المؤلفون: Roman Nowicki, Elżbieta Grubska-Suchanek, Grzegorz Porębski, Marek L. Kowalski, Karina Jahnz-Różyk, Tomasz Matuszewski, Lidia Rudnicka, Marek Kulus, Wioletta Barańska-Rybak, Rafał Czajkowski, Zbigniew Doniec, Cezary Kowalewski, Beata Kręcisz, Magdalena Lange, Joanna Narbutt, Małgorzata Olszewska, Małgorzata Sokołowska-Wojdyło, Aneta Szczerkowska-Dobosz, Radosław Śpiewak, Magdalena Trzeciak, Aleksandra Wilkowska

المصدر: Przegląd Dermatologiczny, Vol 107, Iss 4, Pp 293-307 (2020)

مصطلحات موضوعية: angioedema, icatibant, ecallantide, rituximab, berinert, ruconest, firazyr, landelumab, Medicine, Dermatology, RL1-803

الوصف: Angioedema is a non-inflammatory edema of the subcutaneous tissue and/or mucosal membranes. It commonly coexists with urticaria and is considered a deep form of urticaria. Less commonly, it occurs as an isolated disease in one of two basic forms: acquired angioedema and hereditary angioedema. Four types of acquired angioedema and seven types of hereditary angioedema were difinied. The treatment of angioedema depends on its form and etiological factors, with hereditary angioedema posing a most significant challenge.

وصف الملف: electronic resource

العلاقة: https://www.termedia.pl/Angioedema-Interdisciplinary-diagnostic-and-therapeutic-recommendations-of-the-Polish-Dermatological-Society-PTD-and-Polish-Society-of-Allergology-PTA-,56,42040,1,1.htmlTest; https://doaj.org/toc/0033-2526Test; https://doaj.org/toc/2084-9893Test

الوصول الحر: https://doaj.org/article/bd5150961a7f4f229e027dfc1e81e80eTest

المؤلفون: Zuraw, BL, Cicardi, M, Longhurst, HJ, Bernstein, JA, Li, HH, Magerl, M, Martinez-Saguer, I, Rehman, SMM, Staubach, P, Feuersenger, H, Parasrampuria, R, Sidhu, J, Edelman, J, Craig, T

المصدر: Allergy. 70(10)

مصطلحات موضوعية: Biomedical and Clinical Sciences, Clinical Sciences, Clinical Research, Clinical Trials and Supportive Activities, Evaluation of treatments and therapeutic interventions, 6.1 Pharmaceuticals, Adult, Angioedemas, Hereditary, Complement C1 Inhibitor Protein, Drug Administration Schedule, Female, Humans, Injections, Subcutaneous, Male, Treatment Outcome, Young Adult, Berinert, C1-esterase inhibitor, hereditary angioedema, long-term prophylaxis, subcutaneous treatment, Immunology, Allergy

الوصف: BackgroundHereditary angioedema (HAE) due to C1 inhibitor deficiency manifests as recurrent swelling attacks that can be disabling and sometimes fatal. Long-term prophylaxis with twice-weekly intravenous injections of plasma-derived C1-inhibitor (pdC1-INH) has been established as an effective treatment. Subcutaneous (SC) administration of pdC1-INH has not been studied in patients with HAE.MethodsThis open-label, dose-ranging, crossover study (COMPACT Phase II) was conducted in 18 patients with type I or II HAE who received two of twice-weekly 1500, 3000, or 6000 IU SC doses of highly concentrated volume-reduced CSL830 for 4 weeks each. The mean trough plasma levels of C1-INH functional activity, C1-INH and C4 antigen levels during Week 4, and overall safety and tolerability were evaluated. The primary outcome was model-derived steady-state trough C1-INH functional activity.ResultsAfter SC CSL830 administration, a dose-dependent increase in trough functional C1-INH activity was observed. C1-INH and C4 levels both increased. The two highest dose groups (3000 and 6000 IU) achieved constant C1-INH activity levels above 40% values, a threshold that was assumed to provide clinical protection against angioedema attacks. Compared with intravenous injection, pdC1-INH SC injection with CSL830 showed a lower peak-to-trough ratio and more consistent exposures. All doses were well tolerated. Mild-to-moderate local site reactions were noted with pain and swelling being the most common adverse event.ConclusionsSubcutaneous volume-reduced CSL830 was well tolerated and led to a dose-dependent increase in physiologically relevant functional C1-INH plasma levels. A clinical outcome study of SC CSL830 in patients with HAE warrants further investigation.

