Care of patients with inborn errors of immunity in thirty J Project countries between 2004 and 2021

IntroductionThe J Project (JP) physician education and clinical research collaboration program was started in 2004 and includes by now 32 countries mostly in Eastern and Central Europe (ECE). Until the end of 2021, 344 inborn errors of immunity (IEI)-focused meetings were organized by the JP to raise awareness and facilitate the diagnosis and treatment of patients with IEI.ResultsIn this study, meeting profiles and major diagnostic and treatment parameters were studied. JP center leaders reported patients’ data from 30 countries representing a total population of 506 567 565. Two countries reported patients from JP centers (Konya, Turkey and Cairo University, Egypt). Diagnostic criteria were based on the 2020 update of classification by the IUIS Expert Committee on IEI. The number of JP meetings increased from 6 per year in 2004 and 2005 to 44 and 63 in 2020 and 2021, respectively. The cumulative number of meetings per country varied from 1 to 59 in various countries reflecting partly but not entirely the population of the respective countries. Altogether, 24,879 patients were reported giving an average prevalence of 4.9. Most of the patients had predominantly antibody deficiency (46,32%) followed by patients with combined immunodeficiencies (14.3%). The percentages of patients with bone marrow failure and phenocopies of IEI were less than 1 each. The number of patients was remarkably higher that those reported to the ESID Registry in 13 countries. Immunoglobulin (IgG) substitution was provided to 7,572 patients (5,693 intravenously) and 1,480 patients received hematopoietic stem cell therapy (HSCT). Searching for basic diagnostic parameters revealed the availability of immunochemistry and flow cytometry in 27 and 28 countries, respectively, and targeted gene sequencing and new generation sequencing was available in 21 and 18 countries. The number of IEI centers and experts in the field were 260 and 690, respectively. We found high correlation between the number of IEI centers and patients treated with intravenous IgG (IVIG) (correlation coefficient, cc, 0,916) and with those who were treated with HSCT (cc, 0,905). Similar correlation was found when the number of experts was compared with those treated with HSCT. However, the number of patients treated with subcutaneous Ig (SCIG) only slightly correlated with the number of experts (cc, 0,489) and no correlation was found between the number of centers and patients on SCIG (cc, 0,174).Conclusions1) this is the first study describing major diagnostic and treatment parameters of IEI care in countries of the JP; 2) the data suggest that the JP had tremendous impact on the development of IEI care in ECE; 3) our data help to define major future targets of JP activity in various countries; 4) we suggest that the number of IEI centers and IEI experts closely correlate to the most important treatment parameters; 5) we propose that specialist education among medical professionals plays pivotal role in increasing levels of diagnostics and adequate care of this vulnerable and still highly neglected patient population; 6) this study also provides the basis for further analysis of more specific aspects of IEI care including genetic diagnostics, disease specific prevalence, newborn screening and professional collaboration in JP countries

Acute urticaria: differential diagnosis and treatment

Acute urticaria remains one of the most topical pediatric problems. Acute urticaria lasts for up to 6 weeks, during which blisters appear with varying frequency and intensity. In about 40% of cases, urticaria is accompanied by angioedema. The prevalence of acute urticaria is 1 to 5% in the population, and in the child population the frequency of acute urticaria reaches 6.7%. According to recent estimates, 10 to 20% of the population has had an episode of acute urticaria during their lifetime. About 50% of children with acute urticaria have concomitant allergic diseases. The management of children with acute urticaria presents significant diagnostic and therapeutic challenges. Treatment of acute urticaria in children begins with elimination of the significant trigger – appropriate measures are taken (withdrawal of the drug, administration of an elimination diet, therapy of infectious and inflammatory processes). First-line drugs for acute urticaria are H1-antihistamines, and it is recommended to use H1-antihistamines of the II generation. However, if clinical manifestations develop rapidly, if the patient has generalized urticarial rashes, angioedema, gastrointestinal symptoms, parenteral forms of first-generation antihistamines may be used to relieve the acute allergic reaction. Patients who do not respond to treatment with antihistamines may respond to short-term therapy with systemic glucocorticoids, although the efficacy of this treatment has yet to be tested in controlled clinical trials. The prognosis for acute urticaria is favorable – in most cases, acute spontaneous urticaria remains the only episode in the patient’s life. The disease develops into a chronic form in 5% of patients, and other estimates suggest that the symptoms of urticaria persist in 9.5% of children for up to 6 months

