137 results on '"Jesenak M"'
Search Results
2. Real-life studies and registries of severe asthma: The advent of digital technology
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Urdova, V., primary, Rogers, L., additional, Jesenak, M., additional, and Seys, S.F., additional
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- 2023
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3. Factors attributable to the level of exhaled nitric oxide in asthmatic children
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Banovcin P, Jesenak M, Michnova Z, Babusikova E, Nosal S, Mikler J, Fabry J, and Barreto M
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bronchial asthma ,exhaled carbon monoxide ,exhaled nitric oxide ,children ,Medicine - Abstract
Abstract Background Asthma is a heterogeneous disease with variable symptoms especially in children. Exhaled nitric oxide (FeNO) has proved to be a marker of inflammation in the airways and has become a substantial part of clinical management of asthmatic children due to its potential to predict possible exacerbation and adjust the dose of inhalant corticosteroids. Objectives We analyzed potential factors that contribute to the variability of nitric oxide in various clinical and laboratory conditions. Materials and methods Study population consisted of 222 asthmatic children and 27 healthy control subjects. All children underwent a panel of tests: fractioned exhaled nitric oxide, exhaled carbon monoxide, asthma control test scoring, blood sampling, skin prick tests, and basic spirometry. Results FeNO and other investigated parameters widely changed according to clinical or laboratory characteristics of the tested children. Asthmatics showed increased levels of FeNO, exhaled carbon monoxide, total serum IgE, and higher eosinophilia. Boys had higher FeNO levels than girls. We found a significant positive correlation between FeNO levels and the percentage of blood eosinophils, %predicted of forced vital capacity, total serum IgE levels, and increasing age. Conclusions Various phenotypes of children's asthma are characterized by specific pattern of the results of clinical and laboratory tests. FeNO correlates with total serum IgE, blood eosinophilia, age, and some spirometric parameters with different strength. Therefore, the coexistence of atopy, concomitant allergic rhinitis/rhinoconjunctivitis, and some other parameters should be considered in critical evaluation of FeNO in the management of asthmatic children.
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- 2009
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4. EUFOREA summit in Brussels 2023: inspiring the future of allergy & respiratory care
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Hellings, P. W., primary, Lau, S., additional, Scadding, G. K., additional, Bjermer, L., additional, Backer, V., additional, Chaker, A. M., additional, Conti, D. M., additional, De Corso, E., additional, Diamant, Z., additional, Djukanovic, R., additional, Fokkens, W., additional, Gevaert, P., additional, Gray, C. L., additional, Han, J. K., additional, Heaney, L. G., additional, Hoffmann, H. J., additional, Jesenak, M., additional, Johansen, P., additional, Kumaran, M. S., additional, McDonald, M., additional, Melén, E., additional, Mullol, J., additional, Reitsma, S., additional, Ryan, D., additional, Scadding, G., additional, Schmid-Grendelmeier, P., additional, Teeling, T., additional, Odemyr, M., additional, and Wahn, U., additional
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- 2023
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5. Metabolic pathways in immune senescence and inflammaging: Novel therapeutic strategy for chronic inflammatory lung diseases. An EAACI position paper from the Task Force for Immunopharmacology.
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Roth‐Walter, F., Adcock, I. M., Benito‐Villalvilla, C., Bianchini, R., Bjermer, L., Caramori, G., Cari, L., Chung, K. F., Diamant, Z., Eguiluz‐Gracia, I., Knol, E. F., Jesenak, M., Levi‐Schaffer, F., Nocentini, G., O'Mahony, L., Palomares, O., Redegeld, F., Sokolowska, M., Van Esch, B. C. A. M., and Stellato, C.
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LUNG diseases ,CELLULAR aging ,AGING ,TASK forces ,CHRONIC obstructive pulmonary disease - Abstract
The accumulation of senescent cells drives inflammaging and increases morbidity of chronic inflammatory lung diseases. Immune responses are built upon dynamic changes in cell metabolism that supply energy and substrates for cell proliferation, differentiation, and activation. Metabolic changes imposed by environmental stress and inflammation on immune cells and tissue microenvironment are thus chiefly involved in the pathophysiology of allergic and other immune‐driven diseases. Altered cell metabolism is also a hallmark of cell senescence, a condition characterized by loss of proliferative activity in cells that remain metabolically active. Accelerated senescence can be triggered by acute or chronic stress and inflammatory responses. In contrast, replicative senescence occurs as part of the physiological aging process and has protective roles in cancer surveillance and wound healing. Importantly, cell senescence can also change or hamper response to diverse therapeutic treatments. Understanding the metabolic pathways of senescence in immune and structural cells is therefore critical to detect, prevent, or revert detrimental aspects of senescence‐related immunopathology, by developing specific diagnostics and targeted therapies. In this paper, we review the main changes and metabolic alterations occurring in senescent immune cells (macrophages, B cells, T cells). Subsequently, we present the metabolic footprints described in translational studies in patients with chronic asthma and chronic obstructive pulmonary disease (COPD), and review the ongoing preclinical studies and clinical trials of therapeutic approaches aiming at targeting metabolic pathways to antagonize pathological senescence. Because this is a recently emerging field in allergy and clinical immunology, a better understanding of the metabolic profile of the complex landscape of cell senescence is needed. The progress achieved so far is already providing opportunities for new therapies, as well as for strategies aimed at disease prevention and supporting healthy aging. [ABSTRACT FROM AUTHOR]
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- 2024
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6. Inhibitory Immune Checkpoint Molecules and Exhaustion of T cells in COVID-19
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BARNOVA, M, primary, BOBCAKOVA, A, additional, URDOVA, V, additional, KOSTURIAK, R, additional, KAPUSTOVA, L, additional, DOBROTA, D, additional, and JESENAK, M, additional
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- 2021
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7. COVID-19 and the Differences in Physiological Background Between Children and Adults and Their Clinical Consequences
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KAPUSTOVA, L, primary, PETROVICOVA, O, additional, BANOVCIN, P, additional, ANTOSOVA, M, additional, BOBCAKOVA, A, additional, URBANCIKOVA, I, additional, RENNEROVA, Z, additional, and JESENAK, M, additional
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- 2021
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8. Successful Use of Recombinant Human C1-INH in a Patient with Acquired Angioedema due to C1 Inhibitor Deficiency and an Unusually High Titer of Anti-C1-Inhibitor Autoantibodies
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Jesenak, M, primary, Brndiarova, M, additional, Banovcin, P, additional, Varga, L, additional, and Farkas, H, additional
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- 2021
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9. Pre-asthma: a useful concept for prevention and diseasemodification? A EUFOREA paper. Part 1--allergic asthma.
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Scadding, G. K., McDonald, M., Backer, V., Scadding, G., Bernal-Sprekelsen, M., Conti, D. M., De Corso, E., Diamant, Z., Gray, C., Hopkins, C., Jesenak, M., Johansen, P., Kappen, J., Mullol, J., Price, D., Quirce, S., Reitsma, S., Salmi, S., Senior, B., and Thyssen, J. P.
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- 2024
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10. Omalizumab in An 8-Year-Old Boy With Diabetes Mellitus and Refractory Chronic Spontaneous Urticaria
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Jesenak, M, primary, Ciljakova, M, additional, Janickova, M, additional, and Banovcin, P, additional
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- 2019
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11. RECOMBINANT C1 ESTERASE INHIBITOR FOR SHORT-TERM PROPHYLAXIS IN PATIENTS WITH HEREDITARY ANGIOEDEMA
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Zanichelli, A., primary, Staevska, M., additional, Jesenak, M., additional, Hrubiskova, K., additional, Sobotkova, M., additional, Zachova, R., additional, Hakl, R., additional, Andrejevic, S., additional, Suiter, T., additional, Grivcheva-Panovska, V., additional, Karadza-Lapic, L., additional, Soteres, D., additional, Shapiro, R., additional, Rumbyrt, J., additional, Tachdjian, R., additional, Mehta, V., additional, Hsu, F., additional, and Valerieva, A., additional
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- 2018
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12. The effect of passive smoking on bacterial colonisation of the upper airways and selected laboratory parameters in children
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Bugova, G., additional, Janickova, M., additional, Uhliarova, B., additional, Babela, R., additional, and Jesenak, M., additional
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- 2018
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13. Global case fatality rate of coronavirus disease 2019 (COVID‐19) by continents and national income: a meta‐analysis
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Ghayda RA, Lee KH, Han YJ, Ryu S, Hong SH, Yoon S, Jeong GH, Yang JW, Lee HJ, Lee J, Lee JY, Effenberger M, Eisenhut M, Kronbichler A, Solmi M, Li H, Jacob L., Koyanagi A, Radua J, Park MB, Aghayeva S, Ahmed MLCB, Serouri AA, Al-Shamsi HO, Amir-Behghadami M, Baatarkhuu O, Bashour H, Bondarenko A, Camacho-Ortiz A, Castro F, Cox H, Davtyan H, Douglas K, Dragioti E, Ebrahim S, Ferioli M, Harapan H, Mallah SI, Ikram A, Inoue S, Jankovic S, Jayarajah U, Jesenak M, Kakodkar P, Kebede Y, Kifle M, Koh D, Males VK, Kotfis K, Lakoh S, Ling L, Llibre-Guerra J, Machida M, Makurumidze R, Mamun M, Masic I, Van Minh H, Moiseev S, Nadasdy T, Nahshon C, Ñamendys-Silva SA, Yongsi BN, Nielsen HB, Nodjikouambaye ZA, Ohnmar O, Oksanen A, Owopetu O, Parperis K, Perez GE, Pongpirul K, Rademaker M, Rosa S, Sah R, Sallam D, Schober P, Singhal T, Tafaj S, Torres I, Smith Torres-Roman J, Tsartsalis D, Tsolmon J, Tuychiev L, Vukcevic B, Wanghi G, Wollina U, Xu RH, Yang L, Zaidi Z, Smith L, and Shin JI
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continents ,COVID-19 ,proportion meta-analysis ,case fatality rate (CFR) - Abstract
The aim of this study is to provide a more accurate representation of COVID-19's CFR by performing meta-analyses by continents and income, and by comparing the result with pooled estimates. We used multiple worldwide data sources on COVID-19 for every country reporting COVID-19 cases. Based on the data, we performed random and fixed meta-analyses for CFR of COVID-19 by continents and income according to each individual calendar date. CFR were estimated based on the different geographical regions and level of income using three models: pooled estimates, fixed- and random-model. In Asia, all three types of CFR initially remained approximately between 2.0% and 3.0%. In the case of pooled estimates and the fixed model results, CFR increased to 4.0%, by then gradually decreasing, while in the case of random-model, CFR remained under 2.0%. Similarly, in Europe, initially the two types of CFR peaked at 9.0% and 10.0%, respectively. The random-model results showed an increase near 5.0%. In high income countries, pooled estimates and fixed-model showed gradually increasing trends with a final pooled estimates and random-model reached about 8.0% and 4.0%, respectively. In middle-income, the pooled estimates and fixed-model have gradually increased reaching up to 4.5%. in low-income countries, CFRs remained similar between 1.5% and 3.0%. Our study emphasizes that COVID-19 CFR is not a fixed or static value. Rather, it is a dynamic estimate that changes with time, population, socioeconomic factors and the mitigatory efforts of individuals countries. This article is protected by copyright. All rights reserved.
