Publications by authors named "Marek Niedoszytko"

Mastocytosis is a heterogeneous group of disorders, characterized by accumulation of clonal mast cells which can infiltrate several organs, most often spine (70%). The pathogenesis of mastocytosis bone disease is poorly understood. The main aim of the study was to investigate whether neoplastic mast cells may be the source of sclerostin and whether there is an association between sclerostin and selected bone remodeling markers with mastocytosis related bone disease.

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  • - Patient-reported outcome measures (PROMs) are tools that help assess a patient's health at a specific time and are important for personalized healthcare.
  • - The paper reviews the use of visual analog scales (VAS) for measuring asthma and allergic rhinitis control, particularly focusing on the MASK-air app, which includes electronic VAS forms.
  • - A randomized controlled trial was conducted to validate the electronic VAS, and further evaluations confirmed the effectiveness of VAS questions and additional medication scores for allergic asthma symptoms.
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  • - Mastocytosis is a disorder characterized by an abnormal increase of mast cells in one or more organs, with varying symptoms and outcomes; it's classified into cutaneous, systemic, and MC sarcoma types by the WHO.
  • - The disease often involves a specific mutation (D816V) in most systemic mastocytosis cases, affecting life expectancy significantly based on the type, with those having nonadvanced forms generally living near-normal lifespans, while advanced forms have limited life expectancies.
  • - Recently, a group of experts proposed updated diagnostic criteria and classifications for mastocytosis, aiming to unify different classifications from previous organizations to improve research and clinical comparisons.
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Aims: We aimed to analyse serious cardiac adverse drug reactions to COVID-19 vaccines from the Europe-wide EudraVigilance database.

Methods And Results: In this retrospective, cross-sectional study, the EudraVigilance database was searched to identify suspected serious cardiac post-vaccination adverse drug reactions to COVID-19 vaccines. This data was coupled with the number of total vaccine doses administered in the European Economic Area for Comirnaty (Pfizer BioNTech), Spikevax (Moderna), Vaxzevria (AstraZeneca), Jcovden (Janssen), Nuvaxovid (Novavax), products, available from the European Centre for Disease Prevention and Control 'Vaccine Tracker' database.

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Asthma and chronic obstructive pulmonary disease (COPD) are among the most common chronic respiratory diseases. Chronic inflammation of the airways leads to an increased production of inflammatory markers by the effector cells of the respiratory tract and lung tissue. These biomarkers allow the assessment of physiological and pathological processes and responses to therapeutic interventions.

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Article Synopsis
  • - The traditional healthcare approach often overlooks patients' personal experiences and strengths, focusing mainly on disease treatment. Person-centered care aims to align medical decisions with individual values and preferences, particularly for those with chronic conditions.
  • - This paper seeks to enhance care for rhinitis and asthma by developing digital care pathways and incorporating real-world evidence to create a more patient-centered approach.
  • - Key components of the review include advancements in mHealth, the integration of artificial intelligence, a novel classification system for airway diseases, and proposals for the ARIA 2024 guidelines, all targeting a sustainable and applicable healthcare model.
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Background: The coronavirus disease 2019 (COVID-19) pandemic presented a new challenge in modern medicine: the development of vaccines was followed by massive population vaccinations. A few reports on post-vaccination allergic reactions have made patients and medical personnel uneasy as to COVID-19 vaccines' allergic potential. Most of the studies in this area to date have been small, and some that were based on global databases skipped most of the allergic diseases and concentrated only on anaphylaxis.

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Allergen immunotherapy (AIT) is a well-established and efficient method of causative treatment for allergic rhinitis, asthma and insect venom allergy. Traditionally, a recent history of malignant neoplasm is regarded as a contraindication to AIT due to concerns that AIT might stimulate tumor growth. However, there are no data confirming that the silencing of the Th2 response affects prognosis in cancer.

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Introduction: Diagnostic of aspirin (ASA) hypersensitivity is largely based on provocation tests. However, they have significant limitations including influence of medications, necessity of hospitalization, and safety issues. Basophil activation test (BAT) seems to be a promising in vitro alternative.

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Rationale: It is unclear how each individual asthma symptom is associated with asthma diagnosis or control.

Objectives: To assess the performance of individual asthma symptoms in the identification of patients with asthma and their association with asthma control.

Methods: In this cross-sectional study, we assessed real-world data using the MASK-air app.

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  • Hypersensitivity reactions (HR) are common in mastocytosis, a condition analyzed using data from the European Competence Network on Mastocytosis (ECNM), involving 2485 adults.
  • About 38.1% of patients reported HR, with Hymenoptera venoms being the primary trigger for cutaneous mastocytosis and indolent systemic mastocytosis, while drug reactions were more common in advanced systemic mastocytosis.
  • Key risk factors for HR include lower tryptase levels, minimal mast cell infiltration in bone marrow, and a diagnosis of indolent systemic mastocytosis, with new reactions occurring in 4.8% of patients over four years.
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Certain laboratory abnormalities correlate with subvariants of systemic mastocytosis (SM) and are often prognostically relevant. To assess the diagnostic and prognostic value of individual serum chemistry parameters in SM, 2607 patients enrolled within the European Competence Network on Mastocytosis and 575 patients enrolled within the German Registry on Eosinophils and Mast Cells were analyzed. For screening and diagnosis of SM, tryptase was identified as the most specific serum parameter.

