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1.
Int J Mol Sci ; 24(2)2023 Jan 16.
Artigo em Inglês | MEDLINE | ID: mdl-36675318

RESUMO

Next to cow's milk and eggs, plant foods, i.e., legumes, tree nuts and cereal grains, most often sensitise atopic children. Storage proteins constitutes the most relevant protein fraction of plant foods, causing primary sensitisation. They exhibit strong allergenic properties and immunogenicity. Our goal was to analyse sensitisation to 26 plant storage proteins in a group of 76 children aged 0-5 years with chronic symptoms of atopic dermatitis using Allergy Explorer ALEX2 and to discover changes in serum protein-peptide patterns in allergic patients with the use of MALDI-TOF-MS. We reported that 25% of children were allergic to 2S albumins, 19.7% to 7S globulins, 13.2% to 11S globulins and 1.3% to cereal prolamins. The most common allergenic molecules were Ara h 1 (18.4%), Ara h 2 (17.1%), Ara h 6 (15.8%) and Ara h 3 (11.8%) from peanuts, and the mean serum sIgE concentrations in allergic patients were 10.93 kUA/L, 15.353 kUA/L, 15.359 kUA/L and 9.038 kUA/L, respectively. In children allergic to storage proteins compared to the other patients (both allergic and non-allergic), the cell cycle control protein 50A, testis-expressed sequence 13B, DENN domain-containing protein 5A and SKI family transcriptional corepressor 2 were altered. Our results indicate that the IgE-mediated allergy to storage proteins is a huge problem in a group of young, atopic children, and show the potential of proteomic analysis in the prediction of primary sensitisation to plant foods. It is the next crucial step for understanding the molecular consequences of allergy to storage proteins.


Assuntos
Dermatite Atópica , Proteínas de Plantas , Masculino , Feminino , Animais , Bovinos , Proteômica , Alérgenos , Peptídeos , Proteínas Sanguíneas , Antígenos de Plantas , Arachis
2.
Medicina (Kaunas) ; 57(12)2021 Dec 08.
Artigo em Inglês | MEDLINE | ID: mdl-34946286

RESUMO

Background and Objectives: In paediatric population, atopic asthma is associated with increased eosinophil counts in patients, that correlate with the airway inflammation measured by the concentration of nitric oxide in exhaled air (FeNO). As the FeNO level is a biomarker of atopic asthma, we assumed that polymorphisms in nitric synthases genes may represent a risk factor for asthma development. The purpose of this study was to analyse the association of NOS genetic variants with childhood asthma in the Polish population. Materials and methods: In study we included 443 children-220 patients diagnosed with atopic asthma and 223 healthy control subjects. We have genotyped 4 single nucleotide polymorphisms (SNP) from 3 genes involved in the nitric oxide synthesis (NOS1, NOS2 and NOS3). All analyses were performed using polymerase chain reaction with restriction fragments length polymorphism (PCR-RFLP). Results: We observed significant differences between cases and controls in SNP rs10459953 in NOS2 gene, considering both genotypes (p = 0.001) and alleles (p = 0.0006). The other analyzed polymorphisms did not show association with disease. Conclusions: According to our results, 5'UTR variant within NOS2 isoform may have an impact of asthma susceptibility in the population of Polish children. Further functional studies are required to understand the role of iNOS polymorphism in NOS2 translation and to consider it as a novel risk factor in childhood asthma. The next step would be to apply this knowledge to improve diagnosis and develop novel personalized asthma therapies.


Assuntos
Asma , Óxido Nítrico Sintase Tipo II/genética , Asma/genética , Criança , Expiração , Humanos , Óxido Nítrico , Polônia , Polimorfismo de Nucleotídeo Único , Regiões Promotoras Genéticas , Fatores de Risco
3.
Postepy Dermatol Alergol ; 37(3): 326-332, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32792871

RESUMO

The hyperimmunoglobulin E syndrome (HIES) is a rare multi-system disease with non-immunological as well as immunological abnormalities. The syndrome is characterized by a triad of the most distinctive symptoms, such as pneumonia with pneumatocele formation, recurring staphylococcal skin abscesses and a high serum concentration of IgE. Central mediators of immune responses such as STAT1 and STAT3 affect immune responses and contribute to changes of the skin microbiome which subsequently can amplify the defective immune response against microbial and fungal pathogens. Reactions related to an environmental factor, such as sun-induced skin changes, in individuals during long-term medication therapy have also been reported. The dermatological symptoms, oral status and other health problems of a hyperimmunoglobulin E syndrome paediatric patient are presented. HIES is of great importance to different professionals because sufferers require special preventive and therapeutic management from early infancy in order to avoid complications which can even prove to be life-saving for such patients.