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- 2022
14. Canakinumab as monotherapy for treatment of familial Mediterranean fever – first report in Central and Eastern Europe region
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Jesenak, M., primary, Hrubiskova, K., additional, Kapustova, L., additional, Kostkova, M., additional, and Banovcin, P., additional
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- 2018
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15. Biodegradable versus titanium plates and screws for paediatric facial skeleton fractures
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Janickova, M., primary, Statelova, D., additional, Mikuskova, K., additional, Jesenak, M., additional, and Malachovsky, I., additional
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- 2018
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16. P180 Results from an interim analysis of a recombinant human C1 inhibitor treatment registry in Europe
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Hakl, R., primary, Valerieva, A., additional, Farkas, H., additional, Jesenak, M., additional, Hrubiskova, K., additional, Zanichelli, A., additional, Staevska, M., additional, Bellizzi, L., additional, Relan, A., additional, and Cicardi, M., additional
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- 2017
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17. Successful treatment of extreme acute lead intoxication
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Mikler, J, primary, Banovcin, P, additional, Jesenak, M, additional, Hamzikova, J, additional, and Statelova, D, additional
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- 2009
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18. Our experiences with diagnosis of latent tuberculosis in patients before the initiation of anti-TNF treatment with QuantiFERON-TB Gold test
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Zanova, E., primary, Jesenak, M., additional, Solovic, I., additional, Polanova, V., additional, Rybar, I., additional, and Rovensky, J., additional
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- 2008
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19. EFFECT OF OXIDATIVE STRESS ON THE DEVELOPMENT OF CHILDREN ATOPIC AND NON-ATOPIC ASTHMA: 48 - Poster Symposium
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Babusikova, E., Jesenak, M., Banovcin, P., and Dobrota, D.
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- 2009
20. COUGH REFLEX SENSITIVITY DISCRIMINATES VARIOUS TYPES OF CHILD ASTHMA: 40
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Jesenak, M., Babusikova, E., Michnova, Z., Turcan, T., Rennerova, Z., Havlicekova, Z., Barreto, M., Villa, M. P., and Banovcin, P.
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- 2009
21. Chronic granulomatous disease caused by a novel mutation in a 2-month-old boy with multifocal splenic abscesses
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Jesenak, M., Havlicekova, Z., Peter Banovcin, Stasia, M. J., TheREx, Techniques de l'Ingénierie Médicale et de la Complexité - Informatique, Mathématiques et Applications, Grenoble - UMR 5525 (TIMC-IMAG), VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP )-Centre National de la Recherche Scientifique (CNRS)-Université Joseph Fourier - Grenoble 1 (UJF)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP )-Centre National de la Recherche Scientifique (CNRS)-Université Joseph Fourier - Grenoble 1 (UJF), and Dagher, Marie-Claire
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Male ,Abdominal Abscess ,MESH: Humans ,MESH: Mutation ,MESH: Spleen ,Infant ,NADPH Oxidases ,Granulomatous Disease, Chronic ,MESH: Infant ,[SDV.IMM.II]Life Sciences [q-bio]/Immunology/Innate immunity ,MESH: Male ,Anti-Bacterial Agents ,MESH: Granulomatous Disease, Chronic ,MESH: Abdominal Abscess ,MESH: Anti-Bacterial Agents ,Mutation ,Humans ,MESH: NADPH Oxidase ,[SDV.IMM.II] Life Sciences [q-bio]/Immunology/Innate immunity ,Spleen ,ComputingMilieux_MISCELLANEOUS - Abstract
International audience
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- 2013
22. Food allergens and respiratory symptoms
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Jesenak, M., Rennerova, Z., Babusikova, E., Havlicekova, Z., Jakusova, L., Villa, M. P., Ronchetti, R., and Peter Banovcin
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- 2008
23. Current position of atopy patch test in the diagnosis of food allergy in children
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Jesenak, M, Rennerova, Z, Bavusikova, E, Banovcin, P, Jakusova, L, Havlicekova, Z, Barreto, Mario, Villa, MARIA PIA, and Ronchetti, Roberto
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- 2008
24. National analysis of bacterial meningitis in Slovakia, 1997-2007.
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Hudeckova H, Jesenak M, Maria A, Svihrova V, and Banovcin P
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- 2010
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25. Food allergies, cross-reactions and agroalimentary biotechnologies.
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Ronchetti, R., Kaczmarski, M. G., Hałuszka, J., Jesenak, M., and Villa, M. P.
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SELF-discrepancy ,SPECIALISTS ,ALLERGY diagnosis ,FOOD allergy ,SYMPTOMS ,ALLERGENS ,ALLERGY in children ,POLLEN ,MEDICAL sciences ,AGRICULTURAL biotechnology - Abstract
The discrepancy between what the general public and specialist in allergic diseases regard as a true food allergy can in part depend on the frequent evidence of subjects in whom clinical symptoms elicited by a given food allergen are frequently not reproducible: this suggests the existence of allergens variably present in certain foods. In adults and older children common is a form of food allergy associated with inhaled allergens, especially pollens. In this allergic form pollens and various vegetal food often cross react but the underlying scientific rationale is largely unclear. From the study of the "latex-fruits allergic syndrome" and the "oral allergic syndrome" emerged that the cross reactivity depends on epitopes of pollens and vegetables belonging to one of the 14 classes of the "pathogenesis related proteins" (PRPs). Vegetables produce PRPs in response to infection or after plant injury or application of chemicals: long-term conservation and methods used for rapid artificial ripening of vegetables can cause plant to produce PRPs or other allergens. A genetic selection of vegetables "protecting themselves against infection and infestation" by mean of PRPs production is practiced in agroalimentary biotechnology. We deem it urgent that the two realms, Medical Science (Allergology) and Agricultural Biotechnology begin to communicate openly in order to produce food as efficiently as possible but without harming the large part of the population which is predisposed to allergy and react to PRPs. [ABSTRACT FROM AUTHOR]
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- 2007
26. Cough reflex sensitivity in various phenotypes of childhood asthma
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Jesenak, M., Babusikova, E., Petrikova, M., Turcan, T., Rennerova, Z., Michnova, Z., Havlicekova, Z., Villa, M. P., and Peter Banovcin
27. THERAPEUTIC APPROACH TO A CHILD WITH ACUTE RESPIRATORY DISTRESS SYNDROME: A REPORT OF TWO CASES
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Nosal, S., Peter Durdik, Luptakova, A., Sutovska, M., Nosal, V., Jesenak, M., Havlicekova, Z., Hamzik, J., and Banovcin, P.
28. Food allergies, cross-reactions and agroalimentary biotechnologies
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Ronchetti, R., Maciej Kaczmarski, Hałuszka, J., Jesenak, M., and Villa, M. P.
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food allergy ,cross-reactions ,biotechnology
29. Exhaled carbon monoxide as a new marker of respiratory diseases in children
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Babusikova, E., Jesenak, M., Peter Durdik, Dobrota, D., and Banovcin, P.
30. Cardiac autonomic control in adolescents with primary hypertension
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Havlíceková Z, Tonhajzerová I, Jurko Jr A, Jesenák M, Durdík P, Nosál S, Zelenák K, Antosová M, and Bánovcin P
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primary hypertension ,cardiac autonomic control ,heart rate variability ,adolescents ,Medicine - Abstract
Abstract Background Impairment in cardiovascular autonomic regulation participates in the onset and maintenance of primary hypertension. Objective The aim of the present study was to evaluate cardiac autonomic control using long-term heart rate variability (HRV) analysis in adolescents with primary hypertension. Subjects and methods Twenty two adolescent patients with primary hypertension (5 girls/17 boys) aged 14-19 years and 22 healthy subjects matched for age and gender were enrolled. Two periods from 24-hour ECG recording were evaluated by HRV analysis: awake state and sleep. HRV analysis included spectral power in low frequency band (LF), in high frequency band (HF), and LF/HF ratio. Results In awake state, adolescents with primary hypertension had lower HF and higher LF and LF/HF ratio. During sleep, HF was lower and LF/HF ratio was higher in patients with primary hypertension. Conclusions A combination of sympathetic predominance and reduced vagal activity might represent a potential link between psychosocial factors and primary hypertension, associated with increased cardiovascular morbidity.
