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Objective:To investigate the efficacy of montelukast sodium combined with methylprednisolone in the treatment of pediatric allergic purpura and its effects on inflammatory factors and immune function.Methods:A total of 94 children with allergic purpura who received treatment in Taizhou Women and Children's Hospital and Taizhou Hospital Medical Center (Group) Enze Hospital from March 2019 to March 2021 were included in this study. They were randomly divided into observation and control groups ( n = 47/group). The control group was treated with methylprednisolone. The observation group was treated with montelukast sodium combined with methylprednisolone. The course of treatment was 2 weeks in both groups. Efficacy and changes in inflammatory factors and immune function post-treatment relative to those pre-treatment were compared between the two groups. Results:Total response rate in the observation group [93.62% (44/47)] was significantly higher than that in the control group [74.47% (35/47), Z = 2.15, P < 0.05)]. After treatment, interleukin (IL-4), IL-6, and IL-18 levels in each group were significantly decreased compared with those before treatment ( tobservation group = 21.19, 22.26, 27.20, tcontrol group = 11.10, 13.21, 14.86, all P < 0.05). After treatment, IL-4, IL-6, and IL-8 levels in the observation group were (48.98 ± 5.21) ng/L, (34.10 ± 6.42) ng/L, and (53.29 ± 5.67) ng/L, respectively, which were significantly lower than (65.38 ± 7.08) ng/L, (47.83 ± 4.71) ng/L, (67.83 ± 7.10) ng/L in the control group ( t = 12.79, 11.82, 10.97, all P < 0.05). After treatment, CD3 +, CD4 +, and CD4 +/CD8 + in each group were significantly increased compared with those before treatment ( tobservation group = 14.27, 14.41, 17.61, tcontrol group = 6.90, 5.12, 7.40, all P < 0.05). After treatment, CD3 +, CD4 +, and CD4 +/CD8 + in the observation group were (68.94 ± 2.89)%, (39.94 ± 2.15)%, and (1.79 ± 0.13), respectively, which were significantly higher than (63.86 ± 3.28)%, (35.65 ± 2.31)%, and (1.53 ± 0.16) in the control group ( t = 7.96, 9.32, 8.64, all P < 0.05). After treatment, serum IgG and IgM levels in each group were significantly decreased compared with those before treatment ( tobservation group = 21.00, 7.99, tcontrol group = 8.38, 5.76, both P < 0.05). After treatment, serum IgG and IgM levels in the observation group were (1.43 ± 0.19) g/L and (9.74 ± 0.78) g/L, respectively, which were significantly lower than (1.95 ± 0.37) g/L and (10.89 ± 0.85) g/L in the control group ( t = 8.57, 6.83, both P < 0.05). Conclusion:Montelukast sodium combined with methylprednisolone is highly effective on allergic purpura in children. The combined therapy can reduce inflammatory responses and improve immune function in children.
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Abstract Objective The aim of this study was to evaluate the serum Syndecan-1 (SDC-1) levels in patients with immunoglobulin-A vasculitis (IgAV) in children and its relation with gastrointestinal involvements. Methods Sixty-eight children with IgAV and 48 healthy children were enrolled in this cross-sectional study. Clinical and related laboratory data were collected from a computerized hospital database. Serum SDC-1 was collected on admission prior to treatment. Results Forty-eight patients fully met the IgAV diagnostic criteria at admission (IgAV group), 20 patients with rash only and diagnosed IgAV during hospitalization (Purpura group). In IgAV group, 30 patients with gastrointestinal involvements (IgAV-GI group) and 18 patients without gastrointestinal involvements (IgAV-NGI group). SDC-1 serum levels were significantly higher in the IgAV group (86.37 ng/mL (IQR 59.16-117.14 ng/mL)) than in the controls (20.37 ng/mL (IQR 15.52-26.45 ng/mL)) and the Purpura group (32.66 ng/mL (IQR 14.87-49.89 ng/mL)). Additionally, SDC-1 (OR = 1.08) was independently associated with IgAV with a cut-off value (sensitivity and specificity) of 66.55 ng/mL (68.8%, 95.0%), and the area under the curve was 0.908. The serum SDC-1 levels of the IgAV-GI group (106.92 ± 50.12 ng/mL) were significantly higher than those in the IgAV-NGI group (67.52 ± 17.59 ng/mL). Logistic regression analysis showed that SDC-1 (OR = 1.03) was independently associated with IgAV-GI with a cut-off value of 89.39 ng/mL. Conclusions SDC-1 serum levels may mirror vascular endothelium injury and mucosal damage in IgAV. Its applicability as a surrogate biomarker in IgAV remains to be determined.