وصف الملف: application/pdf

الوصول الحر: https://escholarship.org/uc/item/7740p1ssTest

المؤلفون: Ekaterina Zubareva, Maksim Degterev, Alexander Kazarov, Maria Zhiliaeva, Ksenia Ulyanova, Vladimir Simonov, Ivan Lyagoskin, Maksim Smolov, Madina Iskakova, Anna Azarova, Rahim Shukurov

المصدر: Pharmaceuticals; Volume 14; Issue 11; Pages: 1180

مصطلحات موضوعية: rhC1INH, C1 inhibitor, Berinert ®, Ruconest ®, analytical methods, characterization, CHO cells, hereditary angioedema (HAE), acquired angioedema (AAE)

الوصف: The disfunction or deficiency of the C1 esterase inhibitor (C1INH) is associated with hereditary or acquired angioedema (HAE/AAE), a rare life-threatening condition characterized by swelling in the skin, respiratory and gastrointestinal tracts. The current treatment options may carry the risks of either viral infection (plasma-derived Berinert®) or immune reaction (human recombinant C1INH from rabbit milk, Ruconest®). This study describes the physicochemical and biological characterization of a novel recombinant human C1 esterase inhibitor (rhC1INH) from Chinese hamster ovary (CHO) cells for the treatment of hereditary angioedema compared to the marketed products Berinert® and Ruconest®. The mass spectrometry results of total deglycosylated rhC1INH revealed a protein with a molecular mass of 52,846 Da. Almost full sequence coverage (98.6%) by nanoLC-MS/MS peptide mapping was achieved. The purity and C1s inhibitory activity of rhC1INH from CHO cells are comparable with Ruconest®, although we found differences in charge isoforms distribution, intact mass values, and N-glycans profile. Comparison of the specific activity (IC50 value) of the rhC1INH with human C1 esterase inhibitor from blood serum showed similar inhibitory properties. These data allow us to conclude that the novel rhC1INH molecule could become a potential therapeutic option for patients with HAE/AAE.

وصف الملف: application/pdf

العلاقة: Biopharmaceuticals; https://dx.doi.org/10.3390/ph14111180Test

الإتاحة: https://doi.org/10.3390/ph14111180Test

المؤلفون: Masahiro Morimoto, Noritaka Ohga, Ken-ichiro Sakata, Takuya Asaka, Kazuhiro Matsushita, Jun Sato, Akira Satoh, Yoshimasa Kitagawa

المصدر: Oral and Maxillofacial Surgery Cases, Vol 6, Iss 2, Pp - (2020)

مصطلحات موضوعية: Hereditary angioedema, C1–INH, Berinert P, Tracheotomy, Surgery, RD1-811

الوصف: We report a clinical case of a patient with hereditary angioedema (HAE, Type III) in which, by implementing careful preoperative preparations to prevent the occurrence of edema, good course was achieved without the prophylactic administration of C1-inhibitor (C1–INH). We recommended the prophylactic administration of Berinert P, a human plasma-derived C1–INH formulation; however, due to the risk of developing a viral infection, both the patient and her mother refused to receive Berinert P. In cases where C1–INH cannot be administered as a prophylactic measure, it is essential to make sufficient preparations, including urgent tracheotomy, to prevent sudden occurrence of edema, with the cooperation of the anesthesiologist.

وصف الملف: electronic resource

العلاقة: http://www.sciencedirect.com/science/article/pii/S2214541920300122Test; https://doaj.org/toc/2214-5419Test

الوصول الحر: https://doaj.org/article/83d0b6ff44f64a95a477de85a1975837Test

المؤلفون: Beata Krecisz, Cezary Kowalewski, Marek L. Kowalski, Grzegorz Porebski, Magdalena Lange, Elżbieta Grubska-Suchanek, Lidia Rudnicka, Aneta Szczerkowska-Dobosz, Aleksandra Wilkowska, Radosław Śpiewak, Zbigniew Doniec, Małgorzata Sokołowska-Wojdyło, Rafał Czajkowski, Tomasz Matuszewski, Roman Nowicki, Malgorzata Olszewska, Karina Jahnz-Różyk, Wioletta Barańska-Rybak, Marek Kulus, Magdalena Trzeciak, Joanna Narbutt

المصدر: Przegląd Dermatologiczny, Vol 107, Iss 4, Pp 293-307 (2020)
Advances in Dermatology and Allergology/Postȩpy Dermatologii i Alergologii
Advances in Dermatology and Allergology, Vol 37, Iss 4, Pp 445-451 (2020)

