Henoch-Schonlein Purpura Children Research Articles

Risk due to Elevated Uric Acid Levels in Children With Henoch-Schonlein Purpura.

To compare uric acid levels in children with Henoch-Schonlein purpura (HSP)without nephritis and with renal damage, and at different pathological grades. A total of 451 children were enrolled in this study, including 64 with HSP without nephritis and 387 HSP with kidney damage. Age, gender, uric acid, urea, creatinine and cystatin C levels were reviewed. Pathological findings of those with renal impairment were also reviewed. Among the HSP children with renal damage, 44 were grade I, 167 were grade II and 176 were grade III. There were significant differences in age, uric acid, urea, creatinine and cystatin C levels between the two groups (p<0.05, all). Correlation analysis showed that uric acid levels in children with HSP without nephritis were positively correlated with urea and creatinine levels (p<0.05). Uric acid levels in HSP children with renal damage was positively correlated with age, urea, creatinine and cystatin C levels (p<0.05, all). Regression analysis found that, without adding any correction factors, there were significant differences in uric acid levels between the two groups; however, after adjusting for pathological grade, there was no longer a significant difference. There were significant differences of uric acid levels in children with HSP without nephritis and with renal impairment. Uric acid levels in the renal impairment group were significantly higher than that in the HSP without nephritis group. Uric acid levels were related to only the presence or absence of renal damage, not to the pathological grade.

Analysis of children with Henoch–Schonlein purpura secondary to infection

Henoch-Schonlein purpura (HSP) is the most common childhood vasculitis, infection is the most essential inducement. We hypothesized that infection could impact the blood routine characteristics and/or outcome of vasculitis. Thus, we aim to find the most common infectious agent in HSP patients and identify convenient indicators to predict renal involvement in HSP patients with infection. We conducted a retrospective study of 208 HSP children and 98 healthy children. Clinical parameters were compared in those cases. A total of 68.75% of patients were infected with various pathogens. The mean platelet volume (MPV) (P < 0.02) was lower in HSP patients with infection than patients without infection. Mycoplasma pneumoniae (MP) infection accounted for the largest proportion (45.77%). MPV in HSP nephritis (HSPN) group was lower than in HSP patients (excluded renal involvement) in patients with MP infection. Logistic regression analysis found that age and MPV were risk factors for the occurrence of MP-infected HSPN. The receiver operating characteristic curve (ROC) analysis showed that the combination of MPV with the onset age at the optimal cut-off point had 81% sensitivity in predicting whether HSP patients with MP infection would develop into HSPN. Our research revealed that MP was the most commonly infected pathogen of children's HSP. MPV was an essential predictor of nephritis in HSP patients with MP infection. This discovery can prompt clinical treatments as well as reduce costs. Key Points • Mycoplasma pneumoniae (MP) accounts for the largest proportion in HSP children with infection. • MPV can be used as a predictor for the development of MP-triggered HSP to HSPN.

Correlations of Leukotriene B4 and 25-Hydroxyvitamin D3 Levels with Disease Severity in Children with Henoch-Schonlein Purpura.

The aim was to explore the correlations of leukotriene B4 (LTB4) and 25-hydroxyvitamin D3 [25(OH)D3] levels with disease severity in children with Henoch-Schonlein purpura (HSP). A total of 260 HSP children admitted from January 2017 to December 2018 were selected, and 60 healthy children physically examined in the same period were enrolled as controls. The results of general laboratory tests and expression levels of leukotriene B4 (LTB4) and 25-hydroxyvitamin D3 [25(OH)D3] were compared. The correlations of LTB4 and 25(OH)D3 with immunoglobulin A (IgA) were statistically analyzed, and the expression levels of LTB4 and 25(OH)D3 were compared between Henoch-Schonlein purpura nephritis (HSPN) group and non-Henoch-Schonlein purpura nephritis (NHSPN) group. The optimal cutoff values of LTB4 and 25(OH)D3 to predict HSP were analyzed by receiver operating characteristic (ROC) curves, based on which they were divided into low-, intermediate-, and high-risk groups. The length of hospital stay and recurrence rate within 6 months were compared. The levels of white blood cell count, platelets, C-reactive protein, IgA, IgM, IgE, complement C3, and LTB4 were significantly higher and 25(OH)D3 was lower in HSP group than those in control group (p < 0.05). IgA, LTB4, and 25(OH)D3 levels were independent risk factors for HSP (p < 0.05). LTB4 was positively correlated with IgA (p < 0.05), and 25(OH)D3 was negatively correlated with IgA (p < 0.05). LTB4 level was significantly higher and 25(OH)D3 level was lower in HSPN group than those in NHSPN group (p < 0.05). The optimal cutoff values of LTB4 and 25(OH)D3 were 27.82 pg/mL and 22.10 ng/mL, respectively, the length of hospital stay gradually increased in low-, intermediate-, and high-risk groups, and the recurrence rates within 6 months were 14.2%, 31.5%, and 39.6%, respectively (p < 0.05). LTB4 level increases and 25(OH)D3 level decreases in children with HSP, and the expression levels are significantly correlated with disease severity, suggesting predictive values for prognosis.

