Role of Lung Ultrasonography (LUS) as a Tool for Evaluating Children with Pediatric Inflammatory Multisystem Syndrome Temporally Associated with SARS-CoV-2 (PIMS-TS).

BACKGROUND: Pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) is a novel entity. The inflammatory process involves the circulatory, digestive, respiratory, and central nervous systems, as well as the skin. Making a diagnosis requires extensive differential diagnoses, including lung imaging. The aim of our study was to retrospectively assess the pathologies found in lung ultrasound (LUS) in children diagnosed with PIMS-TS and to evaluate the usefulness of the examination in diagnostics and monitoring. METHODS: The study group consisted of 43 children diagnosed with PIMS-TS, in whom LUS was performed at least three times, including on admission to hospital, on discharge, and 3 months after disease onset. RESULTS: Pneumonia (mild to severe) was diagnosed in 91% of the patients based on the ultrasound image; the same number had at least one pathology, including consolidations, atelectasis, pleural effusion, and interstitial or interstitial-alveolar syndrome. By the time of discharge, the inflammatory changes had completely regressed in 19% of the children and partially in 81%. After 3 months, no pathologies were detected in the entire study group. CONCLUSION: LUS is a useful tool for diagnosing and monitoring children with PIMS-TS. Inflammatory lesions of the lungs resolve completely when the generalized inflammatory process subsides.

Comparison of COVID-19 and RSV Infection Courses in Infants and Children under 36 Months Hospitalized in Paediatric Department in Fall and Winter Season 2021/2022.

BACKGROUND: The study aimed to determine the differences between COVID-19 and Respiratory syncytial virus (RSV) infections in young children hospitalized in the pediatric department. METHODS: This retrospective study included 52 children with COVID-19 and 43 children with RSV infection younger than 36 months hospitalized in a pediatric department between September 2021 and March 2022. Clinical and laboratory findings, methods of treatment and hospitalization length were compared. RESULTS: In the RSV group, significantly higher rates of cough (93.2% vs. 38.5%), rhinitis (83.7% vs. 50%), dyspnea (83.7% vs. 21.1%), crackles (69.8% vs. 5.8%) and wheezes (72.1% vs. 9.6%) were observed. The COVID-19 group had significantly higher rates of fever (80.8% vs. 37.2%) and seizures (13.5% vs. 0%). Patients with RSV infection had significantly higher rates of bronchodilator therapy (88.37% vs. 5.77%) and oxygen therapy (48.8% vs. 7.7%) and required a longer hospital stay (8 vs. 3 days). In admission, the majority of the patients from both groups were not treated with antibiotics, but because of clinical deterioration and suspected bacterial co-infections, antibiotics were administered significantly more frequently in the RSV group (30.2% vs. 9.6%). CONCLUSIONS: RSV infection in infants and small children had a more severe course than COVID-19 infection. RSV infection was associated with a longer hospitalization period and required more elaborate treatment.

COVID-19 in unvaccinated patients with inborn errors of immunity-polish experience.

At the beginning of the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic, patients with inborn errors of immunity (IEI) appeared to be particularly vulnerable to a severe course of the disease. It quickly turned out that only some IEI groups are associated with a high risk of severe infection. However, data on the course of Coronavirus Disease 2019 (COVID-19) in patients with IEI are still insufficient, especially in children; hence, further analyses are required. The retrospective study included 155 unvaccinated people with IEI: 105 children and 50 adults (67.7% and 32.3%, respectively). Male patients dominated in the study group (94 people, 60.6%). At least two comorbidities were found in 50 patients (32.3%), significantly more often in adults (56% vs. 21%). Adult patients presented significantly more COVID-19 symptoms. Asymptomatic and mildly symptomatic course of COVID-19 was demonstrated in 74.8% of the entire group, significantly more often in children (88.6% vs. 46%). Moderate and severe courses dominated in adults (54% vs. 11.4%). Systemic antibiotic therapy was used the most frequently, especially in adults (60% vs. 14.3%). COVID-19-specific therapy was used almost exclusively in adults. In the whole group, complications occurred in 14.2% of patients, significantly more often in adults (30% vs. 6.7%). In the pediatric group, there were two cases (1.9%) of multisystem inflammatory syndrome in children. Deaths were reported only in the adult population and accounted for 3.9% of the entire study group. The death rate for all adults was 12%, 15.4% for adults diagnosed with common variable immunodeficiency, 12.5% for those with X-linked agammaglobulinemia, and 21.4% for patients with comorbidity. The results of our study imply that vaccinations against COVID-19 should be recommended both for children and adults with IEI. Postexposure prophylaxis and early antiviral and anti-SARS-CoV-2 antibody-based therapies should be considered in adults with IEI, especially in those with severe humoral immune deficiencies and comorbidity.

