卷 22, 编号 4 (2025)

BACKGROUND: Anaphylaxis is a severe, life-threatening systemic allergic reaction. Currently, the only biomarker of anaphylaxis recommended for use in Russian and international consensus documents is tryptase, with very limited data on the efficacy of its use in real clinical practice in children.

AIM: To determine the frequency of measurement and diagnostic significance of tryptase level estimation in children with anaphylaxis within the recommended time frame in real clinical practice, as well as its relationship with the age of patients and the severity of symptoms.

METHODS: The study included 128 patients aged 0 to 18 years who were hospitalized urgently in the State Budgetary Institution Morozov Children’s City Clinical Hospital due to an episode of anaphylaxis in the period from May 2022 to April 2025 and were included in the Pediatric Moscow Anaphylaxis Register. The diagnosis of anaphylaxis was verified by an expert allergist-immunologist based on clinical criteria for anaphylaxis. Serum tryptase levels were measured in patients admitted to the hospital within 3 hours of the onset of symptoms of acute allergic reaction. The patients’ clinical and laboratory parameters were entered into an online registry questionnaire and processed using a computer program, which is a system for data recording and analysis.

RESULTS: Of the 128 children, 52 (40.6 %) had their tryptase levels measured within 3 hours of symptom onset. Among these, 15 (28.8 %) showed elevated tryptase levels. For the 76 children who were not assessed (due to delayed hospitalization), a higher frequency of mild anaphylaxis was observed compared to those assessed for tryptase levels (31.6 % vs 15.4 %; p = 0.037). No correlation was found between age and tryptase levels, though there was a trend toward older patients (median 12 years vs 10 years; p = 0.052) being within the timeframe for tryptase measurement. Severity of reaction did not affect tryptase concentrations.

CONCLUSION: In clinical practice, tryptase levels can be measured within 3 hours of symptom onset in less than half of patients, with only one third showing elevated levels. Delayed hospital arrival in patients with mild anaphylaxis is likely to delay diagnosis and treatment.

BACKGROUND: Allergic rhinitis is one of the most common diseases in children and adults worldwide. Allergic rhinitis is often combined with other allergic diseases, including asthma. Indeed, 30 % of patients with Allergic rhinitis develop concomitant asthma throughout their life and 80 % of patients with asthma have allergic rhinitis.

AIM: To evaluate the effect of combination nasal spray containing olopatadine hydrochloride and mometasone furoate on the control of allergic rhinitis and concomitant bronchial asthma in children of different ages.

METHODS: Sixty children 7–17 years old were included in an open-label observational study with a duration of 57 (56–60) days with uncontrolled persistent moderate/severe (seasonal and/or perennial) allergic rhinitis and concomitant, partially or uncontrolled mild or moderate asthma, who were treated with age-appropriate dosage of olopatadine hydrochloride and mometasone furoate nasal spray. Therapy of asthma under the terms of the study protocol remained unchanged. The severity of allergic rhinitis symptoms was assessed using a visual analogue scale. Control of concomitant asthma was determined by validated questionnaires: сACT/ACT, ACQ-5 and quality of life for children with asthma by PAQLQ(s) questionnaire. All study participants underwent the following instrumental and laboratory assessments twice: external respiratory function with bronchodilation test, measurement of nitric oxide in exhaled air, nasal secretion sampling and rhinocytogram, venous blood sampling and clinical blood analysis, and determination of eosinophilic cationic protein level. At intermediate visit after 28 (28–30) days, spirometry was performed. At each visit, patients were asked to fill out questionnaires.

RESULTS: Treatment of allergic rhinitis with nasal spray of a fixed combination olopatadine hydrochloride and mometasone furoate revealed statistically significant reduction of allergic rhinitis and asthma symptoms, functional indices such as first second of forced expiration and bronchial reversibility improved. The level of eosinophils in nasal secretion showed a statistically significant decrease.

CONCLUSION: The rapid and effective achievement of allergic rhinitis control in children with a combined olopatadine hydrochloride and mometasone furoate nasal spray may allow for improved control of concomitant allergic asthma and quality of life without escalation of baseline anti-asthmatic therapy.

