Association between anti-α-1,4-D-polygalacturonic acid antibodies and Henoch-Schönlein purpura in children.

OBJECTIVE: To investigate the relationship between anti-α-1,4-D-polygalacturonic acid (PGA) antibodies, particularly immunoglobulin (Ig)A, and Henoch-Schönlein purpura (HSP) in children. METHODS: This observational case-control study investigated PGA-IgA, PGA-IgG, and PGA/PGA-IgA circulating immune complex (PGA/PGA-IgA CIC) in paediatric patients with HSP versus controls. Children with HSP were also evaluated for food specific IgG and food intolerance. Between-group differences in anti-PGA antibodies were analysed. RESULTS: Serum PGA-IgA and PGA-IgG levels were significantly increased in patients with acute HSP ( n = 251) versus those with urticaria ( n = 48), acute respiratory infections ( n = 95), surgical controls ( n = 53) and neonates ( n = 92). PGA/PGA-IgA CIC levels were also significantly higher in the acute HSP group versus surgical control and neonate groups. Levels of PGA/PGA-IgA CIC and PGA-IgA were significantly correlated ( r = 0.997), and PGA-IgA showed high diagnostic specificity for HSP. No statistically significant differences were observed in PGA-IgA and PGA-IgG between various degrees of food intolerance in children with HSP. CONCLUSION: Increased anti-PGA antibodies, particularly PGA-IgA and PGA/PGA-IgA CIC, were significantly associated with acute HSP in children. Food intolerance was not found to be associated with increased anti-PGA antibodies in children with HSP.

[Association of serum vitamin D level with severity and treatment in children with Henoch-Schönlein purpura].

OBJECTIVE: To investigate the association of serum vitamin D [25-(OH)D3] level with the severity and treatment in children with Henoch-Schönlein purpura (HSP). METHODS: A total of 50 children with newly-diagnosed HSP between January and December, 2015 were enrolled as HSP group, and 49 healthy children were enrolled as control group. Fasting serum samples were collected, and ELISA was used to measure serum 25-(OH)D3 level. According to the serum 25-(OH)D3 level, the HSP group were further divided into normal group (>20 ng/mL) (n=9), insufficiency group (15-20 ng/mL) (n=15), deficiency group (≤15 ng/mL) (n=25), and severe deficiency group (≤5 ng/mL) (n=1). The general data, clinical manifestations, hormone therapy, course of disease before admission, and length of hospital stay were compared between groups. RESULTS: The HSP group had a significantly lower serum 25-(OH)D3 level than the control group (16±6 ng/mL vs 29±5 ng/mL; P<0.01). Compared with the normal and insufficiency groups, the deficiency and severe deficiency groups had significant increases in the incidence rate of renal involvement, rate of hormone application, and median length of hospital stay (P<0.05), while there was no significant difference in course of disease before admission (P>0.05). CONCLUSIONS: Children with HSP have a low serum 25-(OH)D3 level, and such children may have a high risk of renal involvement, a high rate of hormone application, and a prolonged length of hospital stay. However, further studies are needed to investigate whether vitamin D supplementation is helpful to the treatment of HSP and can shorten the course of disease in children with HSP.

[Protective Effect of Total Flavones of Bidens pilosa L. on IgA1 Induced Injury of HUVECs in Henoch-Schönlein Purpura Children Patients].

OBJECTIVE: To explore the protective effect and mechanism of total flavones of Bidens pilosa L. (TFB) on IgA1 induced injury of venous endothelial cells in Henoch-Schönlein purpura (HSP) children patients. METHODS Human umbilical venous endothelial cells (HUVECs) were taken as subject. They were intervened by normal IgA1 and HSP children patients' serum IgA1, and added with different concentrations TFB at the same time. Then they were divided into the blank control group, the normal control group, the HSP IgA1 group, and HSP IgA1 plus TFB (1.0, 0.5, 0.25 mg/mL) groups. Levels of TNF-α and IL-8 in supernate were detected by ELISA. The NO level was detected by nitrate reductase method. mRNA and protein expressions of NF-κB and ICAM-1 in HUVECs were detected by fluorescent quantitative PCR and Western blot respectively. RESULTS: Compared with the normal control group and the blank control group, levels of IL-8, TNF-α, and NO all significantly increased in the HSP group (P < 0.05). Compared with the HSP group, levels of IL-8, TNF-α, and NO significantly decreased after intervention of TFB (1.0 and 0.5 mg/mL; P < 0.05, P < 0.01). Results of fluorescent quantitative PCR and Western blot showed, as compared with the blank control group and the normal control group, mRNA and protein expressions of NF-κB and ICAM-1 in HSP children patients' serum IgA1 induced venous endothelial cells significantly increased with statistical difference (P < 0.05, P < 0.01). Compared with the HSP group, mRNA and protein expressions of NF-KB and ICAM-1 were obviously down-regulated after intervention of TFB (1.0, 0.5, 0.25 mg/mL), with statistical difference (P < 0.05, P < 0.01). CONCLUSION: TFB could protect vascular damage by inhibiting in vivo high expression of NF-κB, reducing the production of IL-8, TNF-α, and NO in vascular endothelial cells of HSP children patients.

