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Hereditary angioedema

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https://www.readbyqxmd.com/read/28069032/glucocorticoid-receptor-gene-polymorphisms-in-hereditary-angioedema-with-c1-inhibitor-deficiency
#1
Zsuzsanna Zotter, Zsolt Nagy, Attila Patócs, Dorottya Csuka, Nóra Veszeli, Kinga Viktória Kőhalmi, Henriette Farkas
BACKGROUND: Hereditary angioedema caused by C1-inhibitor deficiency (C1-INH-HAE) is a rare, autosomal dominant disorder. C1-INH-HAE is characterized by edema-formation, which may occur in response to stress. The individual's response to stress stimuli is partly genetically determined. Activation of the hypothalamic-pituitary-adrenal axis results in the release of cortisol. In turn, the secreted gluco- and mineralocorticoids affect the metabolism, as well as the cardiovascular and immune systems...
January 10, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28051822/novel-usage-of-fresh-frozen-plasma-in-hereditary-angioedema
#2
N Hanizah, C A Affirul, N A Farah, M A Shamila, M I Ridzuan
Hereditary angioedema (HAE) is a rare and potentially life threatening autosomal dominant disease characterized by recurrent episodes of cutaneous and mucosal oedema. It results from reduced expression or loss of function of CI-esterase inhibitors (C1-INH). As opposed to the more common histamine-mediated angioedema, HAE does not respond well to conventional treatments with anti-histamines, steroids and adrenaline. Early recognition and timely intervention with the correct treatment are crucial particularly preventing airway obstruction...
November 2016: La Clinica Terapeutica
https://www.readbyqxmd.com/read/28045547/peptide-macrocycle-inhibitor-of-coagulation-factor-xii-with-subnanomolar-affinity-and-high-target-selectivity
#3
Simon Jan Middendorp, Jonas Wilbs, Claudia Quarroz, Sara Calzavarini, Anne Angelillo-Scherrer, Christian Heinis
Factor XII (FXII) is a plasma protease that has emerged in recent years as a potential target to treat or prevent pathological thrombosis, to inhibit contact activation in extracorporeal circulation, and to treat the swelling disorder hereditary angioedema. While several protein based inhibitors with high affinity for activated FXII (FXIIa) were developed, the generation of small molecule inhibitors has been challenging. In this work, we have generated a potent and selective FXIIa inhibitor by optimizing a peptide macrocycle that was recently evolved by phage display (Ki = 0...
January 3, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28042430/experimental-protocol-of-dental-procedures-in-patients-with-hereditary-angioedema-the-role-of-anxiety-and-the-use-of-nitrogen-oxide
#4
A Rosa, M Miranda, R Franco, M G Guarino, A Barlattani, P Bollero
Hereditary angioedema (HAE) is a rare disease, little known to the medical and dental community, but with a growing rate of hospitalization over the years. HAE is due to a deficit/dysfunction of C1 esterase inhibitor which leads to an increase in vascular permeability and the appearance of edemas widespread in all body areas. The airways are the most affected and laryngeal swelling, which can occur, it is dangerous for the patient's life, is also a sensitive spot in our daily practice, therefore, it is also important to be aware of all the signs of this disease...
April 2016: Oral & Implantology
https://www.readbyqxmd.com/read/28025012/catabolism-of-c1-inhibitor-influences-the-response-to-replacement-therapy-in-hereditary-angioedema
#5
Marco Cicardi, Andrea Zanichelli, Chiara Suffritti, Maddalena A Wu, Thomas Machnig, Annalisa De Silvestri, Mario Regazzi, Carmine Tinelli
Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a disabling and life-threatening disease for which plasma-derived C1 inhibitor (pdC1-INH) is an effective treatment. Poor responses to pdC1-INH are rare. The aim of this prospective study was to evaluate the pharmacokinetics and pharmacodynamics of C1 inhibitor (C1-INH) in a C1-INH-HAE patient with poor response to treatment to investigate the mechanism underlying poor response to pdC1-INH. Seventeen C1-INH-HAE patients with normal responses to treatment served as retrospective controls...
December 23, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28007084/manifestations-of-hereditary-angioedema
#6
Alessandro Testori, Isaac Melamed
No abstract text is available yet for this article.
January 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/27996949/triggers-and-prodromal-symptoms-of-angioedema-attacks-in-patients-with-hereditary-angioedema
#7
T Caballero, M Maurer, H J Longhurst, W Aberer, L Bouillet, V Fabien
No abstract text is available yet for this article.