Biochemical and cardiovascular predictors of PIMS-TS risk in children after COVID-19 recovery: preliminary results of the LATE-COVID-Kids Study.

Introduction: We aimed to characterize biochemical and cardiovascular predictors of the paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) risk based on the data from the LATE-COVID-Kids study. Methods: 148 consecutive COVID-19 convalescents hospitalized for the clinical evaluation after the acute phase of COVID-19 were classified into two groups related to symptoms: 33 children finally diagnosed with PIMS-TS and 115 children without PIMS-TS (control group). Results: PIMS-TS children were significantly younger (6.79 ±4.57 vs. 9.10 ±4.94 years). After adjustment, in comparison to those without, PIMS-TS children had a higher level of antithrombin III (111 ±9.30 vs. 105 ±11.4), higher heart rate (HR)/min (100 (89.0-111) vs. 90 (79.7-100)) and sinus rhythm (p = 0.03) but lower PQ interval (p = 0.02) on admission to hospital. The lymphocytes (absolute count and percentage) were significantly higher in children with PIMS-TS, and the opposite results were obtained for IgA and neutrophils. Furthermore, children with PIMS-TS had a higher level of thyroid stimulating hormone (2.76 (2.16-4.18) vs. 2.36 (1.73-2.83)) and red cell distribution width (p < 0.005) compared to those without. Conclusions: It is the first data on the possible predictors of PIMS-TS risk in the Long-COVID period. These results need to be further validated to next create the PIMS SCORE algorithm, which might enable the effective prediction of children with the risk of PIMS-TS occurrence after COVID-19 recovery.

Toll-like receptor (TLR2, TLR4) polymorphisms and their influence on the incidence of urinary tract infections in children with and without urinary tract malformation.

Introduction: Toll-like receptors (TLRs) contribute to the innate immune system. They are an element of non-specific immunity, which enables organisms to react quickly to foreign antigens, without being previously exposed to them. TLRs are pattern recognition receptors. TLR gene polymorphisms are widely investigated in connection with various infections. The aims of the study were: to investigate the role of TLR2 and TLR4 polymorphisms in the course of urinary tract infections (UTIs); to test for differences in distribution of these polymorphisms between children with urinary tract malformations suffering from recurrent UTI (rUTI), children with malformations but without rUTI and healthy controls; to determine whether these polymorphisms predispose to rUTI; and to analyse how polymorphisms and urine neutrophil gelatinase-associated lipocalin (NGAL) and interleukin 8 (IL-8) concentrations affect one another. Material and methods: The group consisted of 133 children (1-18 years old), 68 female and 65 male. The group was divided into 4 subgroups: A (rUTI with urinary tract malformations), B (urinary tract malformations without rUTI), C (rUTI) and D (healthy controls). Polymorphisms were analysed using PCR-RFLP. IL-8 and NGAL urine concentrations were established using immunoenzymatic methods. Results: TLR2 Arg753Gln and TLR4 Arg299Gly appeared significantly more often among children with rUTI. No correlation between urine IL-8 and urine NGAL and polymorphisms was found. Urine NGAL concentration was significantly higher among children with urinary tract malformations. Conclusions: TLR2 Arg753Gln and TLR4 Asp299Gly may predispose to rUTI. Urine NGAL concentration suggests the presence of kidney tissue injury, of varying degrees, among children with urinary tract malformations.

Nomograms of the fetal thymus for clinical practice.

