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Hereditary angioedema

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https://www.readbyqxmd.com/read/28302171/clinical-characteristics-and-real-life-diagnostic-approaches-in-all-danish-children-with-hereditary-angioedema
#1
Anne Aabom, Klaus E Andersen, Christina Fagerberg, Niels Fisker, Marianne A Jakobsen, Anette Bygum
BACKGROUND: With a potentially early onset, hereditary angioedema (HAE) requires special knowledge also in infancy and early childhood. In children from families with HAE, the diagnosis should be confirmed or refuted early, which can be difficult. Studies of childhood HAE and the diagnostic approaches are limited. Our aim was to investigate the entire Danish cohort of children with HAE and non-HAE children of HAE patients for diagnostic approaches and clinical characteristics. RESULTS: We included 41 children: 22 with HAE and 19 non-HAE...
March 16, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28292712/mr-imaging-of-intestinal-angioedema-related-to-angiotensin-converting-enzyme-inhibitors-report-of-three-cases-and-review-of-literature
#2
Michael R Savino, Pardeep K Mittal, Frank H Miller
Angioedema is a condition in which an increase in vascular permeability leads to the swelling of body tissues. There are both hereditary and acquired forms of the disease, with the latter often associated with the administration of angiotensin-converting enzyme inhibitor medication. Involvement of the intestinal tract is a rare manifestation of angioedema, and can present with abdominal pain, nausea, and vomiting. It is critical for radiologists to be aware of this entity, as they may have the only opportunity to make the diagnosis...
March 8, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28291095/angioedema
#3
Daniel LoVerde, Daniel Clark Files, Guha Krishnaswamy
OBJECTIVES: Angioedema is a potentially life-threatening occurrence that is encountered by critical care providers. The mechanistic understanding of angioedema syndromes has improved in recent years, and novel medications are available that improve outcomes from these syndromes. This clinically focused review will describe the underlying genetics, pathophysiology, classification and treatment of angioedema syndromes, with an emphasis on the novel pharmacologic agents that have recently become available for acute treatment...
April 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28286153/safety-of-c1-inhibitor-concentrate-use-for-hereditary-angioedema-in-pediatric-patients
#4
Paula Busse, James Baker, Inmaculada Martinez-Saguer, Jonathan A Bernstein, Timothy Craig, Markus Magerl, Marc Riedl, Ralph Shapiro, Michael Frank, William Lumry, Jeffrey Rosch, Jonathan Edelman, Debora Williams-Herman, Henrike Feuersenger, Mikhail Rojavin
No abstract text is available yet for this article.
March 9, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28284978/sustained-response-of-recombinant-human-c1-esterase-inhibitor-for-acute-treatment-of-hereditary-angioedema-attacks
#5
Jonathan A Bernstein, Anurag Relan, Joseph R Harper, Marc Riedl
BACKGROUND: Symptoms of hereditary angioedema (HAE) attacks can recur soon after initial treatment; the durability of response for recombinant human C1 esterase inhibitor (rhC1INH) treatment is unknown. OBJECTIVE: To examine the efficacy and durability of rhC1INH for acute HAE attacks. METHODS: In this pooled post hoc analysis of 2 trials, patients with type I or II HAE (functional C1INH levels <50% of normal) and a baseline visual analog scale score of at least 50 mm were included if they had received at least 1 intravenous dose of 50 U/kg of rhC1INH...
March 8, 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/28284781/diagnosis-course-and-management-of-angioedema-in-patients-with-acquired-c1-inhibitor-deficiency
#6
Andrea Zanichelli, Giulia Maria Azin, Maddalena Alessandra Wu, Chiara Suffritti, Lorena Maggioni, Sonia Caccia, Francesca Perego, Romualdo Vacchini, Marco Cicardi
BACKGROUND: Acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a rare disease with no prevalence data or approved therapies. OBJECTIVE: To report data on patients with C1-INH-AAE followed at Angioedema Center, Milan (from 1976 to 2015). METHODS: Diagnostic criteria included history of recurrent angioedema without wheals; decreased C1-INH antigen levels and/or functional activity of C1-INH and C4 antigen less than 50% of normal; late symptom onset (>40 years); no family history of angioedema and C1-INH deficiency...
March 8, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28283277/anabolic-androgen-use-in-the-management-of-hereditary-angioedema-not-so-cheap-after-all
#7
Kevin Y Tse, Bruce L Zuraw, Qiaoling Chen, Sandra C Christiansen
BACKGROUND: Hereditary angioedema due to C1 inhibitor deficiency (HAE) is a rare, life-threatening disease that imposes a significant burden on affected patients. 17α-alkylated androgens (anabolic androgens) decrease attack frequency and severity but carry the risk of potentially serious dose-related adverse effects. Despite the emergence of targeted therapies for HAE, continued anabolic androgen use has been driven in part by their low cost. OBJECTIVE: To examine the hidden cost of anabolic androgen use related to the risk of developing non-HAE comorbidities...
March 7, 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/28279594/blindness-tetraspasticity-and-other-signs-of-irreversible-brain-damage-in-hereditary-angioedema
#8
Konrad Bork, Randolf Brehler, Günther Witzke, Stephan Boor, Wolf Heineke, Jochen Hardt
No abstract text is available yet for this article.
March 6, 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/28258590/health-related-quality-of-life-among-children-with-hereditary-angioedema
#9
Batya Engel-Yeger, Henriette Farkas, Shmuel Kivity, Nóra Veszeli, Kinga Viktória Kőhalmi, Aharon Kesssel
BACKGROUND: The clinical expressions of hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) and its related burden may negatively affect patient quality of life. This study aims to assess health-related quality of life (HRQoL) in children with C1-INH-HAE. METHODS: Children (N=98: 34 C1-INH-HAE patients, 64 healthy controls) aged 3-18 years were recruited in Israel and Hungary. All individuals completed a demographic questionnaire, a disease activity and site questionnaire, and the Pediatric Quality of Life Inventory (PedsQL(™) ) 4...
