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Hereditary angioedema

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https://www.readbyqxmd.com/read/29130992/genetic-determinants-of-c1-inhibitor-deficiency-angioedema-age-of-onset
#1
Panagiota Gianni, Gedeon Loules, Maria Zamanakou, Maria Kompoti, Dorottya Csuka, Fotis Psarros, Markus Magerl, Dimitru Moldovan, Marcus Maurer, Matthaios G Speletas, Henriette Farkas, Anastasios E Germenis
BACKGROUND: In view of the large heterogeneity in the clinical presentation of hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE), great efforts are being made towards detecting measurable biological determinants of disease severity that can help to improve the management of the disease. Considering the central role that plasma kallikrein plays in bradykinin production, we investigated the contribution of the functional polymorphism KLKB1-428G/A to the disease phenotype...
November 9, 2017: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/29128335/hereditary-angioedema-with-normal-c1-inhibitor-and-f12-mutations-in-42-brazilian-families
#2
Camila Lopes Veronez, Adriana S Moreno, Rosemeire Navickas Constantino-Silva, Luana S M Maia, Mariana P L Ferriani, Fábio F M Castro, Solange Rodrigues Valle, Victor Koji Nakamura, Nathália Cagini, Rozana Fátima Gonçalves, Eli Mansour, Faradiba Sarquis Serpa, Gabriela Andrade Coelho Dias, Miguel Alberto Piccirillo, Eliana Toledo, Marli de Souza Bernardes, Sven Cichon, Christiane Stieber, L Karla Arruda, João Bosco Pesquero, Anete Sevciovic Grumach
BACKGROUND: Hereditary angioedema (HAE) with normal C1 inhibitor (C1-INH) is a rare condition with clinical features similar to those of HAE with C1-INH deficiency. Mutations in the F12 gene have been identified in subsets of patients with HAE with normal C1-INH, mostly within families of European descent. OBJECTIVES: Our aim was to describe clinical characteristics observed in Brazilians from 42 families with HAE and F12 gene mutations (FXII-HAE), and to compare these findings with those from other populations...
November 8, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/29112487/pharmacotherapy-for-angiotensin-converting-enzyme-inhibitor-induced-angioedema-a-systematic-review
#3
Claire M Lawlor, Ashwin Ananth, Blair M Barton, Thomas C Flowers, Edward D McCoul
Objective Angioedema is a potentially life-threatening complication of angiotensin-converting enzyme inhibitor (ACEI) use, occurring in up to 0.5% of users. Although the pathophysiology of ACEI-induced angioedema is attributable to elevated serum bradykinin, standard management typically includes corticosteroids and antihistamines. We sought to summarize the evidence supporting pharmacotherapy for ACEI-induced angioedema. Data Sources PubMed, MEDLINE, and Embase portals. Methods A systematic literature review was conducted according to the PRISMA guidelines...
November 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29107952/hereditary-angioedema-assessing-the-hypothesis-for-underlying-autonomic-dysfunction
#4
Maddalena A Wu, Francesco Casella, Francesca Perego, Chiara Suffritti, Nada Afifi Afifi, Eleonora Tobaldini, Andrea Zanichelli, Chiara Cogliati, Nicola Montano, Marco Cicardi
BACKGROUND: Attacks of Hereditary Angioedema due to C1-inhibitor deficiency (C1-INH-HAE)are often triggered by stressful events/hormonal changes. OBJECTIVE: Our study evaluates the relationship between autonomic nervous system (ANS) and contact/complement system activation. METHODS: Twenty-three HAE patients (6 males, mean age 47.5±11.4 years) during remission and 24 healthy controls (8 males, mean age 45.3±10.6 years) were studied. ECG, beat-by-beat blood pressure, respiratory activity were continuously recorded during rest (10') and 75-degrees-head-up tilt (10')...
2017: PloS One
https://www.readbyqxmd.com/read/29093422/comparison-of-the-frequency-of-angioedema-attack-before-and-during-pregnancy-in-a-patient-with-type-i-hereditary-angioedema
#5
Atsushi Satomura, Takayuki Fujita, Tomohiro Nakayama
The patient was a 38-year-old Japanese woman who had been diagnosed with hereditary angioedema type I at 7 years of age based on her family history. She had undergone four pregnancies. She gave birth to a healthy baby girl after her first pregnancy and had reported few episodes of angioedema. However, she subsequently required abortions due to frequent angioedema episodes that occurred during her three subsequent pregnancies. Thus, our patient showed two clinical pregnancy courses. After treating her with C1-inhibitor concentrate, her symptoms of angioedema disappeared...
