Russian Journal of Allergy最新文献

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Cutaneous mastocytosis and allergic diseases in children: a cross-sectional study 儿童皮肤肥大细胞增多症和过敏性疾病:一项横断面研究
Russian Journal of Allergy Pub Date : 2023-12-21 DOI: 10.36691/rja15044
E. Kasikhina
{"title":"Cutaneous mastocytosis and allergic diseases in children: a cross-sectional study","authors":"E. Kasikhina","doi":"10.36691/rja15044","DOIUrl":"https://doi.org/10.36691/rja15044","url":null,"abstract":"BACKGROUND: Mastocytosis is a heterogeneous group of diseases with abnormal accumulation of clonal mast cells in one or more organs. Comorbidities in patients with mastocytosis include IgE-dependent allergies, psychiatric or psychological disorders, and vitamin D deficiency. Despite the clinical similarities between allergies and mastocytosis, only a few European studies have considered comorbidities. \u0000AIMS:to analyze the frequency of concomitant allergic diseases in children with cutaneous mastocytosis. \u0000MATERIALS AND METHODS:We analyzed the data of 248 children aged 6 months to 17 years inclusive with a diagnosis of cutaneous mastocytosis, who were on outpatient treatment and observation at the Moscow Scientific and Practical Center for Dermatovenereology and Cosmetology from January 2019 to June 2023. Serum levels of total IgE and allergen-specific IgE were analyzed in children with and without allergic diseases. In addition, a positive history of allergy was analyzed in close relatives of children with CM. \u0000RESULTS:It was revealed that every third child (36.7%) with cutaneous mastocytosis had a concomitant allergic disease. In 27.4% of the examined children, allergic diseases were in relatives. In children with mastocytomas, the process was significantly more often accompanied by the development of allergic diseases than in children with maculopapular cutaneous mastocytosis (2=45.46, p=0.001). The level of total IgE was not a statistically significant sign of the presence of allergic diseases in mastocytosis (2 =1.161, p=0.3). Allergen-specific IgE were detected in 47.2% of children with allergic diseases. Among children without manifestations of food allergy and pollinosis, asymptomatic low and moderate sensitization to any plant product and birch was detected in 26.5%. Sensitization only to respiratory allergens was found in 8% of children with cutaneous mastocytosis without allergic diseases. \u0000CONCLUSIONS: In Russia, this study for the first time demonstrated a high incidence of allergic diseases in children with cutaneous mastocytosis. The results of the study showed that all clinical forms of mastocytosis in children, including cutaneous mastocytomas, should be subject to interdisciplinary observation and examination. Timely detection of comorbid allergic diseases contributes to the development of individual tactics for the treatment and prevention of complications and anaphylaxis.","PeriodicalId":270411,"journal":{"name":"Russian Journal of Allergy","volume":"26 16","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138950425","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Diagnostic and prognostic importance of biomarkers in the atopic dermatitis endotypes and phenotypes and in the effectiveness assessing of therapy 生物标记物在特应性皮炎内型和表型的诊断和预后以及疗效评估中的重要性
Russian Journal of Allergy Pub Date : 2023-12-21 DOI: 10.36691/rja16905
O. Elisyutina, E. Fedenko, Eugeniy V. Smolnikov, Olga A. Ignatyeva, M. Khaitov
{"title":"Diagnostic and prognostic importance of biomarkers in the atopic dermatitis endotypes and phenotypes and in the effectiveness assessing of therapy","authors":"O. Elisyutina, E. Fedenko, Eugeniy V. Smolnikov, Olga A. Ignatyeva, M. Khaitov","doi":"10.36691/rja16905","DOIUrl":"https://doi.org/10.36691/rja16905","url":null,"abstract":"AD is a highly heterogeneous disease that requires an individualized approach to treatment. Recent studies have characterized a variety of biomarkers that reflect diverse pathophysiological aspects of AD and have provided encouraging evidence of the potential of these biomarkers as tools for personalized diagnosis, severity assessment, and monitoring of treatment efficacy. This review is devoted to the analysis of modern studies of AD biomarkers, their clinical significance and possible prospects not only in determining the endotype of the disease, but also in the development of new therapeutic strategies. The introduction of accessible and simple methods for determining biomarkers into clinical practice is a perspective task of modern allergology and dermatology. Summarizing current data will allow us to identify priority issues for future research that will help to implement the concept of personalized AD therapy, reduce the burden of the disease and improve the quality of life of patients.","PeriodicalId":270411,"journal":{"name":"Russian Journal of Allergy","volume":"21 10","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138952748","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