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- 2009
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31. Initial presenting manifestations in 16,486 patients with inborn errors of immunity include infections and noninfectious manifestations
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Tim Niehues, Catherine Waruiru, Conleth Feighery, Uwe Schauer, Virginie Courteille, Kai Lehmberg, Ingo Müller, I. Esteves, Henner Morbach, Michael Borte, Patrick Hundsdoerfer, Klaus Schwarz, Ewelina Gowin, Alessandro Aiuti, Andreas Holbro, Federica Barzaghi, João Farela Neves, Dagmar Graf, Hannah Tamary, Veneta Milenova, Benedikt Boetticher, Eleonora Gambineri, Vera Goda, Alia Eldash, Jan-Christian Wasmuth, Fabio Candotti, Svetlana O. Sharapova, Markus Metzler, Juergen Brunner, Anna Hilfanova, Brindusa Ruxandra Capilna, Pere Soler-Palacín, Arnau Antolí, Horst von Bernuth, Vassilios Lougaris, Maria Carrabba, Bernd H. Belohradsky, Julian Thalhammer, Nathalie de Vergnes, Peter Olbrich, Peter Kopač, Leif G. Hanitsch, Alexandra Nieters, Filomeen Haerynck, Juliana Gabzdilova, Sezin Aydemir, Rabab El Hawary, Patrick F.K. Yong, Maria Giovanna Danieli, Alberto Tommasini, Sandra Steinmann, Ulrich Baumann, Figen Dogu, Elisabeth Förster-Waldl, Carolina Marasco, Donato Amodio, Lorenzo Lodi, Xavier Solanich, Caterina Cancrini, Brigita Sitkauskiene, Torsten Witte, Clementina Vanessa, Nima Rezaei, Jean-Christophe Goffard, Kirsten Wittke, Emmanouil Liatsis, Helen Baxendale, Susana L. Silva, Bodo Grimbacher, Henrike Ritterbusch, Evangelia Farmaki, Safa Meshaal, Sujal Ghosh, Larysa Kostyuchenko, David Edgar, Simone Cesaro, R Zeuner, Nerea Salmón Rodríguez, Isabella Quinti, Stephan Ehl, Pauline Brosselin, Joerg C. Henes, Pilar Llobet Agulló, Rosa Maria Dellepiane, Andrea Meinhardt, Marina Kojić, Georgios Sogkas, Stephan Borte, Catharina Schuetz, Suheyla Ocak, Karin Marschall, Lukas M. Gasteiger, Stefan Raffac, Sofia Tantou, Sadia Noorani, Matthaios Speletas, Philippe Randrianomenjanahary, Ursula Holzer, Ayca Kiykim, Johannes G. Liese, Angelo Vacca, Gisela Fecker, Ekrem Unal, Koen J. van Aerde, Alba Parra-Martínez, Kaan Boztug, Sophie Stiehler, Sybille Landwehr-Kenzel, Claudio Pignata, Jennifer Neubert, Janine Reichenbach, Shahnaz Parvin, Sarah Goddard, Andrea Schroll, Dirk Holzinger, Asghar Aghamohammadi, Hassan Abolhassani, Johannes Trück, Estela Paz-Artal, Shereen M. Reda, Anna Shcherbina, Maria Raptaki, Jaroslava Orosova, Beata Wolska-Kuśnierz, Tessa Kerre, Gerrit Ahrenstorf, Ben Zion Garty, Dirk Foell, Benjamin Becker, Ulrike F. Demel, Androniki Kapousouzi, Abraham Rutgers, Klaus Warnatz, Gemma Rocamora Blanch, Stephan Rusch, Luis M. Allende, Dalia Abd Elaziz, Safa Baris, Jorisvan Montfrans, Dominik T. Schneider, Raphael Scheible, Juana Gil-Herrera, Gerhard Kindle, Annarosa Soresina, Giovanna Fabio, Uwe Wintergerst, Emilia Faria, Maria Fasshauer, Silvia Ricci, Aisha Elmarsafy, Barbara Pietrucha, Carsten Speckmann, Nizar Mahlaoui, Ulrich Heininger, Isabelle Meyts, Matthew Buckland, Efimia Papadopoulou-Alataki, Robin Kobbe, A Herwadkar, Sebastian F. N. Bode, Ali Sobh, László Maródi, Baldassarre Martire, Chiara Azzari, Maximilian Heeg, Katja Masjosthusmann, Michael H. Albert, Matteo Chinello, Juan Luis Santos-Pérez, Aarnoud Huissoon, Tanya I. Coulter, Hendrik Schulze-Koops, Norbert Graf, Radwa Alkady, Jolanta Bernatoniene, Seraina Prader, Alenka Gagro, Joachim Roesler, Taco W. Kuijpers, Ewa Więsik-Szewczyk, Maria Elena Maccari, Conrad Ferdinand Lippert, Miriam González-Amores, Johannes Dirks, Daniel E Pleguezuelo, Christof M. Kramm, Anders Fasth, Volker Schuster, Olov Ekwall, Nikolaus Rieber, Javier Carbone, Petra Kaiser-Labusch, Diana Ernst, Lucia Augusta Baselli, Luis Ignacio Gonzalez-Granado, Maria Kanariou, Stefanie S. V. Henriet, Sigune Goldacker, Kerstin Felgentreff, Oana Joean, Fine Roosens, Fabian Hauck, Eva C. Schwaneck, Milos Jesenak, Manfred Hoenig, Lenka Kapustova, Christoph Boesecke, Alain Fischer, Sara Pereira da Silva, Julia Körholz, Ansgar Schulz, Carolynne Schwarze-Zander, Mikko Seppänen, Nermeen Galal, Nora Naumann-Bartsch, Tomaz Garcez, Peter Ciznar, Klara M. Posfay-Barbe, Zelimir Pavle Eric, Reinhold E. Schmidt, Hermann J. Girschick, Sabine Heine, Anika-Kerstin Biegner, Annick A. J. M. van de Ven, Stefan Schreiber, J. Merlijn van den Berg, Nurit Assia Batzir, Alexandra Jablonka, Kim Stol, Gregor Dückers, Antonios G.A. Kolios, Ioannis Kakkas, Christian Klemann, Marina N. Guseva, Sofia Grigoriadou, Elif Karakoc-Aydiner, Antonio Marzollo, Peter D. Arkwright, Urs C. Steiner, Sara Sebnem Kilic, Romina Dieli-Crimi, Gergely Kriván, Monika Sparber-Sauer, Marco Cazzaniga, Fulvio Porta, Paraskevi Maggina, Tomas Milota, Robbert G. M. Bredius, Martine Pergent, Klaus Tenbrock, Jana Pachlopnik Schmid, Florentia Dimitriou, Cathal Laurence Steele, Helen Bourne, Anna Bobcakova, Gerd Horneff, Judith Potjewijd, Marc Schmalzing, Tobias Ankermann, Paul Ryan, Oksana Boyarchuk, Necil Kutukculer, Carl Friedrich Classen, Zita Chovancová, Moira Thomas, Cinzia Milito, Michaela Bitzenhofer-Grüber, Faranaz Atschekzei, Eva Hlaváčková, Viviana Moschese, Julie Smet, Hans-Hartmut Peter, Carla Teixeira, Sabine M El-Helou, Suzanne de Kruijf Bazen, Helmut Wittkowski, Donate Jakoby, Marina Garcia-Prat, Esther de Vries, Richard Herriot, Sven Kracker, Alessandro Plebani, Lisa Göschl, Laura Hora Marques, Anna Sediva, Jiri Litzman, Mark M. Gompels, Renate Krüger, Şefika İlknur Kökçü Karadağ, Nadine Binder, Anna Szaflarska, Peter Jandus, Lisa Ibberson, Johann Greil, Ulf Schulze-Sturm, Mehtap Sirin, Aydan Ikinciogullari, Edyta Heropolitańska-Pliszka, Michael E. Weiss, Alla Skapenko, Lukas Wisgrill, Hana Alachkar, Uta Behrends, Silvia Sánchez-Ramón, Maria N. Hatzistilianou, Otilia Petrovicova, Darko Richter, Zoreh Nademi, Jürgen K. Rockstroh, Sohilla Lotfy, Markus G. Seidel, Timothy Ronan Leahy, Audra Blažienė, Translational Immunology Groningen (TRIGR), Paediatric Infectious Diseases / Rheumatology / Immunology, AII - Inflammatory diseases, ARD - Amsterdam Reproduction and Development, University of Zurich, Ehl, Stephan, Thalhammer, J., Kindle, G., Nieters, A., Rusch, S., Seppanen, M. R. J., Fischer, A., Grimbacher, B., Edgar, D., Buckland, M., Mahlaoui, N., Ehl, S., Boztug, K., Brunner, J., Demel, U. F., Forster-Waldl, E., Gasteiger, L. M., Goschl, L., Kojic, M., Schroll, A., Seidel, M. G., Wintergerst, U., Wisgrill, L., Sharapova, S. O., Goffard, J. -C., Kerre, T., Meyts, I., Roosens, F., Smet, J., Haerynck, F., Eric, Z. P., Milenova, V., Gagro, A., Richter, D., Chovancova, Z., Hlavackova, E., Litzman, J., Milota, T., Sediva, A., Elaziz, D. A., Alkady, R. S., El Sayed El Hawary, R., Eldash, A. S., Galal, N., Lotfy, S., Meshaal, S. S., Reda, S. M., Sobh, A., Elmarsafy, A., Brosselin, P., Courteille, V., De Vergnes, N., Kracker, S., Pergent, M., Randrianomenjanahary, P., Ahrenstorf, G., Albert, M. H., Ankermann, T., Atschekzei, F., Baumann, U., Becker, B. C., Behrends, U., Belohradsky, B. H., Biegner, A. -K., Binder, N., Bode, S. F. N., Boesecke, C., Boetticher, B., Borte, M., Borte, S., Classen, C. F., Dirks, J., Duckers, G., El-Helou, S., Ernst, D., Fasshauer, M., Fecker, G., Felgentreff, K., Foell, D., Ghosh, S., Girschick, H. J., Goldacker, S., Graf, N., Graf, D., Greil, J., Hanitsch, L. G., Hauck, F., Heeg, M., Heine, S. I., Henes, J. C., Hoenig, M., Holzer, U., Holzinger, D., Horneff, G., Hundsdoerfer, P., Jablonka, A., Jakoby, D., Joean, O., Kaiser-Labusch, P., Klemann, C., Kobbe, R., Korholz, J., Kramm, C. M., Kruger, R., Landwehr-Kenzel, S., Lehmberg, K., Liese, J. G., Lippert, C. F., Maccari, M. E., Masjosthusmann, K., Meinhardt, A., Metzler, M., Morbach, H., Muller, I., Naumann-Bartsch, N., Neubert, J., Niehues, T., Peter, H. -H., Rieber, N., Ritterbusch, H., Rockstroh, J. K., Roesler, J., Schauer, U., Scheible, R., Schmalzing, M., Schmidt, R. E., Schneider, D. T., Schreiber, S., Schuetz, C., Schulz, A., Schulze-Koops, H., Schulze-Sturm, U., Schuster, V., Schwaneck, E. C., Schwarz, K., Schwarze-Zander, C., Sirin, M., Skapenko, A., Sogkas, G., Sparber-Sauer, M., Speckmann, C., Steinmann, S., Stiehler, S., Tenbrock, K., von Bernuth, H., Warnatz, K., Wasmuth, J. -C., Weiss, M., Witte, T., Wittke, K., Wittkowski, H., Zeuner, R. A., Farmaki, E., Hatzistilianou, M. N., Kakkas, I., Kanariou, M. G., Kapousouzi, A., Liatsis, E., Maggina, P., Papadopoulou-Alataki, E., Raptaki, M., Speletas, M., Tantou, S., Goda, V., Krivan, G., Marodi, L., Abolhassani, H., Aghamohammadi, A., Rezaei, N., Feighery, C., Leahy, T. R., Ryan, P., Batzir, N. A., Garty, B. Z., Tamary, H., Aiuti, A., Amodio, D., Azzari, C., Barzaghi, F., Baselli, L. A., Cancrini, C., Carrabba, M., Cazzaniga, M., Cesaro, S., Chinello, M., Danieli, M. G., Dellepiane, R. M., Fabio, G., Gambineri, E., Lodi, L., Lougaris, V., Marasco, C., Martire, B., Marzollo, A., Milito, C., Moschese, V., Pignata, C., Plebani, A., Porta, F., Quinti, I., Ricci, S., Soresina, A., Tommasini, A., Vacca, A., Vanessa, C., Blaziene, A., Sitkauskiene, B., Gowin, E., Heropolitanska-Pliszka, E., Pietrucha, B., Szaflarska, A., Wiesik-Szewczyk, E., Wolska-Kusnierz, B., Esteves, I., Faria, E., Marques, L. H., Neves, J. F., Silva, S. L., Teixeira, C., Pereira da Silva, S., Capilna, B. R., Guseva, M. N., Shcherbina, A., Bobcakova, A., Ciznar, P., Gabzdilova, J., Jesenak, M., Kapustova, L., Orosova, J., Petrovicova, O., Raffac, S., Kopac, P., Allende, L. M., Antoli, A., Blanch, G. R., Carbone, J., Dieli-Crimi, R., Garcia-Prat, M., Gil-Herrera, J., Gonzalez-Granado, L. I., Agullo, P. L., Olbrich, P., Parra-Martinez, A., Paz-Artal, E., Pleguezuelo, D. E., Rodriguez, N. S., Sanchez-Ramon, S., Santos-Perez, J. L., Solanich, X., Soler-Palacin, P., Gonzalez-Amores, M., Ekwall, O., Fasth, A., Bitzenhofer-Gruber, M., Candotti, F., Dimitriou, F., Heininger, U., Holbro, A., Jandus, P., Kolios, A. G. A., Marschall, K., Schmid, J. P., Posfay-Barbe, K. M., Prader, S., Reichenbach, J., Steiner, U. C., Truck, J., Bredius, R. G., de Kruijf- Bazen, S., de Vries, E., Henriet, S. S. V., Kuijpers, T. W., Potjewijd, J., Rutgers, A., Stol, K., van Aerde, K. J., Van den Berg, J. M., van de Ven, A. A. J. M., Montfrans, J., Aydemir, S., Baris, S., Dogu, F., Ikinciogullari, A., Karakoc-Aydiner, E., Kilic, S. S., Kiykim, A., Kokcu Karadag, S. I., Kutukculer, N., Ocak, S., Unal, E., Boyarchuk, O., Hilfanova, A., Kostyuchenko, L. V., Alachkar, H., Arkwright, P. D., Baxendale, H. E., Bernatoniene, J., Coulter, T. I., Garcez, T., Goddard, S., Gompels, M. M., Grigoriadou, S., Herriot, R., Herwadkar, A., Huissoon, A., Ibberson, L., Nademi, Z., Noorani, S., Parvin, S., Steele, C. L., Thomas, M., Waruiru, C., Yong, P. F. K., and Bourne, H.