November 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29093383/clinical-features-of-hereditary-and-mast-cell-mediated-angioedema-focusing-on-the-differential-diagnosis-in-japanese-patients
#6
Isao Ohsawa, Daisuke Honda, Atsuko Hisada, Hiroyuki Inoshita, Kisara Onda-Tsueshita, Satoshi Mano, Nobuyuki Sato, Yuya Nakamura, Tatsuo Shimizu, Hiromichi Gotoh, Yoshikazu Goto, Yusuke Suzuki, Yasuhiko Tomino
Background The present study was designed to identify the clinical characteristics that permit the differential diagnosis of hereditary angioedema (HAE) and mast cell-mediated angioedema (Mast-AE) during the first consultation. Methods The medical histories and laboratory data of 46 patients with HAE and 41 patients with Mast-AE were compared. Results The average age of onset in the HAE group (19.8±9.0 years) was significantly lower than that in the Mast-AE group (35.2±12.0 years). The incidence of familial angioedema (AE) in the HAE group (73...
November 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29070276/multiple-doses-of-icatibant-used-during-pregnancy
#7
Lauren W Kaminsky, Theodore Kelbel, Fay Ansary, Timothy Craig
BACKGROUND: Hereditary angioedema (HAE) is a life-long disease that often manifests by puberty. Treatment of attacks is essential to improve quality of life and to decrease morbidity and mortality. During pregnancy, treatment is limited because multiple treatment options, including icatibant, are not approved for use during pregnancy. OBJECTIVE: We report the outcomes of three pregnancies during which icatibant was used by a patient with HAE with normal C1-inhibitor for treatment of attacks...
October 1, 2017: Allergy & Rhinology
https://www.readbyqxmd.com/read/29059678/endocan-a-novel-marker-of-endothelial-dysfunction-in-c1-inhibitor-deficient-hereditary-angioedema
#8
Mustafa Demirturk, Timur Selcuk Akpinar, Murat Kose, Aslı Gelincik, Bahattin Colakoğlu, Suna Buyukozturk
BACKGROUND: Hereditary angioedema (HAE) related to C1-inhibitor deficiency is a rare autosomal dominant disorder. Vascular cell adhesion molecules (VCAM) are known as endothelial activation markers. Endocan (also called ESM-1) is proposed as an endothelial dysfunction indicator. We aimed to investigate endothelial activation in attack-free periods in HAE patients by measuring their levels of endocan and VCAM-1. METHODS: Twenty-six HAE patients (22 female, mean age 40 ± 13 years) and 38 healthy control patients (13 female, mean age 36...
2017: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/29057741/pregnancy-and-postpartum-in-hereditary-angioedema-with-c1-inhibitor-deficit-in-women-who-have-no-access-to-therapy
#9
A Mrg Machado, R Mg Pires, R O Martins, A S Grumach
No abstract text is available yet for this article.
2017: Journal of Investigational Allergology & Clinical Immunology
https://www.readbyqxmd.com/read/29046193/health-related-quality-of-life-in-danish-children-with-hereditary-angioedema
#10
Anne Aabom, Dan Nguyen, Niels Fisker, Anette Bygum
BACKGROUND: The potentially life-threatening disease hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) can have considerable impact on the health-related quality of life (HRQoL) in adult patients. Half the patients with C1-INH-HAE develop symptoms before the age of 10 years. However, the HRQoL in children with C1-INH-HAE is almost unexplored. OBJECTIVE: To investigate HRQoL in Danish children with C1-INH-HAE, including possible correlations to disease severity and attack frequency...
November 1, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/29043014/an-open-label-study-to-evaluate-the-long-term-safety-and-efficacy-of-lanadelumab-for-prevention-of-attacks-in-hereditary-angioedema-design-of-the-help-study-extension
#11
Marc A Riedl, Jonathan A Bernstein, Timothy Craig, Aleena Banerji, Markus Magerl, Marco Cicardi, Hilary J Longhurst, Mustafa M Shennak, William H Yang, Jennifer Schranz, Jovanna Baptista, Paula J Busse
BACKGROUND: Hereditary angioedema (HAE) is characterized by recurrent attacks of subcutaneous or submucosal edema. Attacks are unpredictable, debilitating, and have a significant impact on quality of life. Patients may be prescribed prophylactic therapy to prevent angioedema attacks. Current prophylactic treatments may be difficult to administer (i.e., intravenously), require frequent administrations or are not well tolerated, and breakthrough attacks may still occur frequently. Lanadelumab is a subcutaneously-administered monoclonal antibody inhibitor of plasma kallikrein in clinical development for prophylaxis of hereditary angioedema attacks...