March 4, 2017: Pediatric Allergy and Immunology
https://www.readbyqxmd.com/read/28251901/hereditary-angioedema-with-normal-c1-inhibitor-clinical-characteristics-and-treatment-response-with-plasma-derived-human-c1-inhibitor-concentrate-berinert-%C3%A2-in-a-french-cohort
#10
Laurence Bouillet, Isabelle Boccon-Gibod, Anne Gompel, Bernard Floccard, Ludovic Martin, Claire Blanchard-Delaunay, David Launay, Olivier Fain
Hereditary angioedema (HAE) is a rare genetic disorder characterised by episodes of swelling without urticaria. Berinert® (CSL Behring) is a plasma-derived human C1 inhibitor (C1-INH) concentrate, approved for the treatment of HAE with C1-INH deficiency (C1-INH-HAE), however, it is often used off-label in Europe to treat HAE with normal C1-INH. OBJECTIVES: To report the clinical characteristics of patients with HAE with normal C1-INH (with F12 gene mutation; FXII-HAE) or of unknown origin (U-HAE), and their response to Berinert®...
March 1, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28250922/hereditary-angioedema-with-normal-c1-inhibitor-in-a-french-cohort-clinical-characteristics-and-response-to-treatment-with-icatibant
#11
Laurence Bouillet, Isabelle Boccon-Gibod, David Launay, Anne Gompel, Gisele Kanny, Vincent Fabien, Oliver Fain
INTRODUCTION: The clinical characteristics and icatibant-treatment outcomes of patients with hereditary angioedema with normal C1 inhibitor (HAE-nC1 INH) are limited. METHODS: We retrospectively analyzed data from French HAE patients enrolled in the Icatibant Outcome Survey registry (from July 2009 to September 2013) to compare disease characteristics and the effectiveness and safety of acute icatibant-treated angioedema attacks in patients with HAE-nC1 INH, HAE with C1 INH deficiency (type I), or dysfunction (type II)...
March 2017: Immunity, Inflammation and Disease
https://www.readbyqxmd.com/read/28225685/kallikrein-inhibition-for-hereditary-angioedema
#12
EDITORIAL
Hilary J Longhurst
No abstract text is available yet for this article.
February 23, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28225674/inhibiting-plasma-kallikrein-for-hereditary-angioedema-prophylaxis
#13
RANDOMIZED CONTROLLED TRIAL
Aleena Banerji, Paula Busse, Mustafa Shennak, William Lumry, Mark Davis-Lorton, Henry J Wedner, Joshua Jacobs, James Baker, Jonathan A Bernstein, Richard Lockey, H Henry Li, Timothy Craig, Marco Cicardi, Marc Riedl, Ahmad Al-Ghazawi, Carolyn Soo, Ryan Iarrobino, Daniel J Sexton, Christopher TenHoor, Jon A Kenniston, Ryan Faucette, J Gordon Still, Harvey Kushner, Robert Mensah, Chris Stevens, Joseph C Biedenkapp, Yung Chyung, Burt Adelman
Background Hereditary angioedema with C1 inhibitor deficiency is characterized by recurrent, unpredictable swelling episodes caused by uncontrolled plasma kallikrein generation and excessive bradykinin release resulting from cleavage of high-molecular-weight kininogen. Lanadelumab (DX-2930) is a new kallikrein inhibitor with the potential for prophylactic treatment of hereditary angioedema with C1 inhibitor deficiency. Methods We conducted a phase 1b, multicenter, double-blind, placebo-controlled, multiple-ascending-dose trial...
February 23, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28222436/recurrent-angioedema-occurrence-features-and-concomitant-diseases-in-an-italian-single-center-study
#14
Paola Triggianese, Maria Domenica Guarino, Chiara Pellicano, Mauro Borzi, Elisabetta Greco, Stella Modica, Caterina De Carolis, Roberto Perricone
BACKGROUND: Angioedema (AE) is a potentially life-threatening condition with hereditary (HAE), acquired (AAE), or iatrogenic causes. A careful workup allows for the identification of the etiology of attacks and the appropriate management. In this cohort study, based on a clinical practice setting, we aimed at investigating clinical and laboratory findings concerning different features of patients with recurrent AE who were referred to a single, tertiary-level center for HAE. METHODS: Clinical and laboratory data of patients fulfilling the criteria for C1-inhibitor-deficient HAE (C1-INH-HAE), C1-INH-AAE, angiotensin-converting enzyme inhibitor-related AE (ACEI-RA), and idiopathic AAE (I-AAE) were evaluated...
2017: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/28222330/hereditary-c1-inhibitor-deficiency-is-associated-with-high-spontaneous-amidase-activity
#15
Delphine Charignon, Arije Ghannam, Denise Ponard, Christian Drouet
BACKGROUND: Angioedema diagnosis classically targets the complement system (via C1 inhibitor (C1Inh) function and antigenic C4 level) and contact phase activation (via amidase activity). Bradykinin is responsible for angioedema attacks and is produced from contact phase activation secondary to failed C1Inh control. OBJECTIVE: We aimed to compare the diagnostic performances of spontaneous amidase activity and antigenic C4 level in C1Inh hereditary angioedema (C1Inh-HAE) patients...
February 18, 2017: Molecular Immunology
https://www.readbyqxmd.com/read/28202404/recombinant-human-c1-esterase-inhibitor-to-treat-acute-hereditary-angioedema-attacks-in-adolescents
#16
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
#17
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
#18
MULTICENTER STUDY
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
#19
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
#20
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
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