2016: Journal of Investigational Allergology & Clinical Immunology
https://www.readbyqxmd.com/read/27965672/hereditary-angioedema-as-a-metabolic-liver-disorder-novel-therapeutic-options-and-prospects-for-cure
#8
Rohan Ameratunga, Adam Bartlett, John McCall, Richard Steele, See-Tarn Woon, Constance H Katelaris
Hereditary angioedema (HAE) is a rare autosomal dominant disorder caused by mutations of the SERPING1 or the Factor 12 genes. It is potentially fatal, particularly if not identified at an early stage. Apart from androgens, which are contraindicated in children and in pregnant women, a range of effective, albeit very expensive treatments have recently become available for HAE patients. The cost of these new treatments is beyond the reach of most developing countries. At this time, there is no cure for the disorder...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27940765/management-of-children-with-hereditary-angioedema-due-to-c1-inhibitor-deficiency
#9
Michael M Frank, Bruce Zuraw, Aleena Banerji, Jonathan A Bernstein, Timothy Craig, Paula Busse, Sandra Christiansen, Marc Davis-Lorton, H Henry Li, William R Lumry, Marc Riedl
Hereditary angioedema (HAE) is a potentially life-threatening inherited disease characterized by attacks of skin swelling, severe abdominal pain, and upper airway swelling. Attacks typically begin in childhood, but the appropriate diagnosis is often missed. Attacks do not respond to epinephrine, antihistamines, or glucocorticoids. Recently, many effective drugs have been approved for treatment of adults with HAE, and the Medical Advisory Board of the HAE Patient's Association has developed and reported treatment recommendations for adults...
November 2016: Pediatrics
https://www.readbyqxmd.com/read/27936514/comparing-acquired-angioedema-with-hereditary-angioedema-type-i-ii-findings-from-the-icatibant-outcome-survey
#10
Hilary J Longhurst, Andrea Zanichelli, Teresa Caballero, Laurence Bouillet, Werner Aberer, Marcus Maurer, Olivier Fain, Vincent Fabien, Irmgard Andresen
Icatibant is approved for hereditary angioedema with C1 inhibitor deficiency type I/II (C1-INH-HAE type I/II) and has shown promise in treatment of angioedema due to acquired C1 inhibitor deficiency (C1-INH-AAE). Data from the Icatibant Outcome Survey was analyzed to evaluate effectiveness of icatibant in the treatment of patients with C1-INH-AAE and compare disease characteristics with those with C1-INH-HAE type I/II. Key medical history (including prior occurrence of attacks) was recorded upon IOS enrollment...
December 9, 2016: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27931305/subcutaneous-administration-of-human-c1-inhibitor-with-recombinant-human-hyaluronidase-in-patients-with-hereditary-angioedema
#11
Marc A Riedl, William R Lumry, H Henry Li, Aleena Banerji, Jonathan A Bernstein, Murat Ba, Janne Bjrkander, 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 2016: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/27913306/effect-of-bradykinin-receptor-antagonism-on-ace-inhibitor-associated-angioedema
#12
Brittany T Straka, Claudia E Ramirez, James B Byrd, Elizabeth Stone, Alencia Woodard-Grice, Hui Nian, Chang Yu, Aleena Banerji, Nancy J Brown
BACKGROUND: The B2 receptor antagonist icatibant is approved for treatment of attacks of hereditary angioedema. Icatibant has been reported to decrease time-to-resolution of angiotensin-converting enzyme (ACE) inhibitor-associated angioedema in 1 study of European patients. OBJECTIVE: We sought to test the hypothesis that a bradykinin B2 receptor antagonist would shorten time-to-resolution from ACE inhibitor-associated angioedema. METHODS: Patients with ACE inhibitor-associated angioedema (defined as swelling of lips, tongue, pharynx, or face during ACE inhibitor use and no swelling in the absence of ACE inhibitor use) were enrolled at Vanderbilt University Medical Center from October 2007 through September 2015 and at Massachusetts General Hospital in 2012...
November 29, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27905115/treatment-for-hereditary-angioedema-with-normal-c1-inh-and-specific-mutations-in-the-f12-gene-hae-fxii
#13
K Bork, K Wulff, G Witzke, J Hardt
Hereditary angioedema with normal C1 esterase inhibitor and mutations in the F12 gene (HAE-FXII) is associated with skin swellings, abdominal pain attacks, and the risk of asphyxiation due to upper airway obstruction. It occurs nearly exclusively in women. We report our experience treating HAE-FXII with discontinuation of potential trigger factors and drug therapies. The study included 72 patients with HAE-FXII. Potential triggers included estrogen-containing oral contraceptives (eOC), hormonal replacement therapy, or angiotensin-converting enzyme inhibitors...