Differences in Manifestations and Gut Microbiota Composition Between Patients With Different Henoch-Schonlein Purpura Phenotypes.

BackgroundGut microbiota plays an important role in the pathogenesis of immune-mediated diseases. However, the complex pathogenesis of Henoch-Schonlein Purpura (HSP) remains elusive. This study aimed to characterize the gut microbiota in HSP patients and explore the potential association between gut microbiota composition and phenotypic changes in HSP.Methods16SrRNA gene sequencing and bioinformatic analyses were performed using total DNA extracted from the fecal microbiota of 34 children with HSP, including 18 primary cases, 16 recurrent cases, and 23 healthy children.ResultsThe diversity indexes showed significant differences in the microbial community among the primary HSP groups, the recurrent HSP group and healthy controls. The abundance of Escherichia-Shigella in the recurrent HSP group was significantly higher than that in the primary HSP group, and the constructed ROC curve had an AUC value of 0.750. According to the Spearman correlation analysis, the abundance of Bacteroides was positively associated with the serum IgG level in children with HSP, while the abundance of Lachnoclostridium was negatively correlated with the complement component 3 (C3). The diversity indexes of gut microbiota in the HSP group with abdominal symptoms were higher than those in the HSP group without GI involvement, and also higher than those in the healthy control group. In the HSP group with GI involvement, the abundance of Faecalibacterium was decreased, while the abundance of Streptococcus and Fusobacteria was increased, compared to the HSP group without GI involvement.ConclusionsThe gut microbiota of children with HSP was different from that of healthy children. The genus Escherichia-Shigella has a diagnostic value for HSP recurrence. Bacteroides and Lachnoclostridium may affect IgG and complement C3 levels in children with HSP. Abdominal symptoms in HSP children were related to gut microbiota (Streptococcus and butyric acid-producing bacteria).

Proteomic analysis of urine reveals biomarkers for the diagnosis and phenotyping of abdominal-type Henoch-Schonlein purpura.

BackgroundAbdominal-type Henoch-Schonlein purpura (HSP) is a common refractory disease in children. Currently, no specific diagnostic biomarker is available for HSP.MethodsChildren with abdominal type HSP were first diagnosed with three syndromes using Chinese traditional medicine. The urinary proteomes among the three syndromes of patients with abdominal type HSP and healthy controls were compared using two label-free proteomics quantifications, including data-dependent acquisition and data-independent acquisition.ResultsFor the comparison between patients with abdominal type HSP and healthy children, a total of 75 differential urinary proteins were identified by determining the overlap of the two experiments. The ingenuity pathway analysis (IPA) analysis showed that these differential proteins were correlated with the pathogenesis of abdominal type HSP. Of these, 37 proteins were distributed in 13 solid tissues as tissue-enriched proteins. Monitoring changes in these proteins might help us detect uncommon clinical manifestations of HSP. Patients with abdominal type HSP can be further distinguished into three syndromes based on the urine proteome. Finally, a panel of six urinary proteins (P25774, P09417, Q7Z5L0, P60900, P14550 and P09668) was constructed for both the diagnosis and phenotyping of abdominal type HSP.ConclusionsUrinary protein biomarkers for the diagnosis and phenotyping of abdominal type HSP were identified, which will contribute to the personalized treatment of patients with abdominal type HSP.

MicroRNA profiling in Chinese children with Henoch-Schonlein purpura and association between selected microRNAs and inflammatory biomarkers.