A New Climate Nowcasting Tool Based on Paleoclimatic Data

Atmospheric pollutants and environmental indicators are often used to reconstruct historic atmospheric pollution from peat, as it accumulates over time by decomposing plant material, thus recording a history of air pollution. In the present study, three key parameters related to the peat bogs’ surface wetness dynamics in European Russia during the Holocene were investigated using modern statistical analysis. These parameters are: (i) the water table depth (WTD) in relation to the surface, which is reconstructed based on the community structure of the subfossil testate amoeba assemblages; (ii) the peat humification estimated as absorption of alkaline extract that directly reflects moisture at which the peat was formed; (iii) the Climate Moisture Index (CMI) and the Aridity Index derived from pollen-based reconstructions of the mean annual temperature and precipitation and classifying moisture conditions as the ratio between available annual precipitation and potential land surface evapotranspiration. All these parameters provide useful information about the paleoclimate (atmospheric moisture component) dynamics. High values of WTD and peat humification appear to comply with Gutenberg–Richter law. It is noteworthy that this law also seems to reproduce the high values of the modeled climate moisture and aridity indices. The validity of this new result is checked by replacing “conventional time” with “natural time”. On this basis, a new nowcasting tool is developed to more accurately estimate the average waiting time for the extreme values of these climate parameters. This will help to understand climate variability better to address emerging development needs and priorities by implementing empirical studies of the interactions between climatic effects, mitigation, adaptation, and sustainable growth

Особенности статистического учета аллергических болезней у детей на примере г. Москвы