November 6, 2016: Allergy
https://www.readbyqxmd.com/read/27878882/tamoxifen-may-cause-life-threatening-angioedema-attacks-in-patients-with-hereditary-angioedema
#14
LETTER
K Bork, K Wulff, G Witzke, S Rietz, J Hardt
No abstract text is available yet for this article.
November 23, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27873761/hereditary-angioedema-hae-a-cause-for-recurrent-abdominal-pain
#15
Parita Soni, Vivek Kumar, Samson Alliu, Vijay Shetty
A 44-year-old Hispanic woman presented to the emergency room with a 2-day history of sudden onset of severe cramping left lower quadrant abdominal pain associated with ∼20 episodes diarrhoea. Abdominal CT scan exhibited bowel wall oedema and acute extensive colitis. On the basis of the preliminary diagnosis of acute abdomen, the patient was admitted under the surgical team and treated for acute colitis. Since her family history was significant for hereditary angioedema (HAE), complement studies were performed which revealed low complement C4 levels and abnormally low values of C1q esterase inhibitor...
November 14, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27871580/c1-esterase-inhibitor-for-short-term-prophylaxis-in-a-patient-with-hereditary-angioedema-with-normal-c1-inhibitor-function
#16
Savio K H Yu, Jeannie Callum, Asim Alam
Hereditary angioedema with normal C1-esterase inhibitor (HAE-nC1INH) perioperative is a rare condition which could have potential disastrous ramifications for the anesthesiologist in the perioperative period. However, there is limited evidence and/or guidelines on the optimal way to manage these patients. We present the case of a patient with HAE-nC1INH who was successfully managed in the perioperative period with plasma derived C1-esterase inhibitor (pdC1INH). A 29-year-old woman with a diagnosis of HAE-nC1INH presented to the preoperative consultation in preparation for an upcoming total thyroidectomy...
December 2016: Journal of Clinical Anesthesia
https://www.readbyqxmd.com/read/27865714/short-term-prophylactic-use-of-c1-inhibitor-concentrate-in-hereditary-angioedema-findings-from-an-international-patient-registry
#17
Markus Magerl, Michael Frank, William Lumry, Jonathan Bernstein, Paula Busse, Timothy Craig, Inmaculada Martinez-Saguer, Marc A Riedl, Ralph Shapiro, Jonathan Edelman, Debora Williams-Herman, Daniel N Wood, Henrike Feuersenger, Mikhail Rojavin
No abstract text is available yet for this article.
January 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/27864873/hereditary-haemorrhagic-telangiectasia-to-transplant-or-not-to-transplant-is-there-a-right-time-for-liver-transplantation
#18
Yannick D Muller, Roland Oppliger, Romain Breguet, Philippe Meyer, Laura Rubbia-Brandt, Pierre-Auguste Petignat, Thomas Harr, Eric Dayer, Jörg D Seebach
BACKGROUND & AIMS: Hereditary haemorrhagic telangiectasia is characterized by arterio-venous malformations (AVM). It frequently involves the liver without clinical symptoms, but may lead to biliary ischaemia, portal hypertension, or fatal high-output heart failure. The indication of liver transplantation is controversial. METHODS: Herein, we report the case of a 65-year-old female patient with a 'double Osler syndrome' consisting of hereditary haemorrhagic telangiectasia (HHT) and type I hereditary angioedema diagnosed at the age of 25 and 22 years respectively...
December 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27864871/hereditary-haemorrhagic-telangiectasia-to-transplant-or-not-to-transplant
#19
Carlo Sabbà
The Association of hereditary hemorrhagic telangiectasia (HHT) and type I hereditary angioedema is a very rare condition in medicine. The case reported by Muller et al., describes the coexistence of the two diseases and emphasizes the need for listing HHT patients for liver transplantation in case indications occurs, such as the presence of HCC, abnormally increased cardiac output, and gastrointestinal bleeding as reported for this case. The case described by Mueller et al. is anecdotal for the usefulness of liver transplantation in HHT patients and shows that liver transplantation may be the best supportive care and, sometimes, the unique and final therapeutic option for these patients...
December 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27837046/c1-esterase-inhibitor-berinert-for-ace-inhibitor-induced-angioedema-two-case-reports
#20
Maria Leibfried, Alexandra Kovary
OBJECTIVE: To describe 2 cases of angiotensin-converting enzyme inhibitor (ACEI)-induced angioedema treated with C1 esterase inhibitor (human) [Berinert]. SUMMARY: Case 1 is a 60-year-old Caucasian male with angioedema from lisinopril. He was initially treated with a conventional regimen of an antihistamine, methylprednisolone, epinephrine, and fresh frozen plasma. When symptoms did not resolve, intravenous C1 peptide esterase inhibitor (C1INH) was administered, with clinical improvement...
November 11, 2016: Journal of Pharmacy Practice
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