This study aimed to profile the microRNA levels in Chinese Henoch-Schonlein purpura (HSP) children and to explore their association with inflammatory factors and T helper 17 (Th17)/regulatory T (Treg). Forty-five HSP children and 27 healthy controls were enrolled in this study, and microRNA levels were profiled with a microRNA microarray. The levels of selected microRNAs were determined by quantitative real-time PCR, and the levels of serum IgA, interleukin-6, interleukin-10 and interleukin-17A were detected by enzyme-linked immunosorbent assay. Additionally, Th17 and Treg cells were analysed by flow cytometry. There were 9 up-regulated and 27 down-regulated microRNAs in the PBMCs of Chinese HSP children. Among them, miR-1-3p, miR-19b-1-5p and miR-29b-1-5p were up-regulated, while miR-483-5p and miR-1246 were down-regulated. Additionally, these selected microRNAs could differentiate HSP patients from healthy controls. Interestingly, miR-29b-1-5p was correlated with IgA, miR-19b-1-5p, miR-483-5p and miR-1246 were correlated with interleukin-6, while miR-1-3p and miR-1246 were correlated with Th17/Treg. This study reveals that the altered microRNAs could differentiate HSP from the healthy, and were associated with inflammatory factors or Th17/Treg. It is indicated that alteration in these microRNAs may contribute to the HSP pathogenesis and may become therapeutic targets or diagnostic biomarkers for HSP.

Association of the infectious triggers with childhood Henoch–Schonlein purpura in Anhui province, China

BackgroundAlthough the specific etiology of Henoch–Schonlein purpura (HSP) is still unknown, several kinds of infectious triggers have been proved to participate in its pathogenesis. The objectives of present study were to analyze the association of the infectious triggers with childhood HSP in Anhui province, China. Methods1200 HSP children were recruited from January 2015 to December 2017. Serum antistreptolysin O titer, TORCH, Epstein-Barr virus, helicobacter pylori (HP), Mycoplasma antibodies (MP-Ab), tubercle bacillus antibody (TB-Ab), respiratory pathogens (legionella pneumophila, chlamydia pneumoniae, adenovirus, respiratory syncytial virus, influenza A virus, influenza B virus, rickettsia, parainfluenza virus) were determined. Patients’ histories were obtained by interviews and questionnaires. ResultsThe annual incidence of HSP was 8.13–9.17 per 100,000. HSP occurred more commonly in spring and winter than in summer with an obvious west-to-east gradient. On admission, several potential infections were identified in 611 cases (50.92%). The infectious agents including streptococcus, HP, MP, parainfluenza, respiratory syncytial virus, TB and toxoplasma gondii were identified in 205 cases (17.08%), 71 cases (5.92%), 58 cases (4.83%), 6 cases (0.5%), 1 case (0.08%), 1 case (0.08%) and 1 case (0.08%) respectively. 123 cases (10.25%) relapsed or recurred more than one time; the mean number was 2.92, and the mean interval was 11.4 weeks. The infection was the most frequent trigger regardless of clinical phenotypes and relapse/recurrence. Symptomatic treatment plus adjunctive anti-infectious agents could significantly improve the remission rate of purpura in the infectious cases (x2=24.60, p<0.01). ConclusionsStreptococcus is the most frequent infectious agent in HSP children regardless of clinical phenotype or relapse/recurrence. The complete elimination of infectious triggers may help relieve cutaneous purpura.

Effect of miR-21 on the expression of interleukin-10 in B cell of patients with Henoch-Schonlein purpura

Objective: To investigate the effect of microRNAs (miR)-21 on the expression of interleukin (IL)-10 in B cell of patients with Henoch-Schonlein purpura (HSP). Methods: From March 2016 to January 2017, twenty-four children with HSP hospitalized in rheumatology and immunology department of Shenzhen Children's Hospital were enrolled into the study, including 12 males and 12 females. Patients were divided into purpura nephritis group (HSPN, 14 cases) and non-nephritis group (NHSPN, 10 cases). The age-matched 34 healthy children were included as the control group for prospective cohort study. The expression levels of IL-10 in peripheral blood B cells (CD19(+)), transitional B cells (CD19(+) CD24(hi)CD38(hi)) and naïve B cells (CD19(+)CD24(int)CD38(int)) from patients with HSP and healthy children were detected by flow cytometry (FCM). Expression of microRNAs related to IL-10 in B cells were quantitated by real-time PCR, including miR-21-5p, miR-106a-5p, miR-98-3p, miR-142-3p, miR-142-5p, miR-98-5p, miR-155-5p and miR-let7b-5p. Agomir negative control-FAM and agomir-21-5p-FAM were transfected into B cells from patients with HSP. The uptake of miRNA by B cells was observed by laser scanning confocal microscope and FCM, and the expression of IL-10 was detected by FCM after transfection. For quantitative data of normal distribution, t test was used for two samples comparison and multiple comparisons among three groups were conducted by ANOVA. Spearman test was used for correlation analysis. Results: (1) The CD19(+) B cells and its two populations at different differentiation stages all could express IL-10. The expression levels of IL-10 in three B cell populations in patients were significantly lower than those in healthy controls (1.4±0.2 vs. 2.4±0.3, t=3.501, P<0.01; 1.2±0.2 vs. 2.2±0.3, t=2.688, P<0.05; 1.6±0.3 vs. 2.7±0.4, t=2.498, P<0.05). Compared with healthy control and NHSPN groups, the expression of IL-10 in CD19(+) B cells from patients within HSPN group was the lowest, and the difference was statistically significant (1.1±0.2 vs. 2.4±0.3, 1.8±0.3, t=4.006, 2.362, P<0.001, P<0.05). (2) The expression of miR-21-5p in B cell in patients with HSPN was lower than that in healthy control group (1.2±0.9 vs. 3.5±2.8, t=2.962, P<0.01). There was no significant change in the other microRNAs. (3) The expression of IL-10 was positively correlated with the expression of miR-21-5p in the B cells of patients with HSP (r=0.778, P<0.001). (4) The expression of IL-10 in B cells of miR-21-5p group was significantly higher than that in negative control group (2.7±0.2 vs. 1.6±0.3, t=3.091, P<0.05). Conclusion: The insufficient expression of miR-21-5p in peripheral blood B cells of patients with HSP is one of the reasons for the reduction of IL-10 expression in B cells.

