The etiology of CSU in children was mostly idiopathic despite detailed investigation. In childhood, the natural course of CSU was favorable, and nearly half of the patients recovered after 5 years of disease duration. A high UAS7 at admission seemed to be a significant risk factor for the persistence of symptoms.

Objective:To analyze the development of psychopathology in recipients along with their donor and nondonor siblings and the relationship with the bone marrow transplantation (BMT) process.Methods: All children were interviewed using the Kiddie Schedule for Affective Disorders and Schizophrenia to assess psychopathology. The depression and anxiety symptoms and self-esteem of children and adolescents were evaluated using the Children's Depression Inventory, State-Trait Anxiety Inventory for Children, State-Trait Anxiety Inventory, and Rosenberg Self-Esteem Scale.Results: In this study, the depressive symptom level was found significantly higher in the donor group compared with the nondonor group. State anxiety symptoms were higher in the BMT group (P < .05). There were no significant differences in trait anxiety symptoms. Self-respect was higher in children in the donor group compared with those in the BMT group (P < .05). During the transplant process, children with bone marrow transplants had a higher prevalence of depression, anxiety disorder, and attention-deficit/hyperactivity disorder, and nondonor siblings had a higher prevalence of depressive disorder, anxiety disorder, and attention-deficit/hyperactivity disorder compared with society in general. Conclusion:Physicians should deal with the family as a whole, not just their patient, and should be aware of the psychiatric risk of other siblings during the assessment.

Hyper-IgE syndromes comprise a group of inborn errors of immunity. STAT3-deficient hyper-IgE syndrome is characterized by elevated serum IgE levels, recurrent infections and eczema, and characteristic skeletal anomalies. A loss-of-function biallelic mutation in IL6ST encoding the GP130 receptor subunit (p.N404Y) has very recently been identified in a singleton patient (herein referred to as P N404Y ) as a novel etiology of hyper-IgE syndrome. Here, we studied a patient with hyper-IgE syndrome caused by a novel homozygous mutation in IL6ST (p.P498L; patient herein referred to as P P498L ) leading to abrogated GP130 signaling after stimulation with IL-6 and IL-27 in peripheral blood mononuclear cells as well as IL-6 and IL-11 in fibroblasts. Extending the initial identification of selective GP130 deficiency, we aimed to dissect the effects of aberrant cytokine signaling on T-helper cell differentiation in both patients. Our results reveal the importance of IL-6 signaling for the development of CCR6-expressing memory CD4 + T cells (including T-helper 17-enriched subsets) and non-conventional CD8 + T cells which were reduced in both patients. Downstream functional analysis of the GP130 mutants (p.N404Y and p.P498L) have shown differences in response to IL-27, with the p.P498L mutation having a more severe effect that is reflected by reduced T-helper 1 cells in this patient (P P498L ) only. Collectively, our data suggest that characteristic features of GP130-deficient hyper-IgE syndrome phenotype are IL-6 and IL-11 dominated, and indicate selective roles of aberrant IL-6 and IL-27 signaling on the differentiation of T-cell subsets.

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Background: Food protein-induced allergic proctocolitis (FPIAP) is a non‐immunoglobulin E (IgE) mediated food allergy that typically presents with blood-mixed mucoid stool. Objective: To identify the predictors that affect the tolerance development in infants with FPAIP and laboratory as well as clinical differences between patients with early and with late tolerance. Methods: A total of 185 infants with FPIAP were included. The patients were grouped and analyzed based on laboratory tests and clinical characteristics. Results: The median (interquartile range [IQR]) age of onset of symptoms was 2.0 months (1.0‐3.0 months). Symptoms began in severe cases in patients (n = 23) at a younger median (IQR) age (1.5 months [0.7‐2.0 months]) than the group with nonsevere presentation (median 2.0 months [IQR 1.5‐3.0 months]) (p < 0.001). The frequency of neutropenia (<1500/mm3) (p = 0.045) and eosinophilia (450 mm3) (p = 0.018) was increased in severe cases. Concomitant IgE-related food allergy (odds ratio [OR] 3.595 [95% confidence interval {CI}, 1.096‐11.788], p = 0.035), non‐IgE-mediated multiple food allergy (OR 3.577 [95% CI, 1.595‐8.018], p = 0.002), feeding with cow's milk‐based formula (at least once during infancy) (OR 2.517 [95% CI, 1.188‐5.333], p = 0.016), and late complementary feeding (OR 5.438 [95% CI, 2.693‐10.981], p < 0.001) were the predictors for late tolerance development. The estimated optimal cutoff value for introduction of complementary foods for the resolution of allergy was 5.5 months, with 69.4% sensitivity, 74.4% specificity, and an area under the curve of 0.737 (95% CI, 0.626‐0.812) (p < 0.001). Conclusion: This study showed that the early introduction of complementary feeding accelerates tolerance development in FPAIP. A longer duration of an elimination diet has no impact on the resolution of allergy. Physicians should consider conservative avoidance measures and earlier introduction of complementary feeding in FPIAP.

Atopic dermatitis (AD) is the most common chronic inflammatory skin disorder of childhood. Underlying factors that contribute to AD are impaired epithelial barrier, alterations in the lipid composition of the skin, immunological imbalance including increased Th2/Th1 ratio, proinflammatory cytokines, decreased T regulatory cells, genetic mutations, and epigenetic alterations. Atopic dermatitis is a multifactorial disease with a particularly complicated pathophysiology. Discoveries to date may be considered the tip of the iceberg, and the increasing number of studies in this field indicate that there are many points to be elucidated in AD pathophysiology. In this review, we aimed to illustrate the current understanding of the underlying pathogenic mechanisms in AD, to evaluate available treatment options with a focus on recently discovered therapeutic agents, and to determine the personal, familial, and economic burdens of the disease, which are frequently neglected issues in AD. Currently available therapies only provide transient solutions and cannot fully cure the disease. However, advances in the understanding of the pathogenic mechanisms of the disease have led to the production of new treatment options, while ongoing drug trials also have had promising results.