4.
Postepy Dermatol Alergol ; 37(5): 760-765, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-33240017

RESUMO

INTRODUCTION: The development of granulomas is a well-recognized manifestation of immunodeficiency in ataxia-telangiectasia (A-T), resulting from lymphocyte developmental abnormalities, impaired immunosurveillance, and inappropriate innate immune response-driven inflammation. AIM: To better understand pathological and immunological phenomena involved in development of cutaneous and visceral granulomatosis observable in patients with ataxia-telangiectasia. MATERIAL AND METHODS: We retrospectively reviewed medical records of eight A-T children, aged from 2 to 13 years, with regard to clinical, immunological and histopathological features of cutaneous and visceral granulomatosis. RESULTS: In four out of eight A-T patients studied, cutaneous granulomas clinically presented as skin nodules and ulcerated erythematous plaques disseminated on the face, and on trauma-prone areas of upper and lower extremities. Visceral granulomatosis had a severe clinical course and involved the lungs, the spleen, the liver and the larynx. Histologically, cutaneous and laryngeal granulomas showed extensive cellular infiltrations containing T lymphocytes with predominating CD8+ phenotype and with CD68+ histiocytes. The immunological profile with the hyper-IgM phenotype, markedly reduced numbers of B and naive CD4+ and CD8+ T cells with predominating IgM-only memory B cells and skewed repertoire of a T cell receptor was observable in patients with skin and visceral granulomatosis. CONCLUSIONS: In the setting of combined immunodeficiency in A-T, cutaneous and systemic granulomatosis reflects a granulomatous reaction pattern, as a result of inappropriate immune regulation.

5.
Eur J Pediatr ; 178(6): 883-890, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30927097

RESUMO

Lung auscultation is an important part of a physical examination. However, its biggest drawback is its subjectivity. The results depend on the experience and ability of the doctor to perceive and distinguish pathologies in sounds heard via a stethoscope. This paper investigates a new method of automatic sound analysis based on neural networks (NNs), which has been implemented in a system that uses an electronic stethoscope for capturing respiratory sounds. It allows the detection of auscultatory sounds in four classes: wheezes, rhonchi, and fine and coarse crackles. In the blind test, a group of 522 auscultatory sounds from 50 pediatric patients were presented, and the results provided by a group of doctors and an artificial intelligence (AI) algorithm developed by the authors were compared. The gathered data show that machine learning (ML)-based analysis is more efficient in detecting all four types of phenomena, which is reflected in high values of recall (also called as sensitivity) and F1-score.Conclusions: The obtained results suggest that the implementation of automatic sound analysis based on NNs can significantly improve the efficiency of this form of examination, leading to a minimization of the number of errors made in the interpretation of auscultation sounds. What is Known: • Auscultation performance of average physician is very low. AI solutions presented in scientific literature are based on small data bases with isolated pathological sounds (which are far from real recordings) and mainly on leave-one-out validation method thus they are not reliable. What is New: • AI learning process was based on thousands of signals from real patients and a reliable description of recordings was based on multiple validation by physicians and acoustician resulting in practical and statistical prove of AI high performance.


Assuntos
Auscultação/instrumentação , Aprendizado de Máquina , Redes Neurais de Computação , Sons Respiratórios/diagnóstico , Adolescente , Algoritmos , Auscultação/métodos , Criança , Pré-Escolar , Humanos , Lactente , Sons Respiratórios/classificação , Estetoscópios
6.
Postepy Dermatol Alergol ; 35(6): 552-562, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30618521

RESUMO

The observed global climate change is an indisputable cause of the increased frequency of extreme weather events and related natural disasters. This phenomenon is observed all over the world including Poland. Moreover, Polish citizens as tourists are also exposed to climate phenomena that do not occur in our climate zone. Extreme weather events and related disasters can have a significant impact on people with allergic diseases, including asthma. These effects may be associated with the exposure to air pollution, allergens, and specific microclimate conditions. Under the auspices of the Polish Society of Allergology, experts in the field of environmental allergy prepared a statement on climate changes, natural disasters and allergy and asthma to reduce the risk of adverse health events provoked by climate and weather factors. The guidelines contain the description of the factors related to climate changes and natural disasters affecting the course of allergic diseases, the specific microclimate conditions and the recommendations of the Polish Society of Allergology for vulnerable population, patients suffering from asthma and allergy diseases, allergologists and authorities in the event of climate and weather hazards.

