markus M Nöthen
No abstract text is available yet for this article.
October 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28903805/recombinant-human-c1-esterase-inhibitor-for-acute-hereditary-angioedema-attacks-with-upper-airway-involvement
#2
M A M A Riedl, H H Li, M M Cicardi, J R J R Harper, A A Relan
BACKGROUND: Recombinant human C1 esterase inhibitor (rhC1-INH) is approved for treatment of hereditary angioedema (HAE)in adolescents and adults. HAE attacks that involve the upper airway can be life threatening, and data on the administration of rhC1-INH for these types of attacks are currently limited. OBJECTIVE: To evaluate the efficacy and safety of rhC1-INH for treatment of acute HAE attacks with upper airway involvement. METHODS: A pooled analysis of data from three clinical trials with open-label extensions examined rhC1-INH for treatmentof acute HAE attacks with upper airway involvement...
September 13, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28888847/hereditary-angioedema-in-2-sisters-due-to-paternal-gonadal-mosaicism
#3
Didier G Ebo, Athina L Van Gasse, Vito Sabato, Esther Bartholomeus, Edwin Reyniers, Jean-François Vanbellinghen, Hélène A Poirel, Geert Mortier
No abstract text is available yet for this article.
September 6, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28874235/efficacy-of-recombinant-human-c1-esterase-inhibitor-for-the-treatment-of-severe-hereditary-angioedema-attacks
#4
H H Li, A Reshef, J W Baker, J R Harper, A Relan
BACKGROUND: Severe attacks of hereditary angioedema (HAE) are debilitating and potentially life threatening, and can increase anxiety and the use of medical resources. OBJECTIVE: This post hoc assessment evaluated recombinant human C1 esterase inhibitor (rhC1-INH) used to treat acute severe HAE attacks. METHODS: In a double-blind, randomized-controlled trial (RCT), patients with an HAE attack (baseline visual analog scalescore of greater than or equal to 50 mm, with severe attacks defined as greater than or equal to 75 mm) were randomly assigned to receive rhC1-INH (50 IU/kg for patients who weighed <84 kg; 4200 IU for patients who weighed more than or equal to 84 kg) or placebo...
September 5, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28855002/health-related-quality-of-life-in-relation-to-disease-activity-in-adults-with-hereditary-angioedema-in-sweden
#5
undefinedP Nordenfelt, undefinedM Nilsson, undefinedA Lindfors, undefinedC-F Wahlgren, undefinedJ Bjorkander
BACKGROUND: Health-related quality of life (HR-QoL) is impaired in patients with hereditary angioedema (HAE) but has notyet been satisfactorily described. OBJECTIVE: To study HR-QoL in patients with HAE by combining different HR-QoL instruments with disease activity assessment. METHODS: All adults in the Swedish HAE registry were invited to take part in this questionnaire study, which used the generic HR-QoL instruments, EuroQol 5 Dimensions 5 Level (EQ-5D-5L) and the RAND Corporation Short Form 36 (RAND-36), the disease-specific Angioedema Quality of Life instrument (AE-QoL), the recently introduced Angioedema Activity Score (AAS) form, and questionnaires on sick leave and prophylactic medication...
August 30, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28825570/treatment-of-hereditary-angioedema-due-to-c1-inhibitor-deficiency-in-argentina
#6
Eloisa Malbrán, Alejandra Menéndez, Alejandro Malbrán
The benefits of the worldwide approval of new drugs for the treatment of acute C1-INH-HAE attacks may still not reach all patients. Identifying the current barriers in the access to medication, as well as conducting a detailed assessment of the progress in this area, is essential to achieve universal treatment. Two hundred and twenty five patients registered in the Argentina Hereditary Angioedema Patient Association (AHAEPA) were randomly selected and invited to participate in a web based questionnaire on accessibility to icatibant and pdC1-INH, self-treatment, delay to treatment, and coverage...
2017: Medicina
https://www.readbyqxmd.com/read/28818177/angioedema
#7
Janina Hahn, Thomas K Hoffmann, Bastian Bock, Melanie Nordmann-Kleiner, Susanne Trainotti, Jens Greve
BACKGROUND: Acute angioedema of the upper airways can be life-threatening. An important distinction is drawn between mast-cell-mediated angioedema and bradykinin-mediated angioedema; the treatment of these two entities is fundamentally different. METHODS: This review is based on pertinent articles retrieved by a selective search in PubMed and on guidelines concerning the treatment of angioedema. The authors draw on their own clinical experience in their assessment of the literature...