Background. The emergence of new functional capabilities of statistical accounting made it possible to conduct a comparative analysis of the morbidity of allergic pathologies according to the registers of allergists and pediatricians from the Unified Medical Information and Analytical System (UMIAS) of Moscow with data from the Form of Federal Statistical Observation No. 12 (FSO No. 12). The aim of the study is to investigate the potential of using UMIAS for assessing/monitoring the morbidity of allergic diseases, including bronchial asthma in children, using the example of several outpatient clinics (OPCs) in Moscow. Methods. A study of combined design has been carried out. The data of children of several OPCs in Moscow were analyzed — data from UMIAS (observation registers of pediatricians and allergist-immunologists) and from the reporting forms of the FSO No. 12. Results. For a comparative analysis of statistical data from UMIAS and FSO No. 12, we studied the information of 60,851 children under 18 years of age. It was revealed that out of 60,851 children: allergic rhinitis according to FSO No. 12 and UMIAS was established in 1001 and 1059 patients; atopic dermatitis — in 142 and 345; urticaria — in 363 and 33; angioedema — in 4 and 16, respectively; food allergy — in 233 children according (to FSO No. 12) and in none of the children (according to UMIAS). Out of 60,851 children, 619 children were diagnosed with bronchial asthma according to the annual report (FSO No. 12) and 537 according to the pediatrician’s observation registers (UMIAS). At the same time, it was found that the diagnosis of bronchial asthma is not available as a separate nosology in the registry of an allergist-immunologist, and information about children with bronchial asthma is available to this specialist only when analyzing the uploaded information about children with other allergic diseases. Conclusion. A adequate sample ensured a high representativeness of the results obtained. The differences in the incidence rates of allergic diseases revealed by a comparative analysis of data from various sources — UMIAS and FSO No. 12 — indicate the need to improve both the system of statistical registration of incidence and the development of modern algorithms for early diagnosis and dynamic monitoring of children with allergies.Обоснование. Появление новых функциональных возможностей статистического учета позволило провести сравнительный анализ заболеваемости аллергическими патологиями по реестрам аллергологов и педиатров из единой медицинской информационно-аналитической системы (ЕМИАС) г. Москвы и данным из формы федерального статистического наблюдения № 12 (ФСН № 12). Цель исследования — изучить потенциал использования ЕМИАС для оценки/ мониторинга заболеваемости аллергическими патологиями, в том числе и бронхиальной астмой, у детей на примере нескольких амбулаторно-поликлинических центров (АПЦ) г. Москвы. Методы. Проведено исследование комбинированного дизайна. Были проанализированы данные детей нескольких АПЦ г. Москвы — сведения из ЕМИАС (реестров наблюдения педиатров и аллергологов-иммунологов) и из отчетных форм ФСН № 12. Результаты. Для сравнительного анализа статистических данных из ЕМИАС и ФСН № 12 изучили сведения 60 851 ребенка в возрасте до 18 лет. Выявлено, что из 60 851 ребенка аллергический ринит, по данным ФСН № 12 и ЕМИАС, был установлен у 1001 и 1059 пациентов, атопический дерматит — у 142 и 345, крапивница — у 363 и 33; ангиоотек — у 4 и 16 соответственно, пищевая аллергия — у 233 детей (по ФСН № 12) и ни у кого из детей (по данным ЕМИАС). Диагноз бронхиальной астмы из 60 851 ребенка имели 619 детей по данным годового отчета (ФСН № 12) и 537 — по реестрам наблюдения педиатра (ЕМИАС). При этом установлено, что диагноз бронхиальной астмы отсутствует как отдельная нозология в реестре аллерголога-иммунолога, и сведения о детях с астмой доступны этому специалисту только при проведении анализа выгруженных сведений о детях с другими аллергическими болезнями. Заключение. Достаточная выборка обеспечила высокую репрезентативность полученных результатов. Выявленные при сравнительном анализе данных из различных источников — ЕМИАС и ФСН № 12 — отличия в показателях заболеваемости аллергическими болезнями указывают на необходимость как совершенствования системы статистического учета, так и разработки современных алгоритмов ранней диагностики и динамического наблюдения детей с аллергией

Toward personalization of asthma treatment according to trigger factors

International audienceAsthma is a severe and chronic disabling disease affecting more than 300 million people worldwide. Although in the past few drugs for the treatment of asthma were available, new treatment options are currently emerging, which appear to be highly effective in certain subgroups of patients. Accordingly, there is a need for biomarkers that allow selection of patients for refined and personalized treatment strategies. Recently, serological chip tests based on microarrayed allergen molecules and peptides derived from the most common rhinovirus strains have been developed, which may discriminate 2 of the most common forms of asthma, that is, allergen- and virus-triggered asthma. In this perspective, we argue that classification of patients with asthma according to these common trigger factors may open new possibilities for personalized management of asthma

DataSheet_2_Care of patients with inborn errors of immunity in thirty J Project countries between 2004 and 2021.docx

