OBJECTIVES: To investigate the change in the distribution of memory B cell subsets in children with frequently relapsing nephrotic syndrome (FRNS) during the course of the disease. METHODS: A total of 35 children with primary nephrotic syndrome (PNS) who attended the Department of Pediatrics of the Affiliated Hospital of Xuzhou Medical University from October 2020 to October 2021 were enrolled as subjects in this prospective study. According to the response to glucocorticoid (GC) therapy and frequency of recurrence, the children were divided into two groups: FRNS (n=20) and non-FRNS (NFRNS; n=15). Fifteen children who underwent physical examination were enrolled as the control group. The change in memory B cells after GC therapy was compared between groups, and its correlation with clinical indicators was analyzed. RESULTS: Before treatment, the FRNS and NFRNS groups had significantly increased percentages of total B cells, total memory B cells, IgD⁺ memory B cells, and IgE⁺ memory B cells compared with the control group, and the FRNS group had significantly greater increases than the NFRNS group (P<0.05); the FRNS group had a significantly lower percentage of class-switched memory B cells than the NFRNS and control groups (P<0.05). After treatment, the FRNS and NFRNS groups had significant reductions in the percentages of total B cells, total memory B cells, IgM⁺IgD⁺ memory B cells, IgM⁺ memory B cells, IgE⁺ memory B cells, IgD⁺ memory B cells, and IgG⁺ memory B cells (P<0.05) and a significant increase in the percentage of class-switched memory B cells (P<0.05). The FRNS group had a significantly higher urinary protein quantification than the NFRNS and control groups (P<0.05) and a significantly lower level of albumin than the control group (P<0.05). In the FRNS group, urinary protein quantification was negatively correlated with the percentage of class-switched memory B cells and was positively correlated with the percentage of IgE⁺ memory B cells (P<0.05). CONCLUSIONS Abnormal distribution of memory B cell subsets may be observed in children with FRNS, and the percentages of IgE⁺ memory B cells and class-switched memory B cells can be used as positive and negative correlation factors for predicting recurrence after GC therapy in these children.
Child ; Humans ; B-Lymphocyte Subsets/metabolism* ; Immunoglobulin E ; Immunoglobulin M ; Nephrotic Syndrome/immunology* ; Prospective Studies ; Glucocorticoids/therapeutic use*

Urticaria is an immune-mediated allergic disease. This study explored the effect of Jingfang Mixture on spleen T lymphocyte subsets of urticaria mice. A total of 50 Kunming mice were randomized into normal group(C), model group(V), and low-(JF-L, 0.5 g·kg~(-1)), medium-(JF-M, 1 g·kg~(-1)) and high-dose(JF-H, 2 g·kg~(-1)) Jingfang Mixture groups, with 10 mice in each group. The mixture of ovalbumin and aluminum hydroxide(0.1 mg + 0.1 mL) was used(intraperitoneal injection) to induce urticaria in mice. The administration began 6 days after the first immunization, and the second immunization was carried out 10 days after the first immunization. The pruritus index was detected within 30 min after the second immunization. The administration lasted 21 days. After 21 days, the serum was taken to detect the total IgE level. Based on hematoxylin and eosin(HE) staining, the pathological changes of skin tissue were observed, and Western blot was used to detect the levels of p-Janus kinase 2(JAK2)/JAK2 and p-signal transducer and activator of transcription 3(STAT3)/STAT3 in skin tissue. The spleen was taken to detect the spleen index, and flow cytometry was employed to determine the expression of lymphocyte subsets. The results showed that group V had obvious pathological changes in skin tissue compared with group C. Moreover, group V showed more scratches, higher spleen index, and higher level of total serum IgE than group C. In addition, higher levels of p-JAK2 and p-STAT3, lower proportions of CD4~+T, Th1, and Treg, higher proportions of CD8~+T, Th2, and Th17, and lower ratios of CD4~+/CD8~+, Th1/Th2, and Terg/Th17 were observed in group V than in group C. Compared with group V, each administration group showed alleviation of the pathological morphology of skin tissue, obvious epidermal thickening, relatively intact collagen fiber structure of dermal reticular layer, alleviated edema, and relief of vasodilation and peripheral inflammatory cell infiltration. Moreover, less scratching, lower spleen index, lower p-JAK2/JAK2 and p-STAT3/STAT3 were observed in the administration groups than in group V. JF-M group and JF-H group demonstrated lower levels of total IgE, larger proportions of CD4~+T, Th1, and Treg, smaller proportions of CD8~+ T, Th2, and Th17, and higher ratios of CD4~+/CD8~+, Th1/Th2, and Terg/Th17. In conclusion, Jingfang Mixture may improve the symptoms of urticaria mice by regulating the balance of spleen T lymphocyte subsets through JAK2-STAT3 signaling pathway.
Mice ; Animals ; Janus Kinase 2/pharmacology* ; Spleen ; T-Lymphocyte Subsets/metabolism* ; Signal Transduction ; Urticaria ; Immunoglobulin E