July 24, 2017: Deutsches Ärzteblatt International
https://www.readbyqxmd.com/read/28795768/hereditary-angioedema-with-a-mutation-in-the-plasminogen-gene
#8
K Bork, K Wulff, L Steinmüller-Magin, I Braenne, P Staubach-Renz, G Witzke, J Hardt
BACKGROUND: Hereditary angioedema (HAE) with normal C1-INH (HAEnCI) may be linked to specific mutations in the coagulation factor 12 (FXII) gene (HAE-FXII) or functional mutations in other genes that are still unknown. We sought to identify and characterize a hitherto unknown type of HAE with normal C1-INH and without mutation in the F12 gene. METHODS: The study comprised analysis of whole exome sequencing, Sanger sequencing, and clinical data of patients. RESULTS: We detected a mutation in the plasminogen gene in patients with HAEnCI...
August 10, 2017: Allergy
https://www.readbyqxmd.com/read/28791569/pediatric-angioedema
#9
REVIEW
Debendra Pattanaik, Jay Adam Lieberman
PURPOSE OF REVIEW: The aims of this study are to update the clinician on current understanding of angioedema as it presents in the pediatric population and to review proper diagnostic techniques and treatment modalities for various types of angioedema. RECENT FINDINGS: Angioedema is still best classified by whether it is likely histaminergic or kinin-mediated. New guidelines have been published around the world to help diagnose and treat both forms (urticaria/angioedema and hereditary angioedema)...
August 8, 2017: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/28782632/cleaved-kininogen-as-a-biomarker-for-bradykinin-release-in-hereditary-angioedema
#10
Zonne L M Hofman, Steven de Maat, Chiara Suffritti, Andrea Zanichelli, Cassandra van Doorn, Silvie A E Sebastian, Nora Veszeli, Dorottya Csuka, Thomas Renné, Gerard Pasterkamp, Marco Cicardi, Henriette Farkas, C Erik Hack, Coen Maas
No abstract text is available yet for this article.
August 4, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28781749/pediatric-hereditary-angioedema-an-update
#11
REVIEW
Geetika Sabharwal, Timothy Craig
Hereditary angioedema (HAE) with C1-inhibitor (C1-Inh) deficiency (C1-Inh-HAE) is a rare, life-threatening, and disabling genetic disorder characterized by self-limited tissue swelling caused by deficiency or dysfunction of C1-Inh. Our aim in this update is to discuss new advances in HAE therapy, focusing mainly on the various treatment options that have become available recently and also drugs that are under trial for prophylaxis to prevent attacks. There is a paradigm shift to where the treatment of HAE is headed, focusing now on prophylactic treatment rather than abortive management...
2017: F1000Research
https://www.readbyqxmd.com/read/28780879/-effect-of-danazol-treatment-on-growth-in-pediatric-patients-with-hereditary-angioedema-due-to-c1-inhibitor-deficiency
#12
Kinga Viktória Kőhalmi, Nóra Veszeli, Andrea Luczay, Lilian Varga, Henriette Farkas
INTRODUCTION: Attenuated androgens are used for the prevention of angioedema attacks of hereditary angioedema with C1-inhibitor deficiency. After prepuberty, their use can lead to growth retardation. AIM: We assessed the effect of danazol on the growth of pediatric patients with hereditary angioedema. METHOD: In the retrospective study on 42 patients diagnosed with hereditary angioedema, we calculated the deviation from the mid-parental target height, and analyzed it against the gender, the dose and duration of danazol treatment administered before the age of 21 years and before the age of 16 years...
August 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28780207/hereditary-angioedema-an-overlooked-cause-of-recurrent-abdominal-pain-and-free-peritoneal-fluid
#13
Piotr Eder, Maciej Adler, Liliana Lykowska-Szuber
No abstract text is available yet for this article.
August 2, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28763531/new-hereditary-angioedema-therapy
#14
Rebecca Voelker
No abstract text is available yet for this article.