IntroductionThe J Project (JP) physician education and clinical research collaboration program was started in 2004 and includes by now 32 countries mostly in Eastern and Central Europe (ECE). Until the end of 2021, 344 inborn errors of immunity (IEI)-focused meetings were organized by the JP to raise awareness and facilitate the diagnosis and treatment of patients with IEI.ResultsIn this study, meeting profiles and major diagnostic and treatment parameters were studied. JP center leaders reported patients’ data from 30 countries representing a total population of 506 567 565. Two countries reported patients from JP centers (Konya, Turkey and Cairo University, Egypt). Diagnostic criteria were based on the 2020 update of classification by the IUIS Expert Committee on IEI. The number of JP meetings increased from 6 per year in 2004 and 2005 to 44 and 63 in 2020 and 2021, respectively. The cumulative number of meetings per country varied from 1 to 59 in various countries reflecting partly but not entirely the population of the respective countries. Altogether, 24,879 patients were reported giving an average prevalence of 4.9. Most of the patients had predominantly antibody deficiency (46,32%) followed by patients with combined immunodeficiencies (14.3%). The percentages of patients with bone marrow failure and phenocopies of IEI were less than 1 each. The number of patients was remarkably higher that those reported to the ESID Registry in 13 countries. Immunoglobulin (IgG) substitution was provided to 7,572 patients (5,693 intravenously) and 1,480 patients received hematopoietic stem cell therapy (HSCT). Searching for basic diagnostic parameters revealed the availability of immunochemistry and flow cytometry in 27 and 28 countries, respectively, and targeted gene sequencing and new generation sequencing was available in 21 and 18 countries. The number of IEI centers and experts in the field were 260 and 690, respectively. We found high correlation between the number of IEI centers and patients treated with intravenous IgG (IVIG) (correlation coefficient, cc, 0,916) and with those who were treated with HSCT (cc, 0,905). Similar correlation was found when the number of experts was compared with those treated with HSCT. However, the number of patients treated with subcutaneous Ig (SCIG) only slightly correlated with the number of experts (cc, 0,489) and no correlation was found between the number of centers and patients on SCIG (cc, 0,174).Conclusions1) this is the first study describing major diagnostic and treatment parameters of IEI care in countries of the JP; 2) the data suggest that the JP had tremendous impact on the development of IEI care in ECE; 3) our data help to define major future targets of JP activity in various countries; 4) we suggest that the number of IEI centers and IEI experts closely correlate to the most important treatment parameters; 5) we propose that specialist education among medical professionals plays pivotal role in increasing levels of diagnostics and adequate care of this vulnerable and still highly neglected patient population; 6) this study also provides the basis for further analysis of more specific aspects of IEI care including genetic diagnostics, disease specific prevalence, newborn screening and professional collaboration in JP countries.</p

Table_1_Care of patients with inborn errors of immunity in thirty J Project countries between 2004 and 2021.docx

IntroductionThe J Project (JP) physician education and clinical research collaboration program was started in 2004 and includes by now 32 countries mostly in Eastern and Central Europe (ECE). Until the end of 2021, 344 inborn errors of immunity (IEI)-focused meetings were organized by the JP to raise awareness and facilitate the diagnosis and treatment of patients with IEI.ResultsIn this study, meeting profiles and major diagnostic and treatment parameters were studied. JP center leaders reported patients’ data from 30 countries representing a total population of 506 567 565. Two countries reported patients from JP centers (Konya, Turkey and Cairo University, Egypt). Diagnostic criteria were based on the 2020 update of classification by the IUIS Expert Committee on IEI. The number of JP meetings increased from 6 per year in 2004 and 2005 to 44 and 63 in 2020 and 2021, respectively. The cumulative number of meetings per country varied from 1 to 59 in various countries reflecting partly but not entirely the population of the respective countries. Altogether, 24,879 patients were reported giving an average prevalence of 4.9. Most of the patients had predominantly antibody deficiency (46,32%) followed by patients with combined immunodeficiencies (14.3%). The percentages of patients with bone marrow failure and phenocopies of IEI were less than 1 each. The number of patients was remarkably higher that those reported to the ESID Registry in 13 countries. Immunoglobulin (IgG) substitution was provided to 7,572 patients (5,693 intravenously) and 1,480 patients received hematopoietic stem cell therapy (HSCT). Searching for basic diagnostic parameters revealed the availability of immunochemistry and flow cytometry in 27 and 28 countries, respectively, and targeted gene sequencing and new generation sequencing was available in 21 and 18 countries. The number of IEI centers and experts in the field were 260 and 690, respectively. We found high correlation between the number of IEI centers and patients treated with intravenous IgG (IVIG) (correlation coefficient, cc, 0,916) and with those who were treated with HSCT (cc, 0,905). Similar correlation was found when the number of experts was compared with those treated with HSCT. However, the number of patients treated with subcutaneous Ig (SCIG) only slightly correlated with the number of experts (cc, 0,489) and no correlation was found between the number of centers and patients on SCIG (cc, 0,174).Conclusions1) this is the first study describing major diagnostic and treatment parameters of IEI care in countries of the JP; 2) the data suggest that the JP had tremendous impact on the development of IEI care in ECE; 3) our data help to define major future targets of JP activity in various countries; 4) we suggest that the number of IEI centers and IEI experts closely correlate to the most important treatment parameters; 5) we propose that specialist education among medical professionals plays pivotal role in increasing levels of diagnostics and adequate care of this vulnerable and still highly neglected patient population; 6) this study also provides the basis for further analysis of more specific aspects of IEI care including genetic diagnostics, disease specific prevalence, newborn screening and professional collaboration in JP countries.</p