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0301 basic medicine ,Male ,Pediatrics ,syndromic ,Sex Factor ,Disease ,registry ,medicine.disease_cause ,Cohort Studies ,0302 clinical medicine ,Primary Immunodeficiency Disease ,inborn error of immunity ,Immunology and Allergy ,warning signs ,Age Factor ,Registries ,Family history ,presenting symptom ,Child ,Primary immunodeficiency ,Granuloma ,autoimmune ,immune dysregulation ,inflammatory ,Adult ,Autoimmune Diseases ,Female ,Humans ,Infections ,Lymphoproliferative Disorders ,Middle Aged ,Primary Immunodeficiency Diseases ,Sex Factors ,Age Factors ,10177 Dermatology Clinic ,Infections/epidemiology ,3. Good health ,Settore MED/02 ,Warning signs ,Lymphoproliferative Disorder ,2723 Immunology and Allergy ,Infection ,Human ,medicine.medical_specialty ,Immunology ,610 Medicine & health ,Malignancy ,primary immunodeficiency ,Autoimmune Disease ,03 medical and health sciences ,Immunity ,Autoimmune Diseases/epidemiology ,medicine ,2403 Immunology ,business.industry ,warning sign ,Common variable immunodeficiency ,Granuloma/epidemiology ,Immune dysregulation ,medicine.disease ,Primary Immunodeficiency Diseases/epidemiology ,030104 developmental biology ,Lymphoproliferative Disorders/epidemiology ,Cohort Studie ,business ,030215 immunology - Abstract
BACKGROUND: Inborn errors of immunity (IEI) are rare diseases, which makes diagnosis a challenge. A better description of the initial presenting manifestations should improve awareness and avoid diagnostic delay. Although increased infection susceptibility is a well-known initial IEI manifestation, less is known about the frequency of other presenting manifestations.OBJECTIVE: We sought to analyze age-related initial presenting manifestations of IEI including different IEI disease cohorts.METHODS: We analyzed data on 16,486 patients of the European Society for Immunodeficiencies Registry. Patients with autoinflammatory diseases were excluded because of the limited number registered.RESULTS: Overall, 68% of patients initially presented with infections only, 9% with immune dysregulation only, and 9% with a combination of both. Syndromic features were the presenting feature in 12%, 4% had laboratory abnormalities only, 1.5% were diagnosed because of family history only, and 0.8% presented with malignancy. Two-third of patients with IEI presented before the age of 6 years, but a quarter of patients developed initial symptoms only as adults. Immune dysregulation was most frequently recognized as an initial IEI manifestation between age 6 and 25 years, with male predominance until age 10 years, shifting to female predominance after age 40 years. Infections were most prevalent as a first manifestation in patients presenting after age 30 years.CONCLUSIONS: An exclusive focus on infection-centered warning signs would have missed around 25% of patients with IEI who initially present with other manifestations.
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- 2021
32. Complex analysis of the national Hereditary angioedema cohort in Slovakia - Identification of 12 novel variants in SERPING1 gene.
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Markocsy A, Hrubiskova K, Hrubisko M, Freiberger T, Grombirikova H, Dolesova L, Slivka Vavrova L, Lohajova Behulova R, Ondrusova M, Banovcin P, Vorcakova K, and Jesenak M
- Abstract
Background: Hereditary angioedema (HAE) is a rare autosomal dominant genetic disease characterised by acute episodes of non-pruritic skin and submucosal swelling caused by increase in vascular permeability., Objective: Here we present the first complex analysis of the National HAE Slovakian cohort with the detection of 12 previously un-published genetic variants in SERPING1 gene., Methods: In patients diagnosed with hereditary angioedema caused by deficiency or dysfunction of C1 inhibitor (C1-INH-HAE) based on clinical manifestation and complement measurements, SERPING1 gene was tested by DNA sequencing (Sanger sequencing/massive parallel sequencing) and/or multiplex ligation-dependent probe amplification for detection of large rearrangements., Results: The Slovakian national cohort consisted of 132 living patients with confirmed HAE. We identified 51 index cases (32 families, 19 sporadic patients/112 adults, 20 children). One hundred seventeen patients had HAE caused by deficiency of C1 inhibitor (C1-INH-HAE-1) and 15 patients had HAE caused by dysfunction of C1 inhibitor (C1-INH-HAE-2). The prevalence of HAE in Slovakia has recently been calculated to 1:41 280 which is higher than average calculated prevalence. The estimated incidence was 1:1360 000. Molecular-genetic testing of the SERPING1 gene found 22 unique causal variants in 26 index cases, including 12 previously undescribed and unreported., Conclusion: The first complex report about epidemiology and genetics of the Slovakian national HAE cohort expands the knowledge of the C1-INH-HAE genetics. Twelve novel causal variants were present in the half of the index cases. A higher percentage of inframe variants comparing to other studies was observed. Heterozygous deletion of exon 3 found in a large C1-INH-HAE-1 family probably causes the dysregulation of the splicing isoforms balance and leads to the decrease of full-length C1-INH level., Competing Interests: AM has received honoraria for lectures (Takeda). 10.13039/100014294KH has received consulting fees (Takeda). MH has received honoraria for lectures (ALK, Pleuran, Stallergenes, Takeda). TF has received speaker honoraria (Takeda). MJ has received consulting fees (Takeda, Glenmark, Zentiva, Pharming); honoraria for lectures, presentations (Takeda, Zentiva, Pharming, 10.13039/100008322CSL Behring); support for attending meetings and/or travel (Takeda, Zentiva); served as principal investigator in clinical trials (Pharming, Takeda, 10.13039/100014935BioCryst, Kalvista) and honoraria for participation on Advisory Boards (10.13039/100008322CSL Behring, Takeda, 10.13039/100014935BioCryst, Kalvista, Pharming). HG, LD, LSV, RLB, MO, PB, KV have no conflict of interests to declare., (© 2024 The Author(s).)
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- 2024
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33. Association Between Cytometric Biomarkers, Clinical Phenotype, and Complications of Common Variable Immunodeficiency.
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Markocsy A, Bobcakova A, Petrovicova O, Kapustova L, Malicherova Jurkova E, Schniederova M, Petriskova J, Cibulka M, Hyblova M, and Jesenak M
- Abstract
Background: Common variable immunodeficiency (CVID) is a heterogeneous group of immune disorders. The patients are classified according to the clinical manifestation with the infection-only phenotype (CVID
inf ) and CVID with immune dysregulation (CVIDid )., Methods: We performed a retrospective clinical analysis of 64 CVID patients (34 males, 53.13%; mean age: 41.4 years; SD: ±21.4 years). We divided the patients into subgroups according to the clinical manifestation (CVIDinf and CVIDid ) and according to B cell phenotypic profiling after performing flow cytometry with the use of the EUROclass classification. We compared clinical manifestations, selected laboratory parameters, and therapy in these groups. All CVIDid patients were tested after the manifestation of complications associated with immune dysregulation and in eight patients during the immunosuppressive treatment (systemic corticosteroids and hydroxychloroquine)., Results: Two-thirds of patients in our cohort had symptoms resulting from immune dysregulation. Almost half of the patients had autoimmune complications. A higher proportion of marginal zone B cells was associated with autoimmune complications. A lower percentage of naïve B cells was connected to autoimmunity, whereas a lower proportion of transitional B cells was associated with rheumatic diseases and splenomegaly. Patients with lymphadenopathy had a higher percentage of double-negative T cells and a lower percentage of switched memory B cells. We performed molecular-genetic testing in 28% (n = 17) of patients and found a causal pathogenic variant in 23.5% (n = 4) of this group., Conclusion: Based on our results, there is an association between specific cytometric parameters, clinical phenotype, and complications of CVID. The use of the subpopulations of B cells can be helpful in the diagnosis of these specific clinical complications in CVID patients and could help to personalise the therapeutic approach., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Markocsy et al.)- Published
- 2024
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34. Identification of a novel RPGR mutation associated with retinitis pigmentosa and primary ciliary dyskinesia in a Slovak family: a case report.