August 1, 2017: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/28715060/the-complex-interaction-between-polycystic-ovary-syndrome-and-hereditary-angioedema-case-reports-and-review-of-the-literature
#15
Marina Iahn-Aun, Marcelo Vivolo Aun, Antonio Abílio Motta, Jorge Kalil, Pedro Giavina-Bianchi, Sylvia Asaka Hayashida, Edmund Chada Baracat, Gustavo Arantes Maciel
Importance: Hereditary angioedema (HAE) is a rare but severe disease, with high risk of death, and attacks have been associated to high estrogen levels. Polycystic ovary syndrome (PCOS) is a common hyperandrogenic condition, which is frequently treated with combined oral contraceptives. Objective: The aim of this study was to describe 2 clinical cases of young women diagnosed as having PCOS who developed HAE attacks after the introduction of combined estrogen-progestin pills to treat PCOS symptoms...
July 2017: Obstetrical & Gynecological Survey
https://www.readbyqxmd.com/read/28692169/the-relationship-between-anxiety-and-quality-of-life-in-children-with-hereditary-angioedema
#16
Aharon Kessel, Henriette Farkas, Shmuel Kivity, Nóra Veszeli, Kinga Viktória Kőhalmi, Batya Engel-Yeger
BACKGROUND: The severe life-threatening characteristics of hereditary angioedema (HAE) with C1-inhibitor deficiency (C1-INH-HAE) can affect anxiety levels among pediatric patients. This emotional burden together with the physical restrictions of C1-INH-HAE may decrease children's health-related quality of life (HRQoL). OBJECTIVE: (1) To compare anxiety state and trait between children with C1-INH-HAE and healthy controls (2) To examine the relationship between the level of anxiety of children with C1-INH-HAE, their disease activity/affected sites and their HRQoL (3) To predict the HRQoL of children with C1-INH-HAE based on their anxiety level and disease activity/affected sites...
July 10, 2017: Pediatric Allergy and Immunology
https://www.readbyqxmd.com/read/28690642/breakthrough-attacks-in-patients-with-hereditary-angioedema-receiving-long-term-prophylaxis-are-responsive-to-icatibant-findings-from-the-icatibant-outcome-survey
#17
Werner Aberer, Marcus Maurer, Laurence Bouillet, Andrea Zanichelli, Teresa Caballero, Hilary J Longhurst, Amandine Perrin, Irmgard Andresen
BACKGROUND: Patients with hereditary angioedema (HAE) due to C1-inhibitor deficiency (C1-INH-HAE) experience recurrent attacks of cutaneous or submucosal edema that may be frequent and severe; prophylactic treatments can be prescribed to prevent attacks. However, despite the use of long-term prophylaxis (LTP), breakthrough attacks are known to occur. We used data from the Icatibant Outcome Survey (IOS) to evaluate the characteristics of breakthrough attacks and the effectiveness of icatibant as a treatment option...
2017: Allergy, Asthma, and Clinical Immunology
https://www.readbyqxmd.com/read/28687113/pharmacoeconomics-of-orphan-disease-treatment-with-a-focus-on-hereditary-angioedema
#18
REVIEW
William R Lumry
This article discusses orphan diseases, their prevalence, legislative incentives to encourage development of therapies, and the impact of treatment on health care payment systems. Specifically, the cost burden of hereditary angioedema on patients, health care systems, and society is reviewed. The impact of availability of and access to novel and specific therapies on morbidity, mortality, and overall burden of disease is explored. Changes in treatment paradigms to improve effect and reduce cost of treatment are presented...
August 2017: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/28687112/burden-of-illness-and-quality-of-life-measures-in-angioedema-conditions
#19
REVIEW
Teresa Caballero, Nieves Prior
Burden of illness studies and evaluation of health-related quality of life using validated questionnaires have become an important task in the comprehensive management of angioedema conditions, mainly angioedema associated with chronic spontaneous urticaria and hereditary angioedema caused by C1-inhibitor deficiency. A review of the principal tools and studies is presented. Both diseases present a higher proportion of psychiatric disorders, impair work and studies productivity, and produce high direct and indirect costs...
August 2017: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/28687111/emerging-therapies-in-hereditary-angioedema
#20
REVIEW
Meng Chen, Marc A Riedl
Remarkable progress has been made in the treatment of bradykinin-mediated angioedema with the advent of multiple new therapies. Patients now have effective medications available for prophylaxis and treatment of acute attacks. However, hereditary angioedema is a burdensome disease that can lead to debilitating and dangerous angioedema episodes associated with significant costs for individuals and society. The burden of treatment must be addressed regarding medication administration difficulties, treatment complications, and adverse side effects...
August 2017: Immunology and Allergy Clinics of North America
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