keyword
MENU ▼
Read by QxMD icon Read
search

Hereditary angioedema

keyword
https://www.readbyqxmd.com/read/28616043/angioedema-suppressed-by-a-combination-of-anti-histamine-and-leukotriene-modifier
#1
Brendan N Wong, Peter Vadas
RATIONALE: Angioedema without co-existent urticaria is due to a limited number of causes, including hereditary and acquired C1 esterase inhibitor deficiency, drug-induced angioedema or idiopathic histaminergic or non-histaminergic angioedema. We describe a cohort of patients with recurrent angioedema whose clinical features and response to medications are distinct from the causes above. METHODS: Patients were accrued retrospectively from an academic allergy practice between 2007 and 2014...
2017: Allergy, Asthma, and Clinical Immunology
https://www.readbyqxmd.com/read/28601681/mutation-of-angiopoietin-1-gene-associates-with-a-new-type-of-hereditary-angioedema
#2
Valeria Bafunno, Davide Firinu, Maria D'Apolito, Giorgia Cordisco, Stefania Loffredo, Angelica Leccese, Maria Bova, Maria Pina Barca, Rosa Santacroce, Marco Cicardi, Stefano Del Giacco, Maurizio Margaglione
BACKGROUND: Hereditary angioedema (HAE) is a rare genetic disease usually due to mutation within the C1 inhibitor or the coagulation Factor XII gene. However, in a series of patients with HAE no causative variants have been described and the pathophysiology of the disease remains unknown (U-HAE). Identification of causative genes in U-HAE is valuable for understanding the cause of the disease. OBJECTIVE: We conducted genetic studies in Italian patients with U-HAE to identify novel causative genes...
June 7, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28601641/treatment-effect-and-safety-of-icatibant-in-pediatric-patients-with-hereditary-angioedema
#3
Henriette Farkas, Avner Reshef, Werner Aberer, Teresa Caballero, Laura McCarthy, James Hao, Wolfram Nothaft, Jennifer Schranz, Jonathan A Bernstein, H Henry Li
BACKGROUND: Clinical manifestations of hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) usually begin in childhood, often intensifying during puberty. Currently there are insufficient efficacy/safety data for HAE therapies in children and adolescents due to the small number of pediatric patients enrolled in studies. OBJECTIVE: The objective of this phase 3 study was to evaluate the efficacy/safety of a single subcutaneous dose of icatibant (0.4 mg/kg; maximum 30 mg) in pediatric patients with C1-INH-HAE...
June 7, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28595743/the-role-of-the-complement-system-in-hereditary-angioedema
#4
Dorottya Csuka, Nóra Veszeli, Lilian Varga, Zoltán Prohászka, Henriette Farkas
Hereditary angioedema (HAE) is a rare, but potentially life-threatening disorder, characterized by acute, recurring, and self-limiting edematous episodes of the face, extremities, trunk, genitals, upper airways, or the gastrointestinal tract. HAE may be caused by the deficiency of C1-inhibitor (C1-INH-HAE) but another type of the disease, hereditary angioedema with normal C1-INH function (nC1-INH-HAE) was also described. The patient population is quite heterogeneous as regards the location, frequency, and severity of edematous attacks, presenting large intra- and inter-individual variation...
June 5, 2017: Molecular Immunology
https://www.readbyqxmd.com/read/28577900/a-novel-prophylaxis-with-c1-inhibitor-concentrate-in-hereditary-angioedema-during-erythema-marginatum
#5
Kinga Viktória Kőhalmi, Nóra Veszeli, László Cervenak, Lilian Varga, Henriette Farkas
BACKGROUND: Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is a rare, autosomal dominant disorder. The characteristic episodes of subcutaneous/submucosal edema formation may be preceded by erythema marginatum (EM) - the occurrence of a 'map-like' pattern on the skin. EM can occur as an isolated finding or accompanying a hereditary angiooedema (HAE) attack as well. Nevertheless, it is unknown whether a HAE attack can be prevented by the proper prophylactic treatment during EM...
May 31, 2017: Immunology Letters
https://www.readbyqxmd.com/read/28552626/hereditary-angioedema-and-graves-disease-the-first-case-report
#6
Ming-Jer Liu, Shyh-Dar Shyur, Hsin-Hui Chuang, Ping-Hsien Yang
No abstract text is available yet for this article.
May 25, 2017: Journal of the Formosan Medical Association, Taiwan Yi Zhi
https://www.readbyqxmd.com/read/28476524/angioedema-attacks-related-to-endometrial-hyperplasia-in-a-case-of-estrogen-dependent-factor-xii-hereditary-angioedema
#7
Alicia Prieto-García, Dasha Roa Medellín, Maria Luisa Baeza
No abstract text is available yet for this article.
June 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/28465053/successful-management-of-hereditary-angioedema-during-pregnancy-in-a-patient-with-heterozygous-mthfr-mutation
#8
Inmaculada Martinez Saguer, Carmen Escuriola Ettingshausen
No abstract text is available yet for this article.