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Kolkova Z, Durdik P, Holubekova V, Durdikova A, Jesenak M, and Banovcin P
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Background: The mutations in the RPGR (retinitis pigmentosa GTPase regulator) gene are the most common cause of X-linked retinitis pigmentosa (XLRP), a rare genetic disorder affecting the photoreceptor cells in the retina. Several reported cases identified this gene as a genetic link between retinitis pigmentosa (RP) and primary ciliary dyskinesia (PCD), characterised by impaired ciliary function predominantly in the respiratory tract. Since different mutations in the same gene can result in various clinical manifestations, it is important to describe a correlation between the gene variant and the observed phenotype., Methods: Two young brothers from a non-consanguineous Slovak family with diagnosed retinal dystrophy and recurrent respiratory infections were examined. Suspected PCD was diagnosed based on a PICADAR questionnaire, nasal nitric oxide analysis, transmission electron microscopy, high-speed video microscopy analysis, and genetic testing., Results: We identified a novel frameshift RPGR mutation NM_001034853: c.309_310insA, p.Glu104Argfs*12, resulting in a complex X-linked phenotype combining PCD and RP. In our patients, this mutation was associated with normal ultrastructure of respiratory cilia, reduced ciliary epithelium, more aciliary respiratory epithelium, shorter cilia, and uncoordinated beating with a frequency at a lower limit of normal beating, explaining the clinical manifestation of PCD in our patients., Conclusion: The identified novel pathogenic mutation in the RPGR gene expands the spectrum of genetic variants associated with the X-linked PCD phenotype overlapping with RP, highlighting the diversity of mutations contributing to the disorder. The described genotype-phenotype correlation can be useful in clinical practice to recognise a broader spectrum of PCD phenotypes as well as for future research focused on the genetic basis of PCD, gene interactions, the pathways implicated in PCD pathogenesis, and the role of RPGR protein for the proper functioning of cilia in various tissues throughout the body., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2024 Kolkova, Durdik, Holubekova, Durdikova, Jesenak and Banovcin.)
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- 2024
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35. The use of ketotifen as long-term anti-inflammatory prophylaxis in children with PFAPA syndrome.
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Kapustova L, Banovcin P, Bobcakova A, Jurkova Malicherova E, Kapustova D, Petrovicova O, Slenker B, Markocsy A, Oleksak F, Vorcakova K, and Jesenak M
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- Child, Humans, Male, Female, Child, Preschool, Infant, Ketotifen therapeutic use, Syndrome, Anti-Inflammatory Agents, Stomatitis, Aphthous drug therapy, Stomatitis, Aphthous diagnosis, Pharyngitis drug therapy, Lymphadenitis drug therapy, Lymphadenopathy
- Abstract
Introduction: Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA) is the most frequent periodic fever syndrome in children. Its pathogenesis is still unknown, but some disease-modifying factors were observed. Several medications were tested for the long-term prophylaxis of inflammatory flares; however, none are standardly used., Methods: This prospective clinical trial enrolled 142 children (71 girls, 50%) meeting diagnostic criteria for PFAPA syndrome. We analysed selected clinical characteristics and compared laboratory parameters during the flare and attack-free period (at least two weeks after the attack). Moreover, we assessed the possible therapeutic effect of ketotifen on the duration of attack free-periods and clinical picture., Results: The mean age of patients was 6.81 ± 3.03 years and the mean age of onset of symptoms was 2.31 ± 2.02 years. No significant differences were observed between genders.We recorded a positive family history for PFAPA in 31.69% of patients. Attacks lasted for 2.8 ± 1.2 days, with intervals between attacks of 4 ± 1 weeks. We administered ketotifen in 111 (77.8%) patients, and a positive effect was observed in 86 (77.5%) of patients. We observed prolonged attack-free intervals in patients treated with ketotifen (14.7 ± 8.9 days in comparison with 4.4 ± 1.9 days before the treatment; p<0.001). The used dose of ketotifen was 0.08 ± 0.01 mg/kg/day. Mild side effects were observed in four patients (restlessness, irritability, agitation and constipation)., Discussion: Our data supports the use of ketotifen for long-term prophylaxis in children with PFAPA syndrome with positive effects on the attenuation of disease activity and the prolongation of attack-free periods. Further well-designed studies should confirm the preliminary data., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision., (Copyright © 2023 Kapustova, Banovcin, Bobcakova, Jurkova Malicherova, Kapustova, Petrovicova, Slenker, Markocsy, Oleksak, Vorcakova and Jesenak.)
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- 2023
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36. Eosinophils-from cradle to grave: An EAACI task force paper on new molecular insights and clinical functions of eosinophils and the clinical effects of targeted eosinophil depletion.
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Jesenak M, Diamant Z, Simon D, Tufvesson E, Seys SF, Mukherjee M, Lacy P, Vijverberg S, Slisz T, Sediva A, Simon HU, Striz I, Plevkova J, Schwarze J, Kosturiak R, Alexis NE, Untersmayr E, Vasakova MK, Knol E, and Koenderman L
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- Humans, Biomarkers, Eosinophils
- Abstract
Over the past years, eosinophils have become a focus of scientific interest, especially in the context of their recently uncovered functions (e.g. antiviral, anti-inflammatory, regulatory). These versatile cells display both beneficial and detrimental activities under various physiological and pathological conditions. Eosinophils are involved in the pathogenesis of many diseases which can be classified into primary (clonal) and secondary (reactive) disorders and idiopathic (hyper)eosinophilic syndromes. Depending on the biological specimen, the eosinophil count in different body compartments may serve as a biomarker reflecting the underlying pathophysiology and/or activity of distinct diseases and as a therapy-driving (predictive) and monitoring tool. Personalized selection of an appropriate therapeutic strategy directly or indirectly targeting the increased number and/or activity of eosinophils should be based on the understanding of eosinophil homeostasis including their interactions with other immune and non-immune cells within different body compartments. Hence, restoring as well as maintaining homeostasis within an individual's eosinophil pool is a goal of both specific and non-specific eosinophil-targeting therapies. Despite the overall favourable safety profile of the currently available anti-eosinophil biologics, the effect of eosinophil depletion should be monitored from the perspective of possible unwanted consequences., (© 2023 European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.)
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- 2023
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37. EUFOREA pocket guide on the diagnosis and management of asthma: An educational and practical tool for general practitioners, non-respiratory physicians, paramedics and patients.
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Diamant Z, Jesenak M, Hanania NA, Heaney LG, Djukanovic R, Ryan D, Quirce S, Backer V, Gaga M, Pavord I, Antolín-Amérigo D, Assaf S, Bakakos P, Bobcakova A, Busse W, Kappen J, Loukides S, van Maaren M, Panzner P, Pite H, Spanevello A, Stenberg H, Striz I, Thio B, Vasakova MK, Conti D, Fokkens W, Lau S, Scadding GK, Van Staeyen E, Hellings PW, and Bjermer L
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- Humans, Paramedics, Educational Status, Patients, General Practitioners, Asthma diagnosis, Asthma therapy
- Abstract
Competing Interests: Declaration of competing interest ZD: received consultancy fees/lecture fees/fees for attending advisory boards from ALK, Antabio, Foresee Pharmaceuticals, GlaxoSmithKline, Hippo-Dx, QPS-Netherlands, Sanofi-Genzyme; she served as Director Respiratory & Allergy at QPS-NL and this CRO received research grants for clinical trials from HAL Allergy, Janssen Research & Development LLC, Patara pharma, Cerbios, Merck Sharp & Dohme, Novartis, Foresee Pharmaceuticals and ERA4TB (IMI-project). MJ: ALK, Stallergenes-Greer, Chiesi, GSK, Pfizer, Novartis, AstraZeneca and SANOFI. NH: Received honoraria for serving as advisor or consultant for GSK, AstraZeneca, Sanofi, Regeneron, Amgen, Genentech, Novartis and Teva. His institution received research grant support of his behalf from GSK, Genentech, Sanofi, Teva, Novartis, and AstraZeneca. LH: Has received grant funding, participated in advisory boards and given lectures at meetings supported by Amgen, AstraZeneca, Boehringer Ingelheim, Chiesi, Circassia, Hoffmann la Roche, GlaxoSmithKline, Novartis, Theravance, Evelo Biosciences, Sanofi, and Teva; he has received grants from MedImmune, Novartis UK, Roche/ Genentech Inc, and Glaxo Smith Kline, Amgen, Genentech/Hoffman la Roche, Astra Zeneca, MedImmune, Glaxo Smith Kline, Aerocrine and Vitalograph; he has received sponsorship for attending international scientific meetings from AstraZeneca, Boehringer Ingelheim, Chiesi, GSK and Napp Pharmaceuticals; he has also taken part in asthma clinical trials sponsored by AstraZeneca, Boehringer Ingelheim, Hoffmann la Roche, and GlaxoSmithKline for which his institution received remuneration; he is the Academic Lead for the Medical Research Council Stratified Medicine UK Consortium in Severe Asthma which involves industrial partnerships with a number of pharmaceutical companies including Amgen, AstraZeneca, Boehringer Ingelheim, GlaxoSmithKline, Hoffmann la Roche, and Janssen. RD: Declares consulting fees from Synairgen, Sanofi and Galapagos, lecture fees from GSK, AZ and Airways Vista and he holds shares from Synairgen. DR: No COIs related to this work. SQ: ALK, Allergy Therapeutics, AstraZeneca, Chiesi, GSK, Leti, Mundipharma, Novartis, Sanofi-Genzyme, Teva. VB: Has worked as advisor, supervisor, investigator of pharmaceutical studies, unrestricted grants, and others with: AstraZeneca, GSK, MSD & Shering Plough, ALK-Abello; Chiesi,Novartis, Pharmaxis, Pfizer, Boehringer Ingelheim, Aerocrine, Teva, Sanofi, Birk NPC as. MG: No COIs related to this work. IP: In the last 5 years IDP has received speaker’s honoraria for speaking at sponsored meetings from Astra Zeneca, Boehringer Ingelheim, Aerocrine, Almirall, Novartis, Teva, Chiesi, Sanofi/Regeneron, Menarini and GSK and payments for organising educational events from AZ, GSK, Sanofi/Regeneron and Teva. He has received honoraria for attending advisory panels with Genentech, Sanofi/ Regeneron, Astra Zeneca, Boehringer Ingelheim, GSK, Novartis, Teva, Merck, Circassia, Chiesi and Knopp and payments to support FDA approval meetings from GSK. He has received sponsorship to attend international scientific meetings from Boehringer Ingelheim, GSK, AstraZeneca, Teva and Chiesi. He has received a grant from Chiesi to support a phase 2 clinical trial in Oxford. DAA: Has received honoraria as grants/contracts from Sociedad Española de Alergología e Inmunología Clínica (SEAIC), as consulting fees from ALK-Abelló, AstraZeneca, Chiesi and Gebro, as speaker from AstraZeneca, Chiesi, Gebro, GSK, Leti Pharma, Mundipharma, Novartis, Roxall, Sanofi. AS: GSK support for an asthma biologic clinical trial 2021-ongoing. PB: Has received honoraria for presentations and consultancy fees from AstraZeneca, Boehringer Ingelheim, Chiesi, ELPEN, GSK, Menarini, Novartis, Sanofi, and Gilead. AB: Takeda, Novartis, Viatris, ALK, Zentiva, MERCK, Stallergenes Greer, Ewopharma, Astra Zeneca, Chiesi, S&D Pharma, Mundipharma, Berlin Chemie. WB: GlaxoSmithKline, Sanofi, Regeneron. JK: Grants and/or personal fees from ALK, Chiesi, GSK, Novartis, AstraZeneca, Sanofi, Boerhinger, Teva, Viatris, Stallergen, Abbot, all outside the submitted work. SL: Honorarium AstraZeneka, GSK, Chiesi Hellas, Sanofi, Elpen, Menarini, Guidoti. MVM: No COIs related to this work. PP: ALK, AstraZeneca, Stallergenes. HP: No COIs related to this work. AS: Reports lecture fees and/or consultancies from AstraZeneca, Chiesi, B.I., GSK, Merck, Novartis, Zambon, Sanofi. HS: No COIs related to this work. IS: No COIs related to this work. BT: No COIs related to this work. MKV: GlaxoSmithKline, AstraZeneca. Diego Conti: No COIs related to this work. WF: The department of Otorhinolaryngology of the Amsterdam University Medical centre, location AMC received grants for research in Rhinology from: ALK, AllergyTherapeutics, Novartis, EU, GSK, MYLAN, Sanofi-Aventis, and Zon-MW; personal COIs: for consultation and/or speaker fees from Dianosic, GSK, Novartis and Sanofi-Aventis/Regeneron. SL: Advisory Board Sanof-Aventis, GSK and Leo-Pharma. Honoraria by DBV Technologies, Allergopharma, Leti, Nutricia, Sanofi-Aventis. GKS: Honoraria for articles, speaker and advisory boards: ALK, AstraZeneca, Capnia, Church & Dwight, Circassia, Noucor, GSK, Meda/Mylan/Viatris, Merck, Sanofi- Regeneron, Stallergenes. Scientific Chief Editor, Rhinology Section, Frontiers in Allergy. Board Member, Lead for Allergic Rhinitis, EUFOREA. Chair of Data Monitoring Board for Paediatric AR trials of HDM SLIT. EVS: No COIs related to this work. PH: Recipient of research grants, honoraria and/or lecture fees of Sanofi, Regeneron, Viatris, GSK, and Novartis. LB: No COIs related to this work.