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Abstract Introduction Henoch-Schönlein purpura nephritis (HSN) is defined as Henoch-Schönlein purpura with kidney involvement, including hematuria and/or proteinuria. The aim of this study was to evaluate the data of HSN patients who underwent renal biopsy, and compare the main clinical and laboratory parameters that may affect renal biopsy findings, treatment protocols, and short- and long-term outcome of those patients. Methods Biopsies performed in 72 HSN patients between January 2007 to January 2017 were retrospectively evaluated. They were divided into two groups according to renal biopsy classification of the International Study of Kidney Disease in Children. Renal outcome, clinical and laboratory parameters, treatment protocols, and outcome were compared between groups. Short- and long-term follow-up of patients were evaluated. Results Of 72 patients, 47 were male (65.3%) and 44 (61.1%) were ≤10 years of age. Neutrophil-lymphocyte ratio was found higher in patients with scrotal involvement (p=0.042). Short-term unfavorable outcome was significantly higher in patients with scrotal involvement (p=0.038). Patients with hypertension and decreased creatinine clearance were found to have more unfavorable outcomes in long-term follow-up (p=0.029, p=0.040). Conclusion Cyclosporin-A and cyclophosphamide could be effective in steroid unresponsive HSN patients. Patients with scrotal involvement, decreased creatinine clearance, and hypertension should be closely monitored for sequelae of HSN.
Resumo Introdução A nefrite da púrpura de Henoch-Schönlein (NPHS) é definida como púrpura de Henoch-Schönlein com envolvimento renal, incluindo hematúria e/ou proteinúria. O objetivo deste estudo foi avaliar os dados de pacientes com NPHS que foram submetidos à biópsia renal e comparar os principais parâmetros clínicos e laboratoriais que podem afetar os achados da biópsia renal, os protocolos de tratamento e o desfecho de curto e longo prazo desses pacientes. Métodos Foram avaliadas retrospectivamente biópsias realizadas em 72 pacientes com NPHS entre Janeiro de 2007 e Janeiro de 2017. Eles foram divididos em dois grupos de acordo com a classificação de biópsia renal do Estudo Internacional de Doenças Renais em Crianças. O desfecho renal, parâmetros clínicos e laboratoriais, protocolos de tratamento e desfechos foram comparados entre os grupos. Foi avaliado o acompanhamento de pacientes de curto e longo prazo. Resultados De 72 pacientes, 47 eram homens (65,3%) e 44 (61,1%) tinham ≤10 anos de idade. A razão neutrófilo-linfócito foi encontrada mais alta em pacientes com envolvimento escrotal (p=0,042). O desfecho desfavorável de curto prazo foi significativamente maior em pacientes com envolvimento escrotal (p=0,038). Constatou-se que pacientes com hipertensão e diminuição da depuração de creatinina apresentaram desfechos mais desfavoráveis no acompanhamento de longo prazo (p=0,029, p=0,040). Conclusão A ciclosporina-A e a ciclofosfamida podem ser eficazes em pacientes com NPHS não responsivos a esteroides. Pacientes com envolvimento escrotal, diminuição da depuração de creatinina e hipertensão devem ser monitorados de perto para sequelas de NPHS.