Clinical study of the Liangxue-Zhuyu decoction combined with montelukast in the treatment of henoch schonlein purpura

Objective To study the efficacy of Liangxue-Zhuyu decoction combined with montelukast in the treatment of henoch schonlein purpura (HSP) and its influence on immunologic function. Methods A total of 95 HSP children treated in department of pediatrics from February 2014 to December 2016 were enrolled, and then were randomly divided into observation group (n=48) and control group (n=47), meanwhile another 48 healthy children admitted to a medical examination were selected as the healthy group. The patients in the control group were given the treatment of oral montelukast sodium chewable tablets before sleeping (the dose:2-6 years old 4 mg/d, 6-14 years old 5 mg/d), and the patients in the observation group accepted the therapy of Liangxue-Zhuyu decoction based on the treatment of the control group. The course of the treatment in the two groups were 30d. The time of clinical symptom regression and adverse reactions were recorded, and the clinical efficacy was evaluated. Monoclonal antibody immunofluorescence method and Enzyme-linked immunosorbent assay (ELISA) were used to detect the levels of serum T lymphocyte subsets and INF-γ and IL-10 before and after treatment, and their differences among three groups were compared. Results The effective rates of the observation group was 95.8% (46/48), which was significantly higher than 76.6% (36/47) of control group (χ2=7.441, P<0.05). The subsided time of the symptoms, including purpura (5.0 ± 1.3 d vs. 6.6 ± 2.6 d, t=3.816), abdominal pain (3.1 ± 2.1 d vs. 4.1 ± 2.6 d, t=2.139), joint pain (4.3 ± 1.5 d vs. 5.4 ± 1.9 d, t=3.416), fecal occult blood (4.5 ± 1.5 d vs. 6.1 ± 1.9 d, t=4.404) of the observation group were significantly shorter than those of the control group (P<0.05). After the treatment, the CD4 (45.14% ± 9.32% vs. 41.02% ± 9.97%), CD8 (25.10% ± 4.69% vs. 21.52% ± 5.71%) of the observation group was significantly higher than those of the control group, and the CD4/CD8 (1.62 ± 0.32 vs. 1.95 ± 0.35) and IL-10 (33.07 ± 1.86 pg/ml vs. 53.92 ± 2.98 pg/ml) of the observation group was significantly lower than this of the control group (P<0.05). Conclusions The efficacy of the Liangxue-Zhuyu decoction combined with montelukast for HSP was definite, and the effect was rapid with little side effects. The mechanism might be related to regulating the secretion of T lymphocyte and IL-10, thereby to correct Th1/Th2 cell balance and disturbance of immune function. Key words: Purpura, schoenlein-henoch; Liangxue-Zhuyu decoction; Montelukast sodium chewable tablets; T-Lymphocytes; Interferon-gamma; Interleukin-10

Biomarkers identification by a combined clinical and metabonomics analysis in Henoch-Schonlein purpura nephritis children.