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38. Asthma in the era of COVID-19.
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Assaf S, Stenberg H, Jesenak M, Tarasevych SP, Hanania NA, and Diamant Z
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- Humans, SARS-CoV-2, Comorbidity, Adrenal Cortex Hormones therapeutic use, COVID-19 epidemiology, COVID-19 complications, Asthma diagnosis
- Abstract
Since its global invasion in 2019, COVID-19 has affected several aspects of patients' lives and posed a significant impact on the health care system. Several patient populations were identified to be at high risk of contracting SARS-CoV-2 infection and/or developing severe COVID-19-related sequelae. Conversely, anyone who has contracted SARS-CoV-2 is at risk to experience symptoms and signs consistent with post-COVID manifestations. Patients with asthma were initially thought to be at increased risk and severity for SARS-CoV-2 infection. However, accumulating evidence demonstrates that asthma endotypes/phenotypes and comorbidities influence the risk stratification in this population. Furthermore, initial concerns about the potentially increased risk of poor outcomes with asthma treatments such as inhaled corticosteroids and biologics have not been substantiated. In this review, we provide an update on COVID-19 and asthma, including risk of susceptibility, clinical manifestations and course in this population as well as discuss recommendations for management., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: SA: Her institution received research support on her behalf as site PI for a GSK clinical trialHS: No COIs related to this workMJ: ALK, Stallergenes-Greer, Chiesi, GSK, Pfizer, Novartis, AstraZeneca and SANOFISPT: No COIs related to this workNAH: Received honoraria for serving as advisor or consultant for GSK, AstraZeneca, Sanofi, Regeneron, Amgen, Genentech, Novartis and Teva. His institution received research grant support of his behalf from GSK, Genentech, Sanofi, Teva, Novartis, and AstraZenecaZD: received consultancy fees/lecture fees/fees for attending advisory boards from ALK, Antabio, Foresee Pharmaceuticals, GlaxoSmithKline, Hippo-Dx, QPS-Netherlands, Sanofi-Genzyme; she served as Director Respiratory & Allergy at QPS-NL and this CRO received research grants for clinical trials from HAL Allergy, Janssen Research & Development LLC, Patara pharma, Cerbios, Merck Sharp & Dohme, Novartis, Foresee Pharmaceuticals and ERA4TB (IMI-project)., (Copyright © 2023. Published by Elsevier Ltd.)
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39. Systematic Approach Revealed SERPING1 Splicing-Affecting Variants to be Highly Represented in the Czech National HAE Cohort.
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Grombirikova H, Bily V, Soucek P, Kramarek M, Hakl R, Ballonova L, Ravcukova B, Ricna D, Kozena K, Kratochvilova L, Sobotkova M, Zachova R, Kuklinek P, Kralickova P, Krcmova I, Hanzlikova J, Vachova M, Krystufkova O, Dankova E, Jesenak M, Novackova M, Svoboda M, Litzman J, and Freiberger T
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- Humans, Czech Republic epidemiology, RNA Splicing, RNA, Messenger, Complement C1 Inhibitor Protein genetics, Angioedemas, Hereditary diagnosis, Angioedemas, Hereditary epidemiology, Angioedemas, Hereditary genetics
- Abstract
Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a rare and life-threatening condition characterized by recurrent localized edema. We conducted a systematic screening of SERPING1 defects in a cohort of 207 Czech patients from 85 families with C1-INH-HAE. Our workflow involved a combined strategy of sequencing extended to UTR and deep intronic regions, advanced in silico prediction tools, and mRNA-based functional assays. This approach allowed us to detect a causal variant in all families except one and to identify a total of 56 different variants, including 5 novel variants that are likely to be causal. We further investigated the functional impact of two splicing variants, namely c.550 + 3A > C and c.686-7C > G using minigene assays and RT-PCR mRNA analysis. Notably, our cohort showed a considerably higher proportion of detected splicing variants compared to other central European populations and the LOVD database. Moreover, our findings revealed a significant association between HAE type 1 missense variants and a delayed HAE onset when compared to null variants. We also observed a significant correlation between the presence of the SERPING1 variant c.-21 T > C in the trans position to causal variants and the frequency of attacks per year, disease onset, as well as Clinical severity score. Overall, our study provides new insights into the genetic landscape of C1-INH-HAE in the Czech population, including the identification of novel variants and a better understanding of genotype-phenotype correlations. Our findings also highlight the importance of comprehensive screening strategies and functional analyses in improving the C1-INH-HAE diagnosis and management., (© 2023. The Author(s).)
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40. Dysfunctional mucociliary clearance in asthma and airway remodeling - New insights into an old topic.
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Jesenak M, Durdik P, Oppova D, Franova S, Diamant Z, Golebski K, Banovcin P, Vojtkova J, and Novakova E
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- Humans, Mucociliary Clearance physiology, Hyperplasia, Inflammation, Airway Remodeling, Asthma drug therapy
- Abstract
Bronchial asthma is a heterogeneous respiratory condition characterized by chronic airway inflammation, airway hyperresponsiveness and airway structural changes (known as remodeling). The clinical symptoms can be evoked by (non)specific triggers, and their intensity varies over time. In the past, treatment was mainly focusing on symptoms' alleviation; in contrast modern treatment strategies target the underlying inflammation, even during asymptomatic periods. Components of airway remodeling include epithelial cell shedding and dysfunction, goblet cell hyperplasia, subepithelial matrix protein deposition, fibrosis, neoangiogenesis, airway smooth muscle cell hypertrophy and hyperplasia. Among the other important, and frequently forgotten aspects of airway remodeling, also loss of epithelial barrier integrity, immune defects in anti-infectious defence and mucociliary clearance (MCC) dysfunction should be pointed out. Mucociliary clearance represents one of the most important defence airway mechanisms. Several studies in asthmatics demonstrated various dysfunctions in MCC - e.g., ciliated cells displaying intracellular disorientation, abnormal cilia and cytoplasmic blebs. Moreover, excessive mucus production and persistent cough are one of the well-recognized features of severe asthma and are also associated with defects in MCC. Damaged airway epithelium and impaired function of the ciliary cells leads to MCC dysfunction resulting in higher susceptibility to infection and inflammation. Therefore, new strategies aimed on restoring the remodeling changes and MCC dysfunction could present a new therapeutic approach for the management of asthma and other chronic respiratory diseases., Competing Interests: Declaration of competing interest No conflict of interest., (Copyright © 2023 Elsevier Ltd. All rights reserved.)
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41. Biologics in severe asthma: A pragmatic approach for choosing the right treatment for the right patient.
