Invasive meningococcal diseases (IMD) caused by Neisseria meningitidis are generally rare. They affect mostly selected age categories and risk groups of patients (in terms of age, comorbidities, or applied therapy), and the immune system and its defects may play an important modifying role. Meningococcal infections could be the first and only clinical sign of unrecognised immunodeficiency. IMD are a typical clinical presentation of inborn errors of immunity with low concentrations or dysfunction of the terminal components of complement cascade. Meningitis is present in approximately 40% of the patients with terminal complement components deficiencies and in 6% of the patients with properdin deficiency. Despite evident advances in the understanding of the pathogenesis of meningococcal infections and the mechanisms of immune defence against this pathogen, patients with defects in the alternative or terminal complement pathway are highly predisposed to invasive and recurrent meningococcal infections, usually with a mild course. Therefore, it is recommended that each patient with IMD, especially recurrent, should undergo an immunological examination to rule out complement deficiencies.
- Klíčová slova
- Neisseria meningitides, Neisseria meningitidis, complement immunodeficiencies, complement system, immunodeficiency, immunological examination, invasive meningococcal diseases,
- MeSH
- lidé MeSH
- meningokokové infekce * imunologie diagnóza MeSH
- recidiva MeSH
- syndromy imunologické nedostatečnosti komplikace imunologie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
BACKGROUND: Systemic autoinflammatory disorders (SAIDs) represent a growing spectrum of diseases characterized by dysregulation of the innate immune system. The most common pediatric autoinflammatory fever syndrome, Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA), has well defined clinical diagnostic criteria, but there is a subset of patients who do not meet these criteria and are classified as undefined autoinflammatory diseases (uAID). This project, endorsed by PRES, supported by the EMERGE fellowship program, aimed to analyze the evolution of symptoms in recurrent fevers without molecular diagnosis in the context of undifferentiated AIDs, focusing on PFAPA and syndrome of undifferentiated recurrent fever (SURF), using data from European AID registries. METHODS: Data of patients with PFAPA, SURF and uSAID were collected from 3 registries including detailed epidemiological, demographic and clinical data, results of the genetic testing and additional laboratory investigations with retrospective application of the modified Marshall and PRINTO/Eurofever classification criteria on the cohort of PFAPA patients and preliminary SURF criteria on uSAID/SURF patients. RESULTS: Clinical presentation of PFAPA is variable and some patients did not fit the conventional PFAPA criteria and exhibit different symptoms. Some patients did not meet the criteria for either PFAPA or SURF, highlighting the heterogeneity within these groups. The study also explored potential overlaps between PFAPA and SURF/uAID, revealing that some patients exhibited symptoms characteristic of both conditions, emphasizing the need for more precise classification criteria. CONCLUSIONS: Patients with recurrent fevers without molecular diagnoses represent a clinically heterogeneous group. Improved classification criteria are needed for both PFAPA and SURF/uAID to accurately identify and manage these patients, ultimately improving clinical outcomes.
- Klíčová slova
- AID-Net, Autoinflammatory diseases, Eurofever, JIR-cohort, PFAPA, SURF,
- MeSH
- aftózní stomatitida * diagnóza epidemiologie MeSH
- dědičné zánětlivé autoimunitní nemoci * diagnóza MeSH
- dítě MeSH
- faryngitida * diagnóza MeSH
- horečka etiologie diagnóza MeSH
- kojenec MeSH
- lidé MeSH
- lymfadenitida * diagnóza epidemiologie MeSH
- mladiství MeSH
- předškolní dítě MeSH
- recidiva MeSH
- registrace * MeSH
- retrospektivní studie MeSH
- Check Tag
- dítě MeSH
- kojenec MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Evropa epidemiologie MeSH
The concept of pre-diabetes has led to provision of measures to reduce disease progression through identification of subjects at risk of diabetes. We previously considered the idea of pre-asthma in relation to allergic asthma and considered that, in addition to the need to improve population health via multiple measures, including reduction of exposure to allergens and pollutants and avoidance of obesity, there are several possible specific means to reduce asthma development in those most at risk (pre- asthma). The most obvious is allergen immunotherapy (AIT), which when given for allergic rhinitis (AR) has reasonable evidence to support asthma prevention in children (2) but also needs further study as primary prevention. In this second paper we explore the possibilities for similar actions in late onset eosinophilic asthma.
- Klíčová slova
- S. aureus biofilm, chronic rhinosinusitis with nasal polyps, eosinophils, late onset asthma, mast cells, non-allergic rhinitis, virulence genes,
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
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.