INTRODUCTION: The fetal thymus may be visualized using ultrasonography (USG) and is typically located in the mediastinum. In the past years, the size of the fetal thymus has served not only as a marker of genetic or heart defects but also as a predictive factor for intrauterine growth restriction, premature birth, preeclampsia, chorioamnionitis or even neonatal sepsis. MATERIAL AND METHODS: A total of 410 fetuses were qualified for the study. Fetuses with heart defects were excluded from the study. The fetal thymus was evaluated with ultrasonography between the 14th and 40th week of gestation. After obtaining a standard transverse view encompassing the three great vessels, thymus measurements were attempted, i.e. maximal transverse diameter, circumference and surface area. Linear regression was used for statistical analysis, yielding 3 models, each with a different dependent variable. The confidence interval for each model was set at 80% to aid the comparison with centile grid growth charts for neonates and children. The test was regarded as statistically significant when p < 0.05. RESULTS: From a total of 410 fetuses the thymus transverse diameter, circumference and area were successfully measured in 410, 320 and 330 cases, respectively. The probabilities are lower than 0.0005 for each model, which means that each model is quite statistically significant. CONCLUSIONS: The coverage of healthy thymus nomograms in the fetal population may be the basis for the identification of fetuses at risk of hypoplasia or thymic hyperplasia, which seems particularly important from the point of view of the detection of potential inborn immunological disorders.

CSF levels of myelin basic protein in pediatric patients with ventriculoperitoneal shunt infection.

INTRODUCTION: Hydrocephalus is a common disorder of the central nervous system (CNS) in the pediatric population. Surgical treatment options involve ventriculoperitoneal shunt (VPS) placement. VPS infection is the most common complication of surgically treated hydrocephalus in pediatric patients [1, 2],which may lead to neuronal damage. Myelin basic protein (MBP) has been proposed as a marker of neuronal injury in a variety of contexts, and MBP levels in the cerebrospinal fluid (CSF) may be used to assess the severity of neuronal damage [1, 3, 4]. Therefore, the aim of this study was to evaluate the CSF level of myelin basic protein (MBP) in a group of pediatric patients with VPS infection. MATERIAL AND METHODS: Thirty CSF samples were collected from pediatric patients with VPS infection. CSF levels of MBP were measured at three time points, marked by contamination detection, obtention of the first sterile CSF culture, and VPS shunt implantation. The collected data were compared with those of the control group composed of children with active congenital hydrocephalus and valid CSF values. RESULTS: The MBP level in the study group was higher than the corresponding control values in the second and third measurements. The highest MBP level was reached in the study group in the second and third measurements. CONCLUSIONS: The lack of normalization of MBP level in the CSF of children with shunt infection could be connected with ongoing brain damage. It takes longer than the normalization of CSF protein level and pleocytosis. The delay is associated with a prolonged reaction of the immunological system.

Vaccination in children with chronic severe neutropenia - review of recommendations and a practical approach.

While the management of childhood neutropenia associated with a modifiable factor should be appropriate for the primary cause, there are misconceptions regarding the management of severe congenital neutropenia, immune neutropenia and cases classified as "idiopathic". Antibiotic prophylaxis or granulocyte-colony stimulating factor (G-CSF) are prescribed by specialists in pediatric hematology or immunology, whereas immunization may be conducted by primary care physicians should clear recommendations by provided. There is a belief that severe neutropenia, as an immunodeficiency, is associated with compromised effectiveness and increased rate of complications of immunization. The immunization might be delayed or omitted, increasing the risk of unnecessary infection. We discuss the available data and recommendations regarding vaccination of children with chronic severe neutropenia. While there are virtually no studies addressing the safety and effectiveness of vaccination in neutropenia, expert opinions provide information on immunization policy in "phagocytic cells defects" or explicitly neutropenia. There are no contraindications for inactivated vaccines in neutropenia. Live bacterial vaccines are contraindicated. While in general the vaccination with live viral vaccines is encouraged, occasionally neutropenia might be associated with defects of adaptive immunity, which would preclude the administration of such vaccines. Although this should be easily phenotypically identified, we propose assessing immunoglobulin levels and performing a low-cost flow cytometry test for major lymphocyte subpopulations to exclude significant defects in adaptive immunity before administration of live viral vaccines to such patients. This can improve the adherence of patients' guardians and physicians to proposed vaccination policy and the professional and legal safety associated with the procedure.

Unexpected eosinophilia in children affected by hydrocephalus accompanied with shunt infection.