BackgroundIn children with Henoch-Schonlein purpura (HSP), the severity of Henoch-Schonlein purpura nephritis (HSPN) is considered responsible for the prognosis of HSP. The pathological process from HSP to HSPN is not clear yet and current diagnostic tools have shortcomings in accurate diagnosis of HSPN. This study aims to assess clinical characteristics of HSP and HSPN, to identify metabolic perturbations involved in HSP progress, and to combine metabolic biomarkers and clinical features into a better prediction for HSPN.MethodsA total of 162 children were recruited, including 109 HSP patients and 53 healthy children (HC). The clinical characteristics were compared between HSPN and HSP without nephritis (HSPWN). The serum metabonomics analysis was performed to determine the metabolic differences in HSP and HC.ResultsAmong 109 HSP children, 57 progressed to HSPN. The increased D-dimer level was significantly associated with renal damage in HSP. The metabonomic profiles revealed alterations between various subgroups of HSP and HC, making it possible to investigate small-molecule metabolites related to the pathological process of HSP. In total, we identified 9 biomarkers for HSP vs. HC, 7 for HSPWN vs. HC, 9 for HSPN vs. HC, and 3 for HSPN vs. HSPWN.Conclusions(S)-3-hydroxyisobutyric acid, p-Cresol sulfate, and 3-carboxy-4-methyl-5-pentyl-2-furanpropanoic acid were found associated with the progress of HSP to HSPN. Moreover, resulting biomarkers, when combined with D-dimer, allowed improving the HSPN prediction with high sensitivity (94.7%) and specificity (80.8%). Together these findings highlighted the strength of the combination of metabonomics and clinical analysis in the research of HSP.

Parainfluenza infection is associated with Henoch-Schönlein purpura in children

Abstract Background To investigate the association of respiratory tract viruses with Henoch-Schonlein purpura (HSP) in children. Methods 40 children with HSP and 42 controls with upper respiratory tract infections were enrolled in the study. In both groups, the serum IgM antibodies and viral antigens in peripheral blood mononuclear cells (PBMCs) of common respiratory tract viruses including parainfluenza virus (PIV), influenza, respiratory syncytial virus (RSV), and adenovirus were detected. Children with HSP nephritis (HSPN) received renal biopsy, and antigens of the aforementioned viruses were examined. Results Viral IgM antibodies in sera and antigens in PBMCs were detected in HSP and control groups (55% vs. 42.9% and 60% vs. 42.9% respectively), but the differences were not significant. The prevalence of PIV IgM antibody and antigen in PBMCs in HSP group was significantly higher than that in controls (P Conclusions The presence of respiratory tract viruses was common in HSP children. PIV may be an important contributing factor in the pathogenesis of HSP.

Current views of the relationship between Helicobacter pylori and Henoch-Schonlein purpura in children.

Helicobacter pylori (H. pylori) is one of the factors involved in the pathogenesis of various gastrointestinal diseases and may play a potential role in certain extra-intestinal diseases. H. pylori infection are mainly acquired during childhood, and it has been reported that in endemic areas of China the infection rates are extraordinarily higher in HSP children, particular those with abdominal manifestations. Furthermore, eradication therapy may ameliorate Henoch-Schonlein purpura (HSP) manifestations and decrease the recurrence of HSP. Therefore, results suggested that detection of H. pylori infection by appropriate method ought to be applied in HSP children. Current evidences indicate that local injury of gastric mucosa and immunological events induced by H. pylori infection are involved in the development of HSP. Increased serum IgA, cryoglobulins, C3 levels, autoimmunity, proinflammatory substances and molecular mimicry inducing immune complex and cross-reactive antibodies caused by H. pylori infection might play their roles in the course of HSP. However, there are no investigations confirming the causality between H. pylori infection and HSP, and the pathogenesis mechanism is still unclear. More bench and clinical studies need to be executed to elaborate the complex association between H. pylori and HSP.

MEFV gene mutations in Egyptian children with Henoch-Schonlein purpura.

BackgroundDue to an increased frequency of vasculitis in FMF patients, many investigators have studied MEFV mutations in patients with HSP. The aim of the study is to investigate the frequency and clinical significance of MEFV mutations in Egyptian children with Henoch-Schonlein purpura (HSP). Investigating MEFV mutations in controls may help in estimating the prevalence of MEFV mutation carrier rate in Egyptian children.MethodsThe study enrolled 90 individuals, sixty children with Henoch-Schonlein purpura (HSP), together with 30 sex-and age-matched apparently healthy controls. The entire study group was screened for 12 common MEFV mutations using a reverse hybridization assay of biotinylated PCR products.ResultsPatients with HSP had a significantly higher frequency of MEFV mutations (61.7%), when compared to the apparently healthy control population (36.7%). V726A was the most frequent mutation with an allelic frequency of 10.8%. Ninety- one percent of patients with MEFV mutations were heterozygous for one mutation, while 8.1% had a compound heterozygous MEFV gene mutations. The mutation V726A, followed by E148Q, were the leading mutations, present in 16.6% and in 13.3% of controls.ConclusionsMEFV mutations may be related to HSP susceptibility in children. The mutations were not associated with any clinical and laboratory manifestations. Screening for MEFV mutations in larger number of HSP children may be beneficial to evaluate any possible relationship between certain types of MEFV mutations and HSP, and compare the HSP MEFV mutations to the types of MEFV mutations associated with FMF.Electronic supplementary materialThe online version of this article (doi:10.1186/1546-0096-12-41) contains supplementary material, which is available to authorized users.