مصطلحات موضوعية: Allergy, medicine.medical_specialty, berinert, landelumab, Acquired angioedema, Lanadelumab, Dermatology, Berinert, ecallantide, 030207 dermatology & venereal diseases, 03 medical and health sciences, chemistry.chemical_compound, Ecallantide, Special Article, 0302 clinical medicine, rituximab, Icatibant, immune system diseases, firazyr, medicine, Immunology and Allergy, cardiovascular diseases, skin and connective tissue diseases, Internal medicine, General Environmental Science, Angioedema, business.industry, angioedema, icatibant, food and beverages, medicine.disease, RC31-1245, chemistry, RL1-803, Hereditary angioedema, Etiology, General Earth and Planetary Sciences, Medicine, Rituximab, Ruconest, Allergists, medicine.symptom, business, Firazyr, medicine.drug, ruconest

الوصف: Angioedema is a non-inflammatory oedema of the subcutaneous tissue and/or mucosal membranes. It most commonly coexists with urticaria wheals and is considered to be a deep form of urticaria. Less commonly, it occurs in isolation and can take two basic forms: acquired angioedema and hereditary angioedema. Currently, there are 4 defined types of acquired angioedema and 7 types of hereditary angioedema. Treatment of angioedema depends on its form and etiological factors. Especially the genetic form, i.e. hereditary angioedema, is a considerable challenge for medical specialists, particularly dermatologists and allergists.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1931591a6f1546eb842f3bde385017f9Test
https://www.termedia.pl/Angioedema-Interdisciplinary-diagnostic-and-therapeutic-recommendations-of-the-Polish-Dermatological-Society-PTD-and-Polish-Society-of-Allergology-PTA-,56,42040,1,1.htmlTest

المؤلفون: E. V. Zubareva, Ivan Lyagoskin, Maria Zhiliaeva, Rahim Shukurov, Maksim Degterev, Madina Iskakova, Ksenia Ulyanova, Maksim Smolov, Vladimir Simonov, Alexander Kazarov, Anna Azarova

المصدر: Pharmaceuticals
Volume 14
Issue 11
Pharmaceuticals, Vol 14, Iss 1180, p 1180 (2021)

مصطلحات موضوعية: Ruconest®, Pharmaceutical Science, Berinert®, Article, rhC1INH, C1-inhibitor, law.invention, Blood serum, Pharmacy and materia medica, law, Drug Discovery, medicine, characterization, IC50, C1 inhibitor, biology, Molecular mass, acquired angioedema (AAE), Chemistry, Chinese hamster ovary cell, CHO cells, medicine.disease, Molecular biology, RS1-441, analytical methods, Hereditary angioedema, hereditary angioedema (HAE), biology.protein, Recombinant DNA, Molecular Medicine, Medicine, Specific activity

الوصف: The disfunction or deficiency of the C1 esterase inhibitor (C1INH) is associated with hereditary or acquired angioedema (HAE/AAE), a rare life-threatening condition characterized by swelling in the skin, respiratory and gastrointestinal tracts. The current treatment options may carry the risks of either viral infection (plasma-derived Berinert®) or immune reaction (human recombinant C1INH from rabbit milk, Ruconest®). This study describes the physicochemical and biological characterization of a novel recombinant human C1 esterase inhibitor (rhC1INH) from Chinese hamster ovary (CHO) cells for the treatment of hereditary angioedema compared to the marketed products Berinert® and Ruconest®. The mass spectrometry results of total deglycosylated rhC1INH revealed a protein with a molecular mass of 52,846 Da. Almost full sequence coverage (98.6%) by nanoLC-MS/MS peptide mapping was achieved. The purity and C1s inhibitory activity of rhC1INH from CHO cells are comparable with Ruconest®, although we found differences in charge isoforms distribution, intact mass values, and N-glycans profile. Comparison of the specific activity (IC50 value) of the rhC1INH with human C1 esterase inhibitor from blood serum showed similar inhibitory properties. These data allow us to conclude that the novel rhC1INH molecule could become a potential therapeutic option for patients with HAE/AAE.

وصف الملف: application/pdf

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4aa34c4254abd99833665427e9ce8e82Test
https://pubmed.ncbi.nlm.nih.gov/34832963Test

مصطلحات موضوعية: angioedema, icatibant, ecallantide, rituximab, Berinert, Ruconest, Firazyr, Lanadelumab

الوصف: Angioedema is a non-inflammatory oedema of the subcutaneous tissue and/or mucosal membranes. It most commonly coexists with urticaria wheals and is considered to be a deep form of urticaria. Less commonly, it occurs in isolation and can take two basic forms: acquired angioedema and hereditary angioedema. Currently, there are 4 defined types of acquired angioedema and 7 types of hereditary angioedema. Treatment of angioedema depends on its form and etiological factors. Especially the genetic form, i.e. hereditary angioedema, is a considerable challenge for medical specialists, particularly dermatologists and allergists.