Atopic dermatitis (AD) is among the most common skin disorders in humans. Although a variety of regimens are available for the treatment of AD, preventive approaches are limited. Recent studies have demonstrated that certain naturally-occurring herbal medicines are effective in preventing the development of AD via divergent mechanisms, such as inhibiting cytokine and chemokine expression, IgE production, inflammatory cell infiltration, histamine release, and/or enhancement of epidermal permeability barrier function. Yet, they exhibit few adverse effects. Since herbal medicines are widely available, inexpensive and generally safe, they could represent an ideal approach for preventing the development of AD, in both highly developed and developing countries.
Animals ; Chemokines ; metabolism ; Dermatitis, Atopic ; prevention & control ; Disease Models, Animal ; Herbal Medicine ; Humans ; Immunoglobulin E ; metabolism ; Inflammation ; pathology

BACKGROUNDCryopyrin-associated periodic syndrome (CAPS) is a group of rare, heterogeneous autoinflammatory disease characterized by interleukin (IL)-1β-mediated systemic inflammation and clinical symptoms involving skin, joints, central nervous system, and eyes. It encompasses a spectrum of three clinically overlapping autoinflammatory syndromes including familial cold autoinflammatory syndrome, Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease. CAPS is associated with gain-of-function missense mutations in NOD-like receptor family pyrin domain-containing protein 3 (NLRP3), the gene encoding NLRP3. Moreover, most mutations leading to MWS occurred in exon 3 of NLRP3 gene. Here, we reported a novel mutation occurred in exon 1 of NLRP3 gene in an MWS patient and attempted to explore the pathogenic mechanism.

METHODSGenetic sequence analysis of NLRP3 was performed in an MWS patient who presented with periodic fever, arthralgia, and multiform skin lesions. NLRP3 was also analyzed in this patient's parents and 50 healthy individuals. Clinical examinations including X-ray examination, skin biopsy, bone marrow aspiration smear, and blood test of C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), serum levels of IL-1β, immunoglobulin E (IgE), antineutrophil cytoplasmic antibodies, antinuclear antibodies, and extractable nuclear antigen were also analyzed. The protein structure of mutant NLRP3 inflammasome was calculated by SWISS-MODEL software. Proteins of wild type and mutant components of NLRP3 inflammasome were expressed and purified, and the interaction abilities between these proteins were tested by surface plasmon resonance (SPR) assay.

RESULTSX-ray examination showed no abnormality in the patient's knees. Laboratory tests indicated an elevation of CRP (233.24 mg/L) and ESR (67 mm/h) when the patient had fever. Serum IL-1β increased to 24.37 pg/ml, and serum IgE was higher than 2500.00 IU/ml. Other blood tests were normal. Bone marrow aspiration smear was normal. A novel point mutation c.92A>T in exon 1 of NLRP3 gene was identified, which caused a p.D31V mutation in pyrin domain (PYD) of NLRP3. SPR assay showed that this point mutation may strengthen the interaction between the PYD of NLRP3 and the PYD of the apoptosis-associated speck-like protein. The mutation c.92A>T in exon 1 of the NLRP3 gene was not found in the patient's parents and 50 healthy individuals.