Image_1_Care of patients with inborn errors of immunity in thirty J Project countries between 2004 and 2021.jpeg

IntroductionThe J Project (JP) physician education and clinical research collaboration program was started in 2004 and includes by now 32 countries mostly in Eastern and Central Europe (ECE). Until the end of 2021, 344 inborn errors of immunity (IEI)-focused meetings were organized by the JP to raise awareness and facilitate the diagnosis and treatment of patients with IEI.ResultsIn this study, meeting profiles and major diagnostic and treatment parameters were studied. JP center leaders reported patients’ data from 30 countries representing a total population of 506 567 565. Two countries reported patients from JP centers (Konya, Turkey and Cairo University, Egypt). Diagnostic criteria were based on the 2020 update of classification by the IUIS Expert Committee on IEI. The number of JP meetings increased from 6 per year in 2004 and 2005 to 44 and 63 in 2020 and 2021, respectively. The cumulative number of meetings per country varied from 1 to 59 in various countries reflecting partly but not entirely the population of the respective countries. Altogether, 24,879 patients were reported giving an average prevalence of 4.9. Most of the patients had predominantly antibody deficiency (46,32%) followed by patients with combined immunodeficiencies (14.3%). The percentages of patients with bone marrow failure and phenocopies of IEI were less than 1 each. The number of patients was remarkably higher that those reported to the ESID Registry in 13 countries. Immunoglobulin (IgG) substitution was provided to 7,572 patients (5,693 intravenously) and 1,480 patients received hematopoietic stem cell therapy (HSCT). Searching for basic diagnostic parameters revealed the availability of immunochemistry and flow cytometry in 27 and 28 countries, respectively, and targeted gene sequencing and new generation sequencing was available in 21 and 18 countries. The number of IEI centers and experts in the field were 260 and 690, respectively. We found high correlation between the number of IEI centers and patients treated with intravenous IgG (IVIG) (correlation coefficient, cc, 0,916) and with those who were treated with HSCT (cc, 0,905). Similar correlation was found when the number of experts was compared with those treated with HSCT. However, the number of patients treated with subcutaneous Ig (SCIG) only slightly correlated with the number of experts (cc, 0,489) and no correlation was found between the number of centers and patients on SCIG (cc, 0,174).Conclusions1) this is the first study describing major diagnostic and treatment parameters of IEI care in countries of the JP; 2) the data suggest that the JP had tremendous impact on the development of IEI care in ECE; 3) our data help to define major future targets of JP activity in various countries; 4) we suggest that the number of IEI centers and IEI experts closely correlate to the most important treatment parameters; 5) we propose that specialist education among medical professionals plays pivotal role in increasing levels of diagnostics and adequate care of this vulnerable and still highly neglected patient population; 6) this study also provides the basis for further analysis of more specific aspects of IEI care including genetic diagnostics, disease specific prevalence, newborn screening and professional collaboration in JP countries.</p