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Objective:To investigate the clinical significance and the diagnostic value of detecting kidney injury biomarkers in urine and serum of children with Henoch-Sch?nlein purpura nephritis (HSPN).Methods:A total of 216 children with untreated HSPN, who were admitted in Beijing Children′s Hospital of Capital Medical University from January 2018 to December 2019, were recruited in this retrospective study. Two hundred and sixteen healthy children were selected as the healthy control group. We determined the levels of six biomarkers of kidney injury, including transferrin (TRF), immunoglobulin (IgG), microalbumin (mAlb), alpha-1 microglobulin (α1-MG), N-acetyl-β-D-glucosaminidase (NAG) in urine and cystatin C (CysC) in serum. The data from the two groups were analyzed, the diagnostic value of each biomarker was evaluated and a logistic regression model for the diagnosis of HSPN was established. In addition, 60 children with HSPN, who were admitted to our hospital from November 2021 to February 2022 and 60 healthy children, who underwent healthy check up in the same period were included to validate the diagnostic performance of the established logistic model. Receiver operating characteristic (ROC) curve was used to analyze the diagnostic value of each biomarker.Results:The urine levels of TRF, IgG, mAlb, α1-MG and NAG and the serum level of CysC were significantly higher in the HSPN group than those in healthy control group (all P<0.05). The area under the ROC curve (AUC) of TRF, IgG, mAlb, α1-MG, NAG and the serum levels of CysC was 0.749, 0.719, 0.810, 0.648, 0.828 and 0.790 (all P<0.05). Logistics regression analysis showed that IgG, mAlb and TRF were the three diagnostic determinants of HSPN ( OR=1.083, 1.105, 1.704,all P<0.001), and the AUC was 0.916 of the established logistic model based on these three biomarkers. The sensitivity was 87.4% and the specificity reached 96.2%. The logistic model was validated by independent cohorts, and the AUC was 0.973, the sensitivity was 95.0% and the specificity was 98.3%. Conclusions:The levels of urine TRF, IgG, mAlb, α1-MG, NAG and serum CysC were higher in children with HSPN. The established logistic regression model based on three biomarkers including IgG, mAlb and TRF in this study has satisfactory clinical value in diagnosing HSPN in children.
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Objective:To evaluate the efficacy of Jiedu-Huaban Decoction combined with montelukast sodium chewable tablets in the treatment of children with henoch schonlein purpura (HSP). Methods:A total of 80 children with HSP and blood heat syndrome who met the inclusion criteria, from January 2017 to December 2019, were randomly divided into two groups by random number table method, 40 in each group. The control group took montelukast sodium chewable tablets at night, and the study group took Jiedu-Huaban Decoction on the basis of the control group. Both groups were treated for 2 weeks. The disappearance time of gastrointestinal disease, skin purpura, kidney disease, joint swelling and pain were observed. The improvement score of skin purpura was evaluated before and after treatment. The serum levels of IL-6, IL-4, interferon-γ (IFN-γ) and TNF-α were detected by ELISA, and the levels of IgG, IgA and IgM. The T lymphocyte subsets (CD4 + and CD8 +) were measured by nephelometry, and the CD4 +/CD8 +values were calculated. The clinical efficacy was evaluated. Results:The total effective rate was 87.5% (35/40) in the study group and 67.5% (27/40) in the control group, with significant difference between the two groups ( χ2 =4.588, P=0.032). The disappearance time of gastrointestinal disease, skin purpura, kidney disease and joint swelling and pain in the study group were significantly earlier than those in the control group ( t=7.802, 12.167, 7.309, 9.365, all Ps<0.001). After treatment, the serum levels of IL-6, IL-4, IFN-γ and TNF-α in the study group were significantly lower than those in the control group ( t=9.319, 6.738, 8.221, 6.553, all Ps<0.001). The improvement score of skin purpura at 1 week after treatment (2.75 ± 0.69 vs. 3.92 ± 0.83, t=6.856) and 2 weeks after treatment (0.41 ± 0.15 vs. 1.55 ± 0.37, t=18.095) in the study group were significantly lower than those in the control group ( P<0.01). After treatment, the level of IgG, CD4 +, CD4 +/CD8 + in the study group were significantly higher than those in the control group ( t=5.160, 4.558, 3.442, all Ps<0.01), the level of IgA, IgM, CD8 + in the study group were significantly lower than those in the control group ( t=2.614, 6.712, 5.468, all Ps< 0.05). During the treatment, the incidence of adverse reactions in the control group was 17.5% (7/40), and that of the study group was 15.0% (6/40), wherer there was no statistical difference between the two groups ( χ2=0.092, P=0.762). Conclusion:Jiedu-Huaban Decoction combined with montelukast sodium chewable tablets can improve the clinical symptoms of children with HSP and blood heat syndrome, reduce the body inflammatory reaction, improve immunity, with good safety.
