The Pulmonary Manifestation of Mastocytosis: Experiences of the National Reference Centre of Excellence.

Patients with mastocytosis may present with exacerbated respiratory symptoms and lung diseases resulting from mast cell mediator release. However, their prevalence and severity level remain under debate. The study aims to analyze the prevalence of respiratory symptoms and the usefulness of lung function tests like spirometry, diffusing capacity of the lung for carbon monoxide (DLCO), and high-resolution computed tomography (HRCT) of the chest in mastocytosis patients presenting with dyspnea, cough, and exercise intolerance.

Comprehensive analyses of immune activity in COVID-19-vaccinated idiopathic pulmonary fibrosis patients.

Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal disease, characterized by impaired wound repair, tissue remodeling and fibrosis. Immune system may participate in the development and progression of the disease as indicated by altered activity in IPF sufferers. This study investigates the immune response to the BNT162b2 COVID-19 vaccine in patients with IPF compared to healthy controls, with a particular focus on evaluation of antibody responses, interferon-gamma release, cytokine profiling and a broad panel of immune cell subpopulations.

Catamenial Pneumothorax-Still an Unveiled Disease.

This review presents current opinions on an uncommon condition called catamenial pneumothorax (CP), which is usually associated with thoracic endometriosis syndrome (TES). TES is characterized by the presence of endometriotic lesions in pleura and lung parenchyma and presents with various clinical signs and symptoms, including catamenial pneumothorax. Their diagnosis is often delayed.

Hyper eosinophilic diseases - diagnosis and therapeutic approach - practical position of the Polish working group.

Hypereosinophilic diseases represent a heterogeneous group that poses a significant medical challenge. An increased number of eosinophils in the blood can be a dominant symptom of hematologic diseases, such as myeloid hematopoietic cancers or lymphomas. However, it is more commonly associated with other health conditions, such as allergies, infections, and systemic inflammatory diseases.

Co-existence of ANCA-associated vasculitides with immune-mediated diseases: a single-center observational study.

Background: Antineutrophil cytoplasmic antibody-associated vasculitides (AAV) is a group of systemic necrotizing small vessel autoimmune diseases, with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) being the two most common. The co-existence of AAV with different immune-mediated diseases (autoimmune disesases - AID) might affect the clinical presentation of the primary disease. The purpose of the study was to assess the co-existence of AAV with AID and to investigate whether it affects the characteristics and the course of AAV.

Venom Immunotherapy Does Not Affect Survival of Patients with Malignant Tumor in Poland.

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.

The influence of nutritional habits, body mass index and intestinal microbiota in mastocytosis on clinical symptoms using conventional culture and next generation sequencing.

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.

Characteristics of Interstitial Pneumonia With Autoimmune Features (IPAF): Protocol for a Multicenter Prospective Study.

Background: "Interstitial lung disease" (ILD) is a broad term encompassing diseases of different backgrounds. "Interstitial pneumonia with autoimmune features" (IPAF) is a recent term that implies the presence of autoimmunity.

Objective: This study aims to determine the characteristics of Polish patients with IPAF, compare them with patients with other interstitial pneumonias, and search for the prognostic and diagnostic biomarkers of IPAF in serum and bronchoalveolar lavage fluid (BALF).

Polish Adaptation and Psychometric Validation of the PREM-C9 Questionnaire for Patients with Chronic Obstructive Pulmonary Disease.

: Chronic obstructive pulmonary disease (COPD) is a common, preventable, and treatable disease. The first PREM (patient-reported outcome measure)-type questionnaire that has been dedicated to assess the experience of care in COPD is the PREM-C9. : The aim of this study was to create a Polish version of the PREM-C9 and determine its psychometric characteristics.

Value-Based Integrated Care (VBIC) Concept Implementation in a Real-World Setting-Problem-Based Analysis of Barriers and Challenges.

Healthcare effectiveness measurement and value in health have been common topics in public health literature since 2006 when value-based healthcare (VBHC) was first defined by Porter and Teisberg. The aim of this study was to identify the barriers and challenges related to the implementation of VBHC solutions in the example of Poland. A case presentation was used as a method.

Inhaled pirfenidone solution (AP01) for IPF: a randomised, open-label, dose-response trial.