Changes and significance of serum interleukin-17 and matrix metalloproteinase-9 in children of henoch-schonlein purpura

Objective To investigate the chanegs and correla iton between IL-17 and MMP-9 in c hildren of henoch-schonlein purpura,and to explore the pathogenesis of teh IL-17 and MMP-9 in henoch-schonlein pur-pura /henoch-schonlein purpura nephritis.Methods The level of serum IL-17 andMMP-9 in 74 henoch-schon-lein purpura childr en in initial stage and 30 cases of healthy children（ control group） were detetced by ELISA. Results （1） The level of plasma IL-17 was significantly higher in children of henoch-schonlein purpura thna tha t foh ealthy control group （86.59 ±35.50） fg/L vs.（62.38 ±14.65）fg/L,P〈0.01].（2） The level of plasma MMP-9 was significantly higher in children of henoch-schonlein pupru ar than that o fhealt hy control group [（201.82 ±105.87） fg/L vs（89.27 ±27.99）fg/L,P 〈0.01].（3） Henoch-schonlein purpura children were followed up for6 months,dependin g on whether the kidney was involved,they were devided into non-henoch-schonlein purpura nephritsi grou p nad henoch-s chonlein purpura nephritis group.In initial phase of HSP,the level of plasma IL-17 was significant lyhigher in henoch-schonlein purpura nephritis group than non-henoch-schonlein purpura nephrits group [（101.67 ±39.55） fg/L vs.（101.67 ±39.55）fg/L,P〈0.05].The level of p lasma MMP-9 was significantly higher in henoch-schonlein purpura nephritsi group than no n-henoc h-schonlein purpura nephritis group [（249.63 ±97.57） Pg/L vs.（185.30 ±104.39） pg/L,P〈0.05 ].（4） IL-17 and MMP-9 correlation aanlysis didn＇t show sign ificant correlation（r=0.184,P〉0.05）.Conclusion IL -17 and MMP-9 are involved in the pathogenesis of henohc-schonlein purpura/henoch-schonlein pur pura nephritis. Key words: Henoch-Schonlein purpura; Interleukin-17; Matrix metalloproteinase-9

Expression of plasmacytoid dendritic cells in peripheral blood and renal tissues in children with Henoch-Schönlein purpura

Objective To investigate the expression and distribution of plasmacytoid dendritic cells(pDC) in peripheral blood and renal tissues in children with Henoch-Schonlein purpura(HSP), and explore the role of pDCs in the pathogenesis of Henoch-Schonlein purpura nephritis(HSPN). Methods Among the 40 children with HSP, 28 cases were in the active phase(renal biopsy performed in 8 cases of them)and the other 12 in remission phase.Peripheral blood mononuclear cells were isolated, and the expression of pDC was detected by flow cytometry.The normal control group was established (n=15). Total RNA of peripheral blood was extracted and transcripted into cDNA.Sybr green dye based real-time quantitative PCR method was used to compare the expression(indicated as 2-ΔCt value) of CXC motif chemokine 10 (CXCL10), CC chemokine ligand 5 (CCL5), chemokine CXC subfamily receptor 3(CXCR3), CC chemokine receptor 5(CCR5) in children with HSP and those in the controls.Immunohistochemistry labeling technique was used to detect the distribution of pDC in renal tissues from renal biopsy, and the normal controls were established(n=3). Results The expression percentage of pDC in peripheral blood in active phase was 0.051± 0.039, signi-ficantly lower than those in remission phase(0.181±0.082) and the normal controls(0.166±0.079)(P<0.000 1). Chemokines genes CXCL10 and CCL5 were overexpressed in peripheral blood ceils of acute phase HSP children, but chemokine receptors CXCR3, CCR5 were lowly expressed compared with normal controls.There was almost no expression of pDC in the normal control renal tissues, while pDC was infiltrated in glomeruli of HSPN children. Conclusions The number of pDC and chemokines' expression in peripheral blood is abnormal, and the pathogenesis of nephritis may be involved with the pDC in peripheral blood to migrate to the renal tissues. Key words: Henoch-Schonlein purpura; Plasmacytoid dendritic cells; Chemokines

Effect and possible mechanism of hemoperfusion treatment for severe Henoch-Schönlein purpura