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Abstract Introduction: Henoch Schönlein purpura (HSP) is the most common type of vasculitis in childhood. HSP affects small blood vessels, and it rarely leads to serious complications such as bullous small vessel vasculitis, as it occurred in the case presented here. Case presentation: 5-year-old male who was brought to a primary healthcare center due to having arthralgia and purple skin lesions on his lower limbs. After the patient was diagnosed with HSP, he developed bullous lesions, so he was hospitalized and analgesic and topical management was started. During his hospital stay, the patient's renal function was monitored, and since he did not experience other complications, he was discharged. Conclusion: The available literature on HSP suggests that its cutaneous bullous manifestation rarely occurs in pediatric population and that, unlike normal HSP cases, it is not always associated with renal and/or gastrointestinal involvement. However, regardless of the dermatological severity of this type of vasculitis, the function of the gastrointestinal and renal systems must be always monitored in these patients.
Resumen Introducción. La púrpura Henoch-Schönlein (PHS) es la forma más común de vasculitis en la infancia; esta se da en pequeños vasos sanguíneos y no es frecuente que genere complicaciones graves como la vasculitis bullosa, tal como sucedió en el caso que se presenta a continuación. Presentación del caso. Paciente masculino de cinco años que fue traído a un centro de atención primaria con un cuadro clínico consistente en artralgias y aparición de lesiones purpúricas en miembros inferiores. Luego de ser diagnosticado con PHS, presentó lesiones bullosas, por lo que fue hospitalizado y se inició manejo analgésico y tópico; durante su estadía en el hospital se vigiló su función renal y, ya que no presentó otras complicaciones, se dio de alta. Conclusión. Las publicaciones disponibles sobre PHS sugieren que su presentación cutánea bullosa en pediatría no es frecuente y que no siempre se relaciona con un compromiso renal y/o gastrointestinal como la variante clásica; sin embargo, siempre debe vigilarse la función de estos sistemas sin importar la gravedad dérmica de esta vasculitis.