7.
J Asthma ; 54(7): 699-705, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28282228

RESUMO

INTRODUCTION: Noninvasive and easy-to-use tools to monitor airway inflammation in asthma are needed to maintain disease control, particularly in pediatric population. The aim of the study was to evaluate exhaled breath temperature (EBT) in pediatric respiratory clinic setting. METHODS: We evaluated 37 children and adolescents with asthma (5-17 years; median: 11 years). The patients were followed up in stable condition and during exacerbations (paired observations in n = 19 subjects). We evaluated medication use, EBT, fractional exhaled nitric oxide (FeNO), spirometry and atopic status of patients. RESULTS: EBT was significantly higher in children with asthma exacerbation {entire group: median [interquartile range (IQR)]: 32.3 [1.1]°C vs. 33.8 [1.7]°C; p < 0.001 and mean ± SD: 33.1 ± 1.0°C vs. 33.6 ± 1.1°C; p = 0.038 for paired observations}. Significant correlation was observed between EBT and FeNO in the entire group (r = 0.22; p = 0.03). No difference was observed in EBT median values in atopic and non-atopic subjects in the entire group (median [IQR]: 32.6 [1.6] vs. 32.7 [2.0]; p = 0.88) and in subgroups. There was no difference in EBT values in patients receiving systemic or inhaled glucocorticosteroids (p = 0.45 and 0.83). There was no significant correlation between EBT and body or room temperature. The only significant predictor of exacerbation in logistic regression model was EBT {aOR = 2.4; 95% [confidence interval (CI)]: 1.4-4.1}. ROC analysis demonstrated applicability of EBT as a marker of asthma exacerbation in children (AUC = 0.748; p < 0.001; cut-off = 33.3°C; sensitivity: 64.3%; specificity: 82.1%). CONCLUSIONS: We suggest that EBT may serve as marker and predictor of asthma exacerbation in children. EBT follow-up may be useful in asthma monitoring in children and adolescents.


Assuntos
Asma/metabolismo , Testes Respiratórios/métodos , Temperatura , Adolescente , Antiasmáticos/uso terapêutico , Asma/tratamento farmacológico , Asma/fisiopatologia , Biomarcadores , Temperatura Corporal , Criança , Pré-Escolar , Expiração , Feminino , Humanos , Masculino , Óxido Nítrico/metabolismo , Espirometria
8.
Postepy Dermatol Alergol ; 33(3): 206-10, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27512356

RESUMO

INTRODUCTION: Beekeepers are a group of people with high exposure to honeybee stings and with a very high risk of allergy to bee venom. Therefore, they are a proper population to study the correlations between clinical symptoms and results of diagnostic tests. AIM: The primary aim of our study was to assess the correlations between total IgE, venom- and phospholipase A2-specific IgE and clinical symptoms after a bee sting in beekeepers. The secondary aim was to compare the results of diagnostic tests in beekeepers and in individuals with standard exposure to bees. MATERIAL AND METHODS: Fifty-four individuals were divided into two groups: beekeepers and control group. The levels of total IgE (tIgE), venom-specific IgE (venom sIgE), and phospholipase A2-specific IgE (phospholipase A2 sIgE) were analyzed. RESULTS: Our study showed no statistically significant correlation between the clinical symptoms after a sting and tIgE in the entire analyzed group. There was also no correlation between venom sIgE level and clinical symptoms either in beekeepers or in the group with standard exposure to bees. We observed a statistically significant correlation between phospholipase A2 sIgE level and clinical signs after a sting in the group of beekeepers, whereas no such correlation was detected in the control group. Significantly higher venom-specific IgE levels in the beekeepers, as compared to control individuals were shown. CONCLUSIONS: In beekeepers, the severity of clinical symptoms after a bee sting correlated better with phospholipase A2 sIgE than with venom sIgE levels.