[Controlled study on therapeutic effect of vessel pricking therapy and western medication for treatment of Henoch-Schonlein purpura nephritis].

OBJECTIVE: To compare the difference of therapeutic effects between vessel pricking therapy and Prednisone for treatment of Henoch-Schonlein purpura nephritis. METHODS: Seventy cases of acute purpura nephritis syndrome were randomly divided into an observation group (40 cases) and a control group (30 cases). Patients in observation group were differentiated into sthenia and asthenia syndromes. Vessel pricking therapy was applied at Hegu (LI 4), Quchi (LI 11), Xuehai (SP 10) etc. by triangular needle for sthenia symptom; shallow needling was used at Pishu (BL 20), Shenshu (BL 23), Zusanli (ST 36) etc. by filiform needle. The control group was treated with oral admi-nidtration of Prednisone. The symptom score of TCM, 24 h urinary protein, red blood cell count of urinary sediment of both groups were observed before and after treatment and therapeutic effects were compared. RESULTS: The total effective rate of 92.5% (37/40) in observation group was superior to that of 80.0% (24/30) in control group, and there was a significant difference between two groups (P < 0.05); the symptom score of TCM, 24 h urinary protein, red blood cell count of urinary sediment were all improved in both groups after treatment (all P < 0.05), and moreover, the improvement in observation group was superior to that of control group (all P < 0.05); after treatment, the symptom score of TCM of sthenia syndrome was lower than that of asthenia syndrome in observation group (P < 0.05). CONCLUSION: Vessel pricking therapy has a significant therapeutic effect for treatment of Henoch-Schonlein purpura nephritis, superior to that of oral administration of Prednisone, and the therapeutic effect is better for treating sthenia syndrome than for asthenia syndrome.

[Effects of Astragalus membranaceus on cytokine secretion of peripheral dendritic cells in children with Henoch-Schonlein purpura in the acute phase].

OBJECTIVE: To explore the effects of Astragalus membranaceus (AM) on the cytokines secretion of peripheral dendritic cells (DC), including interleukin-10, -12, and -18 (IL-10, IL-12 and IL-18), in children with Henoch-Schonlein purpura (HSP) in the acute phase; and to study the immunological regulation mechanism of AM. METHODS: Peripheral blood mononuclear cells (PBMC) were obtained from 28 children with acute HSP by density gradient centrifugation, and each sample was divided into two parts, one untreated and one treated with AM. All cells were developed to mature DC through treating with recombinant human granulocyte macrophage colony stimulating factor (GM-CSF), interleukin-4 (IL-4) and tumor necrosis factor-alpha (TNF-alpha). Expression of CD83 in the surface of mature DC was detected by flow cytometry, and levels of IL-10, IL-12 and IL-18 in the supernatant were measured by ELISA. RESULTS: The supernatant level of IL-12 was higher [(141.58 +/- 100.19) ng/L vs (96.18 +/- 76.65) ng/L, t = 3.90, P<0.01], while levels of IL-10 and IL-18 were lower (t = 2.70, P<0.05; t = 4.07, P<0.01) in AM treated PBMCs than those in the untreated ones. CONCLUSION: AM can correct the immunologic dysfunction of HSP children through increasing the IL-12, and decreasing the IL-10 and IL-18 secretions of PBMCs.

Effect of composite salvia injection on platelet parameters in children with anaphylactoid purpura.

OBJECTIVE: To explore the effect of composite salvia injection (CSI) on platelet parameters in children with anaphylactoid purpura (AP) and its clinical significance. METHODS: One hundred and fifty children with AP were assigned to two groups, 80 in Group A and 70 in Group B. They were treated, respectively, with conventional therapy only or conventional therapy combined with CSI. Their platelet parameters, including blood platelet count (BPC), mean platelet volume (MPV), platelet distribution width (PDW) and plateletcrit (PCT) were determined at the acute stage and convalescent stage, respectively. RESULTS: At the acute stage, the BPC in AP children of both groups was in the normal range, but significant abnormality was shown in the levels of MPV, PDW and PCT. As for comparisons of these parameters at the convalescent stage, significant difference between the two groups was also shown in terms of MPV, PDW and PCT (P<0.05 or P<0.01). CONCLUSION: Although platelet shows no quantitative change in the pathogenic process of AP, important functional changes are surely existent. CSI could promote the normalization of platelet function.

[Effect of danshao granule on serum superoxide dismutase activity and malonyldialdehyde content in children with Henoch-Schonlein purpura nephritis].