PURPOSE: The aim of the article is to describe an immunological reaction to shunt infection in children with hydrocephalus. The main cause of shunt infection involves methicillin resistant Staphylococcus epidermidis (Bhatia et al. Indian J Med Microbiol 35:120-123, 2017; Hayhurst et al. Childs Nerv Syst 24:557-562, 2008; Martínez-Lage et al. Childs Nerv Syst 26: 1795-1798, 2010; Simon et al. PLoS One, 2014; Snowden et al. PLoS One 8:e84089, 2013; Turgut et al. Pediatr Neurosurg 41:131-136, 2005), a bacterial strain which is responsible for the formation of biofilm on contaminated catheters (Snowden et al. PLoS One 8:e84089, 2013; Stevens et al. Br J of Neurosurg 26: 792-797, 2012). METHODS: The study group involved 30 children with congenital hydrocephalus after shunt system implantation, whose procedures were complicated by S. epidermidis implant infection. Thirty children with congenital hydrocephalus awaiting their first-time shunt implantation formed the control group. The level of eosinophils in peripheral blood was assessed in both groups. Cerebrospinal fluid (CSF) was examined for protein level, pleocytosis, interleukins, CCL26/Eotaxin-3, IL-5, IL-6, CCL11/Eotaxin-1, CCL3/MIP-1a, and MBP. Three measurements were performed in the study group. The first measurement was obtained at the time of shunt infection diagnosis, the second one at the time of the first sterile shunt, and the third one at the time of shunt reimplantation. In the control group, blood and CSF samples were taken once, at the time of shunt implantation. RESULTS: In the clinical material, the highest values of eosinophils in peripheral blood and CSF pleocytosis were observed in the second measurement. It was accompanied by an increase in the majority of analyzed CSF interleukins. CONCLUSION: CSF pleocytosis observed in the study group shortly after CSF sterilization is presumably related to an allergic reaction to Staphylococcus epidermidis, the causative agent of ventriculoperitoneal shunt infection.

Lectin pathway factors in patients suffering from juvenile idiopathic arthritis.

Both complement activation and certain infections (including those with Yersinia sp.) may contribute to the pathogenesis of juvenile idiopathic arthritis (JIA). We investigated factors specific for the lectin pathway of complement: mannose-binding lectin (MBL), ficolins and MBL-associated serine protease-2 (MASP-2), in 144 patients and 98 controls. One hundred and six patients had oligoarticular disease and 38 had polyarticular disease. In 51 patients (out of 133 tested), Yersinia-reactive antibodies were found (JIA Ye+ group). MBL deficiency was significantly more frequent in the JIA Ye+ group than in patients without Yersinia-reactive antibodies or in controls. Median serum ficolin-2 level was significantly lower (and proportion of values deemed ficolin-2 insufficient greater) in JIA patients irrespective of their Yersinia antibody status. The minority (C) allele at -64 of the FCN2 gene was less frequent among JIA patients than among control subjects. No differences were found in the frequency of FCN3 gene +1637delC or MASP2 +359 A>G mutations nor for median values of serum ficolin-1, ficolin-3 or MASP-2. However, high levels of serum ficolin-3 were under-represented in patients, in contrast to MBL. MBL, ficolin-1, ficolin-2, ficolin-3 and MASP-2 were also readily detectable in synovial fluid samples but at a considerably lower level than in serum. Our findings suggest a possible role for the lectin pathway in the pathogenesis of JIA, perhaps secondary to a role in host defence, and indicate that investigations on the specificity of lectin pathway recognition molecules towards specific infectious agents in JIA might be fruitful.

Body mass index and waist-to-height ratio among schoolchildren with visual impairment: A cross-sectional study.

Children and adolescents with visual impairments may be predisposed to excessive body mass due to restrictions in everyday functioning and the ability to take part in physical activity. This study aimed to estimate the prevalence of obesity, overweight, and abdominal obesity (AO) among blind and partially sighted schoolchildren and to determine whether sociodemographic factors and participation in physical education classes (PEC) are associated with excessive body weight or AO in this group.A cross-sectional sample of 141 partially sighted or blind schoolchildren aged 7 to 18.9 years were included in this study. Anthropometric measurements were performed, and sociodemographic variables and ability to attend PEC were recorded. Overweight and obesity were noted among 21.3% and 14.9% of students, respectively. Although more males than females had excessive body weight (39.2% vs 32.3%), the difference was not significant (chi square test [ch] = 3.197; probability value [P] = 0.362). There was a significant association between mean body mass index standard deviation score and age (results of ANOVA analysis [F] = 5.620; P = 0.0045). A waist-to-height ratio (WHtR) ≥0.50 was observed among 27.7% of pupils. The prevalence of AO in boys and girls was 32.9% and 21.0%, respectively; this difference was not significant (ch = 2.48; P = 0.12). There was a significant relationship between mean WHtR and age (7-9 years: 0.477 ±â€Š0.050; 10-13 years: 0.484 ±â€Š0.065; ≥14 years: 0.454 ±â€Š0.061; results of Kruskal-Wallis test [H] = 8.729; P = 0.023, respectively).Multivariate logistic regression analysis showed that none of the sociodemographic variables examined (except "having siblings") were significantly associated with the occurrence of overweight, obesity, and AO. Subjects with no siblings were 4 times more likely to have WHtR ≥ 0.5 (odds ratio [OR] = 4.22; 95% confidence interval [CI] = 1.33-17.8; P = 0.01).Overweight and obesity were almost 4 times more frequent (OR = 3.74; 95% CI 0.81-17.4) and AO 3 times more frequent (OR = 3.18, 95% CI 0.71-14.2) among students not participating in PEC. Excessive body mass and AO represent an urgent health problem among schoolchildren with visual impairments. Health education concerning healthy eating habits and physical activity should be provided to this group to reduce potential future health costs.

