Polysomnographic Profile and Clinical Phenotypes of Osa in Association with Residual Sleep Apnea in Children.

: Residual obstructive sleep apnea (OSA) is defined as persistence of the AHI at ≥1 respiratory event per hour of sleep after otorhinolaryngology intervention in pediatric population. In terms of OSA phenotypes in children, we recognize the common, adult, and congenital phenotypes. We studied 34 pediatric patients with OSA diagnosed by standard overnight polysomnography (PSG).

Self-reported Clinical Outcomes and Quality of Life in Agammaglobulinemia: the Importance of an Early Diagnosis.

Purpose: Patients with (X-linked) agammaglobulinemia (XLA) suffer from severe, recurrent infections potentially leading to life-threatening complications such as sepsis, meningoencephalitis and chronic lung disease. Early diagnosis and timely treatment can prevent infections and secondary complications, emphasizing a role for early detection of XLA via newborn screening (NBS). Our international multicenter survey study aimed to evaluate self-reported outcomes and parental perspectives in XLA patients to determine whether an early diagnosis is associated with better quality of life (QoL).

Disparities in Seasonal Influenza Vaccination in Europe.

Background: Seasonal influenza remains a major public health challenge in Europe, associated with high morbidity, mortality, and socioeconomic burden. Despite the proven efficacy and cost-effectiveness of vaccination, coverage rates vary substantially across European countries and population groups, often falling short of the World Health Organization's target of 75% for older adults.

Objective: This narrative review aims to identify and explore disparities in seasonal influenza vaccination coverage across European countries and among different population subgroups.

Long-term safety and efficacy of once-daily berotralstat in patients with hereditary angioedema: APeX-S final results.

Background: Berotralstat is a once-daily oral medication for the prophylaxis of hereditary angioedema (HAE) attacks in patients aged 12 years and older.

Objective: To assess the long-term safety and efficacy of berotralstat in patients with HAE caused by C1 inhibitor deficiency.

Methods: APeX-S was a global, open-label phase 2 study (NCT03472040) assessing berotralstat 150 and 110 mg for up to 96 weeks in the United States and 240 weeks elsewhere.

Diagnosis, management, and monitoring of interleukin-1 mediated diseases in Central and Eastern Europe: real-world data.

Background: Global healthcare disparities, stemming from organizational differences in healthcare systems, lead to variable availability and funding, resulting in a gap between recommended and implemented practices for interleukin (IL)-1-mediated autoinflammatory diseases. We aimed to assess diagnostic, treatment and follow-up options for these diseases in Central and Eastern European countries, comparing them with the 2021 recommendations of the European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR).

Methods: In 2023, a structured collaborative effort was organized with representatives from 10 Central and Eastern European countries to address autoinflammatory diseases.

Combination of Allergen-Specific Immunotherapy With Biologics in Severe Asthma: Counterintuitive or Rational?

Although numbers vary, most patients with severe asthma have eosinophilic asthma. Many of them have the allergic asthma endotype, particularly those with concomitant allergic rhinitis (AR). Allergen immunotherapy (AIT) is the only disease-modifying therapy offering long-term effectiveness for allergic respiratory disorders, including asthma, but it is contraindicated in patients with uncontrolled disease.

Long-term efficacy and safety of colchicine and anti-IL-1 blockers in FMF: results from the Eurofever multicenter observational study.

Introduction: The majority of currently available data on familial Mediterranean fever (FMF) come from retrospective national or international studies.

Methods: An observational study collected data on the Eurofever international FMF cohort. Patients fulfilling genetic and clinical Eurofever criteria were considered as FMF+.

Effect of a Pleuran-Based Supplement on Salivary IgA Secretion in Children With Recurrent Respiratory Infections.

Background: ß-glucans isolated from natural sources have demonstrated pluripotent immunomodulatory potential, making them a promising supportive treatment for the management of recurrent respiratory infections (RRIs) in children. This study aimed to evaluate the effects of a pleuran-based supplement (ß-glucan isolated from in combination with vitamin D and zinc) on mucosal immunity -through modulating salivary secretory immunoglobulin A (sIgA) levels -in children with RRIs.

Methods: This monocentric, prospective, open-label pilot study investigated the effect of an orally administered pleuran/vitamin D/zinc supplement (1-2 chewable tablets daily depending on body weight) on the dynamics of sIgA secretion measured in saliva samples collected at three timepoints: at baseline and after 4-6 and 8-10 days.

Beneficial effects of pleuran on asthma control and respiratory tract-infection frequency in children with perennial asthma.

The aim of this study was to evaluate the effects of pleuran (β-glucan isolated from Pleurotus ostreatus) on asthma control and respiratory morbidity in children on conventional GINA-based asthma treatment who had partially controlled perennial asthma. A double-blind, placebo-controlled multicentre clinical trial with a 2-arm, parallel design was conducted across three countries; 230 children aged 7 to 17 years were randomised (1:1) into an active group (receiving a pleuran/vitamin C combination) or a placebo group (receiving vitamin C only). This study consisted of 24 weeks of treatment (2 capsules a day) and then 24 weeks of follow-up.

Lymphocyte Inhibition Mechanisms and Immune Checkpoints in COVID-19: Insights into Prognostic Markers and Disease Severity.