9.
Pneumonol Alergol Pol ; 82(4): 364-7, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-24964240

RESUMO

Primary Epstein-Barr virus infection in children typically presents as infectious mononucleosis and in immunocompetent individuals severe pneumonitis proves to be a rare complication. Chronic active Epstein-Barr virus infection (CAEBV) is associated with multiple life-threatening conditions, including interstitial lung disease with fibrosis and lymphoid and lymphohistiocytic infiltrations. We report on a pediatric patient in whom CAEBV resulted in severe pneumopathy with a fatal outcome.


Assuntos
Infecções por Vírus Epstein-Barr/complicações , Síndromes de Imunodeficiência/complicações , Pneumopatias/diagnóstico , Pneumopatias/etiologia , Adolescente , Doença Crônica , Evolução Fatal , Humanos , Masculino
10.
Cent Eur J Immunol ; 39(3): 392-5, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-26155153

RESUMO

Severe combined immunodeficiency (SCID) is characterized by the absence of functional T lymphocytes and impairment of adaptive immunity. While heterogeneity of the genetic background in SCID leads to the variability of immune phenotypes, most of affected newborns appear healthy but within the first few months they develop life-threatening opportunistic respiratory or gastrointestinal tract infections. The objective of the study was to define the presenting features and etiology of infections in children with SCID. We retrospectively reviewed five children in whom the diagnosis of SCID had been established in our pediatric immunology clinic over the last 10-year period. A viral respiratory tract infection was the first manifestation of SCID in all the children studied. Cytomegalovirus (CMV) pneumonia was recognized in as many as 4 cases and coronavirus pulmonary infection was diagnosed in one case, whereas Pneumocystis jiroveci was identified as a co-pathogen in one CMV-infected patient. Severe combined immunodeficiency is a pediatric emergency condition and given the significant impact of pulmonary CMV infection in SCID children, establishing an accurate etiological diagnosis is of essential importance in instituting the specific treatment and improving the outcome.

11.
Adv Respir Med ; 92(4): 254-262, 2024 Jun 24.
Artigo em Inglês | MEDLINE | ID: mdl-39051187

RESUMO

In recent years, questionnaires were published in English to assess the quality of life of patients with PCD (Primary Ciliary Diskinesia) for adults, adolescents aged 13-17 years, and children aged 6-12 years and their caregivers. This study aimed to prepare Polish versions of the questionnaires and validate them in specific age groups with the participation of Polish patients with PCD. The individual questionnaires were translated and discussed with the involvement of the creator of the original questionnaire in English. Patients completed the questionnaires according to their affiliation with one of the groups. Validation was based on internal consistency analysis (Cronbach's alpha coefficient and split-half reliability) and test-retest reliability (intraclass correlation coefficient-ICC). The internal consistency of all questionnaires was from moderate to very good (Cronbach's alpha 0.67-0.91, split-half reliability 0.53-0.95). The consistency of the measurements showed excellent repeatability (ICC 0.67-0.91). The surveyed Polish PCD patients rated their quality of life quite well (63-77%). QOL questionnaires for patients with PCD can be used routinely during each medical check-up as a simple tool to provide the doctor with an indication of the effectiveness of treatment and the impact of the disease on the patient's quality of life.


Assuntos
Qualidade de Vida , Humanos , Qualidade de Vida/psicologia , Inquéritos e Questionários/normas , Polônia , Adolescente , Criança , Masculino , Feminino , Reprodutibilidade dos Testes , Adulto , Transtornos da Motilidade Ciliar
12.
Biomedicines ; 11(7)2023 Jul 06.
Artigo em Inglês | MEDLINE | ID: mdl-37509558