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Objective@#To explore the effect of Qishen-Xiaodian Decoction combined with laser acupoint irradiation on oxidative stress and renal function on children with recurrent henoch schonlein purpura (HSP).@*Methods@#A total of 120 children with recurrent HSP in the dermatology department clinic of Hospital of Xinle City from January 2015 to November 2017 were divided into two groups according to the random number table method, with 60 cases in each group. The control group took loratadine, Troxerutin and vitamin C orally, while the treatment group took Chinese medicine combined with laser acupoint irradiation based onthe control group. Drugs were taken orally for 4 weeks, laser acupoint irradiation treatment for 2 weeks. The level of glutathione peroxidase (GSH-Px) in plasma was detected by ELISA, the levels of MDA and SOD in plasma were detected by thiobarbituric acid colorimetry and hydroxylamine method, the level of IgG, micro albumin (Alb), β2-microglobulin (β2-MG) in urine were detected by radioimmunoassay, and the clinical effect was evaluated. The recurrence was recorded.@*Results@#After treatment, the plasma GSH-Px (90.45 ± 15.36 μmol/L vs. 81.62 ± 13.68 μmol/L, t=3.318), SOD (99.64 ± 18.66 IU/ml vs. 84.21 ± 16.73 IU/ml, t=4.769) in the treatment group were significantly higher than the control group (P<0.01); the MDA (5.58 ± 1.31 μmol/L vs. 4.37 ± 1.36 μmol/L, t=4.964) was significantly lower than the control group (P<0.01); the ratio of urine IgG (3.48 ± 0.95 mg/L vs. 6.56 ± 1.47 mg/L, t=13.630), Alb (7.80 ± 2.94 mg/L vs. 12.73 ± 4.26 mg/L, t=7.378), β2-MG (4.02 ± 1.61 mg/L vs. 6.95 ± 2.10 mg/L, t=8.577) were significantly lower than those of the control group (P<0.01). The total effective rate was 95.0% (57/60) in the treatment group and 80.0% (48/60) in the control group. There was statistically significant difference between two groups (χ2=6.171, P=0.013). Follow-up of 6 and 12 months, the recurrence rate of the treatment group were significantly lower than that of the control group (χ2 value were 4.931, 4.574, P<0.05).@*Conclusions@#Qishen-Xiaodian Decoction combined with laser acupoint irradiation can improve the oxidative stress of children with recurrent HSP, protect the renal function, improve the clinical efficacy and reduce the recurrence rate.
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Resumen La púrpura de Shönlein-Henoch es una vasculitis de la infancia caracterizada por una púrpura palpable no trombocitopénica y manifestaciones sistémicas, acompañadas de dolor abdominal, sangrado gastrointestinal, glomerulonefritis, artritis y artralgias. Está relacionada con patologías desencadenantes como las infecciones y las picaduras de insectos. Se presenta el caso de un niño de 4 años de edad, que presentaba cuadros alérgicos de rinitis y urticaria. Se le había diagnosticado una trombocitopenia inmune primaria de evolución crónica. A pesar del empleo de diferentes alternativas de tratamiento, recurrió con trombocitopenia muy severa, asociada a cuadro de púrpura y lesiones maculo-papulares y urticarianas. Los complementarios realizados, a excepción del conteo de plaquetas, estuvieron en rangos normales. La evolución fue satisfactoria en un periodo de tres semanas. Se presenta el caso porque es una forma atípica de esta enfermedad.
Abstract Shönlein-Henoch purpura is a common vasculatis in childhood, characterized by a non-trombocytopenic palpable purpura and systemic manifestations, accompanied by abdominal pain, gastro-intestinal bleeding, glomerulonephritis, arthritis and arthralgias. It is the most common of diseases due to disturbances in the vascular component in children. It is related to triggering pathologies as infections and insect bites. A case of a 4 year old boy is presented who presented allergic episodes of rhinitis and urticaria. He had been diagnosed with an chronic primary inmmune thrombocytopenia. In spite of the different treatment approaches, the patient had a severe thrombocytopenia, associated to purpura and macolu-papular lesions and uticaria. Lab tests, with the exception of platelet count, were within the normal limits. The progress was satisfactory in a three week period.