Objective To research the changes of clinical symptoms and serum free radical in children with severe Henoch-Schonlein purpura (HSP) between before and after accepted hemoperfusion. And evaluate the curative effect of hemoperfusion treatment for severe HSP and discuss the mechanism. Methods Twenty-three severe HSP patients in Children's Medical Center of the Second Xiangya Hospital of Central South University which were divided into 2 groups: 13 cases were divided into traditional treatment group, 10 cases were divided into hemoperfusion group; 11 healthy children were divided into healthy control group. The alleviate situation of clinical symptoms were observed and recorded such as purpura, abdominal pain, joint pain, hematuria, albuminuria, and the changes of urine RBC count, 24 hour urine protein quantitative before and after hemoperfusion and traditional treatment. Collected the serum before and after the first time hemoperfusion treatment, after the second time hemoperfusion treatment, after the third time hemoperfusion treatment in hemoperfusion group; Collected the serum before and after conventional therapy in traditional treatment group; Collected only once serum in healthy control group. And then their superoxide anion(), hydroxy radical(·OH), hydrogen dioxide(H2O2), malonaldehyde(MDA) values were detected with spectrophotometry. The diffe-rences of each index among each group were compared respectively. Results After treatment, the clinical symptoms of 23 severe HSP children such as rash, abdominal pain, joint pain, hematuria, albuminuria which were reliefed compared to before treatment. The urine erythrocyte count, 24 hour urine protein quantitative were reduced in 2 groups, but the symptoms of perfusion group children relieved faster, and the clinical index decreased more obviously. The serum , ·OH, H2O2, MDA levels of 23 HSP children were significantly higher than those of the healthy control group(all P<0.01). In the traditional treatment group, compared between before and after treatment, the indexes were decreased(all P<0.05); After 3 times of hemoperfusion, all indexes were decreased in hemoperfusion group, but only after the third hemoperfusion the indexes were decreased statistically significant(all P<0.05). But compared with the hemoperfusion group, the index were decreased more apparently in after the third hemoperfusion, and it was statistically significant(P<0.05). Conclusions 1. The serum free radicals are increased in severe HSP children, they may play a role in vasculitis.2. For severe HSP, the recent therapeutic effect of hemoperfusion ally with traditional treatment is better than the alone traditional treatment.3. Hemoperfusion ally with traditional treatment can remove more effectly the serum free radicals, reduce lipid peroxide products, then mitigate the damage of the oxidate stress to the vascular endothelial. Key words: Henoch-Schonlein purpura; Hemoperfusion; Vascular endothelial cell; Free radicals; Oxidate stress

Apoptosis of human umbilical vein endothelial cells (HUVEC) induced by IgA1 isolated from Henoch-Schonlein purpura children

It had been shown that apoptosis of vascular endothelial cells played an important role in the pathogenesis of HSP. The present study was designed to investigate the apoptosis of vascular endothelial cells induced by isolated IgA1 from sera of HSP patients. HUVEC were cultured in 3 different types of media with IgA1 from HSP patients, normal healthy children and simply medium (blank control). Serum IgA1 was purified by jacalin affinity chromatography. The rates of apoptosis in HUVEC incubated with IgA1 were determined by the TUNEL method and flow cytometry, respectively. The expression of bax/bcl-2 and p53 was detected with the methods of Real-time PCR and Westernblot, respectively. The results showed that the apoptosis rate of HUVEC by IgA1 isolated from HSP patients was higher than that the normal controls (14.77±2.23% vs 9.97±1.48%) and blank controls (14.77±2.23% vs 2.25±0.77%) (P <0.01). Moreover the rate of HUVEC by IgA1 from normal healthy children was higher than the blank controls (9.97±1.48% vs 2.25±0.77%) (P <0.01). In addition, the bax and P53 expression were up-regulated and the Bcl-2 expression was down-regulated in HUVEC co-cultured with IgA1 isolated from HSP patients for 24 hours. These findings suggested that IgA1 from HSP patients could induce the apoptosis of HUVEC, which might be related to the vascular endothelial injury of HSP.

Henoch Schonlein Purpura in children: clinical analysis of 120 cases

Henoch Schonlein Purpura (HSP) is a systemic vasculitic disease which is common in children. It is very important to understand the clinical features of this disease for doctors and nurses. To study the clinical characteristics of HSP in children. Collect the clinical data of the HSP children, and analyze the clinical characteristics of these HSP patients. The ratio of M:F was 1.9:1. The mean age was 6.6 ± 1.6 years. The typical onset seasons were spring, winter and autumn. Infection and food allergy were the main etiological factors. The first symptom was skin purpura and these purpura mainly concentrated the lower extremities and buttocks. The dominant digestive clinical features were abdominal pains and vomiting. The knee joint and ankle joint were most frequently affected. The typical kidney symptoms were microscopic hematuria and albuminuria. An increased ESR was reported in 68 patients (56.7%). Serum C3 decreased in 13 cases (10.8%). ASO titer was higher in 57 children (47.5%). There were gender, season and area differences for the HSP patients. The etiological factors were diverse. HSP patients could have various clinical symptoms and rare complications.