June 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/28453344/pediatric-hereditary-angioedema-as-a-cause-of-acute-compartment-syndrome-of-the-hand-and-forearm-a-case-report
#9
Chelsea Venditto, Zachary Jager, John LoGiudice, Hani Matloub
BACKGROUND: Compartment syndrome of the upper extremity is a surgical emergency that, when left untreated, can have dire consequences. Its causes are numerous, one of which is the uncommon entity hereditary angioedema, an autosomal dominant disease resulting in edema in a variety of potential locations, including the extremities. This is only the second time hereditary angioedema has been mentioned in the literature as a cause of compartment syndrome. METHODS: We present a case of hereditary angioedema leading to hand and forearm compartment syndrome in a 13-year-old pediatric patient...
May 2017: Hand: Official Journal of the American Association for Hand Surgery
https://www.readbyqxmd.com/read/28452302/subcutaneous-administration-of-human-c1-inhibitor-with-recombinant-human-hyaluronidase-in-patients-with-hereditary-angioedema
#10
Marc A Riedl, William R Lumry, H Henry Li, Aleena Banerji, Jonathan A Bernstein, Murat Baş, Janne Björkander, 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 1, 2016: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28441992/safety-of-a-c1-inhibitor-concentrate-in-pregnant-women-with-hereditary-angioedema
#11
James Fox, Arthur B Vegh, Inmaculada Martinez-Saguer, Walter A Wuillemin, Jonathan Edelman, Debora Williams-Herman, Mikhail Rojavin, Tanja Rosenberg
BACKGROUND: Increased estrogen levels during pregnancy can exacerbate hereditary angioedema (HAE), yet disease and treatment ramifications remain poorly studied in pregnant women. OBJECTIVE: Data from the international Berinert Patient Registry were used to evaluate outcomes of pregnancies exposed to plasma-derived, pasteurized, nanofiltered C1-inhibitor concentrate (pnfC1-INH) during routine HAE management. METHODS: This observational registry, conducted between 2010 and 2014 at 30 U...
May 1, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28433622/diminished-capacity-of-opsonization-and-immune-complex-solubilization-and-detection-of-anti-c1q-antibodies-in-sera-from-patients-with-hereditary-angioedema
#12
Daisuke Honda, Isao Ohsawa, Nobuyuki Sato, Hiroyuki Inoshita, Satoshi Mano, Yasuhiko Tomino, Yusuke Suzuki
BACKGROUND: Hereditary angioedema (HAE) is an autosomal dominant disease caused by deficiency of C1 esterase inhibitor. Symptoms of HAE include edema, which can potentially cause suffocation. Some patients with HAE exhibit immunological abnormalities, which could prevent an accurate diagnosis. Low levels of complement components are characteristic of HAE and in other settings are thought to reduce elimination of apoptotic cells and immune complex (IC). Thus, we aimed to experimentally clarify the mechanism of immunological abnormalities using sera from HAE patients...
April 19, 2017: Allergology International: Official Journal of the Japanese Society of Allergology
https://www.readbyqxmd.com/read/28412283/complement-factor-c4-activation-in-patients-with-hereditary-angioedema
#13
Anne Aabom, Anette Bygum, Claus Koch
OBJECTIVES: Low complement factor C4 is usually considered a valuable screening tool for patients with the potentially life-threatening Hereditary Angioedema with C1-inhibitor (C1-INH) deficiency (C1-INH-HAE). However, there are patients with C1-INH-HAE presenting with normal C4 levels. This means, that C1-INH-HAE may potentially be overlooked, if screening is performed only by measurement of C4. It has been suggested that measurement of C4 activation products is better suited to avoid false negative results...
April 12, 2017: Clinical Biochemistry
https://www.readbyqxmd.com/read/28405953/angioedema-in-the-emergency-department-a-practical-guide-to-differential-diagnosis-and-management
#14
REVIEW
Jonathan A Bernstein, Paolo Cremonesi, Thomas K Hoffmann, John Hollingsworth
BACKGROUND: Angioedema is a common presentation in the emergency department (ED). Airway angioedema can be fatal; therefore, prompt diagnosis and correct treatment are vital. OBJECTIVE OF THE REVIEW: Based on the findings of two expert panels attended by international experts in angioedema and emergency medicine, this review aims to provide practical guidance on the diagnosis, differentiation, and management of histamine- and bradykinin-mediated angioedema in the ED...
December 2017: International Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28381322/efficacy-and-safety-of-an-intravenous-c1-inhibitor-concentrate-for-long-term-prophylaxis-in-hereditary-angioedema
#15
Timothy Craig, Ralph Shapiro, Arthur Vegh, James W Baker, Jonathan A Bernstein, Paula Busse, Markus Magerl, Inmaculada Martinez-Saguer, Marc A Riedl, William Lumry, Debora Williams-Herman, Jonathan Edelman, Henrike Feuersenger, Thomas Machnig, Mikhail Rojavin
BACKGROUND: The plasma-derived, pasteurized, nanofiltered C1-inhibitor concentrate (pnfC1-INH) is approved in the United States as an intravenous (IV) on-demand treatment for hereditary angioedema (HAE) attacks, and, in Europe, as on demand and short-term prophylaxis. OBJECTIVE: This analysis evaluated Berinert Patient Registry data regarding IV pnfC1-INH used as long-term prophylaxis (LTP). METHODS: The international registry (2010-2014) collected prospective and retrospective usage, dosing, and safety data on individuals who used pnfC1-INH for any reason...