CONCLUSIONSThe mutation c.92A>T in exon 1 of the NLRP3 gene is a novel mutation associated with MWS. The p.D31V mutation might promote the activation of NLRP3 inflammasome and induce MWS in this patient.

Adolescent ; Cryopyrin-Associated Periodic Syndromes ; genetics ; metabolism ; Exons ; genetics ; Humans ; Immunoglobulin E ; blood ; Interleukin-1beta ; blood ; Male ; Mutation ; genetics ; NLR Family, Pyrin Domain-Containing 3 Protein ; genetics ; Surface Plasmon Resonance

Immunoglobulin A (IgA) vasculitis is the most common leukocytoclastic small-vessel vasculitis in children and mainly involves the small vessels in the skin, joints, digestive tract, and kidneys. Its pathogenesis is still unclear. Currently, it is believed that environmental factors can cause autoimmune dysfunction and lead to the deposition of IgA-containing immune complexes on the wall of arterioles on the basis of genetic factors. This article reviews the research advances in the role of immune factors in the pathogenesis of IgA vasculitis.
Autoantibodies ; analysis ; Complement System Proteins ; physiology ; Cytokines ; physiology ; Glycosylation ; Humans ; Immunoglobulin A ; analysis ; Immunoglobulin E ; metabolism ; Vasculitis ; etiology ; immunology

BACKGROUNDImmunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic disease that can involve multiple organs and various clinical phenotypes. The purpose of this study was to analyze different types of organ involvement in IgG4-RD patients in China.

METHODSWe conducted a prospective cohort study on IgG4-RD patients to analyze the clinical manifestations and rare features of IgG4-RD. Patients were grouped into different types according to organ involvement regarding organ number and organ site. The constituent ratio in different types was also analyzed.

RESULTSA total of 200 IgG4-RD patients, with a male:female ratio of 2.08:1, were grouped into different types. Cases having involvement of two or three organs were the most common whereas the fewest number of patients had multi-organ (≥4) involvement. Serum IgG4 and IgE levels, IgG4/IgG ratio, and percentage of eosinophils increased as the number of involved organs increased. In addition, constituent ratio analysis revealed that patients with salivary gland/lacrimal gland swelling, who also constituted the largest number of IgG4-RD patients, had higher serum IgG4 concentrations and IgG4/IgG values, had higher percentage of Eos, and were more likely to have had a history of allergies relative to patients with internal organ involvement.

CONCLUSIONSThe characteristic feature of IgG4-RD is multiple organ involvement with various clinical manifestations and different types. Although serum IgG4 levels increased with the number of involved organs, serum IgG4 levels were higher for those patients with salivary gland/lacrimal gland swelling compared with those with internal organ involvement. Thus, valuable clues to the differential diagnosis of IgG4-RD could be obtained by examining the clinical patterns of organ involvement.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Autoimmune Diseases ; complications ; pathology ; Eosinophils ; metabolism ; Female ; Humans ; Immunoglobulin E ; blood ; Immunoglobulin G ; blood ; Kidney ; pathology ; Lacrimal Apparatus ; pathology ; Lung ; pathology ; Male ; Middle Aged ; Pituitary Gland ; pathology ; Prospective Studies ; Prostate ; pathology ; Salivary Glands ; pathology ; Thyroid Gland ; pathology ; Uterus ; pathology ; Young Adult