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Rogers L, Jesenak M, Bjermer L, Hanania NA, Seys SF, and Diamant Z
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- Adult, Child, Humans, Biomarkers, Phenotype, Precision Medicine, Biological Products therapeutic use, Asthma, Anti-Asthmatic Agents therapeutic use
- Abstract
The development of monoclonal antibody therapies targeting specific components of the pathways relevant to asthma pathophysiology has revolutionized treatment of severe asthma both in adults and children and helped to further unravel the heterogeneity of this disease. However, the availability of multiple agents, often with overlapping eligibility criteria, creates a need for pragmatic guidance for specialists undertaking care of patients with severe asthma. In this review, we provide an overview of the data supporting the clinical efficacy of biologics in distinct asthma phenotypes/endotypes. We also focus on the role of biomarkers and treatable traits, including comorbidities, in the choice of asthma biologics, highlight which treatments have been demonstrated to be steroid sparing in corticosteroid dependent asthma, and provide practical guidance that can drive shared decision making on treatment choice with patients. In addition, we summarize what is known to date regarding long-term safety of these drugs, and lastly, discuss future directions in biologics research., Competing Interests: Declaration of competing interest Dr. Linda Rogers receives research funding including salary support from Sanofi (Global) and Gene DX (formerly Sema 4). She has served on advisory boards and performed consulting work for Astra Zeneca, Sanofi (US) and Teva Pharmaceuticals. She has received travel funding and lecture honoraria from the American College of Chest Physicians. Dr. Sven Seys company receives grants/contracts from Sanofi, Novartis, GSK, and is an employee of Galenus Health and Hippo Dx, and received speakers fee from Teva Pharmaceuticals. Dr. Milos Jenesak has performed consulting for Novartis, Sanofi Genzyme, Astra Zeneca, and GSK, has received payment/honoraria for lectures, presentatinos, speakers bureaus, manuscript writing or educational events from Sanofi Genzyme and Novartis. Dr. Hanania's institution receives grants or contract from Astra Zeneca, GSK, Sanofi, Genentech, and Teva and consulting fees from Astra Zeneca, GSK, Sanofi, Genentech, Teva, and Amgen, andspeaking fees from Regeneron. Dr. Bjermer has no conflicts to declare in relation to this manuscript. Dr. Zuzana Diamant received speaker or consultant honoraria and/or served on advisory boards at: Antabio, Arcede, Foresee Pharmaceuticals, GlaxoSmithKline, Hippo-Dx, QPS-Netherlands, Sanofi-Genzyme-Regeneron, all outside the submitted work. During the last 3 years of her assignment as Research Director Respiratory and Allergy, QPS-Netherlands received European grant from ERA4TB and funding from Foresee Pharmaceuticals for early clinical studies. She serves as associate editor for Allergy and Respiratory Medicine and acts as Chair of the Asthma Expert Panel at EUFOREA. submitted work., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)
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42. Real-world outcomes of mepolizumab treatment in severe eosinophilic asthma patients - retrospective cohort study in Slovakia.
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Jesenak M, Vanecek V, Ondrusova M, Urdova V, Dostalova K, and Hochmuth L
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- Adult, Humans, Retrospective Studies, Slovakia, Adrenal Cortex Hormones therapeutic use, Anti-Asthmatic Agents therapeutic use, Asthma drug therapy, Asthma complications, Eosinophilia complications, Eosinophilia drug therapy
- Abstract
Aims: Mepolizumab, a fully-humanized recombinant IgG1 kappa monoclonal antibody directed against IL-5, has shown improved asthma control and lung function in randomised controlled trials. The aim of this study was to evaluate real-world clinical experience in patients with severe eosinophilic asthma treated with mepolizumab in Slovakia., Methods: A retrospective, non-interventional study based on medical records of all adult asthma patients initiating mepolizumab between November 1, 2017 and January 31, 2019, completing 12 months of treatment. At baseline, general and clinical profile data were recorded 12 months prior to treatment. Primary and secondary endpoints described the results of mepolizumab use at 2, 6, and 12 months after the initiation and compared to baseline. Statistical testing of individual change (in each patient) in selected parameters was performed., Results: The cohort included 17 patients with particularly severe asthma at baseline, with frequent severe exacerbations (SE, median 5 [IQR 4-6]/patient/year), high blood eosinophil counts (median 0.6x10
9 /L), frequent oral corticosteroid (OCS) dependence (82.35%), median dose 15 (IQR 7.5-20) mg/day, impaired lung function, and a spectrum of comorbidities. In a one-year follow-up, the data showed reductions in median SE (0 [IQR 0-1] patient/year, eosinophilia (median 0.175x109 /L) and OCS maintenance dose (median 6.25 [IQR 2.5-20] mg/day), all statistically significant after 12 months on mepolizumab. Improved and stabilised lung functions throughout the cohort and a reduced incidence of nasal polyposis were observed., Conclusions: The results provide clinical evidence of mepolizumab efficacy in a real sample of patients with severe asthma when administered in routine care settings in Slovakia., Competing Interests: The authors report no conflicts of interest in this work.- Published
- 2023
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43. A EUFOREA comment on a lost comorbidity of asthma.
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Conti DM, Hellings PW, Diamant Z, Bjermer L, Jesenak M, Backer V, Fokkens W, Lau S, Van Staeyen E, and Scadding GK
- Abstract
"Epidemiology of comorbidities and their association with asthma control" (Tomisa, G., Horváth, A., Sánta, B. et al. Epidemiology of comorbidities and their association with asthma control. Allergy Asthma Clin Immunol 17, 95 (2021). https://doi.org/10.1186/s13223-021-00598-3 ) is an interesting paper reflecting data collection from more than 12,000 asthmatic patients in Hungary regarding their condition and associated comorbidities. We found it valuable that the paper provides an overview of asthma comorbidities not usually considered in similar reports. Nevertheless, we believe that chronic rhinosinusitis (CRS) with or without nasal polyps (CRSwNP or CRSsNP) should have been listed due to its high incidence and prevalence, its association with asthma which is also endorsed in both GINA and EPOS, as well as in several peer-reviewed scientific papers, and to reflect the role of this comorbidity in poor control and a most severe presentation of asthma for the patient. Consequently, several targeted therapies (especially monoclonal antibodies) used for several years in severe forms of asthma are now indicated also for the effective treatment of nasal polyps., (© 2023. The Author(s).)
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44. New insights into the pathophysiology and therapeutic targets of asthma and comorbid chronic rhinosinusitis with or without nasal polyposis.
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Striz I, Golebski K, Strizova Z, Loukides S, Bakakos P, Hanania NA, Jesenak M, and Diamant Z
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- Humans, Comorbidity, Chronic Disease, Inflammation drug therapy, Nasal Polyps complications, Nasal Polyps drug therapy, Asthma complications, Asthma drug therapy, Asthma epidemiology, Sinusitis complications, Sinusitis drug therapy, Rhinitis complications, Rhinitis drug therapy, Biological Products therapeutic use
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Asthma and chronic rhinosinusitis with nasal polyps (CRSwNP) or without (CRSsNP) are chronic respiratory diseases. These two disorders often co-exist based on common anatomical, immunological, histopathological, and pathophysiological basis. Usually, asthma with comorbid CRSwNP is driven by type 2 (T2) inflammation which predisposes to more severe, often intractable, disease. In the past two decades, innovative technologies and detection techniques in combination with newly introduced targeted therapies helped shape our understanding of the immunological pathways underlying inflammatory airway diseases and to further identify several distinct clinical and inflammatory subsets to enhance the development of more effective personalized treatments. Presently, a number of targeted biologics has shown clinical efficacy in patients with refractory T2 airway inflammation, including anti-IgE (omalizumab), anti-IL-5 (mepolizumab, reslizumab)/anti-IL5R (benralizumab), anti-IL-4R-α (anti-IL-4/IL-13, dupilumab), and anti-TSLP (tezepelumab). In non-type-2 endotypes, no targeted biologics have consistently shown clinical efficacy so far. Presently, multiple therapeutical targets are being explored including cytokines, membrane molecules and intracellular signalling pathways to further expand current treatment options for severe asthma with and without comorbid CRSwNP. In this review, we discuss existing biologics, those under development and share some views on new horizons., (© 2023 The Author(s).)
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45. Unusual Cause of Thrombocytopenia and Renal Failure in a 14-Year-Old Boy (MYH9-Associated Disorders).
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Granak K, Brndiarova M, Vnucak M, Plamenova I, Lohajova RB, Valencikova R, Jesenak M, and Dedinska I
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MYH9-associated disorders represent rare group of autosomal dominant diseases and are caused by pathogenic mutations in the MYH9 gene. Clinically, they are represented by macro-platelet-thrombocytopenia, various degrees of renal dysfunction, hearing loss, and early onset cataracts. We describe the case of 14-year-old boy in medical follow-up from birth for thrombocytopenia. Systolic hypertension and nephrotic proteinuria were detected at preventive health check. Renal biopsy revealed sing of segmental glomerulosclerosis. Dialysis treatment was needed. Before transplantation due to the finding of chronic tonsillitis with positive bacterial capture in the culture examination, tonsillectomy was indicated. Postoperative period was complicated with arterial post-tonsillectomy hemorrhage. Six months after tonsillectomy, the patient underwent primary deceased-donor kidney transplantation without complication. Blood platelets showed fluctuating character in the zone of severe thrombocytopenia. However, no signs of bleeding were present. Three months after successful transplantation gene sequencing of whole exon was performed. The presence of the variant c.2105G>A [p.(Arg702HIS)] in exon 17 of the MYH9 gene has been detected. The variant c.2105G>A may be clinically manifested by progressive proteinuria with rapid deterioration of renal function. This case is an example of the delayed diagnosis of rare disease and highlights the usefulness of genetic testing., Competing Interests: The authors have no conflicts of interest to declare., (© 2023 The Author(s). Published by S. Karger AG, Basel.)
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46. Editorial: Definition of the immune parameters related to COVID-19 severity.
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Tincati C, de Vries R, Jesenak M, and Marchetti G
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- Humans, COVID-19 immunology
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Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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- 2023
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47. Allergen immunotherapy for allergic asthma: The future seems bright.
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Diamant Z, van Maaren M, Muraro A, Jesenak M, and Striz I
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- Animals, Humans, Desensitization, Immunologic, Allergens, Pollen, Antigens, Dermatophagoides therapeutic use, Pyroglyphidae, Asthma etiology, Rhinitis, Allergic drug therapy, Sublingual Immunotherapy
- Abstract
Allergen specific immunotherapy (AIT) is the only causal therapeutic option for allergic airway diseases including asthma and allergic rhinitis. AIT has been shown to restore the allergen immune tolerance, can modify both the early and late-onset allergen-specific airway hyperreactivity, helps to achieve disease control/remission and prevents new sensitisations. Recent real life data on long-term effectiveness of house dust mite (HDM) AIT in a large group of patients with HDM-driven asthma further underscored its unique therapeutic potential as well as confirmed previous data with pollen AIT. More widespread use of this causal treatment in select patient populations should further move this promising therapeutic field. In this mini-review, we discuss updates on new insights based on real world patient data., (Copyright © 2023 Elsevier Ltd. All rights reserved.)
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48. Nonpharmaceutical interventions reduce the incidence and mortality of COVID-19: A study based on the survey from the International COVID-19 Research Network (ICRN).