Introduction: Oral pirfenidone reduces lung function decline and mortality in patients with idiopathic pulmonary fibrosis (IPF). Systemic exposure can have significant side effects, including nausea, rash, photosensitivity, weight loss and fatigue. Reduced doses may be suboptimal in slowing disease progression.

Development and validation of an electronic daily control score for asthma (e-DASTHMA): a real-world direct patient data study.

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).

Digitally-enabled, patient-centred care in rhinitis and asthma multimorbidity: The ARIA-MASK-air approach.

MASK-air , a validated mHealth app (Medical Device regulation Class IIa) has enabled large observational implementation studies in over 58,000 people with allergic rhinitis and/or asthma. It can help to address unmet patient needs in rhinitis and asthma care. MASK-air is a Good Practice of DG Santé on digitally-enabled, patient-centred care.

Comprehensive care for people living with heart failure and chronic obstructive pulmonary disease-Integration of palliative care with disease-specific care: From guidelines to practice.

Heart failure (HF) and chronic obstructive pulmonary disease (COPD) are the leading global epidemiological, clinical, social, and economic burden. Due to similar risk factors and overlapping pathophysiological pathways, the coexistence of these two diseases is common. People with severe COPD and advanced chronic HF (CHF) develop similar symptoms that aggravate if evoking mechanisms overlap.

The role of the clinical and psychological symptoms of mastocytosis in the patient's quality of life.

Introduction: Patients with mastocytosis have various clinical and psychological symptoms, for example, life-threatening anaphylactic reactions or anxiety, resulting in decreased quality of life (QoL).

Aim: To assess the clinical and psychological symptoms (such as depression, anxiety) as well as the quality of life and satisfaction with life in patients with mastocytosis.

Material And Methods: The study group included 85 patients with mastocytosis (57 women and 28 men) treated at the Department of Allergology, Medical University of Gdansk, Poland.

Therapeutic decisions in a cohort of patients with idiopathic pulmonary fibrosis: a multicenter, prospective survey from Poland.

Background: Pirfenidone and nintedanib are considered as the standard of care in idiopathic pulmonary fibrosis (IPF), but there is no consensus as to which of these two agents should be regarded as first-line treatment.

Objective: To provide real-world data on therapeutic decisions of pulmonary specialists, particularly the choice of the antifibrotic drug in patients with IPF.

Methods: This was a multicenter, prospective survey collecting clinical data of patients with IPF considered as candidates for antifibrotic treatment between September 2019 and December 2020.

Protocol of safe vaccination against COVID-19 in patients with high risk of allergic reactions.

Background: Sars-CoV-2 infections are hazardous, especially to the elderly and patients with comorbidities. With no efficient treatment available, newly developed vaccines are the only way to change the course of the pandemic. However, reports of allergic reactions resulted in some patients and practicing physicians being concerned about the safety of vaccine administration, particularly in people with severe anaphylactic reactions to multiple or unknown factors in their medical history.

Benefits of Telemonitoring of Pulmonary Function-3-Month Follow-Up of Home Electronic Spirometry in Patients with Duchenne Muscular Dystrophy.

Background: In patients with Duchenne Muscular Dystrophy (DMD), the respiratory system determines the quality and length of life; therefore, the search for easy and safe everyday monitoring of the pulmonary function is currently extremely important, particularly in the COVID-19 pandemic. The aim of the study was to evaluate the influence of a three-month home electronic spirometry (e-spirometry) monitoring of the pulmonary function and strength of respiratory muscles as well as the patients' benefits from this telemetric program.

Methods: Twenty-one boys with DMD (aged 7-22; non-ambulatory-11) received a remote electronic spirometer for home use with a special application dedicated for patients and connected with a doctor platform.

miRNA profiles change during grass pollen immunotherapy irrespective of clinical outcome.

Subcutaneous immunotherapy (SCIT) is widely used in the treatment of allergic rhinitis (AR). This study aimed to determine the expression of 48 miRNAs in patients with AR undergoing grass pollen SCIT and investigate relations with clinical outcomes. Expression of selected miRNAs was determined using RT-PCR in the full blood of 16 patients with AR and seven healthy controls.