RESUMO

An IgE-mediated food allergy (FA) in atopic dermatitis (AD) children should be easily differentiated from other immune-mediated adverse effects related to food. Specific IgEs for particular protein components has provided additional diagnostic value. However, component-resolved diagnostics (CRD) has not solved all diagnostic problems either. We analysed the serum profile of 42 amino acids (AAs) in 76 AD children aged 2-60 months with an IgE-mediated FA (n = 36), with a non-IgE-mediated FA (n = 15) and without an FA (n = 25) using high-performance liquid chromatography coupled with mass spectrometry (LC-MS/MS) and an aTRAQ kit. We identified homocitrulline (Hcit), sarcosine (Sar) and L-tyrosine (Tyr) as features that differentiated the studied groups (one-way ANOVA with least significant difference post hoc test). The Hcit concentrations in the non-IgE-mediated FA group were significantly decreased compared with the IgE-mediated FA group (p = 0.018) and the control group (p = 0.008). In AD children with a non-IgE-mediated FA, the Tyr levels were also significantly reduced compared with the controls (p = 0.009). The mean concentration of Sar was the highest in the non-IgE-mediated FA group and the lowest in the IgE-mediated FA group (p = 0.047). Future studies should elucidate the involvement of these AAs in the molecular pathway of IgE- and non-IgE-mediated allergic responses.

13.
Diagnostics (Basel) ; 13(14)2023 Jul 11.
Artigo em Inglês | MEDLINE | ID: mdl-37510079

RESUMO

Aim: Recently, the most commonly used for multiple breath washout device, the Exhalyzer D, has been shown to overestimate lung clearance index (LCI) results due to a software error. Our study aimed to compare the predictive values of LCI in the CF pulmonary exacerbations (PE) calculated with the updated (3.3.1) and the previous (3.2.1) version of the Spiroware software. Materials and Methods: The measurements were performed during 259 visits in CF pediatric patients. We used 39ΔPE pairs (PE preceded by stable visit) and 138ΔS pairs (stable visit preceded by stable visit) to compare the LCI changes during PE. The areas under the receiver operating curves (AUCROC) and odds ratios were calculated based on the differences between ΔPEs and ΔSs. The exacerbation risk was estimated using a logistic regression model with generalized estimating equations (GEE). Results: There were statistically significant differences in LCI 2.5% median values measured using the two versions of the software in the stable condition but not during PE. The AUCROC for changes between the two consecutive visits for LCI did not change significantly using the updated Spiroware software. Conclusions: Despite the lower median values, using the recalculated LCI values does not influence the diagnostic accuracy of this parameter in CF PE.

14.
J Asthma ; 49(4): 329-35, 2012 May.
Artigo em Inglês | MEDLINE | ID: mdl-22468730

RESUMO

OBJECTIVES: Asthma is a heterogenous complex disorder caused by chronic inflammation of the airways. The key issue in genetic association studies of complex disorders is the identification of multiple low-risk genes that individually have little impact on the phenotype, but in combination account for the clinical manifestation of asthma. Since neurogenic inflammation is emerging as a candidate factor in the pathogenesis of asthma, the aim of the study was to investigate whether genetic variants of neurotrophin genes are associated with asthma disease severity or asthma-related phenotypes in a pediatric population. METHODS: We genotyped 27 polymorphisms located in neurotrophin genes, using TaqMan SNP genotyping assays or Polymerase Chain Reaction - Restriction Fragments Lengths Polymorphism (PCR-RFLP) in 200 children diagnosed with asthma and 226 controls. Interactions between 27 polymorphic loci and asthma-related phenotypes were determined using the Multifactor Dimensionality Reduction (MDR) method. RESULTS: In single marker analysis, we observed an association of MAP3K1 gene polymorphisms (rs702689 and rs889312) with asthma. We also observed that four Single Nucleotide Polymorphisms (SNPs) were associated with severe asthma. Analysis stratified by asthma-related phenotype revealed an association between atopy and NGFR (rs3785931), while BDNF (rs7124442), NTRK2 (rs1212171), NGFR (rs2072446), and FYN (rs3730353) variants were associated with increased exhaled nitric oxide (exNO). In addition, gene-gene interaction analysis revealed a significant epistatic interaction between MAPK (rs889312) and NGF (rs11102930) variants in asthma susceptibility. CONCLUSIONS: Our results suggest that genetic variants of MAP3K1 and NGF genes involved in the regulation of neurogenic inflammation may contribute to asthma, possibly via enhanced NGF expression and MAPK signaling pathway activation.