PURPOSE: In addition to regulating calcium and phosphorus homeostasis and bone metabolism, vitamin D is known as an immune modulator. Recently, there has been increased worldwide interest in the association between low levels of vitamin D and allergic diseases. The purpose of this study was to assess the relationship between serum vitamin D levels and allergic/vasomotor rhinitis (AR/VR) in children. METHODS: This study included 164 patients. The sample included 59 patients with AR, 42 patients with VR, and 63 controls. Their ages ranged from 0 to 16 years. We examined the levels of 25-hydroxyvitamin D, Immunoglobulin E, specific IgE, and eosinophil cationic protein; peripheral blood eosinophil count; and the results of a skin prick test. RESULTS: Serum 25-hydroxyvitamin D levels were 19.0+/-8.5 ng/mL in the AR group, 25.5+/-10.9 ng/mL in the VR group, and 26.9+/-10.7 ng/mL in the control group. After adjustment for body mass index and season at the time of blood sampling, vitamin D levels in the AR group were lower than those of the VR group (P=0.003) and control group (P<0.001). Vitamin D levels were inversely correlated with Immunoglobulin E levels (r=-0.317, P<0.001). AR patients with food allergy or atopic dermatitis did not have lower levels of 25-hydroxyvitamin D than AR patients without these diseases. CONCLUSION: This study demonstrates a possible relationship between vitamin D levels and allergic rhinitis in Korean children.
Body Mass Index ; Calcium ; Child* ; Dermatitis, Atopic ; Eosinophil Cationic Protein ; Eosinophils ; Food Hypersensitivity ; Homeostasis ; Humans ; Immunoglobulin E ; Immunoglobulins ; Metabolism ; Phosphorus ; Rhinitis ; Rhinitis, Vasomotor* ; Seasons ; Skin ; Vitamin D* ; Vitamins*

PURPOSE: The purpose of this study was to evaluate the effects of essential oil on oxidative stress, immunity, and skin condition in atopic dermatitis (AD) induced mice. METHODS: This study was a 3x3 factorial design. Factors were oil type (Lavender, Thyme, and 2:1 mixture of lavender and thyme oil [blending oil]) and treatment period (0 day, 7 days, and 21 days). The samples were 45 mice with AD and randomly assigned to nine groups of five mice per group. The dependent variables such as superoxide radical, IgE, degranulated mast cells, and epidermal thickness were measured. Data were collected from February to April in 2014. Descriptive statistics, One-way ANOVA, Two-way ANOVA, and Tukey's HSD test were performed using the SPSS WIN 20.0 program. RESULTS: Dependent variables were not statistically significantly different by the three oil types (p >.05). Essential oils such as lavender, thyme, and blending oil were all effective in reducing AD symptoms and especially 2:1 blending oil were most effective. There were statistically significant differences by the three treatment periods in all dependent variables (p <.001). There were statistically significant interactions between oil types and treatment periods in all dependent variables (p <.01). For decreasing superoxide radical, degranulated mast cells, and epidermal thickness, 2:1 mixed oil should be applied for at least 21 days. Otherwise to reduce IgE, 2:1 mixed oil should be used for at least 7 days. CONCLUSION: These findings provide bases for developing effective interventions for AD patients to manage their AD symptoms.
Animals ; Dermatitis, Atopic/chemically induced/*drug therapy/pathology ; Disease Models, Animal ; *Immunity/drug effects ; Immunoglobulin E/blood ; Lavandula/*chemistry/metabolism ; Mast Cells/cytology/metabolism ; Mice ; Oils, Volatile/chemistry/pharmacology/therapeutic use ; *Oxidative Stress/drug effects ; Picryl Chloride/toxicity ; Plant Oils/chemistry/pharmacology/*therapeutic use ; Singlet Oxygen/metabolism ; Skin/drug effects/pathology ; Thymus Plant/*chemistry/metabolism

OBJECTIVETo investigate the changes of the levels of galectin-3 (Gal-3) in serum and bronchoalveolar lavage fluid (BALF) of children with asthma whose have different serum levels of 25-hydroxyl-vitamin D₃[25(OH)D₃].