The paper presents the modern concept of airway inflammation formation in T2 endotype asthma under the exposure to ambient air particulate matter (PM). It was shown that PM exposure leads to disruption of the epithelial barrier integrity and epithelial cells damage, triggering the alarmins production with subsequent activation of dendritic cells, Th2 lymphocytes, and/or type 2 innate lymphoid cells. The role of PM in eosinophilic inflammation in both allergic and non-allergic asthma phenotypes was highlighted. Moreover, evidence suggests that PM may modify the structure and activity of certain aeroallergens. Furthermore, a correlation was demonstrated between PM concentrations and asthma incidence. Prenatal PM exposure leads to increased risk for childhood asthma. An association was found between PM concentration and disease progression, exacerbation frequency, and emergency care visits.

The results of experimental, epidemiological, and clinical data show the significant role of PM in driving airway inflammation in the T2-endotype asthma. This highlights the need for further research to develop preventive strategies and novel therapeutic approaches.

There are various pheno-, endotypes of chronic rhinitis, which can clinically proceed in the same way and are difficult to distinguish even with a comprehensive approach to diagnosis. Since the prognosis and treatment of chronic rhinitis (allergic, non-infectious non-allergic rhinitis and local allergic rhinitis) most often differ, differential diagnosis of these diseases is important, especially when choosing drugs for each clinical situation. It is possible to obtain more accurate information about the state of the patient’s nasal mucosa at the moment by examining the patient’s nasal mucus cytology. Rhinocytology is a simple and non-invasive test that can help doctors differentiate various forms of chronic rhinitis.

The purpose of this review is to analyze current concepts of the role of rhinocytology in chronic rhinitis in children and adults. It should be assumed that the assessment of the cellular composition in nasal mucus (the type and number of different cells, the presence of bacteria, fungi, etc.) will allow us to establish not only the type of inflammation (for example, allergic rhinitis, infectious rhinitis etc.), but will also help in choosing the most effective treatment individually for each patient. The review also examines in detail the features of using this diagnostic method in the daily practice of Russian doctors and abroad; issues related to changes in the cytology of nasal mucus in various forms of chronic rhinitis are discussed; the advantages and disadvantages of rhinocytology are highlighted. Researchers admit that due to its limited use in daily practice, the role of rhinocytology is underestimated. Meanwhile, this is a method that can really help clinicians in diagnosing and treating patients with various pheno-, endotypes of chronic rhinitis.

Сommon variable immunodeficiency is the most prevalent form of inborn errors of immunity, however, genetic cause may be identified in less than a third of the cases. Pathogenic variants in the subunit 1 of nuclear factor κB are thought to be one of the most frequent monogenic causes of common variable immunodeficiency. Besides the humoral immunodeficiency, NFKB1 defect is characterized by a wide range of autoimmune, autoinflammatory and hematological features. To date, there are no standardized guidelines for immunosuppressive or anti-inflammatory therapy in patients with NFKB1 deficiency.

We report on a 62 year old woman with common variable immunodeficiency due to heterozygous mutation in the NFKB1 gene, complicated by a systemic form of Still’s disease. Since initiation of the anti-cytokine therapy with the interleukin 6 antagonist olokizumab, resolution of constitutional symptoms has been achieved. Over the 1.5-years follow-up there has been an improvement on lymphadenopathy and joint syndrome according to computed tomography scan and ultrasound.

Variety of manifestations of immune disregulation in complex of symptoms of NFKB1 defect forces a neccessity of awareness of the specialists and requires a multidisciplinary approach to selection of an optimal tactic to patient treatment.

Epidermolysis bullosa is a group of rare genetic skin diseases, the common feature of which is a tendency to form blisters and/or erosions on the skin and mucous membranes due to minimal trauma. Phenotypic manifestations and severity of epidermolysis bullosa depend on the genotype, while pathogenic variants in the same gene can lead to different forms of epidermolysis bullosa, inherited in both autosomal dominant and autosomal recessive patterns. Currently, more than twenty genes are known, pathogenic variants in which can lead to the development of various forms of epidermolysis bullosa. It is worth noting that causal variants in different genes can cause similar clinical phenotypes, which significantly complicates the understanding of the pathogenetic mechanisms of the disease. In addition to the association of individual genes with certain phenotypes, a correlation has also been established between specific variants within the same gene and the clinical severity of the disease. Phenotype variability in epidermolysis bullosa is observed both between different subtypes and within each of them — from cases with minimal and barely noticeable manifestations to severe forms with pronounced cutaneous and systemic lesions caused by significant disruption of the dermal-epidermal junction, including the basement membrane, adjacent basal keratinocytes and connective tissue structures.

This article presents cases of clinically identical manifestations — pruriginous rashes in different genotypes occurring in epidermolysis bullosa children.