- Klíčová slova
- cell metabolism, immune senescence, immunometabolism, inflammaging, senolytic drugs, senomorphic drugs,
- MeSH
- chronická nemoc MeSH
- chronická obstrukční plicní nemoc metabolismus farmakoterapie imunologie MeSH
- lidé MeSH
- metabolické sítě a dráhy * MeSH
- plicní nemoci etiologie farmakoterapie metabolismus imunologie MeSH
- stárnutí buněk * účinky léků MeSH
- stárnutí imunologie metabolismus MeSH
- zánět metabolismus imunologie MeSH
- zvířata MeSH
- Check Tag
- lidé MeSH
- zvířata MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- přehledy MeSH
In March 2023, the European Forum for Research and Education in Allergy and Airways diseases (EUFOREA) organized its bi-annual Summit in Brussels with expert panel members of EUFOREA, representatives of the EUFOREA patient advisory board, and the EUFOREA board and management teams. Its aim was to define the research, educational and advocacy initiatives to be developed by EUFOREA over the next 2 years until the 10th anniversary in 2025. EUFOREA is an international non-for-profit organization forming an alliance of all stakeholders dedicated to reducing the prevalence and burden of chronic allergic and respiratory diseases via research, education, and advocacy. Based on its medical scientific core competency, EUFOREA offers an evidence-supported platform to introduce innovation and education in healthcare leading to optimal patient care, bridging the gap between latest scientific evidence and daily practice. Aligned with the mission of improving health care, the expert panels of asthma, allergic rhinitis (AR), chronic rhinosinusitis (CRS) & European Position Paper on Rhinosinusitis and Nasal Polyps (EPOS), allergen immunotherapy (AIT) and paediatrics have proposed and elaborated a variety of activities that correspond to major unmet needs in the allergy and respiratory field. The current report provides a concise overview of the achievements, ambitions, and action plan of EUFOREA for the future, allowing all stakeholders in the allergy and respiratory field to be up-dated and inspired to join forces in Europe and beyond.
- Klíčová slova
- EUFOREA, allergen immunotherapy, allergic rhinitis, asthma, paediatrics, patient advisory board, pocket guide, rhinosinusitis,
- Publikační typ
- časopisecké články MeSH
Asthma, which affects some 300 million people worldwide and caused 455,000 deaths in 2019, is a significant burden to suffers and to society. It is the most common chronic disease in children and represents one of the major causes for years lived with disability. Significant efforts are made by organizations such as WHO in improving the diagnosis, treatment and monitoring of asthma. However asthma prevention has been less studied. Currently there is a concept of pre- diabetes which allows a reduction in full blown diabetes if diet and exercise are undertaken. Similar predictive states are found in Alzheimer's and Parkinson's diseases. In this paper we explore the possibilities for asthma prevention, both at population level and also investigate the possibility of defining a state of pre-asthma, in which intensive treatment could reduce progression to asthma. Since asthma is a heterogeneous condition, this paper is concerned with allergic asthma. A subsequent one will deal with late onset eosinophilic asthma.
- Klíčová slova
- asthma, asthma natural history, pre-asthma, predisposition, quality of life, risk factors,
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
COVID-19 (Coronavirus Disease) is an infectious disease caused by the coronavirus SARS-CoV-2 (Severe acute respiratory syndrome Coronavirus 2), which belongs to the genus Betacoronavirus. It was first identified in patients with severe respiratory disease in December 2019 in Wuhan, China. It mainly affects the respiratory system, and in severe cases causes serious lung infection or pneumonia, which can lead to the death of the patient. Clinical studies show that SARS-CoV-2 infection in critical cases causes acute tissue damage due to a pathological immune response. The immune response to a new coronavirus is complex and involves many processes of specific and non-specific immunity. Analysis of available studies has shown various changes, especially in the area of specific cellular immunity, including lymphopenia, decreased T cells (CD3+, CD4+ and CD8+), changes in the T cell compartment associated with symptom progression, deterioration of the condition and development of lung damage. We provide a detailed review of the analyses of immune checkpoint molecules PD-1, TIM-3, LAG-3 CTLA-4, TIGIT, BTLA, CD223, IDO-1 and VISTA on exhausted T cells in patients with asymptomatic to symptomatic stages of COVID-19 infection. Furthermore, this review may help to better understand the pathological T cell immune response and improve the design of therapeutic strategies for patients with SARS-CoV-2 infection.
- MeSH
- COVID-19 imunologie metabolismus virologie MeSH
- fenotyp MeSH
- interakce hostitele a patogenu MeSH
- lidé MeSH
- proteiny kontrolních bodů imunitní reakce metabolismus MeSH
- SARS-CoV-2 imunologie patogenita MeSH
- signální transdukce MeSH
- T-lymfocyty imunologie metabolismus MeSH
- zvířata MeSH
- Check Tag
- lidé MeSH
- zvířata MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
- Názvy látek
- proteiny kontrolních bodů imunitní reakce MeSH
The SARS-CoV-2 pandemic has indeed been one of the most significant problems facing the world in the last decade. It has affected (directly or indirectly) the entire population and all age groups. Children have accounted for 1.7 % to 2 % of the diagnosed cases of COVID-19. COVID-19 in children is usually associated with a mild course of the disease and a better survival rate than in adults. In this review, we investigate the different mechanisms which underlie this observation. Generally, we can say that the innate immune response of children is strong because they have a trained immunity, allowing the early control of infection at the site of entry. Suppressed adaptive immunity and a dysfunctional innate immune response is seen in adult patients with severe infections but not in children. This may relate to immunosenescence in the elderly. Another proposed factor is the different receptors for SARS-CoV-2 and their differences in expression between these age groups. In infants and toddlers, effective immune response to viral particles can be modulated by the pre-existing non-specific effect of live attenuated vaccines on innate immunity and vitamin D prophylaxis. However, all the proposed mechanisms require verification in larger cohorts of patients. Our knowledge about SARS-CoV-2 is still developing.