Assuntos
Asma/genética , Estudos de Associação Genética , Hipersensibilidade Imediata/genética , MAP Quinase Quinase Quinase 1/genética , Inflamação Neurogênica/genética , Adolescente , Estudos de Casos e Controles , Criança , Feminino , Genótipo , Humanos , Masculino , Fatores de Crescimento Neural/genética , Fenótipo , Polimorfismo Genético , Testes de Função Respiratória , Índice de Gravidade de Doença
15.
Artigo em Inglês | MEDLINE | ID: mdl-35805534

RESUMO

The main risk factor for the development of food allergies (FAs) in children is atopic dermatitis (AD). AD is usually recognized as the Th1/Th2 paradigm of allergic disease. Recently, the Th1/Th2 paradigm in allergy and autoimmunity has been revised, including the role of the Th17 cell population and related cytokines. However, there are only a few studies that have found Th17 cytokine involvement in the allergic inflammatory response, especially with food allergens. This research aimed to analyze the serum profile of cytokines involved in the T-helper cell type 17 immune response pathway in young, atopic children with an IgE-mediated and delayed-type FA. The study involved 76 children (0−5 years old) with chronic AD. We used the Bio-Plex system to simultaneously determine the concentrations of 15 different cytokines in one experiment. In accordance with complete dermatological and allergological examination, including OFC testing and ALEX2 assays, participants were divided into 3 groups: IgE-mediated FA, delayed-type FA, and the control group. Data were analyzed using univariate statistical tests. In the IgE-mediated FA group, the circulating levels of tested cytokines had increased compared with those of other patients; however, a statistically significant difference was only obtained for IL-1beta (p < 0.05). According to the ROC curves, IL-1beta may be considered an effective predictor of IgE-mediated FA in AD children (p < 0.05; AUC = 0.67). In the delayed-type FA group, the concentration of most cytokines had slightly decreased compared to the control group. The obtained results suggest that FA influences the Th17-related cytokine profile in the serum of AD children. More advanced studies are needed to confirm the involvement of Th17 cytokines in the allergic inflammatory response and to prove their usefulness in clinical practice.


Assuntos
Dermatite Atópica , Hipersensibilidade Alimentar , Pré-Escolar , Citocinas/metabolismo , Dermatite Atópica/etiologia , Humanos , Imunidade , Imunoglobulina E , Lactente , Recém-Nascido , Células Th17/metabolismo , Células Th2/metabolismo
16.
J Allergy Clin Immunol ; 125(2): 424-432.e8, 2010 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-20159255

RESUMO

BACKGROUND: The hyper-IgE syndrome (HIES) is a primary immunodeficiency characterized by infections of the lung and skin, elevated serum IgE, and involvement of the soft and bony tissues. Recently, HIES has been associated with heterozygous dominant-negative mutations in the signal transducer and activator of transcription 3 (STAT3) and severe reductions of T(H)17 cells. OBJECTIVE: To determine whether there is a correlation between the genotype and the phenotype of patients with HIES and to establish diagnostic criteria to distinguish between STAT3 mutated and STAT3 wild-type patients. METHODS: We collected clinical data, determined T(H)17 cell numbers, and sequenced STAT3 in 100 patients with a strong clinical suspicion of HIES and serum IgE >1000 IU/mL. We explored diagnostic criteria by using a machine-learning approach to identify which features best predict a STAT3 mutation. RESULTS: In 64 patients, we identified 31 different STAT3 mutations, 18 of which were novel. These included mutations at splice sites and outside the previously implicated DNA-binding and Src homology 2 domains. A combination of 5 clinical features predicted STAT3 mutations with 85% accuracy. T(H)17 cells were profoundly reduced in patients harboring STAT3 mutations, whereas 10 of 13 patients without mutations had low (<1%) T(H)17 cells but were distinct by markedly reduced IFN-gamma-producing CD4(+)T cells. CONCLUSION: We propose the following diagnostic guidelines for STAT3-deficient HIES. Possible: IgE >1000IU/mL plus a weighted score of clinical features >30 based on recurrent pneumonia, newborn rash, pathologic bone fractures, characteristic face, and high palate. Probable: These characteristics plus lack of T(H)17 cells or a family history for definitive HIES. Definitive: These characteristics plus a dominant-negative heterozygous mutation in STAT3.