IgE mediated food allergy and celiac disease: An updated Review IgE 介导的食物过敏和乳糜泻:最新综述
Russian Journal of Allergy Pub Date : 2023-12-21 DOI: 10.36691/rja16499
Hasna Ait Said, L. Elmoumou, M. Guennouni, Bouchra Rherissi, Nadia El Kadmiri
{"title":"IgE mediated food allergy and celiac disease: An updated Review","authors":"Hasna Ait Said, L. Elmoumou, M. Guennouni, Bouchra Rherissi, Nadia El Kadmiri","doi":"10.36691/rja16499","DOIUrl":"https://doi.org/10.36691/rja16499","url":null,"abstract":"Celiac disease is a chronic autoimmune disorder that affects the small intestine. It is triggered in genetically predisposed individuals by consumption of gluten, a protein found in wheat, barley and rye. Celiac disease typically manifests in young pediatric patients as malabsorption of nutrients, gastrointestinal symptoms, and other health complications, which is associated with partial or total villous atrophy of the proximal small intestine. Food allergy, on the other hand, is an abnormal immune response to a specific food protein that causes inflammation and a range of symptoms, from mild hives to life-threatening anaphylaxis. \u0000Although the mechanisms behind celiac disease and food allergy are different, both conditions involve an immune response to food proteins. The current study revealed the possibility of IgE-A coexistence in celiac disease and food allergy screening should be considered for people with celiac disease, especially when symptoms persist even after implementing a gluten-free. However, the relationship between celiac disease and food allergy is not fully understood, and more research is needed to explore this link. This review aims to examine the available literature for the occurrence of food allergy in subjects with celiac disease.","PeriodicalId":270411,"journal":{"name":"Russian Journal of Allergy","volume":"25 12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138948091","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Moisturizers for treatment and prophylaxis of atopic dermatitis exacerbations 用于治疗和预防特应性皮炎恶化的保湿剂
Russian Journal of Allergy Pub Date : 2023-12-21 DOI: 10.36691/rja16900
Daria D. Chernushevich, O. Shtyrbul, Elena I. Grebenchenko, E. Fedenko
{"title":"Moisturizers for treatment and prophylaxis of atopic dermatitis exacerbations","authors":"Daria D. Chernushevich, O. Shtyrbul, Elena I. Grebenchenko, E. Fedenko","doi":"10.36691/rja16900","DOIUrl":"https://doi.org/10.36691/rja16900","url":null,"abstract":"Atopic dermatitis (AD) is a global problem with increasing prevalence rates worldwide. Genetic predisposition to allergies, epidermal barrier dysfunction, and T2 immune response, underlies AD. Xerosis, itchy skin and exacerbation are the main AD symptoms, leading to increased permeability of allergens and microbes through the damaged epidermis and to the development of sensitization. The main purpose of AD therapy is to achieve long-term remission and to improve quality of life. Topical treatment includes glucocorticosteroids, topical calcineurin inhibitors and emollients for reducing inflammation, xerosis and prevention of infection. Daily use of effective emollients as basic skin care can improve epidermal barrier function, provide hydration of the skin end reduce the topical glucocorticosteroids needs.","PeriodicalId":270411,"journal":{"name":"Russian Journal of Allergy","volume":"20 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138952787","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
CLINICAL PREDICTORS OF ANTIHISTAMINE RESISTANCE IN THE PATIENTS WITH CHRONIC SPONTANEOUS URTICARIA 慢性自发性荨麻疹患者抗组胺药耐药性的临床预测因素
Russian Journal of Allergy Pub Date : 2023-12-21 DOI: 10.36691/rja7951
C. Popova, V. Zaborova, V. Kurshev, Elena V. Kovalkova, Serdotetskova Serdotetskova, D. Fomina, E. Borzova