METHODSFifty children with asthma between January 2013 and December 2014 were enrolled as the asthma group, and they were classified into 25(OH)D₃sufficient (n=7), insufficient (n=12) and deficient subgroups (n=31) according to the serum levels of 25(OH)D₃. Twenty children with abnormal airway or tracheal foreign bodies served as the control group. The levels of 25(OH)D₃, Gal-3 and total IgE in serum and Gal-3 levels in BALF were measured using ELISA.

RESULTThe serum levels of 25(OH)D₃in the asthma group were lower than in the control group (P<0.05). The 25(OH)D₃deficient subgroup displayed the highest percentages of neutrophils, eosinophils and epithelial cells in BALF, followed by the 25(OH)D₃insufficient subgroup and the 25(OH)D₃sufficient subgroup (P<0.05). The percentages of neutrophils, eosinophils and epithelial cells in BALF in the three subgroups were all higher than in the control group (P<0.05). In children with asthma, serum levels of 25(OH)D₃were negatively correlated with the percentages of neutrophils, eosinophils and epithelial cells in BALF (r=-0.683, -0.795 and -0.670 respectively; P<0.05); and a negative correlation was also seen between serum 25(OH)D₃levels and serum Gal-3 and total IgE levels (r=-0.759 and -0.875 respectively; P<0.05).

CONCLUSIONSThe children with asthma have low serum levels of 25(OH)D₃. 25(OH)D₃and Gal-3 may be involved in the airway inflammation and the development of asthma.

Asthma ; etiology ; metabolism ; Bronchoalveolar Lavage Fluid ; chemistry ; Child ; Child, Preschool ; Female ; Galectin 3 ; analysis ; blood ; physiology ; Humans ; Immunoglobulin E ; blood ; Infant ; Male ; Vitamin D ; analogs & derivatives ; blood ; physiology

OBJECTIVES: The seroprevalence of hepatitis E virus (HEV) among high-risk groups overseas is high, but studies in these groups are rare in South Korea. We conducted the present study from April to November 2012 to obtain data on the seroprevalence and associated risk factors for HEV among slaughterhouse workers in South Korea. METHODS: Slaughterhouse workers from 80 workplaces nationwide were surveyed in South Korea in 2012. The subjects comprised 1848 cases: 1434 slaughter workers and 414 residual products handlers. By visiting 80 slaughterhouses, which were mixed with 75 of which also performed residual products handling, we conducted a questionnaire survey for risk factors and obtained blood samples in order to determine the seropositivity and seroprevalence of HEV. Anti-HEV IgG and IgM were measured using HEV IgG and IgM enzyme-linked immunospecific assay kits and HEV antigen was measured by reverse transcription polymerase chain reaction (RT-PCR). RESULTS: The seropositivity of anti-HEV IgG was 33.5% (slaughter workers 32.8% and residual products handlers 36.2%), and among the seropositive individuals the seroprevalence of anti-HEV IgM was 0.5% (slaughter workers 0.5%, residual products handlers 0.7%). The response rate of HEV-antigen as measured by RT-PCR was 0.2%. Risk factors significantly related to anti-HEV IgG seropositivity were age, sex , and working duration (slaughter workers only). CONCLUSIONS: There were significant risk factors (sex, age, and working duration) for HEV identified in our study. All three positive cases for HEV-antigen by RT-PCR were related to pig slaughter but without statistical significance. To prevent HEV, an educational program and working guidelines may be needed for high risk groups.
Abattoirs ; Adult ; Aged ; Enzyme-Linked Immunosorbent Assay ; Female ; Hepatitis Antibodies/blood ; Hepatitis E/*diagnosis/epidemiology/virology ; Hepatitis E virus/genetics/*immunology/metabolism ; Humans ; Immunoglobulin G/blood ; Immunoglobulin M/blood ; Male ; Middle Aged ; Multivariate Analysis ; Prevalence ; Republic of Korea/epidemiology ; Reverse Transcriptase Polymerase Chain Reaction ; Risk Factors ; Workplace