A5074941284- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Autoimmune Bullous Skin Diseases
- Vitamin K Research Studies
- Hemophilia Treatment and Research
- Mast cells and histamine
- Urticaria and Related Conditions
- Peptidase Inhibition and Analysis
- Immunodeficiency and Autoimmune Disorders
- PI3K/AKT/mTOR signaling in cancer
- HIV/AIDS drug development and treatment
- HIV Research and Treatment
- Chronic Myeloid Leukemia Treatments
- Pneumocystis jirovecii pneumonia detection and treatment
- Traditional Chinese Medicine Studies
- Glycogen Storage Diseases and Myoclonus
- Dermatological and COVID-19 studies
- Ophthalmology and Eye Disorders
- Orbital Angular Momentum in Optics
- Birth, Development, and Health
- Enzyme function and inhibition
- Ocular Diseases and Behçet’s Syndrome
- Herpesvirus Infections and Treatments
- Cytomegalovirus and herpesvirus research
- Cardiovascular Function and Risk Factors
- Cardiovascular Health and Risk Factors
University of California, Irvine
2009-2024
UC Irvine Health
2019-2021
University of California System
2020
Parkland Memorial Hospital
2020
American College of Physicians
2020
American College of Allergy, Asthma and Immunology
2020
American Academy of Allergy, Asthma and Immunology
2020
Einstein Medical Center Philadelphia
2020
CHOC Foundation
2019
University of California, Irvine Medical Center
2018
Hereditary angioedema is a disabling, potentially fatal condition caused by deficiency (type I) or dysfunction II) of the C1 inhibitor protein. In phase 2 trial, use CSL830, nanofiltered preparation that suitable for subcutaneous injection, resulted in functional levels activity would be expected to provide effective prophylaxis attacks.We conducted an international, prospective, multicenter, randomized, double-blind, placebo-controlled, dose-ranging, 3 trial evaluate efficacy and safety...
Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) impairs health-related quality of life (HRQoL).The objective this study was to assess HRQoL outcomes in patients self-administering subcutaneous C1-INH (C1-INH[SC]; HAEGARDA) for routine prevention HAE attacks.Post hoc analysis data from the placebo-controlled, crossover phase III COMPACT (Clinical Studies Optimal Management Preventing Angioedema Low-Volume Subcutaneous C1-Inhibitor Replacement Therapy). Ninety C1-INH-HAE were...
<h3>Background</h3> For the prevention of attacks hereditary angioedema (HAE), efficacy and safety subcutaneous human C1-esterase inhibitor (C1-INH[SC]; HAEGARDA, CSL Behring) was established in 16-week Clinical Study for Optimal Management Preventing Angioedema with Low-Volume Subcutaneous C1-Inhibitor Replacement Therapy (COMPACT). <h3>Objective</h3> To assess long-term safety, occurrence attacks, use rescue medication C1-INH(SC). <h3>Methods</h3> Open-label, randomized, parallel-arm...
Abstract Background Long-term prophylaxis with subcutaneous C1-inhibitor (C1-INH[SC]; HAEGARDA, CSL Behring) in patients hereditary angioedema (HAE) due to C1-INH deficiency (C1-INH-HAE) was evaluated an open-label extension follow-up study the international, double-blind, placebo-controlled COMPACT study. The current analysis patient-reported health-related quality of life (HRQoL) data from 126 randomized treatment C1-INH(SC) 40 IU/kg (n = 63) or 60 twice weekly for 52 weeks. HRQoL at...
Case Reports1 January 1948WEBER-CHRISTIAN'S DISEASE: REPORT OF A CASEIVAN L. BUNNELL, M.D., DEXTER S. LEVY, M.D.IVAN M.D.Search for more papers by this author, authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-28-1-169 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptWeber-Christian's disease, or relapsing febrile nodular, non-suppurative panniculitis, is a disease of unknown...