Clinical characteristics of pancreatic gland damage in children with Henoch-Schönlein purpura

Objective To explore the clinical characteristics of pancreatic gland damage in children with Henoch-Schonlein purpura(HSP). Methods The serum and urine analysis of 95 examples were detected by the automa-tic biochemical analyzer, which were diagnosed as HSP from Aug.2009 to Jun.2010 in Department of Nephrology, Hunan Children's Hospital, and the clinical characteristics of them were analyzed.All children were treated with anti-infection and anti-allergic drugs.The pancreatic morphology of patients was observed by B ultrasonic, and the clinical features of skin, joints, digestive tract, and kidney damage were observed.And the relationship between damage of pancreas and damage of other systems was analyzed. Results There were 64 cases suffering from pancreatic gland damage in 95 HSP children: 34 cases were male(53.1%), 30 cases were female(46.9%), and there was no significant difference(χ2=0.56, P>0.05). The incidence of pancreatic gland damage of the patients with allergic purpura combined with abdominalgia was 82.8%(53 cases), which was obviously higher than that in the patients without abdominalgia (11 cases, 17.2%)(χ2=14.24, P 0.05). Conclusions HSP children usually were accompanied with pancreatic gland damage, and if they get abdominalgia and multi-system damage they are more likely to be accompanied with pancreatic gland damage, which should be paid attention to. Key words: Henoch-Schonlein purpura; Pancreatic gland damage; Child

Is Helicobacter pylori infection associated with Henoch-Schonlein purpura in Chinese children? a meta-analysis

The prevalence of Helicobacter pylori (H. pylori) infection is high in China. It not only causes the damage of gastric epithelium, but also plays a potential pathogenic role in several extraintestinal diseases. Henoch-Schonlein purpura (HSP) is one of the most common vasculitis syndromes affecting children. Although its cause is unclear, HSP is often considered to be associated with infectious agents. This metaanalysis of previously published studies was conducted using a predefined protocol to evaluate the underlying association between H. pylori infection and HSP in Chinese children. Predefined search strategy and inclusion criteria were set up to select studies reporting the prevalence of H. pylori infection among HSP children and control groups. Included studies were subjected to quality assessment and data extraction by two independent reviewers. The pooled odds ratio (OR) was calculated as the effect size via both traditional and cumulative meta-analysis. Heterogeneity was investigated by subgroup analysis, and the nonparametric "trim and fill" method was performed to adjust the overall estimate for the existence of publication bias. Ten eligible studies covering 749 HSP children and 560 controls were included for metaanalysis. Observational epidemiology studies clearly aimed at detecting the potential association between H. pylori infection and HSP with retrospective data collection from the children enrolled consecutively. Overall, 49.27% (369/749) of HSP children had evidence of H. pylori infection compared with 23.39% (131/560) of children in the control group. The pooled OR of H. pylori infection in HSP children (10 studies with 749 HSP children) was 3.80 [95% confidence interval (CI): 2.54-5.68, P<0.001], and the overall estimate from the cumulative meta-analysis confirmed the association with more narrow confidence interval (OR=3.35, 95% CI: 2.95-3.81). In HSP children mainly with abdominal manifestations (8 studies with 337 HSP children), the pooled OR was 4.62 (95% CI: 2.66-8.01, P<0.001). The adjusted pooled OR was 2.04 (95% CI: 1.48-2.82, P<0.001), determined by the nonparametric "trim-andfill" method for eliminating the effect of publication bias. H. pylori eradication therapy (4 studies with 266 HSP children) was capable of reducing the recurrence of HSP (RR=0.38, 95% CI: 0.25-0.58, P<0.001). Although the subgroup analysis for heterogeneity suggested that diagnostic methods and geographical diversity might be account for the heterogeneity, statistical analysis of differences revealed no differences between subgroups, indicating their limited impact on the overall estimates. These results suggest the necessity of screening H. pylori infection in HSP children, particularly in those with gastrointestinal manifestations in China. Eradication therapy may reduce the recurrence of HSP in children with H. pylori infection. However, further mechanistic and more clinical studies in different populations and regions are needed to confirm this association and the effect of eradication of H. pylori in HSP children.