{"title":"CLINICAL PREDICTORS OF ANTIHISTAMINE RESISTANCE IN THE PATIENTS WITH CHRONIC SPONTANEOUS URTICARIA","authors":"C. Popova, V. Zaborova, V. Kurshev, Elena V. Kovalkova, Serdotetskova Serdotetskova, D. Fomina, E. Borzova","doi":"10.36691/rja7951","DOIUrl":"https://doi.org/10.36691/rja7951","url":null,"abstract":"BACKGROUND: Сhronic spontaneous urticaria is a disease characterized by itchy wheals and/or angioedema on the skin over a period of 6 weeks or longer. A large-scale international AWARE study, with the participation of researchers from Russia, indicates the ineffectiveness of therapy with standard doses of second-generation antihistamines in more than 50% of patients with CSU. Clinical predictors for antihistamine resistance include low quality of life based on CU-Q2oL, baseline UAS7 16, combination with chronic inducible urticaria, and autoimmune comorbidity. Currently, research into clinical predictors of antihistamine resistance, including body mass index (BMI), in CSU patients is ongoing. AIMS: to examine the degree of symptoms control depending on BMI and the level of physical activity in CSU patients treated with second-generation antihistamines. MATERIALS AND METHODS: The examination of CSU patients (n=34) included the clinical history and the use of international questionnaires: UAS7 (urticaria activity score for 7 days), UCT (Urticaria Control Test), DLQI (The Dermatology life Quality Index) and GPAQ (Questionnaire on Physical Activity of the World Health organizations, WHO). The questionnaires were analyzed in subgroups of patients with controlled and uncontrolled CSU, as well as in subgroups of CSU patients with normal weight, overweight, and obesity. RESULTS: Uncontrolled symptoms were observed in 70.6% (24) CSU patients, including 45.8% (11) with a resistance to 4-fold doses of second-generation antihistamines. In 52.9% of CSU patients the BMI exceeded normal values. In our study, thre was an inverse correlation between BMI and the degree of control of CSU symptoms. CONCLUSIONS: Due to the negative correlation between BMI and the degree of symptoms control, there is a need for further research into the role of BMI as a clinical predictor of antihistamine-resistant CSU. In the future, the personalized therapy for CSU may take into account the degree of disease control, clinical predictors of antihistamine resistance and proinflammatory biomarkers.","PeriodicalId":270411,"journal":{"name":"Russian Journal of Allergy","volume":"19 10","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139166239","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
CHANGES IN UPPER RESPIRATORY TRACT MICROBIOTA IN PATIENTS WITH RESPIRATORY ALLERGY ON THE BACKGROUND OF SENSITIZATION TO BACTERIAL STAPHYLOCOCCAL SERINE PROTEINASE BEFORE AND AFTER BASELINE AND ALLERGEN-SPECIFIC THERAPY (ASIT) 对细菌葡萄球菌丝氨酸蛋白酶过敏的呼吸道过敏患者在接受基线和过敏原特异性疗法(ASIT)前后上呼吸道微生物群的变化
Russian Journal of Allergy Pub Date : 2023-12-21 DOI: 10.36691/rja14875
Yury A Tyurin, A. A. Sharifullina, I. Reshetnikova, Rustam Amiryanovich Minnibayev, Ruslan Zufarovich Khairullin, Rustem Salakhovich Fassakhov
{"title":"CHANGES IN UPPER RESPIRATORY TRACT MICROBIOTA IN PATIENTS WITH RESPIRATORY ALLERGY ON THE BACKGROUND OF SENSITIZATION TO BACTERIAL STAPHYLOCOCCAL SERINE PROTEINASE BEFORE AND AFTER BASELINE AND ALLERGEN-SPECIFIC THERAPY (ASIT)","authors":"Yury A Tyurin, A. A. Sharifullina, I. Reshetnikova, Rustam Amiryanovich Minnibayev, Ruslan Zufarovich Khairullin, Rustem Salakhovich Fassakhov","doi":"10.36691/rja14875","DOIUrl":"https://doi.org/10.36691/rja14875","url":null,"abstract":"Allergic rhinitis (AR) is a common allergic disease in children and adults, often combined with other allergic diseases. Objective. To study the changes in the local microbiota of the upper respiratory tract (URT) in patients with respiratory allergy in the dynamics of the disease, as well as after therapy, and the possible participation of some components of opportunistic microbiota, in particular, S. aureus in the formation of allergic inflammation. The study included 182 patients with respiratory allergy (seasonal and year-round allergic rhinitis and combined forms with atopic bronchial asthma) aged 6 to 54 years. The study was a case-control study for 5 years. Methods. A complex of microbiologic, immunologic and allergologic methods of research was applied in the course of the study. Results. In patients in remission of allergic rhinitis who received ASIT in the form of SLIT, in contrast to patients who received baseline therapy, we found an increase in the occurrence of commensal staphylococci (S. epidermidis) and a decrease in the occurrence of hemolytic forms of bacteria and Staphylococcus aureus. Conclusions. The formation of sensitization to the bacterial component of staphylococci (serine proteinase of S. aureus - SplA) in seasonal and year-round allergic rhinitis on the background of dysbiosis of the local microbiota of the mucous membrane of the respiratory tract and nasopharynx has been established.","PeriodicalId":270411,"journal":{"name":"Russian Journal of Allergy","volume":"26 12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139166072","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Practical issues of using emollients for the prevention of exacerbations of atopic dermatitis during the flowering season: Expert resolution 在花季使用润肤剂预防特应性皮炎恶化的实际问题:专家决议