March 1, 2017: Allergy & Rhinology
https://www.readbyqxmd.com/read/28377172/mimicker-of-hereditary-angioedema-idiopathic-systemic-capillary-leak-syndrome-successfully-treated-with-intravenous-immunoglobulin
#16
Xiao Chloe Wan, Andrew Lai, Tejaswi Kompala, Rosa Ten
No abstract text is available yet for this article.
May 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/28370444/the-icatibant-outcome-survey-experience-of-hereditary-angioedema-management-from-six-european-countries
#17
T Caballero, W Aberer, H J Longhurst, M Maurer, A Zanichelli, A Perrin, L Bouillet, I Andresen
BACKGROUND: Hereditary angioedema (HAE) due to C1-inhibitor deficiency (C1-INH-HAE) is a rare, potentially fatal, bradykinin-mediated disease. Icatibant is a bradykinin B2 receptor antagonist originally approved in 2008 in the European Union and 2011 in the United States as an acute therapy option for HAE attacks in adults. OBJECTIVE: To compare demographics, disease characteristics and treatment outcomes of icatibant-treated HAE attacks in patients with C1-INH-HAE enrolled in the Icatibant Outcome Survey across six European countries: Austria, France, Germany, Italy, Spain and the UK...
April 1, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28346966/factor-xii-contact-activation
#18
Clément Naudin, Elena Burillo, Stefan Blankenberg, Lynn Butler, Thomas Renné
Contact activation is the surface-induced conversion of factor XII (FXII) zymogen to the serine protease FXIIa. Blood-circulating FXII binds to negatively charged surfaces and this contact to surfaces triggers a conformational change in the zymogen inducing autoactivation. Several surfaces that have the capacity for initiating FXII contact activation have been identified, including misfolded protein aggregates, collagen, nucleic acids, and platelet and microbial polyphosphate. Activated FXII initiates the proinflammatory kallikrein-kinin system and the intrinsic coagulation pathway, leading to formation of bradykinin and thrombin, respectively...
March 27, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/28345520/-management-of-patients-with-bradykinin-mediated-angioedema-in-oral-and-maxillofacial-surgery
#19
J Rohart, L Bouillet, H Moizan
Bradykinin-mediated angioedema (AE) is a rare disease characterized by recurrent cutaneous or mucosal angioedema. This hereditary or acquired disease is of rapid installation, non-pruritic, usually painless and can affect the face, lips, larynx, gastrointestinal tract or extremities. When the affected area involves the upper respiratory tract, laryngeal angioedema can lead to imminent death by asphyxia. This is the reason for the high mortality rate (30 %) in undiagnosed or improperly managed patients. High-risk situations in oral and maxillofacial surgery procedures should be identified preoperatively...
February 22, 2017: Journal of Stomatology, Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28328347/prevention-of-hereditary-angioedema-attacks-with-a-subcutaneous-c1-inhibitor
#20
RANDOMIZED CONTROLLED TRIAL
Hilary Longhurst, Marco Cicardi, Timothy Craig, Konrad Bork, Clive Grattan, James Baker, Huamin H Li, Avner Reshef, James Bonner, Jonathan A Bernstein, John Anderson, William R Lumry, Henriette Farkas, Constance H Katelaris, Gordon L Sussman, Joshua Jacobs, Marc Riedl, Michael E Manning, Jacques Hebert, Paul K Keith, Shmuel Kivity, Sergio Neri, Donald S Levy, Maria L Baeza, Robert Nathan, Lawrence B Schwartz, Teresa Caballero, William Yang, Ioana Crisan, María D Hernandez, Iftikhar Hussain, Michael Tarzi, Bruce Ritchie, Pavlina Králíčková, Mar Guilarte, Syed M Rehman, Aleena Banerji, Richard G Gower, Debra Bensen-Kennedy, Jonathan Edelman, Henrike Feuersenger, John-Philip Lawo, Thomas Machnig, Dipti Pawaskar, Ingo Pragst, Bruce L Zuraw
BACKGROUND: Hereditary angioedema is a disabling, potentially fatal condition caused by deficiency (type I) or dysfunction (type II) of the C1 inhibitor protein. In a phase 2 trial, the use of CSL830, a nanofiltered C1 inhibitor preparation that is suitable for subcutaneous injection, resulted in functional levels of C1 inhibitor activity that would be expected to provide effective prophylaxis of attacks. METHODS: We conducted an international, prospective, multicenter, randomized, double-blind, placebo-controlled, dose-ranging, phase 3 trial to evaluate the efficacy and safety of self-administered subcutaneous CSL830 in patients with type I or type II hereditary angioedema who had had four or more attacks in a consecutive 2-month period within 3 months before screening...
March 23, 2017: New England Journal of Medicine
keyword
keyword
5755
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"