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Objective To determine the proportion of CD4+ CD25+ regulatory T (Treg) cells,mRNA expression of the forkhead box protein 3 (Foxp3) gene,and DNA methylation status of the Foxp3 promoter in peripheral CD4+ T cells from patients with Henoch-Sch(o)nlein purpura.Methods Totally,20 inpatients with Henoch-Sch(o)nlein purpura and 20 healthy controls were enrolled from Department of Dermatology,the Second Xiangya Hospital of Central South University between 2015 and 2016,and there were no significant differences in the gender and age between the two groups (both P ＞ 0.05).CD4+ T cells were isolated from the peripheral blood samples of these subjects.Real-time fluorescence-based quantitative PCR was performed to detect the mRNA expression of the Foxp3 gene,flow cytometry to determine the proportion of CD4 + CD25+ Treg cells,and sodium bisulfite sequencing PCR (BSP) to determine the DNA methylation status of the Foxp3 promoter.Statistical analysis was carried out with SPSS16.0 software by using two-sample t test for the comparison between the two groups,and linear correlation analysis for evaluating the correlations of the DNA methylation status of the Foxp3 promoter with clinical severity scores and the proportion of CD4+CD25+ Treg cells.Results Compared with the healthy control group,the Henoch-Sch(o)nlein purpura group showed significantly decreased mRNA expression of the Foxp3 gene in CD4+ T cells (0.380 ± 0.226 vs.1,t =9.503,P ＜ 0.01),proportion of CD4+CD25+ Treg cells (1.668％ ± 0.959％ vs.2.741％ ± 1.131％,t =2.552,P ＜ 0.05),but significantly increased DNA methylation status of the Foxp3 promoter (0.712 ± 0.164 vs.0.453 ± 0.147,t =3.610,P ＜ 0.01).In the Henoch-Sch(o)nlein purpura group,the DNA methylation status of the Foxp3 promoter was negatively correlated with the percentage of CD4+CD25+ Treg cells (r =-0.490,P ＜ 0.05),but positively correlated with the clinical severity scores (r =0.486,P ＜ 0.05).The DNA methylation level of the Foxp3 promoter was significantly higher in the patients with renal impairment than in those without renal impairment (P ＜0.05).Conclusion The patients with Henoch-Sch(o)nlein purpura showed increased DNA methylation status of the Foxp3 promoter in CD4+ T cells,decreased mRNA expression of the Foxp3 gene and proportion of CD4+CD25+ Treg cells,which may be related to the occurrence of Henoch-Sch(o)nlein purpura,and affect disease development and prognosis.
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Objective To study the relationships between pathological grade of Henoch-Schonlein purpura nephritis (HSPN) and levels of serum transforming growth factor-beta 1 (TGF-β1),monocyte chemoattractant protein 1 (MCP-1),interleukin (IL)-17 and prognosis in adults.Methods 98 HSPN patients treated in our hospital from June 2015 to December 2017 were selected as the study group,65 IgA nephritis patients were selected as the IgA nephritis group,and 60 healthy people who came to our hospital for physical examination during the same period were selected as the control group.The levels of TGF-β1,MCP-1 and IL-17 in serum of the three groups were detected,and the Cox regression analysis was used to analyze the risk factors affecting the patient's condition.Results The levels of serum TGF-β1,MCP-1 and IL-17 in the study group were significantly higher than those in IgA nephritis group and control group (P ＜ 0.05).The levels of serum TGF-β1,MCP-1 and IL-17 in IgA nephritis group were significantly higher than those in control group (P ＜ 0.05).There was no significant difference in age,sex,hemoglobin,albumin,urinary protein and renal phenotype among groups (P ＜ 0.05).Platelets of type Ⅲ were significantly lower than those of type Ⅱ (P ＜0.05);C-reactive protein (CRP) level of type Ⅳ,Ⅴ and Ⅵ was significantly higher than that of type Ⅱ (P ＜ 0.05).The degree of glomerulosclerosis in patients with type Ⅲ,Ⅳ,Ⅴ and Ⅵ was significantly higher than that in patients with type Ⅱ,and the degree of glomerulosclerosis in patients with type Ⅴ and Ⅵ was also significantly higher than that in patients with type Ⅲ (P ＜ 0.05).The formation of crescents in patients with type Ⅲ,Ⅳ,Ⅴ and Ⅵ was significantly higher than that in patients with type Ⅱ,and the formation of crescents in patients with type Ⅳ,Ⅴ and Ⅵ was also significantly higher than that in patients with type Ⅲ (P ＜ 0.05).The levels of serum TGF-β1,MCP-1 and IL-17 were the lowest in type Ⅱ patients and the highest in type Ⅴ and Ⅵ patients.The levels of TGF-β1,MCP-1 and IL-17 in type Ⅲ,Ⅳ,Ⅴ and Ⅵ were significantly higher than those in type Ⅱ (P ＜0.05),and the level of TGF-β1 in type Ⅳ,Ⅴ and Ⅵ was significantly higher than that in type Ⅲ (P ＜ 0.05);Serum IL-17 level of type Ⅴ and Ⅵ was significantly higher than that of type Ⅲ (P ＜ 0.05).Cox regression analysis showed that TGF-β1 and IL-17 were risk factors for pathological grading.Conclusions The higher the pathological grade of Henoch-Schonlein purpura nephritis in adults,the higher the levels of serum TGF-β1 and IL-17.TGF-β1 and IL-17 are the risk factors affecting the pathological grade of Henoch-Schonlein purpura nephritis.