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Background: In allergic rhinitis and asthma, adolescents and young adult patients are likely to differ from older patients. We compared adolescents, young adults and adults on symptoms, control levels, and medication adherence.

Methods: In a cross-sectional study (2015-2022), we assessed European users of the MASK-air mHealth app of three age groups: adolescents (13-18 years), young adults (18-26 years), and adults (>26 years).

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Background: Mastocytosis is a rare neoplastic disease of the bone marrow associated with the proliferation and accumulation of mast cells in various internal organs, including the gastrointestinal tract. There are few studies describing the gut microbiome of patients with mastocytosis using next generation sequencing supported using traditional culture methods. The aims of the study were, firstly, the determination of nutrition habits, composition of the intestinal microflora and BMI in mastocytosis, and secondly, analysis of mastocytosis severity and symptoms depending on the composition of the intestinal microflora.

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Introduction: COVID-19 vaccines became a relevant element of prevention during COVID-19 pandemic. It is worth highlighting the importance of severe allergic post-vaccination reactions.

Aim: To evaluate the usability of skin reaction tests using skin prick tests with Comirnaty (Pfizer, USA) vaccine in risk detection of the post-vaccine immediate hypersensitivity reaction (anaphylaxis) after administration of this vaccine [PvIHR(A)].

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  • - Mastocytosis is a disease linked to mast cells that can vary in severity, with the presence of the KIT p. D816V mutation playing a key role in its development, alongside potentially significant epigenetic factors that influence gene expression.
  • - The study aimed to investigate differences in DNA methylation profiles between patients with indolent systemic mastocytosis (ISM) and healthy individuals, analyzing blood samples to identify regions of differing methylation (DMRs) that could indicate disease progression and management strategies.
  • - Through analysis using a MethylationEPIC BeadChip kit and statistical methods like Limma, researchers assessed co-methylated regions to establish potential epigenetic predictors that could aid in understanding and treating mast
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Drug hypersensitivity reactions can be classified as immediate or delayed. While diagnostic options for immediate reactions are well developed and standardized, delayed reactions (in many cases type IV according to Gell and Coombs) are a challenge for allergy work-up. In recent years, some in vitro markers have been proposed and used for delayed reactions, such as contact dermatitis.

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Physiological levels of basal serum tryptase vary among healthy individuals, depending on the numbers of mast cells, basal secretion rate, copy numbers of the TPSAB1 gene encoding alpha tryptase, and renal function. Recently, there has been a growing debate about the normal range of tryptase because individuals with the hereditary alpha tryptasemia (HαT) trait may or may not be symptomatic, and if symptomatic, uncertainty exists as to whether this trait directly causes clinical phenotypes or aggravates certain conditions. In fact, most HαT-positive cases are regarded as asymptomatic concerning mast cell activation.

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  • The EQ-5D-5L is a health-related quality-of-life tool with five domains, and this study investigates how rhinitis and asthma control affect these domains.
  • Data from the MASK-air mHealth app (2015-2021) was used to evaluate the relationship between rhinitis/asthma control and EQ-5D-5L levels through visual analog scales and a symptom-medication score.
  • Findings reveal that poor control of rhinitis and asthma worsens quality of life, particularly in pain/discomfort, activity levels, anxiety/depression, and mobility.
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Biomarkers for the diagnosis, treatment and follow-up of patients with rhinitis and/or asthma are urgently needed. Although some biologic biomarkers exist in specialist care for asthma, they cannot be largely used in primary care. There are no validated biomarkers in rhinitis or allergen immunotherapy (AIT) that can be used in clinical practice.

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Background: Validated questionnaires are used to assess asthma control over the past 1-4 weeks from reporting. However, they do not adequately capture asthma control in patients with fluctuating symptoms. Using the Mobile Airways Sentinel Network for airway diseases (MASK-air) app, we developed and validated an electronic daily asthma control score (e-DASTHMA).

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In 2002, the European Competence Network on Mastocytosis (ECNM) was launched as a multidisciplinary collaborative initiative to increase the awareness and to improve diagnosis and management of patients with mast cell (MC) disorders. The ECNM consists of a net of specialized centers, expert physicians, and scientists who dedicate their work to MC diseases. One essential aim of the ECNM is to timely distribute all available information about the disease to patients, doctors, and scientists.

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Asthma is a heterogeneous chronic disorder of the airways, with inflammation and bronchial hyperresponsiveness as its major underlying phenomena. Asthmatics vary in terms of inflammation pattern, concomitant pathologies, and factors aggravating the course of the disease. As a result, there is a need for sensitive and specific biomarkers that could facilitate diagnosing asthma as well as phenotyping in everyday practice.

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