العلاقة: Postępy Dermatologii i Alergologii = Advances in Dermatology and Allergology, T. 37, nr 4, s. 445-451; https://ruj.uj.edu.pl/xmlui/handle/item/264453Test

الإتاحة: https://doi.org/10.5114/ada.2020.98226Test
https://ruj.uj.edu.pl/xmlui/handle/item/264453Test

المؤلفون: Ken-ichiro Sakata, Yoshimasa Kitagawa, Masahiro Morimoto, Noritaka Ohga, Takuya Asaka, Akira Satoh, Kazuhiro Matsushita, Jun Sato

المصدر: Oral and Maxillofacial Surgery Cases, Vol 6, Iss 2, Pp-(2020)

مصطلحات موضوعية: medicine.medical_treatment, lcsh:Surgery, Intravenous sedation, 03 medical and health sciences, 0302 clinical medicine, Tracheotomy, C1–INH, Berinert P, Edema, medicine, Hereditary Angioedema Type III, Hereditary angioedema, business.industry, 030206 dentistry, lcsh:RD1-811, medicine.disease, Otorhinolaryngology, 030220 oncology & carcinogenesis, Anesthesia, Wisdom tooth extraction, Surgery, Clinical case, Oral Surgery, medicine.symptom, business

الوصف: We report a clinical case of a patient with hereditary angioedema (HAE, Type III) in which, by implementing careful preoperative preparations to prevent the occurrence of edema, good course was achieved without the prophylactic administration of C1-inhibitor (C1–INH). We recommended the prophylactic administration of Berinert P, a human plasma-derived C1–INH formulation; however, due to the risk of developing a viral infection, both the patient and her mother refused to receive Berinert P. In cases where C1–INH cannot be administered as a prophylactic measure, it is essential to make sufficient preparations, including urgent tracheotomy, to prevent sudden occurrence of edema, with the cooperation of the anesthesiologist.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::491f34e30755af38c8d009291f88c88cTest
http://www.sciencedirect.com/science/article/pii/S2214541920300122Test

المؤلفون: Hikaru Yamamoto, Kaoru Komita, Kenji Okami, Masahiro Iida, Masashi Hamada, Motoki Sekine, Shoji Kaneda

المصدر: Practica oto-rhino-laryngologica. Suppl.. 2016, 147:56

المؤلفون: Pham, Hoang, Santucci, Stephanie, Yang, William H

مصطلحات موضوعية: Berinert®, Hereditary angioedema, HAE, C1 inhibitor concentrate, C1-INH, Short-term prophylaxis, Critical care

الوصف: Hereditary angioedema (HAE) is a rare autosomal dominant disease most commonly associated with defects in C1 esterase inhibitor (C1-INH). HAE manifests as recurrent episodes of edema in various body locations. Atypical symptoms, such as ascites, acute respiratory distress syndrome, and hypovolemic shock, have also been reported. Management of HAE conventionally involves the treatment of acute attacks, as well as short- and long-term prophylaxis. Since attacks can be triggered by several factors, including stress and physical trauma, prophylactic therapy is recommended for patients undergoing surgery. Human plasma-derived C1-INH (pdC1-INH) concentrate is indicated for the treatment of both acute HAE attacks and pre-procedure prevention of HAE episodes in patients undergoing medical, dental, or surgical procedures. We report the first case of a patient with HAE who experienced an abdominal attack precipitated by a retroperitoneal bleed while being converted from warfarin to heparin in preparation for surgery. Subsequently, the patient had a protracted course in hospital with other complications, which included hypovolemic shock, ascites, severe sepsis from nosocomial pneumonia, renal and respiratory failure. Despite intensive interventions, the patient remained in a critical state for months; however, after a trial of daily intravenous infusion of pdC1-INH concentrate (Berinert®, CSL Behring GmbH, Marburg, Germany), clinical status improved, particularly renal function. Therefore, pdC1-INH concentrate may be an effective treatment option to consider for critically-ill patients with HAE.

العلاقة: http://www.aacijournal.com/content/10/1/62Test

الإتاحة: http://www.aacijournal.com/content/10/1/62Test