: Immune checkpoint inhibitors such as PD-1 and TIM-3 play an important role in regulating the host immune response and are proposed as potential prognostic markers and therapeutic targets in severe cases of COVID-19. We evaluated the expression of PD-1 and TIM-3 on T cells, as well as the concentration of sPD-1 in plasma, to clarify the role of these molecules in patients infected with SARS-CoV-2. In this retrospective observational study, we analysed the expression of PD-1 and TIM-3 on CD4 and CD8 T cells upon admission and after 7 days of hospitalisation in 770 adult patients.

Promoting Prevention and Targeting Remission of Asthma: A EUFOREA Consensus Statement on Raising the Bar in Asthma Care.

Asthma is a common, multifaceted respiratory disease with a major impact on quality of life. Despite increased insights into mechanisms underlying various asthma phenotypes and endotypes and the availability of targeted biologic treatment options, the disease remains uncontrolled in a substantial proportion of patients with risk of exacerbations, requiring systemic corticosteroids, and with progressive disease. Current international guidelines advocate for a personalized management approach to patients with uncontrolled severe asthma.

Atypical Manifestation of X-linked Agammaglobulinemia - the Importance of Genetic Testing.

X-linked agammaglobulinemia (XLA) was one of the first inborn errors of immunity to be described. It is caused by pathogenic variants in the gene for Bruton tyrosine kinase (BTK), which has important functions in B cell development and maturation. Recurrent bacterial infections in the first two years of life and hypogammaglobulinemia with absent B cells in male patients are the most common symptoms.

fSCIG 10% in pediatric primary immunodeficiency diseases: a European post-authorization safety study.

Background: The safety, tolerability, and immunogenicity of hyaluronidase-facilitated subcutaneous immunoglobulin (fSCIG) 10% (dual-vial unit of human immunoglobulin 10% and recombinant human hyaluronidase [rHuPH20]) were assessed in children with primary immunodeficiency diseases (PIDs).

Methods: This phase 4, post-authorization, prospective, interventional, multicenter study (NCT03116347) conducted in the European Economic Area, enrolled patients aged 2 to < 18 years with a documented PID diagnosis who had received immunoglobulin therapy for ≥ 3 months before enrollment. New fSCIG 10% starters underwent fSCIG 10% dose ramp-up for ≤ 6 weeks (epoch 1) before receiving fSCIG 10% for ≤ 3 years (epoch 2); patients pretreated with fSCIG 10% entered epoch 2 directly.

Choosing the right biologic treatment for individual patients with severe asthma - Lessons learnt from Picasso.

Severe asthma represents a true challenge for clinicians from two basic perspectives, i.e.: a rational assessment of the underlying endo/phenotype and the subsequent selection of the best fitted (personalized) and effective treatment.

EUFOREUM Berlin 2023: Optimizing care for type 2 inflammatory diseases from clinic to AI: A pediatric focus.

The European Forum for Research and Education in Allergy and Airways diseases (EUFOREA) organized its bi-annual forum EUFOREUM in Berlin in November 2023. The aim of EUFOREUM 2023 was to highlight pediatric action plans for prevention and optimizing care for type 2 inflammatory conditions starting in childhood, with a focus on early-stage diagnosis, ensuring neither under- nor overdiagnosis, optimal care, and suggestions for improvement of care. EUFOREA is an international not-for-profit organization forming an alliance of all stakeholders dedicated to reducing the prevalence and burden of chronic respiratory diseases through the implementation of optimal patient care via educational, research, and advocacy activities.

Recommendations for asthma monitoring in children: A PeARL document endorsed by APAPARI, EAACI, INTERASMA, REG, and WAO.

Monitoring is a major component of asthma management in children. Regular monitoring allows for diagnosis confirmation, treatment optimization, and natural history review. Numerous factors that may affect disease activity and patient well-being need to be monitored: response and adherence to treatment, disease control, disease progression, comorbidities, quality of life, medication side-effects, allergen and irritant exposures, diet and more.

Complex analysis of the national Hereditary angioedema cohort in Slovakia - Identification of 12 novel variants in gene.

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 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, gene was tested by DNA sequencing (Sanger sequencing/massive parallel sequencing) and/or multiplex ligation-dependent probe amplification for detection of large rearrangements.

Association Between Cytometric Biomarkers, Clinical Phenotype, and Complications of Common Variable Immunodeficiency.

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) and CVID with immune dysregulation (CVID).

Methods: We performed a retrospective clinical analysis of 64 CVID patients (34 males, 53.

Identification of a novel mutation associated with retinitis pigmentosa and primary ciliary dyskinesia in a Slovak family: a case report.

Background: The mutations in the (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.

Adherence to application technique of inhaled corticosteroid in patients with asthma and COVID-19 improves outcomes.

Background: Inhaled corticosteroids have been widely reported as a preventive measure against the development of severe forms of COVID-19 not only in patients with asthma.

Methods: In 654 Czech and Slovak patients with asthma who developed COVID-19, we investigated whether the correct use of inhaler containing corticosteroids was associated with a less severe course of COVID-19 and whether this had an impact on the need for hospitalisation, measurable lung functions and quality of life (QoL).

Results: Of the studied cohort 51.

The use of ketotifen as long-term anti-inflammatory prophylaxis in children with PFAPA syndrome.

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.

Biologics in severe asthma: A pragmatic approach for choosing the right treatment for the right patient.

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.