Components of the lectin pathway of complement activation in paediatric patients of intensive care units.

Infections are a major cause of childhood mortality. We investigated components of the lectin pathway of complement activation in the context of sepsis at both genetic and protein levels in neonates, infants and older children. Major components of the lectin pathway and two genes for Toll-like receptors were studied in 87 neonates with confirmed sepsis and compared with 40 babies with infections who did not develop sepsis (disease controls) and 273 infection-free neonatal controls. A second cohort comprised 47 older children with sepsis and 87 controls. Low MBL-conferring genotypes (LXA/O+O/O) were more frequent in sepsis patients than in healthy controls but no significant differences in the frequency of SNPs of other lectin pathway genes (FCN1, FCN2, FCN3, MASP1/3, MASP2) or TLR receptor genes (TLR2, TLR4) were found. One case of primary MASP-2 deficiency was found among healthy pre-terms and one neonate suffering from SIRS was heterozygous for the rare FCN1 gene mutation, +6658 G>A. Generally, sepsis was associated with low serum MBL and low ficolin-2 concentrations on admission. Among neonates, ficolin-1 and MASP-2 levels were elevated in sepsis relative to healthy, but not disease, controls. Unlike neonates, ficolin-3 and MASP-2 levels were lower in older patients than in healthy controls while no difference was found for ficolin-1. With the possible exception of MBL, inherited lectin pathway insufficiencies do not seem to predispose to sepsis, rather changes in protein concentrations reflect alterations in disease course.

Small intestinal bacterial overgrowth syndrome in children.

INTRODUCTION: Small intestinal bacterial overgrowth syndrome (SIBO) is defined as an increased number of nonpathogenic bacteria over 10(5) organisms in 1 millilitre of small intestine content. The most common predisposing factors include, among others, gut motility disorders and chronic use of proton pump inhibitors. The results of recent studies indicate the importance of SIBO in gastrointestinal diseases. AIM: To assess the prevalence of SIBO in children with abdominal pain. MATERIAL AND METHODS: One hundred children (59 girls and 41 boys) aged from 4 to 17 years (mean age: 10.47 ±3.73 years), hospitalised due to abdominal pain, were enrolled in the study. Hydrogen breath test (HBT) with lactulose was established among all patients. Expired air was analysed using a Gastrolyzer (Bedfont). RESULTS: The HBT result was positive in 63 (63%) children with abdominal pain; including 40 girls (67.8%) and 23 boys (56.1%). The test was positive in the group of 29 (46%) children aged under 10 years and in the group of 34 (54%) children aged over 10 years. Among the patients who reported for the control study 88% achieved a normalisation of HBT after treatment. CONCLUSIONS: The prevalence of positive HBT results in the group of patients with abdominal pain is over 60%. Small intestinal bacterial overgrowth syndrome should be considered as one of the causes of abdominal pain in children. The SIBO in children shows a good response to treatment.

[Evaluation of selected parameters of specific immunity in children with osteosarcoma at various stages of antitumour treatment].

