keyword
MENU ▼
Read by QxMD icon Read
search

Hereditary angioedema

keyword
https://www.readbyqxmd.com/read/28476524/angioedema-attacks-related-to-endometrial-hyperplasia-in-a-case-of-estrogen-dependent-factor-xii-hereditary-angioedema
#1
Alicia Prieto-García, Dasha Roa Medellín, Maria Luisa Baeza
No abstract text is available yet for this article.
May 2, 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
#2
Inmaculada Martinez Saguer, Carmen Escuriola Ettingshausen
No abstract text is available yet for this article.
April 29, 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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
T Caballero, W Aberer, H J Longhurst, M Maurer, A Zanichelli, A Perrin, L Bouillet, I Andresen
BACKGROUND: Hereditary angioedema (HAE) due to C1 INH 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 United Kingdom...
April 1, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28346966/factor-xii-contact-activation
#12
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
#13
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
#14
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
https://www.readbyqxmd.com/read/28302171/clinical-characteristics-and-real-life-diagnostic-approaches-in-all-danish-children-with-hereditary-angioedema
#15
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
#16
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...
May 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28291095/angioedema
#17
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
#18
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
#19
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 IU/kg of rhC1INH...
April 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
#20
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
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"