Search results for: C1INH
#39357560 
2024/09/30
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Angioedema due to acquired C1-inhibitor deficiency associated with monoclonal gammopathies of undetermined significance.
No specific description of monoclonal gammopathies of undetermined significance (MGUS)-associated angioedema due to acquired C1 inhibitor deficiency (AAE-C1-INH) has been reported yet.Constance Lahuna, Federica Defendi, Laurence Bouillet, Isabelle Boccon-Gibod, Arsene Mekinian, Paul Coppo, Henri Adamski, Stephanie Amarger, Guillaume Armengol, Magali Aubineau, Beatrice Bibes, Claire Blanchard-Delaunay, Gilles Blaison, Benoit Brihaye, Pascal Cathebras, Olivier Caubet, Claire Demoreuil, Julien Desblache, Francois Durupt, Stephane Gayet, Guillaume Gondran, Jerome Hadjadj, Galith Kalmi, Gisele Kanny, Marion Lacoste, David Launay, Kim Heang Ly, Chloé McAvoy, Ludovic Martin, Yann Ollivier, Fabien Pelletier, Aylsa Robbins, Damien Roos-Weil, Olivier Fain, Delphine Gobert
1035 related Products with: Angioedema due to acquired C1-inhibitor deficiency associated with monoclonal gammopathies of undetermined significance.
1mg100.00 ug200 ug100 ul100 ul200ug100 ul200 ug200 ug1 mg100 ug200 ug
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#39309672 2024/09/06
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A real-world study of hereditary angioedema patients due to C1 inhibitor deficiency treated with danazol in the Brazilian Public Health System.
Hereditary angioedema (HAE) due to C1 inhibitor (C1-INH) deficiency is an ultra-rare autosomal dominant inherited disease that affects 1 in 67,000 people in the world. The attacks are based on subcutaneous and submucosal edema that can lead to death if not properly managed. Considering the lack of information on the clinical management of Brazilian patients with HAE, this study aimed to identify and characterize patients with HAE-C1-INH that used danazol prophylactic treatment in the Brazilian Public Health System (SUS) and the healthcare resource utilization (HCRU).Alessandra Mileni Versuti Ritter, Suelen Silva, Robson de Paula, Juliana Senra, Fabio Carvalho, Tatiane Ribeiro, Solange Oliveira Rodrigues Valle
2551 related Products with: A real-world study of hereditary angioedema patients due to C1 inhibitor deficiency treated with danazol in the Brazilian Public Health System.
20 0.1 ml100ug Lyophilized20 200ul100 10 mg100 assays
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#39295925 2024/09/11
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Edema of the Scrotum and Penile Shaft: An Uncommon Initial Presentation of Acquired Angioedema With Low C1-Inhibitor.
Acquired angioedema with low C1-inhibitor (AAE-C1-INH) is a rare disorder characterized by an acquired deficiency in the C1 esterase inhibitor (C1-INH). This case report describes a 79-year-old patient presenting to the emergency department for painless swelling of his scrotum, penile shaft, and left lower and upper extremities with lab values consistent with acquired angioedema without identifiable lymphoreticular or rheumatic disorder on history, exam, or total body PET scan. Proper diagnosis of AAE-C1-INH is essential to prevent life-threatening airway compromise, ensure proper therapy, and exclude lymphoreticular disorders as the etiology of AAE-C1-INH.Meghan V Matheny, Timothy Craig, Joseph Y Clark
2004 related Products with: Edema of the Scrotum and Penile Shaft: An Uncommon Initial Presentation of Acquired Angioedema With Low C1-Inhibitor.
200ul100ug Lyophilized100ug25 mg1000 tests50ul (1mg/ml)100ul100.00 ul100 10 mg
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#39272138 2024/09/13
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Uncovering a novel SERPING1 pathogenic variant: insights into the aggregation of C1-INH in hereditary angioedema.
Hereditary angioedema (HAE) is a rare autosomal dominant genetic disease characterized by recurrent edema and a potentially fatal risk. Despite its severity, there is a notable lack of effective methods for predicting and preventing HAE attacks. This study aims to thoroughly investigate the underlying pathological mechanisms of HAE and identify potential biomarkers that could aid in its prediction and prevention.Lingxi Jiang, Chao Dai, Suyang Duan, Tingting Wang, Chunbao Xie, Luhan Zhang, Zimeng Ye, Xiumei Ma, Yi Shi
1294 related Products with: Uncovering a novel SERPING1 pathogenic variant: insights into the aggregation of C1-INH in hereditary angioedema.
100.00 ug100 assays96 assays 100μg100ug Lyophilized1mg200 20 20 20 mg5 mg
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#39255718 2024/09/03
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Elevated C1s/C1-INH in serum and plasma of myasthenia gravis patients.
Myasthenia Gravis (MG) is an autoimmune neuromuscular disorder where acetylcholine receptor (AChR) antibodies induce membrane attack complex formation at the muscle membrane. The C1-inhibitor (C1-INH) regulates the classical pathway and is a promising marker in other autoimmune disorders. Treatment options for AChR antibody MG include complement inhibitors; nevertheless, the early pathway activation in MG remains unclear. Serum and plasma C1s-C1-INH levels were higher in MG patients than in matched healthy controls, supporting early classical pathway activation in most MG patients. These findings allow prospective validation studies of activated C1s as a putative treatment target and potential accompanying biomarker in MG.Yu-Fang Huang, Caitlin M Briggs, Sankalp Gokhale, Anna Rostedt Punga
1940 related Products with: Elevated C1s/C1-INH in serum and plasma of myasthenia gravis patients.