2017: Clinical and Translational Allergy
https://www.readbyqxmd.com/read/29017903/successful-perinatal-management-of-hereditary-angioedema-with-normal-c1-esterase-inhibitor-and-factor-xii-mutation-using-c1-esterase-inhibitor-therapy
#12
Kathleen R Gibbons, Tina Abraham, Monica Sandhu, Brian P Peppers, Julia F Girzhel, Robert W Hostoffer
No abstract text is available yet for this article.
October 7, 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/28994183/angiotensin-converting-enzyme-inhibitor-induced-angioedema-a-review-of-the-literature
#13
REVIEW
Teresa Brown, Jimmy Gonzalez, Catherine Monteleone
According to the National Health and Nutrition Examination Survey 2012, one third of antihypertensive prescriptions in the United States in the past decade were for angiotensin-converting enzyme inhibitors (ACEIs). An important and serious side effect of ACEIs is angioedema caused by a reduction in bradykinin degradation. In a national medical chart abstraction study conducted at the US Veterans Affairs Health Care System in 2008, 0.20% of ACEI initiators developed angioedema while on the medication. The angiotensin-converting enzyme is a part of the renin-angiotensin system that converts angiotensin I to angiotensin II...
October 10, 2017: Journal of Clinical Hypertension
https://www.readbyqxmd.com/read/28986975/complete-kinetic-follow-up-of-symptoms-and-complement-parameters-during-a-hereditary-angioedema-attack
#14
Nóra Veszeli, Kinga Viktória Kőhalmi, Erika Kajdácsi, Dominik Gulyás, György Temesszentandrási, László Cervenak, Henriette Farkas, Lilian Varga
We studied the kinetics of C1-inhibitor (C1-INH) and other complement parameters in a self-limited edematous attack (EA) in a patient with hereditary angioedema due to C1-INH deficiency to better understand the pathomechanism of the evolution, course, and complete resolution of EAs. C1-INH concentration and functional activity (C1-INHc+f ), C1(q,r,s), C3, C4, C3a, C4a, C5a and SC5b-9 levels were measured in blood samples obtained during the 96-hour observation period. The highest C1-INHc+f , C4, and C1(q,r,s) levels were measured at baseline, and their continuous decrease was observed during the entire observation period...
October 7, 2017: Allergy
https://www.readbyqxmd.com/read/28966616/factor-xii-driven-inflammatory-reactions-with-implications-for-anaphylaxis
#15
REVIEW
Lysann Bender, Henri Weidmann, Stefan Rose-John, Thomas Renné, Andy T Long
Anaphylaxis is a life-threatening allergic reaction. It is triggered by the release of pro-inflammatory cytokines and mediators from mast cells and basophils in response to immunologic or non-immunologic mechanisms. Mediators that are released upon mast cell activation include the highly sulfated polysaccharide and inorganic polymer heparin and polyphosphate (polyP), respectively. Heparin and polyP supply a negative surface for factor XII (FXII) activation, a serine protease that drives contact system-mediated coagulation and inflammation...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28950264/an-abc-of-the-warning-signs-of-hereditary-angioedema
#16
REVIEW
Anete Sevciovic Grumach, Natasha Ferraroni, Maria Margarita Olivares, Maria Concepción López-Serrano, Anette Bygum
Hereditary angioedema (HAE) with C1 inhibitor deficiency is a genetic disorder that clinically manifests with attacks of angioedema in the subcutaneous and submucosal tissues, mainly in the extremities, abdomen, and upper airway. During attacks, vascular permeability is increased due to increased bradykinin (BK). This means that special therapies are needed for attacks that do not respond to traditional antiallergic therapies involving antihistamines, corticosteroids, and epinephrine. The recurring attacks may disable patients and lead to frequent visits to emergency rooms where misdiagnoses are common...
2017: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/28939396/novel-therapies-for-angiotensin-converting-enzyme-inhibitor-induced-angioedema-a-systematic-review-of-current-evidence
#17
Heidi M Riha, Bryant B Summers, Jessica V Rivera, Megan A Van Berkel
BACKGROUND: Angiotensin-converting enzyme inhibitor (ACEI)-induced angioedema can occur at any point during therapy and, when severe, can require mechanical ventilation. Standard agents for anaphylactic reactions have limited efficacy for bradykinin-mediated angioedema and, therefore, agents approved for hereditary angioedema are increasingly prescribed for these patients. OBJECTIVE OF THE REVIEW: This systematic review critically evaluates evidence describing the off-label use of fresh frozen plasma (FFP), prothrombin complex concentrate (PCC), complement 1 esterase inhibitor (C1-INH), icatibant, and ecallantide for treatment of ACEI-induced angioedema...
November 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28905884/clinical-utility-gene-card-for-hereditary-angioedema-with-normal-c1-inhibitor-haenc1
#18
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
#19
M A Riedl, H Li, M Cicardi, J R Harper, 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
#20
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
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