Russian Journal of Allergy Pub Date : 2023-07-09 DOI: 10.36691/rja13056
E. Fedenko, I. Zakharova, O. V. Zaytseva, A. Pampura, O. Tamrazova
{"title":"Practical issues of using emollients for the prevention of exacerbations of atopic dermatitis during the flowering season: Expert resolution","authors":"E. Fedenko, I. Zakharova, O. V. Zaytseva, A. Pampura, O. Tamrazova","doi":"10.36691/rja13056","DOIUrl":"https://doi.org/10.36691/rja13056","url":null,"abstract":"In February 2023, a meeting of the Council of Experts was held, attended by leading specialists in the fields of allergology, pediatrics and dermatology. \u0000The Working Group discussed the problem of sensitization to pollen allergens in patients with atopic dermatitis and exacerbation of the disease during the pollination season of plants, as well as the formation of epicutaneous sensitization through a damaged epidermal barrier. The experts comprehensively considered the criteria that a modern emollient used for the care of atopic dermatitis patients' skin should meet, discussed the role of moisturizers in preventing seasonal exacerbations of atopic dermatitis based on previously conducted research, and developed unified recommendations on the principles of managing this type of patients. \u0000Suggestions were made for further informational and organizational measures aimed at expanding the knowledge of patients and medical specialists about the problem of epicutaneous sensitization to aeroallergens in atopic dermatitis patients, their role in the development of seasonal exacerbations of atopic dermatitis, and the possibility of their prevention using modern emollients.","PeriodicalId":270411,"journal":{"name":"Russian Journal of Allergy","volume":"14 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"131492620","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Allergic rhinitis masks: Nasal polyposis in two preschool children 过敏性鼻炎口罩:两个学龄前儿童鼻息肉
Russian Journal of Allergy Pub Date : 2023-07-09 DOI: 10.36691/rja1595
D. Macharadze, V. Popadiuk
{"title":"Allergic rhinitis masks: Nasal polyposis in two preschool children","authors":"D. Macharadze, V. Popadiuk","doi":"10.36691/rja1595","DOIUrl":"https://doi.org/10.36691/rja1595","url":null,"abstract":"Nasal symptoms such as nasal congestion, rhinorrhea, and cough are especially common in children. Despite symptomatic treatment (various nasal sprays, physiotherapy, antibiotics and topical glucocorticosteroids), nasal congestion can persist for a long time. In such cases, several diseases are most likely: adenoids, allergic rhinitis, various anomalies in the structure of intranasal structures and sinuses (for example, curvature of the nasal septum), rhinosinusitis (acute, subacute). If a child has two main symptoms ― congestion and discharge from the nose ― and such additional symptoms as a headache with predominant localization in the paranasal sinuses and a deterioration in smell (possibly also the taste of food) for more than 3 months, it is necessary to exclude the diagnosis of chronic rhinosinusitis, which can be combined with a nasal polyp. \u0000The purpose of this article is to analyze clinical cases of chronic rhinosinusitis with nasal polyp in two preschool children, including one of them with intolerance to non-steroidal anti-inflammatory drugs, and to raise physicians' awareness of this nosology. \u0000In children with symptoms such as prolonged nasal congestion, rhinorrhea, possibly impaired sense of smell, headache, intolerance to non-steroidal anti-inflammatory drugs, in the absence of sensitization and immunodeficiency, a computed tomography scan of the paranasal sinuses is recommended to exclude nasal polyp.","PeriodicalId":270411,"journal":{"name":"Russian Journal of Allergy","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129966058","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Is Quifenadine an antihistamine of the first generation? Do we know everything… 喹非那定是第一代抗组胺药吗?我们知道一切吗?
