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Lung biopsy was performed in 19% of GLILD patients, all of whom had histopathologic diagnosis of Lymphoid Interstitial Pneumonia (LIP). Conclusions GLILD is the major cause of lung disease in CVID. Computed tomography is useful for diagnosis but not necessary in follow-up, in which functional tests should have better correlation with clinical evolution, reducing radiation exposure. Biopsy should be indicated when the clinical diagnosis is unclear. Treatment should be considered whenever there is clear evidence of disease progression.Background The chemical modification of allergens with glutaraldehyde improves safety while maintaining clinical efficacy, which permits the administration of higher doses of immunotherapy, reducing the risk of adverse reactions. The aim of this study is to evaluate the immunogenic capacity of a new cat dander polymer by immunizing mice and quantifying immunoglobulins in serum, in comparison with the non-modified allergen. Methods The study consists of the immunization of three mice groups with the polymerized and the native extract, together with a negative control group. The immunoglobulin levels in serum have been measured by indirect ELISA. By means of the non-parametric Mann-Whitney U test, it was determined if there were significant differences in the values of specific antibodies between groups. Results The group immunized with the allergoid showed significantly higher specific IgG and IgG1 values to dander allergens and specific IgG to the major allergen Fel d 1, while there were no significant changes in IgG2a and IgE values. These results could be due to a higher immunization dose. The vaccine formulation was based on the optimal defined dose for clinical efficacy of allergen immunotherapy. Conclusions This preclinical study carried out with the present assay has established that the allergoid of cat dander extract, as designed for its optimal use in allergen immunotherapy, produces a higher specific IgG than the native extract, in addition to showing significantly higher specific IgG1 levels, evidencing a greater effectiveness in immunization.Objective To assess the Quality of Life (QoL) of children with Atopic Dermatitis (AD) and their families and the impact of the mothers' illness perceptions on the family QoL. Materials and methods Seventy-five children with AD (54 infants and 21 children) and their mothers participated in the study. The following questionnaires were administrated 1. Brief Illness Perception Questionnaire (Brief IPQ); 2. Infant's Dermatitis Quality of Life Index (IDQOL); 3. Children's Dermatology Life Quality Index (CDLQI); 4. Dermatitis Family Impact Questionnaire (DFIQ) and 5. The Severity Scoring of Atopic Dermatitis (SCORAD). Results Atopic dermatitis had a moderate impact on the QoL of the infants (6.67±5.30), children (7.86±7.19) and their families (9.42±7.03). The DFIQ was associated with certain dimensions of the Brief IPQ, specifically, with Illness Identity (greater symptom burden) (r=0.615, p=0.000), beliefs about the Consequences of the illness (r=0.542, p=0.000), the Concerns (r=0.421, p=0.000) and the Emotional Representations (r=0.510, p=0.000). Correlation was demonstrated between IDQOL and DFIQ (r=0.662, p=0.000) and between CDLQI and DFIQ (r=0.832, p=0.000), and a weaker correlation between SCORAD and DFIQ (r=0.255, p=0.035). The chronicity of the AD showed negative association with DFIQ (p less then 0.001). Conclusions The QoL of families with a child with AD is associated with the mother's illness perceptions about AD, the children's QoL and with both the severity and the chronicity of the disease. Therefore, clinicians should pay attention not only to the clinical characteristics of the children, but also to the parents' beliefs and emotions, to improve the family QoL.Introduction and objectives As well as increased susceptibility to infections, autoimmune and inflammatory manifestations also eventuate due to dysregulation of immune system in a substantial proportion of patients with primary immunodeficiency (PID). Autoimmune and inflammatory manifestations can occur prior or after diagnosis of PID. This study aimed to evaluate autoimmune and inflammatory complications among all types of PID patients in childhood and to emphasize the importance of these findings as a warning sign to diagnose PIDs. Methods Medical records of 1036 patients with PID, followed up between 2003 and 2019, were retrospectively screened for occurrence of autoimmunity and inflammation. During this time, demographic features, autoimmune/inflammatory findings and initial time, genetic mutations, laboratory and clinical follow up findings, treatment regimens and outcomes were recorded. Results Autoimmune and inflammatory manifestations were observed in 83 patients (10.1%). The median age of autoimmunitncy and patients with known PIDs should be evaluated for autoimmune and inflammatory complications.Background The mite alimentary canal contains plenty of microbiota. BIBW2992 It is accepted that some of the microbial products function as adjuvants to speed up immune responses. Objectives We identified five bacterial proteins from dust mite, and Enterobacterial fimbriae H (FimH) was one of them. This study aims to test a hypothesis that the FimH protein enforces immunotherapy in asthmatic mice. Methods Asthmatic mice were treated by allergen specific immunotherapy (ASIT) with rDer f1/f2 or rDer f1/f2 plus FimH. Changes in inflammatory cell infiltration, airway hyperreactivity, frequency of Tregs, splenic CD4+IFN-γ+ cells, and serum levels of TGF-β, IL-10, IL-13 and IL-17A of asthmatic mice were checked. Results ASIT with rDer f1/f2 plus FimH reduced inflammatory cell infiltration, airway hyperreactivity (AHR), and levels of IgE and IgG1 compared to ASIT with rDer f1/f2 alone, but the levels of IgG2a increased. Asthmatic mice that underwent ASIT with rDer f1/f2 plus FimH showed increased frequency of Tregs, splenic CD4+IFN-γ+ cells, serum levels of TGF-β and IL-10; and deceased splenic CD4+IL-4+ cells, and serum levels of IL-13 and IL-17A. In vitro study showed FimH triggered IL-10 expression in a concentration dependent manner and facilitated the differentiation of Tregs. Conclusion Used as an adjuvant, FimH enforces the effect of ASIT in asthmatic mice via augmenting Tregs.

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