UNLABELLED: Typical cells of specific immunity are lymphocytes. T cells may specifically recognize antigens using T Cell Receptors (TCRs). Antigen presentation by specialized cells is a necessary element of specific immunity. It leads to initiating an immune response. After antigen dependent activation T cells transform to memory and effector lymphocytes. Cells engaged directly in destruction of the tumour are CD8+cytotoxic T lymphocytes, CD4+ lymphocytes, Tγδ lymphocytes, NK, NKT cells and indirectly B lymphocytes. One of the main methods of osteosarcoma treatment in children is chemotherapy. The goal is to destroy the cancer cells by putting them in a state of apoptosis. All of the medications used as chemotherapy carry the risk of short-term and long-term problems including leukopenia, immune disorders such as immunodeficiency. THE AIM OF STUDY: was evaluation by flow cytometry selected elements of specific cellular immunity in children with osteosarcoma at various stages of antitumour treatment. MATERIALS AND METHODS: The study was performed on the group of 44 children with osteosarcoma, aged from 6 to 20 years (average 14.9 years; median 15.0 years). T and B lymphocytes and subpopulations: CD4+, CD8+, CD3+?HLA-DR+, CD3+γδ; NK, NKT cells were analyzed in peripheral blood with use of flow cytometry method with monoclonal antibodies. Examinations were performed before the therapy - in diagnostic period (examination I), after the neoadjuvant chemotherapy (examination II), 10-14 days after the surgery (examination III), 5 months after the surgery (after adjuvant chemotherapy, examination IV). RESULTS: The number of T and B lymphocytes was decreasing after each stage of cytostatic therapy, with the biggest differences for CD19+ cells (medians: I examination - 205.0; II exam. - 62.0; IV exam. - 24.0 cells/mL); in single cases the number of cells decreased even under 10/mL (norm 200-500 cells/mL). CONCLUSIONS: 1. In children and youth with osteosarcoma antineoplastic treatment contributes to the suppression of the immune system, decreasing definitely the number and percentage of B lymphocytes, T helper lymphocytes and NK cells. 2. Decreased number of CD3+, CD4+, CD19+ and NK lymphocytes during chemotherapy may contribute to the progression of neoplastic disease in the future, after treatment. 3. Evaluation of immunologic status in patients with osteosarcoma may be helpful in monitoring of antineoplastic therapy effectiveness, may prevent the formation of unfavourable clinical changes and may be the basis for correction of the cytostatic agents' administration.

Evaluation of HLA-DQ2/DQ8 genotype in patients with celiac disease hospitalised in 2012 at the Department of Paediatrics.

INTRODUCTION: Celiac disease (CD) is a permanent intolerance to gluten that occurs in genetically predisposed individuals and leads to small intestinal mucosa damage. According to ESPGHAN guidelines from 2012, CD can be diagnosed in a patient with characteristic clinical symptoms, in whom, anti-tissue transglutaminase antibodies (> 10 times the upper limit) are found, endomysial antibodies (EMA) is confirmed and a positive genetic test is obtained. In these conditions no small-bowel biopsies are required. AIM: Evaluation of the presence of HLA-DQ2 and HLA-DQ8 haplotypes in children with previously diagnosed CD, hospitalised in 2012 at the Department of Paediatrics and Immunology and/or the Gastroenterological Outpatient Clinic, and their relatives. MATERIAL AND METHODS: Blood samples of 22 subjects, including 9 children with CD diagnosed on the basis of clinical symptoms, serological investigations and small-intestine biopsy, 7 diagnosed on the basis of clinical symptoms and serological investigations, 2 with the suspicion of CD on the basis of clinical symptoms and 4 relatives of a child with CD. METHODS: HLA-DQ2/DQ8 test, automatic evaluation by EUROArrayScan. RESULTS: The presence of HLA-DQ2 and/or HLA-DQ8 genotype was confirmed in 16 children with CD diagnosed on the basis of clinical symptoms and serological tests with/without intestinal biopsy, in 2 with the suspicion of CD and in 1 relative of a celiac child. CONCLUSIONS: The evaluation of HLA-DQ2/DQ8 haplotype confirms the genetic predisposition to CD in subjects with the disease diagnosed previously on the basis of clinical symptoms, serological tests or intestinal biopsy. Genetic testing is particularly indicated for the diagnosis of CD in infants consuming gluten for a short time and in small amounts.

Correlation between serum interleukin-31 level and the severity of disease in children with atopic dermatitis.