Abstract Background Hereditary angioedema (HAE) is caused by a SERPING1 gene defect resulting in decreased (Type I) or dysfunctional II) C1 esterase inhibitor (C1-INH). The prevalence of autoimmune diseases (ADs) patients with HAE appears to be higher than the general population. A systematic literature review was conducted examine co-occurrence between and ADs. Methods PubMed/EMBASE were searched for English-language reviews, case reports, observational studies, retrospective randomized...
Background: Hereditary angioedema (HAE) is a rare condition marked by swelling episodes in various body parts, including the extremities, upper airway, face, intestinal tract, and genitals. Long-term prophylaxis (LTP), prescribed to control recurring HAE attacks, integral its management. Previously, attenuated androgens (AAs) were only oral LTP options. However, 2020, berotralstat, an plasma kallikrein inhibitor, was approved United States. A 2018 survey of adults with type I or II showed...
Background: Hereditary angioedema (HAE) due to C1 inhibitor (C1INH) deficiency is characterized by recurrent attacks of edema the skin and mucosal tissues. Symptoms usually present during childhood (mean age at first attack, 10 years). Earlier symptom onset may predict a more severe disease course. Subcutaneous (SC) C1INH indicated for routine prophylaxis prevent HAE in adolescents adults. We analyzed long-term efficacy subjects ≤17 years old treated an open-label extension (OLE) pivotal...
Abstract Background Women with hereditary angioedema due to C1-inhibitor deficiency (HAE-C1INH) experience more frequent and severe attacks compared men. Fluctuations in female sex hormones can influence HAE attack frequency severity. Subcutaneous C1-INH (C1-INH [SC]) is indicated as routine prophylaxis prevent attacks. In this post hoc subgroup analysis, we evaluated the efficacy safety of (SC) subjects HAE-C1INH enrolled an open-label extension pivotal phase III COMPACT trial. Methods...
Abstract Background While many studies of effective hereditary angioedema (HAE) therapy have demonstrated improved health-related quality life (HRQoL) using validated instruments, specific reasons behind the scores never been investigated qualitative methods. A non-interventional, research study was designed to investigate for improvements in HRQoL while prophylaxis, this case subcutaneous C1INH (C1INH[SC]) replacement therapy. Methods Adult patients with HAE-C1INH type 1 or 2 who had...
Hereditary angioedema (HAE) is a debilitating disorder resulting from C1-esterase inhibitor (C1-INH) deficiency. In the COMPACT phase 3 study prophylactic use of subcutaneous C1 (C1-INH [SC], HAEGARDA®, CSL Behring) twice weekly significantly reduced frequency acute edema attacks. Analysis treatment effects by subgroups, onset effect, and other exploratory analysis have not been reported. This post hoc on data randomized, placebo-controlled study. 90 patients with C1-INH-HAE were randomized...
Abstract Adrenergic urticaria (AU) is a rare type of physical triggered by stress. It frequently confused with IgE‐mediated or other urticarias. This report describes case localized adrenergic sweat chloride test in an adolescent male multiple atopic disorders. A pruritic papular rash at the site prompted evaluation for allergic and urticarias using skin methods. positive intradermal to noradrenaline, which reproduced observed during test, lead diagnosis urticaria. first describing immediate...
In this letter to the editor, we present results of claims data analysis. This analysis supports a hypothesis that in patients with hereditary angioedema due C1-esterase inhibitor (C1-INH) deficiency, occurrence and/or symptomatology coexisting autoimmune disease may be positively influenced by replacement therapy plasma derived C1-INH.
Hereditary angioedema (HAE) is a debilitating condition caused by functional C1-inhibitor (C1-INH) deficiency and characterized clinically episodes of subcutaneous or submucosal swelling. C1-INH replacement highly effective for preventing HAE attacks can improve health-related quality life. Once available only intravenous use, now as formulation self-administration, shown to provide sustained plasma levels reducing the monthly median attack rate 95% versus placebo in phase 3 COMPACT study....
"The Effect of Topical Hydrocortisone Acetate Ointment at the Site Intracutaneous Tuberculin Reaction." American Review Tuberculosis and Pulmonary Diseases, 79(5), pp. 666–668