OBJECTIVE: To explore the effect and mechanism of Danshao Granule (DSG) in treating Henoch-Schonlein purpura nephritis (HSPN) in children. METHODS: The 63 patients with HSPN were randomly divided into two groups. The 32 patients in the treated group were treated with DSG and the 31 patients in the control group were treated with Tripterygium polyglycosides tablet and composite Salviae tablet. The therapeutic course for both groups was one month. The skin purpura, macroscopic hematuria, hypertension and edema subsidence time, 24 hrs urinary protein content, serum levels of immunoglobulins (IgA, IgG, IgM), superoxide dismutase (SOD) activity and malonyldialdehyde (MDA) content were observed before and after treatment. RESULTS: Therapeutic effect in the treated group was better than that in the control group in curing skin purpura and macroscopic hematuria (P < 0.05). The 24 hrs urinary protein content and serum levels of IgA, SOD and MDA were improved after treatment in both groups significantly (P < 0.01). However, the improvement of 24 hrs urinary protein, serum SOD and MDA in the treated group was more significant than that in the control group respectively (P < 0.05). CONCLUSION: DSG can alleviate the injury of free radicals in organism, so it is an ideal remedy for treatment of HSPN.

[Scanning electron microscopy of peripheral blood erythrocytes in the diagnosis and treatment of hemorrhagic vasculitis in children]. / Metod skaniruiushchei élektronnoi mikroskopii éritrotsitov perifericheskoi krovi v diagnostike i lechenii gemorragicheskogo vaskulita u detei.

Twenty-four children aged 3-15 years were examined, 16 of these with cutaneous and articulo-cutaneous hemorrhagic vasculitis (HV) and 8 normal controls. The patients were divided into 2 groups: 10 patients treated by basic therapy and 6 children whose treatment protocols were supplemented by membranoprotector locheine. The children were repeatedly examined 1 month after discharge from hospital. Scanning electron microscopy of peripheral blood erythrocytes provides valuable diagnostic data on erythrocyte membrane morphology and function in children with HV and can serve as a method for monitoring the efficiency of new approaches to therapy of this disease.

An open-label study of the role of adjuvant hemostatic support with protein C replacement therapy in purpura fulminans-associated meningococcemia.

Activated protein C (APC) is a natural anticoagulant that plays a pivotal role in coagulation homeostasis. Severe inherited or acquired deficiency results in a clinical syndrome called purpura fulminans. In addition, APC also appears to have potent cytokine-modifying properties and is protective in animal models of sepsis. The dual functional properties of APC are particularly relevant to severe meningococcemia, where acquired PC deficiency is accompanied by multiorgan failure and purpura fulminans. The authors conducted an open-label prospective study assessing the efficacy of PC replacement therapy in patients with severe meningococcal septicemia, purpura fulminans, and multiorgan failure. The morbidity and mortality were compared with predicted morbidity using the Glasgow Meningococcal Septicemia Prognostic Score. Thirty-six patients with a mean age of 12 years (range 3 months to 72 years) were enrolled in the study. The mean +/- SD for plasma PC was 18 +/- 7 IU/mL. PC was significantly lower than antithrombin or protein S and was also significantly lower than PC levels in a cohort of patients who developed meningococcemia without multiorgan failure and purpura fulminans. A total of 3 of 36 (8%) patients died, which compares favorably with predicted mortality of 18 of 36 (50%). Amputations were required in 4 of 33 (12%) survivors and in 2 of 31 (6.5%) patients who received PC within 24 hours of admission into the hospital, in comparison with the predicted amputation rate of 11 of 33 (30%). In conclusion, PC replacement therapy in severe meningococcal septicemia was associated with a reduction in predicted morbidity and mortality. The beneficial effect of PC replacement may reflect both the anticoagulant and anti-inflammatory properties of the PC pathway. (Blood. 2000;96:3719-3724)

[Significance of hypercoagulation index in treatment of allergic purpura by Huoxue Huayu decoction].

OBJECTIVE: To explore the therapeutic mechanism of Huoxue Huayu Decoction (HXHYD) in treating allergic purpura (AP) and the significance of hypercoagulation index (HCI). METHODS: Patients of AP were divided into two groups. On the basis of conventional treatment of western medicine, the HXHYD was given to the treated group additionally, and the cure rate, change of hypercoagulation index of patients were compared with those of the control group treated by western conventional treatment alone. RESULTS: The cure rate of the treated group was 86%, higher than that of control group (48%) significantly. HCI of the treated group was lowered markedly after treatment, as compared with that before treatment, the difference was significant (P < 0.01). CONCLUSIONS: By the comprehensive effect of promoting anticoagulation activity, maintaining the integrity of vascular endothelia and reducing aggregation of platelets, HXHYD could reduce the HCI and improve hypercoagulative state of AP patients, so as to elevate the cure rate significantly.