INTRODUCTION: Atopic dermatitis (AD) is a chronic inflammatory skin disease, characterized by intense itch, typical localization and a specific image of skin lesions. Pathogenesis of pruritus in AD is not fully understood, but recent studies emphasize the role of interleukin-31 (IL-31). This relatively recently described cytokine is considered to be a potential mediator inducing pruritus in AD. AIM: To assess the correlation of serum IL-31 level and the disease severity in children with AD. MATERIAL AND METHODS: Twenty-five children (16 girls and 9 boys) with AD aged from 4 months to 17 years (mean age: 4.2 years) were enrolled in the study. Disease severity in children with AD was assessed using the SCORAD (Severity SCORing of Atopic Dermatitis) index. Serum IL-31 levels were measured using ELISA with standard kits from EIAab R&D Systems. RESULTS: Serum IL-31 level was significantly higher in AD children than in healthy children. There was no statistic correlation between serum IL-31 level and the disease severity or itch intensity. CONCLUSIONS: The disease severity and itch intensity do not correlate with serum IL-31 level in children with atopic dermatitis.

Is there any association between secretory IgA and lactoferrin concentration in mature human milk and food allergy in breastfed children.

BACKGROUND: Breastfeeding is recommended as a protective method against the development of allergy. However, some studies have reported an increased risk of allergies development in breastfed infants of atopic mothers, which implies that atopic mothers may have an altered composition of breast milk. AIM: The aim of the study was to determine the concentration of secretory immunoglobulin A (S-IgA) and lactoferrin in human mature milk and to evaluate the association between the levels of these proteins in breast milk with food allergy in children, depending on the allergy status of the breastfeeding mother. MATERIAL AND METHODS: Medical data was collected from birth to 24 months of age from 84 mother-child pairs participating in an EU-funded project "EuroPrevall - The prevalence, cost and basis of food allergy across Europe". The diagnosis of food allergy in children was based on the positive result of a double-blind placebo-controlled food challenge (DBPCFC). S-IgA and lactoferrin levels were measured in the whey of mature breast milk with commercial enzyme-linked immunosorbent assay (ELISA) kits. Statistical analysis (the U Mann-Whitney and Kruskal-Wallis tests as well as the Spearman's rank correlation coefficient) was performed using STATISTICA 8.0 PL (Statsoft, Tulsa, USA). RESULTS: Ten out of eighty four participating children had positive skin prick tests (SPT) and/or sIgE to food antigens and in 7 (8.4%) DBPCFC confirmed food allergy. the median concentration of S-IgA was 476,83 µg/ml (range 6.51-1359.61 µg/ml). the median concentration of Lf was 15.68 µg/ml (range 11.68-36.43 µg/ml). The concentrations of S-IgA and Lf showed a moderate, negative, correlation R=-0.28; p=0.05. CONCLUSIONS: Mature breast milk of mothers of children with food allergy and of healthy children showed similar concentrations of both proteins. The level of S-IgA in the mature milk of mothers with atopic allergy was significantly lower, compared to non-atopic mothers. More studies are needed to reveal the mystery of the lack of protective effect of breastfeeding on allergy development in children.

Successful haploidentical PBSCT with subsequent T-cell addbacks in a boy with HyperIgM syndrome presenting as severe congenital neutropenia.

HIGM syndrome is a group of primary immunodeficiency disorders characterized by recurrent bacterial and opportunistic infections; it is also associated with normal to elevated serum IgM levels and a concomitant deficiency of IgG, IgA, and IgE. In this report, we give account of a boy with X-linked HIGM and a novel Y172C mutation within his CD40LG gene. He presented with severe neutropenia as the dominating symptom. His bone marrow showed maturation arrest at the promyelocyte/myelocyte stage, typical of congenital neutropenia. This boy suffered from life-threatening infections and required high doses of rhG-CSF, and a haploidentical PBSCT was also successfully performed, thus leading to reconstitution of CD40L expression on activated CD4+ T cells (as assessed with flow cytometry six months after the procedure). Two low-dose T-cell addbacks were required to re-establish full donor chimerism and clear CMV reactivation. The report demonstrates that in select cases, alternative donor allogeneic HSCT supported by DLI may be effective in correcting the defect in X-linked HIGM, and HSCT in HIGM children is not necessarily limited to matched sibling donor transplantation.

[Can the concentration of immunomodulating factors in mature breastmilk be associated with food allergy in breastfed children?]. / Czy istnieje zwiazek miedzy stezeniem czynników immunomodulujacych w ludzkim mleku dojrzalym i wystapieniem alergii pokarmowej u dzieci karmionych piersia?