Assuntos
Síndrome de Job/diagnóstico , Síndrome de Job/genética , Fator de Transcrição STAT3/genética , Adolescente , Adulto , Separação Celular , Criança , Pré-Escolar , Ensaio de Imunoadsorção Enzimática , Feminino , Citometria de Fluxo , Humanos , Imunoglobulina E/sangue , Lactente , Interleucina-17/imunologia , Síndrome de Job/imunologia , Masculino , Pessoa de Meia-Idade , Mutação , Reação em Cadeia da Polimerase , Guias de Prática Clínica como Assunto , Linfócitos T Auxiliares-Indutores/imunologia , Adulto Jovem
17.
Pol Merkur Lekarski ; 30(179): 349-51, 2011 May.
Artigo em Polonês | MEDLINE | ID: mdl-21675140

RESUMO

Antibiotics have significant role in the treatment of respiratory diseases. The main aim of their use is to treat infection, but anti-inflammatory properties of macrolides are also beneficial in selected diseases. The role of antibiotics in the therapy of asthma exacerbation can be neglected and should be limited to exceptional situations of bacterial infections which are very rare. During last few years the role of atypical infections in asthma inception, induction of exacerbation and modification of chronic course of disease has been discussed. Antibiotics play significant role in cystic fibrosis therapy. They are especially recommended during exacerbation, when new pathogens are revealed and in chronic Pseudomonas aeruginosa infection. This paper includes the principal rules of antibiotics therapy in patients with cystic fibrosis. The role of azithromycin in antiinflammatory therapy in this group of patients is also presented.


Assuntos
Antibacterianos/uso terapêutico , Asma/complicações , Asma/tratamento farmacológico , Fibrose Cística/complicações , Infecções Respiratórias/tratamento farmacológico , Anti-Inflamatórios/uso terapêutico , Azitromicina/uso terapêutico , Criança , Humanos , Infecções Respiratórias/complicações
18.
Pol Merkur Lekarski ; 31(186): 335-9, 2011 Dec.
Artigo em Polonês | MEDLINE | ID: mdl-22239001

RESUMO

UNLABELLED: Although pneumothorax has been thoroughly described since the beginning of 19th century there are few studies investigating its epidemiology in pediatric patients. The aim of this study was to evaluate incidence and clinical picture of pneumothorax in pediatric patients. MATERIALS AND METHOD: A retrospective review of medical records of all patients treated in the Department of Pediatric Pulmonology, Allergy and Clinical Immunology of Karol Marcinkowski Medical University from January 1999 to December 2008 with the diagnosis of pneumothorax. Data analyzed include anthropometric parameters, present comorbidities, clinical presentation, treatment, length of hospital stay as well as presence and type of complications. RESULTS: 27 episodes of pneumothorax in 21 patients were treated in the given period. There were 52% of episodes of spontaneous primary pneumothorax, 37% of secondary spontaneous pneumothorax and 11% of pneumothorax due to non-penetrating trauma. 59% of patients were males. Presenting symptoms included dyspnoe (59%), chest pain (48%) and cough (44%). 74% of cases required treatment with chest tube drainage: 80% episodes of primary spontaneous pneumothorax, 67% episodes of non-penetrating trauma pneumothorax and 71% episodes of secondary spontaneous pneumothorax. Mean time of chest tube drainage was 6.2 +/- 5.1 days: 4.5 +/- 0.7 days for non-penetrating trauma pneumothorax, 5.7 +/- 6.4 days for secondary and 7.2 +/- 1.4 days for primary spontaneous pneumothorax. Chest tube drainage was successful in 80% of cases. 4 patients were referred to thoracic surgeons. One child was treated with chemical pleurodesis. Mean hospital stay was 22.2 +/- 13.7 days: 11.1 +/- 0.7 days for patients with non-penetrating trauma pneumothorax and 29.7 +/- 1.4 days for patients with spontaneous secondary pneumothorax. CONCLUSIONS: Primary and secondary spontaneous pneumothorax is a rare event in children and the majority of patients are male. Secondary spontaneous pneumothorax is a complication of underlying chronic pulmonary conditions, most frequently cystic fibrosis and pulmonary infections. Children presenting with spontaneous secondary pneumothorax tended to be younger and required longer hospital stay.