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Park SH, Hong SH, Kim K, Lee SW, Yon DK, Jung SJ, Abdeen Z, Ghayda RA, Ahmed MLCB, Serouri AA, Al-Herz W, Al-Shamsi HO, Ali S, Ali K, Baatarkhuu O, Nielsen HB, Bernini-Carri E, Bondarenko A, Cassell A, Cham A, Chua MLK, Dadabhai S, Darre T, Davtyan H, Dragioti E, East B, Edwards RJ, Ferioli M, Georgiev T, Ghandour LA, Harapan H, Hsueh PR, Mallah SI, Ikram A, Inoue S, Jacob L, Janković SM, Jayarajah U, Jesenak M, Kakodkar P, Kapata N, Kebede Y, Khader Y, Kifle M, Koh D, Maleš VK, Kotfis K, Koyanagi A, Kretchy JP, Lakoh S, Lee J, Lee JY, Mendonça MDLL, Ling L, Llibre-Guerra J, Machida M, Makurumidze R, Memish ZA, Mendoza I, Moiseev S, Nadasdy T, Nahshon C, Ñamendys-Silva SA, Yongsi BN, Nicolasora AD, Nugmanova Z, Oh H, Oksanen A, Owopetu O, Ozguler ZO, Parperis K, Perez GE, Pongpirul K, Rademaker M, Radojevic N, Roca A, Rodriguez-Morales AJ, Roshi E, Saeed KMI, Sah R, Sakakushev B, Sallam DE, Sathian B, Schober P, Ali PSS, Simonović Z, Singhal T, Skhvitaridze N, Solmi M, Subbaram K, Tizaoui K, Tlhakanelo JT, Torales J, Torres-Roman JS, Tsartsalis D, Tsolmon J, Vieira DN, Rosa SGV, Wanghi G, Wollina U, Xu RH, Yang L, Zia K, Zildzic M, Il Shin J, and Smith L
- Subjects
- Humans, SARS-CoV-2, Incidence, Cross-Sectional Studies, Quarantine, COVID-19 epidemiology
- Abstract
The recently emerged novel coronavirus, "severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2)," caused a highly contagious disease called coronavirus disease 2019 (COVID-19). It has severely damaged the world's most developed countries and has turned into a major threat for low- and middle-income countries. Since its emergence in late 2019, medical interventions have been substantial, and most countries relied on public health measures collectively known as nonpharmaceutical interventions (NPIs). We aimed to centralize the accumulative knowledge of NPIs against COVID-19 for each country under one worldwide consortium. International COVID-19 Research Network collaborators developed a cross-sectional online survey to assess the implications of NPIs and sanitary supply on the incidence and mortality of COVID-19. The survey was conducted between January 1 and February 1, 2021, and participants from 92 countries/territories completed it. The association between NPIs, sanitation supplies, and incidence and mortality were examined by multivariate regression, with the log-transformed value of population as an offset value. The majority of countries/territories applied several preventive strategies, including social distancing (100.0%), quarantine (100.0%), isolation (98.9%), and school closure (97.8%). Individual-level preventive measures such as personal hygiene (100.0%) and wearing facial masks (94.6% at hospitals; 93.5% at mass transportation; 91.3% in mass gathering facilities) were also frequently applied. Quarantine at a designated place was negatively associated with incidence and mortality compared to home quarantine. Isolation at a designated place was also associated with reduced mortality compared to home isolation. Recommendations to use sanitizer for personal hygiene reduced incidence compared to the recommendation to use soap. Deprivation of masks was associated with increased incidence. Higher incidence and mortality were found in countries/territories with higher economic levels. Mask deprivation was pervasive regardless of economic level. NPIs against COVID-19 such as using sanitizer, quarantine, and isolation can decrease the incidence and mortality of COVID-19., (© 2022 Wiley Periodicals LLC.)
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49. Care of patients with inborn errors of immunity in thirty J Project countries between 2004 and 2021.
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Abolhassani H, Avcin T, Bahceciler N, Balashov D, Bata Z, Bataneant M, Belevtsev M, Bernatowska E, Bidló J, Blazsó P, Boisson B, Bolkov M, Bondarenko A, Boyarchuk O, Bundschu A, Casanova JL, Chernishova L, Ciznar P, Csürke I, Erdős M, Farkas H, Fomina DS, Galal N, Goda V, Guner SN, Hauser P, Ilyina NI, Iremadze T, Iritsyan S, Ismaili-Jaha V, Jesenak M, Kelecic J, Keles S, Kindle G, Kondratenko IV, Kostyuchenko L, Kovzel E, Kriván G, Kuli-Lito G, Kumánovics G, Kurjane N, Latysheva EA, Latysheva TV, Lázár I, Markelj G, Markovic M, Maródi L, Mammadova V, Medvecz M, Miltner N, Mironska K, Modell F, Modell V, Mosdósi B, Mukhina AA, Murdjeva M, Műzes G, Nabieva U, Nasrullayeva G, Naumova E, Nagy K, Onozó B, Orozbekova B, Pac M, Pagava K, Pampura AN, Pasic S, Petrosyan M, Petrovic G, Pocek L, Prodeus AP, Reisli I, Ress K, Rezaei N, Rodina YA, Rumyantsev AG, Sciuca S, Sediva A, Serban M, Sharapova S, Shcherbina A, Sitkauskiene B, Snimshchikova I, Spahiu-Konjusha S, Szolnoky M, Szűcs G, Toplak N, Tóth B, Tsyvkina G, Tuzankina I, Vlasova E, and Volokha A
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- Infant, Newborn, Humans, Administration, Intravenous, Educational Status, Egypt, Europe, Immunoglobulin G
- Abstract
Introduction: The J Project (JP) physician education and clinical research collaboration program was started in 2004 and includes by now 32 countries mostly in Eastern and Central Europe (ECE). Until the end of 2021, 344 inborn errors of immunity (IEI)-focused meetings were organized by the JP to raise awareness and facilitate the diagnosis and treatment of patients with IEI., Results: In this study, meeting profiles and major diagnostic and treatment parameters were studied. JP center leaders reported patients' data from 30 countries representing a total population of 506 567 565. Two countries reported patients from JP centers (Konya, Turkey and Cairo University, Egypt). Diagnostic criteria were based on the 2020 update of classification by the IUIS Expert Committee on IEI. The number of JP meetings increased from 6 per year in 2004 and 2005 to 44 and 63 in 2020 and 2021, respectively. The cumulative number of meetings per country varied from 1 to 59 in various countries reflecting partly but not entirely the population of the respective countries. Altogether, 24,879 patients were reported giving an average prevalence of 4.9. Most of the patients had predominantly antibody deficiency (46,32%) followed by patients with combined immunodeficiencies (14.3%). The percentages of patients with bone marrow failure and phenocopies of IEI were less than 1 each. The number of patients was remarkably higher that those reported to the ESID Registry in 13 countries. Immunoglobulin (IgG) substitution was provided to 7,572 patients (5,693 intravenously) and 1,480 patients received hematopoietic stem cell therapy (HSCT). Searching for basic diagnostic parameters revealed the availability of immunochemistry and flow cytometry in 27 and 28 countries, respectively, and targeted gene sequencing and new generation sequencing was available in 21 and 18 countries. The number of IEI centers and experts in the field were 260 and 690, respectively. We found high correlation between the number of IEI centers and patients treated with intravenous IgG (IVIG) (correlation coefficient, cc, 0,916) and with those who were treated with HSCT (cc, 0,905). Similar correlation was found when the number of experts was compared with those treated with HSCT. However, the number of patients treated with subcutaneous Ig (SCIG) only slightly correlated with the number of experts (cc, 0,489) and no correlation was found between the number of centers and patients on SCIG (cc, 0,174)., Conclusions: 1) this is the first study describing major diagnostic and treatment parameters of IEI care in countries of the JP; 2) the data suggest that the JP had tremendous impact on the development of IEI care in ECE; 3) our data help to define major future targets of JP activity in various countries; 4) we suggest that the number of IEI centers and IEI experts closely correlate to the most important treatment parameters; 5) we propose that specialist education among medical professionals plays pivotal role in increasing levels of diagnostics and adequate care of this vulnerable and still highly neglected patient population; 6) this study also provides the basis for further analysis of more specific aspects of IEI care including genetic diagnostics, disease specific prevalence, newborn screening and professional collaboration in JP countries., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Abolhassani, Avcin, Bahceciler, Balashov, Bata, Bataneant, Belevtsev, Bernatowska, Bidló, Blazsó, Boisson, Bolkov, Bondarenko, Boyarchuk, Bundschu, Casanova, Chernishova, Ciznar, Csürke, Erdős, Farkas, Fomina, Galal, Goda, Guner, Hauser, Ilyina, Iremadze, Iritsyan, Ismaili-Jaha, Jesenak, Kelecic, Keles, Kindle, Kondratenko, Kostyuchenko, Kovzel, Kriván, Kuli-Lito, Kumánovics, Kurjane, Latysheva, Latysheva, Lázár, Markelj, Markovic, Maródi, Mammadova, Medvecz, Miltner, Mironska, Modell, Modell, Mosdósi, Mukhina, Murdjeva, Műzes, Nabieva, Nasrullayeva, Naumova, Nagy, Onozó, Orozbekova, Pac, Pagava, Pampura, Pasic, Petrosyan, Petrovic, Pocek, Prodeus, Reisli, Ress, Rezaei, Rodina, Rumyantsev, Sciuca, Sediva, Serban, Sharapova, Shcherbina, Sitkauskiene, Snimshchikova, Spahiu-Konjusha, Szolnoky, Szűcs, Toplak, Tóth, Tsyvkina, Tuzankina, Vlasova and Volokha.)
- Published
- 2022
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50. Beta-(1,3/1,6)-D-glucan from Pleurotus ostreatus in the prevention of recurrent respiratory tract infections: An international, multicentre, open-label, prospective study.
- Author
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Rennerova Z, Picó Sirvent L, Carvajal Roca E, Paśnik J, Logar M, Milošević K, Majtan J, and Jesenak M
- Abstract
Preschool children are particularly susceptible to recurrent upper and lower respiratory tract infections due to their immune immaturity and other contributing factors. Preventing and/or treating children suffering from recurrent respiratory tract infections (RRTIs) is challenging, and it is important to provide more clinical evidence about the safety and efficacy of natural immunomodulating preparations, including β-glucans. The aim of the present study was to assess the incidence of respiratory tract infections (RTIs) in children with a history of RRTIs for a period of 6 months (3 months of pleuran supplementation and 3 months of follow-up) compared with the same period from October to March of the previous year prior to enrolment in the study. A total of 1,030 children with a mean age of 3.49 ± 1.91 years from seven countries were included in this study. The total number of RTIs observed during the study period was significantly lower compared to the same period of the previous year (7.07 ± 2.89 vs. 3.87 ± 3.19; p < 0.001). Analysis of each type of RTI revealed significant reductions in the mean number and duration of infections for all RTI subtypes compared to the previous year. This study also confirmed the beneficial safety profile of pleuran supplementation. In conclusion, pleuran supplementation represents an interesting and prospective supplement in preventing respiratory infections and reveals new strategies for supporting immune functions in the paediatric population., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2022 Rennerova, Picó Sirvent, Carvajal Roca, Paśnik, Logar, Milošević, Majtan and Jesenak.)
- Published
- 2022
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