OBJECTIVES: The aim of the study was to determine the concentration of such immunomodulating factors as transforming growth factor beta1 (TGF-ß1), interleukin 10 (IL-10), tumor necrosis factor alpha (TNF-α) in mature human milk and to relate the levels of the above mentioned cytokines in mature breast milk to the occurence of food allergy in children during the first 24 months of life. MATERIALS AND METHODS: Data on breastfeeding, symptoms of food allergy in children and breast milk samples were collected prospectively from birth to 24 months of age from 84 mothers participating in the Polish birth cohort of "EuroPrevall" study, in the years 2005-2007. Cytokine levels were measured in the whey with commercial enzyme-linked immunosorbent assays (ELISA) kits. RESULTS: Ten out of the eighty four (11.9%) participating children had positive SPT and/or sIgE to food antigens. In 7 out of 84 (8.4%) children DBPCFC confirmed the diagnosis of food allergy. The median concentration of TGF-ß1 was 21.94 pg/ml (range 10.47-83.19), TNF-α 1.46 pg/ml (range 0.35-16.50), IL- 101.83 pg/ml (range 0.58-31.04). There was a positive correlation between the concentration of IL-10 and TGF-ß1. The level of TNF-α correlated positively with the duration of lactation (p=0.04). There was no significant difference between the concentration of IL-10, TGF-ß1, TNF-α, in the mature breast milk of mothers of children with symptoms of allergy and positive SPT and/or sIgE, mothers of children with positive DBPCFC and in the milk of mothers of control children. CONCLUSIONS: There was no significant difference between the concentration of IL-10, TGF-ß1, TNF-α, in the mature breast milk of mothers of children with food allergy and in the breast milk of mothers of control children.

[Evaluation of selected parameters of cellular immunity in children with osteosarcoma at diagnosis]. / Ocena stanu immunologicznego dzieci I mlodzinezy z miesakiem kosciopochodnym w momencie rozpoznania choroby.

UNLABELLED: The causes of osteosarcoma (OS) and effector mechanisms of the immune response against OS and other neoplastic diseases remain unknown. According to current knowledge, the major role is attributed to cytotoxic T lymphocytes, NK, NKT and Taä lymphocytes, which are engaged directly in the destruction of the tumour cells. Helper T lymphocytes (CD4+) and indirectly B lymphocytes are of special importance. There is sparse data on the state and efficiency of the immune system in children with neoplastic disease, with bone tumours in particular. THE AIM OF THE STUDY was the evaluation of selected elements of cellular immunity in children with osteosarcoma at the time of diagnosis. MATERIALS AND METHODS: The study was performed on a group of 44 children with osteosarcoma, aged from 6 to 20 years (median 15.0 years). The control group consisted of 22 children of the same age (median 14.5 years) without the diagnosis of neoplastic disease and active inflammatory state. T lymphocytes with their subpopulations, activated T lymphocytes (CD3+/HLA-DR+), B lymphocytes, NK and NKT cells were analyzed in peripheral blood using the flow cytometry method. Examinations were performed before the therapy - in the diagnostic period. RESULTS: A lower number of peripheral blood lymphocyte population in children with osteosarcoma at diagnosis, compared to the control group was observed. The differences concerned T lymphocytes CD3+(1609.0 vs 3038.0 kom/µl, p<0.001) CD4+(598.0 vs 1071.0 kom/l; p<0.001) and their cytotoxic subpopulation CD8+ (386.0 vs. 866.0 cells/µL; p<0.001), activated T lymphocytes CD3+/HLA-DR+(39.0 vs. 81.0 cells/µL; p<0.025), B lymphocytes CD19+(205.0 vs. 381.0 cells/µL; p<0.025) and NK cells (161.0 vs. 339.0 cells/µL; p<0.005). The number and percentage of peripheral blood lymphocytes in children and youth with osteosarcoma at diagnosis is over 50% lower compared to the patients without neoplastic disease. CONCLUSIONS: 1. The general analysis of peripheral blood without differentiating lymphocyte subpopulations is insufficient to determine disturbances which are forming in the immune system of patients developing the neoplastic disease. 2. The course of the neoplastic disease (osteosarcoma) in patients of developmental age is very diverse, and associated with individual biological variation. 3. The evaluation of the immunologic status in patients with osteosarcoma may have prognostic meaning for the further course of the disease, may prevent the formation of unfavourable clinical changes, and be the basis for correcting the administration of cytostatic agents.