Assuntos
Pneumotórax/diagnóstico , Pneumotórax/epidemiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Tempo de Internação , Masculino , Pneumotórax/terapia , Polônia/epidemiologia , Estudos Retrospectivos
19.
Przegl Lek ; 68(1): 29-32, 2011.
Artigo em Polonês | MEDLINE | ID: mdl-21563441

RESUMO

Broncholitis in infant is most commonly results from viral infection, typically RS virus will be responsible. Treatment is difficult due to limited efficacy of available methods of causal and symptomatic therapies. Therefore it is specially important to seek preventive measures. This is crucial in case of preterm infants, infants with broncho-pulmonary dysplasia, cystic fibrosis, hemodynamically significant congenital heart defects and immunodeficiencies who are likely to undergo a severe course of the disease. In the above mentioned cases the effects of passive immunotherapy of prophylaxis with initially intravenous anti RSV-immunoglobulin G, then intramuscular monoclonal antibody palivizumab have been assessed. Palivizumab is currently recommended by American Academy of Pediatrics for RSV infection prophylaxis with children at risk like infants with broncho-pulmonary dysplasia, preterm infants depending on gestational age and infant age in infection season as well as infants with hemodynamically significant congenital heart defects. So far there is no possibility to prevent infection within the whole population.


Assuntos
Bronquiolite/prevenção & controle , Anticorpos Monoclonais/administração & dosagem , Anticorpos Monoclonais Humanizados , Antivirais/administração & dosagem , Bronquiolite/tratamento farmacológico , Bronquiolite/virologia , Displasia Broncopulmonar/complicações , Criança , Fibrose Cística/complicações , Cardiopatias Congênitas/complicações , Humanos , Imunoglobulina G/administração & dosagem , Imunoterapia , Lactente , Recém-Nascido , Doenças do Prematuro/prevenção & controle , Injeções Intramusculares , Injeções Intravenosas , Palivizumab
20.
Int J Immunopathol Pharmacol ; 35: 2058738421990493, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33626956

RESUMO

Allergic rhinitis (AR) and allergic asthma (AA) exhibit similar inflammatory response in the airways. However, the remodelling is more extensive in the lower airways, suggesting that the inflammation itself is not sufficient for allergic phenotype. We aimed to analyse whether the expression of selected 27 inflammatory and fibrosis-related proteins may be altered in AR and AA in the paediatric population and whether the expression pattern is either similar (due to the inflammation) or disease-specific (due to the remodelling). We analysed 80 paediatric subjects: 39 with AA, 21 with AR and 20 healthy children. The diagnosis of AR and AA was based on clinical manifestation, lung function, positive skin prick tests and increased immunoglobulin E levels. Serum levels of selected inflammatory proteins were measured with custom Magnetic Luminex Assay. Statistical analysis was performed in Statistica v.13. CCL2/MCP1, GM-CSF, gp130 and periostin concentrations were significantly lower, whereas IL-5 levels were higher in AA compared to the control group. CD-40L, CHI3L1/YKL-40, EGF, GM-CSF and periostin levels were significantly decreased in patients with AR than in the control group. Comparison of AA and AR patients revealed significant changes in CHI3L1/YKL-40 (P = 0.021), IL-5 (P = 0.036), periostin (P = 0.013) and VEGFα (P = 0.046). Significantly altered proteins were good predictors to distinguish between AA and AR (P < 0.001, OR 46.00, accuracy 88.57%). Our results suggest that the expression of four fibrotic proteins was significantly altered between AA and AR, suggesting possible differences in airway remodelling between upper and lower airways.


Assuntos
Asma/sangue , Rinite Alérgica/sangue , Adolescente , Asma/imunologia , Asma/patologia , Asma/fisiopatologia , Moléculas de Adesão Celular/sangue , Criança , Proteína 1 Semelhante à Quitinase-3/sangue , Receptor gp130 de Citocina/sangue , Citocinas/sangue , Feminino , Fibrose , Humanos , Imunoglobulina E/sangue , Masculino , Sistema Respiratório/patologia , Rinite Alérgica/imunologia , Rinite Alérgica/patologia , Rinite Alérgica/fisiopatologia , Testes Cutâneos , Espirometria , Fator A de Crescimento do Endotélio Vascular/sangue
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