- MeSH
- COVID-19 imunologie patofyziologie terapie virologie MeSH
- dítě MeSH
- dospělí MeSH
- hodnocení rizik MeSH
- imunitní systém růst a vývoj patofyziologie virologie MeSH
- interakce hostitele a patogenu MeSH
- kojenec MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- novorozenec MeSH
- předškolní dítě MeSH
- prognóza MeSH
- rizikové faktory MeSH
- SARS-CoV-2 imunologie patogenita MeSH
- stupeň závažnosti nemoci MeSH
- věkové faktory MeSH
- vývoj dítěte * MeSH
- vývoj mladistvých * MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- kojenec MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- novorozenec MeSH
- předškolní dítě MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
AIM: To evaluate microbial colonization of upper aerodigestive tract in children and to investigate the influence of adenoid hypertrophy, allergy and exposition to cigarette smoke on presence of pathogens. METHODS: In 43 children with adenoid hypertrophy and 17 healthy children bacterial culture was performed by a swab from middle nasal meatus, nasopharynx and tonsils. The effect of adenotomy, presence of allergy and exposure to passive smoking on bacterial colonization were investigated. RESULTS: Identification of potentially pathogenic bacteria in upper aerodigestive tract was significantly higher in children with adenoid hypertrophy compare to control group. Adenotomy was associated with significantly decreased colonization by potential pathogens. Allergy was diagnosed in 33 % children with adenoid hypertrophy. Presence of allergy and exposure to tobacco smoke were associated with significantly more often colonization by potentially pathogenic bacteria in the upper aerodigestive tract. CONCLUSION: Increased colonization of upper aerodigestive tract by potential pathogens and their significant decrease after adenotomy indicate the role of pathogenic bacteria in the etiopathogenesis of adenoid hypertrophy. Allergy and tobacco smoke exposure are related to increased colonization by potentially pathogenic bacteria in the upper aerodigestive tract.
- Klíčová slova
- pathogens - upper airways - adenoid hypertrophy - allergy - smoking.,
- MeSH
- adenoidy * patologie MeSH
- alergie * MeSH
- dítě MeSH
- fyziologie bakterií * MeSH
- infekce dýchací soustavy * epidemiologie MeSH
- krční mandle mikrobiologie MeSH
- lidé MeSH
- nazofarynx mikrobiologie MeSH
- nos mikrobiologie MeSH
- rizikové faktory MeSH
- vystavení vlivu životního prostředí MeSH
- znečištění tabákovým kouřem * MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- Názvy látek
- znečištění tabákovým kouřem * MeSH
AIM: The complex influence of internal and external environmental factors on the individual and his/her immune system and the lack of suitable markers to assess and reduce the risk of the development of allergies during the lifetime can explain the continuous increase in the number of people affected by some form of allergy. According to the results of some studies, cord blood IgE level could be a useful early marker for assessing the risk of atopic diseases, but the studies showed controversial results. In addition, several authors discuss the origin of these antibodies (synthesis in utero, peripartum contamination from maternal blood or placental transfer). The aim of our pilot study was to investigate the possible influence of modifying factors on cord blood IgE level. MATERIAL AND METHODS: Our group of patients consisted of 184 retrospectively selected neonates (98 boys, 53.3% and 86 girls, 46.7%) from whom cord blood was collected and cord blood IgE level was measured 25 years ago (PRIST method). The impact of selected modifying factors (sex, type of delivery or month of birth) on cord blood IgE level was assessed retrospectively. RESULTS: Higher cord blood IgE levels were found in boys than in girls, in neonates born by Caesarean section than in those born by natural delivery, and in those born in the winter months than in other seasons of the year. Our findings are in agreement with those of other authors. CONCLUSION: Based on our results and those of others, we assume that the selected factors affect the cord blood IgE levels to varying degrees. These facts should be taken into consideration while interpreting the cord blood IgE levels.
- Klíčová slova
- mmunoglobulin E - public health hypothesis - cord blood - modifying factors - prevention.,
- MeSH
- alergie MeSH
- fetální krev chemie MeSH
- imunoglobulin E krev MeSH
- lidé MeSH
- novorozenec MeSH
- pilotní projekty MeSH
- roční období MeSH
- těhotenství MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- novorozenec MeSH
- těhotenství MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Názvy látek
- imunoglobulin E MeSH