Russian Journal of Allergy Pub Date : 2023-07-09 DOI: 10.36691/rja11546
D. Sychev, N. Nenasheva, O. V. Sebekina, M. Peredelskaya
{"title":"Is Quifenadine an antihistamine of the first generation? Do we know everything…","authors":"D. Sychev, N. Nenasheva, O. V. Sebekina, M. Peredelskaya","doi":"10.36691/rja11546","DOIUrl":"https://doi.org/10.36691/rja11546","url":null,"abstract":"Quifenadine was developed in the 1970s in the laboratory of M.D. Mashkovsky and is one of the first examples of the creation of a new class of non-sedative antihistamine with polyfunctional action that combines high selective activity to block histamine receptor type 1 and the ability to block serotonin action and increase tissue histaminase activity. \u0000There is a large experience in the use of quifenadine (based on scientific clinical studies and real clinical practice). The total number of patients involved in the trials reached approximately 2000. The studies evaluated the quifenadipharmacokinetics, pharmacodynamics, clinical efficacy, and safety of the grug. The results of a clinical study of quiphenadine showed high therapeutic efficacy in a variety of allergic diseases (allergic rhinitis, atopy and atom-atopic dermatitis, chronic urticaria). \u0000Quiphenadine meets the current requirements for antihistamines, however, the question remains open, which generation of drugs can be classified as quinuclidine, taking into account all its characteristics and clinical effects.","PeriodicalId":270411,"journal":{"name":"Russian Journal of Allergy","volume":"108 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133465560","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Organization of medical care for patients with hereditary angioedema and experience in treating patients with lanadelumab in the Sverdlovsk region 斯维尔德洛夫斯克地区遗传性血管性水肿患者的医疗护理组织和使用lanadelumab治疗患者的经验
Russian Journal of Allergy Pub Date : 2023-07-09 DOI: 10.36691/rja8732
G. A. Bykova, Evgeny K. Beltyukov, V. Naumova, M. Karakina
{"title":"Organization of medical care for patients with hereditary angioedema and experience in treating patients with lanadelumab in the Sverdlovsk region","authors":"G. A. Bykova, Evgeny K. Beltyukov, V. Naumova, M. Karakina","doi":"10.36691/rja8732","DOIUrl":"https://doi.org/10.36691/rja8732","url":null,"abstract":"BACKGROUND: Hereditary angioedema is a rare autosomal dominant disease that can cause edema of skin and mucous membranes, including life-threatening localization, in pathogenesis of which plasma kallikrein is also important. The main therapy aim is to achieve complete control over symptoms and improve quality of life. In Russia, the first available specialized drug for long-term prophylaxis is lanadelumab blocking plasma kallikrein. AIM: The aim is to show principles of medical care for patients with hereditary angioedema in Sverdlovsk region and to determine lanadelumab efficacy in patients with hereditary angioedema in real clinical practice. MATERIALS AND METHODS: We conducted a single-center prospective non-interventional study. There are 22 patients with hereditary angioedema in register of primary immunodeficiencies in Sverdlovsk region. The analysis of patients' therapy was performed. Lanadelumab efficacy was studied in 5 patients received drug for at least 4 months. The validated questionnaires AAS, AECT, AE-QoL were used to assess lanadelumab efficacy. RESULTS: Educational program for practitioners and implemented diagnostic algorithm led to increase hereditary angioedema detection: the number of hereditary angioedema patients doubled over past 3.5 years (11 patients in 2019, 22 patients in 2023). School for patients education was organized. Lanadelumab has been introduced into clinical practice since December 2021. All patients included in lanadelumab efficacy study were female. According to AAS28 scale, patients had high disease activity before and there was an almost 10-fold decrease in disease activity in 4 month of lanadelumab therapy. A positive effect was observed after the first month of treatment with lanadelumab. Hereditary angioedema activity decreased to 0 after 4 months of therapy. The average score on AEST scale was 5.402.41, which indicated an uncontrolled course of hereditary angioedema. By the 4th month of therapy, control was achieved ― 13.602.61 points on AEST scale. Quality of life on AE-Qol scale improved from 81% to 38% by the 4th month of therapy (p=0.050). CONCLUSION: Current system of medical care for patients with hereditary angioedema in Sverdlovsk Region makes it possible to improve hereditary angioedema detection and provide patients with special pharmacotherapy betimes. Lanadelumab significantly reduces hereditary angioedema activity, allows achieving controlled course and high quality of life in real clinical practice.","PeriodicalId":270411,"journal":{"name":"Russian Journal of Allergy","volume":"10 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114183738","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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