96T96T1mg96T100 10mg100 100 5 mg1 ml96T1 kit(96 Wells)
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#39224758 2024/08/31
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Hereditary angioedema presented as isolated ascending and transverse colon swelling mimicking acute abdomen.
Hereditary angioedema (HAE) is a rare autosomal dominant condition characterized by C1-INH gene mutations, leading to recurrent angioedema episodes affecting various body parts, including the gastrointestinal tract. This case report describes a 24-year-old female presenting with symptoms mimicking an acute abdomen, characterized by severe abdominal cramps, anorexia, and diarrhea, with a significant past medical history of angioedema flares and emergency intubation for asphyxiation at age 11. Despite initial treatment with antihistamines showing no improvement, her symptoms spontaneously resolved. Further investigation revealed low complement C4 levels and reduced C1-INH function, confirming HAE with an unusual isolated involvement of the ascending and transverse colon. This case underscores the importance of considering HAE in patients presenting with acute abdominal symptoms, especially with a history suggestive of angioedema. It highlights the need for emergency physicians and gastroenterologists to be aware of HAE's clinical manifestations to avoid misdiagnosis and unnecessary interventions. Moreover, the case emphasizes the significance of patient education on recognizing symptoms and seeking timely medical attention to prevent severe complications. This report adds to the existing literature by detailing an uncommon presentation of HAE, aiming to enhance early diagnosis and management of this potentially life-threatening condition.Mohammad Marrawani, Mustafa Alatawneh, Fouad Asafra, Omar H Salloum, Ahmad M Abuayash, Mosab Samamra
1122 related Products with: Hereditary angioedema presented as isolated ascending and transverse colon swelling mimicking acute abdomen.
100μl100ug1 kit100 reactions100tests250 assays1000 assays50 assays
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#39165045 2024/08/20
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Circulating levels of endogenous complement inhibitors correlate inversely with complement consumption in systemic lupus erythematosus.
Systemic lupus erythematosus (SLE) is marked by excessive complement activation, contributing to tissue damage. Complement activation can be detected in many organs including the skin, kidney, and brain. The involvement of the central nervous system is particularly relevant to understanding neuropsychiatric SLE (NPSLE), one of the poorest understood manifestations of SLE for which no biomarkers are available. We studied the levels of complement inhibitors in SLE in relation to disease activity and as possible biomarkers to identify NPSLE. Serum levels of complement inhibitors C1-inhibitor (C1-INH), C4b-binding protein (C4BP), Factor I, and Factor H were measured in 345 SLE patients (including 102 with NPSLE) and 108 healthy controls. Compared with controls, SLE patients had higher C1-INH and C4BP but lower Factor I and H levels. All inhibitors positively correlated with total C3 and C4 levels. While correlating with the SLE Disease Activity Index (SLEDAI), no distinction in inhibitor levels was found between SLE and NPSLE patients. Over time, C1-INH and Factor H levels normalized, but no significant changes were observed for C4BP and Factor I. In SLE the levels of circulating complement inhibitors are inversely correlated to complement consumption but do not serve as biomarkers for NPSLE.Stef van der Meulen, Rory C Monahan, Kyra A Gelderman, Cees van Kooten, Y K Onno Teng, Tom W J Huizinga, Gerda M Steup-Beekman, Leendert A Trouw
2840 related Products with: Circulating levels of endogenous complement inhibitors correlate inversely with complement consumption in systemic lupus erythematosus.
100 μg10ml6 inhibitors25ml1 mL100 ul25ml50ml100ug25ml8 inhibitors
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#39127675 2024/08/10
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A human centred innovative approach based on persona in hereditary angioedema.
Hereditary Angioedema (HAE) due to C1-inhibitor deficiency (C1INH) is a rare condition, clinically characterised by recurrent swelling. The unpredictability of attacks affects the patients' quality of life (QoL). HAE patients and their families have vast unmet physical, psychological, and social needs. A human-centred design (HCD) approach to describing the needs of different user types is to utilise personas, a data-driven narrative tool for communicating user archetypes that capture the individuals' attitudes, goals, and behaviours. The aim of this study was to create and analyse personas based on HAE patients' and their caregivers' interviews. Semi-structured interviews were conducted through anthropological conversations with patients, patient-caregivers (double role of patient and caregiver), and non-affected caregivers. Qualitative and quantitative insights from analyses formed the basis to create personas.Francesca Perego, Lorenza Chiara Zingale, Azzurra Cesoni Marcelli, Luca Ranucci, Lorenzo Rimoldi, Nurgul Nsanbayeva, Maria Rosaria Natale, Laura Adelaide Dalla Vecchia, Alessandra Gorini
1051 related Products with: A human centred innovative approach based on persona in hereditary angioedema.
100 μg100 μg100 μg100 μg20 100 μg100 μg100 μg100 μg100 μg100 μg100 μg
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#39071003 2024/06/28
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Navigating the swells: A case report of hereditary angioedema.
Hereditary angioedema (HAE) is a rare genetic disorder characterized by recurrent episodes of localized edema caused by a deficiency or dysfunction of C1 inhibitor (C1-INH). This case report presents the clinical features, diagnostic evaluation, and management of a 23-year-old man with HAE. We discuss the challenges of diagnosing and treating this condition, emphasizing the importance of early recognition and appropriate therapeutic interventions.Kamlesh Taori, Vijendra Kirnake, Parmeshwar Junare, Vishal Padwale