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Objective To investigate the clinical characteristics of diffused capillary endothelial proliferative Henoch-Sch?nlein purpura nephritis (DEP-HSPN) children with or without crescents formation. Methods The clinical data of 110 DEP-HSPN children diagnosed by renal biopsy from February 2013 to June 2017 in Shangluo Maternal and Child Health Family Planning Service Center were retrospectively analyzed. Among them, 24 cases had no crescents formation (group A), and 86 cases had crescents formation (group B). The children of 2 groups were treated with standard regimen, and the clinical characteristics and prognosis between 2 groups were compared. Results The rates of Ⅴ type and gross hematuria in group B were significantly higher than those in group A: 59.3% (51/86) vs. 0 and 83.7% (72/86) vs. 29.2% (7/24), the levels of urine red blood cell count, 24 h urine protein and serum creatinine were significantly higher than those in group A: (112.4 ± 20.3)/HP vs. (45.2 ± 10.6)/HP, (2 471.6 ± 242.0) mg vs. (1 358.5 ± 109.3) mg and (44.9 ± 9.6) μmol/L vs. (32.3 ± 5.2) μmol/L, the level of serum albumin was significantly lower than that in group A: (22.8 ± 3.8) g/L vs. (35.1 ± 5.7) g/L, and there were statistical differences (P＜0.01 or ＜0.05). There were no statistical differences in rate of nephrogenous albuminuria, glomerular pathology type and immunoglobulin deposition condition between 2 groups (P＞0.05). There were 17 cases of complete remission and 7 cases of asymptomatic hematuria in group A, and 50 and 36 cases in group B, respectively. There was no significant difference between 2 groups (P > 0.05). Conclusions When DEP-HSPN is accompanied by crescent formation, gross hematuria, urine red blood cell count and the proportion of massive albuminuria can increase significantly. Combination therapy with immunosuppressive agents in acute stage and long- term sequential treatment could achieve good prognosis.
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Objective@#To observe the change and clinical significance of the peripheral blood T-lymphocytes CD45RA+ and CD45RO+ in children with Henoch-Schonlein purpura (HSP) under different clinical classification.@*Methods@#From October 2015 to July 2017, the clinical data of 80 children with HSP in the Affiliated Hospital of North Sichuan Medical College were retrospectively analyzed.According to the clinical classification, they were divided into three groups: skin involvement group (35 cases), abdominal type group (36 cases), and renal type group (9 cases). Another 80 healthy children were selected as the control group.The changes and clinical significance of peripheral blood T-lymphocytes CD45RA+ , CD45RO+ and CD45RA+ /CD45RO+ ratio were analyzed.@*Results@#Among the 80 children, 40 cases were male and 40 cases were female, with age of (7.2±2.3)years old.The CD45RA+ and CD45RO+ rates in the HSP group were (13.19±7.09)%, (12.07±3.46)%, respectively, which were significantly lower than those in the control group [(23.26±6.01)%, (21.74±3.46)%], the differences were statistically significant (t=9.69, 16.42, all P<0.05). The CD45RA+ ratio, CD45RA+ count and CD45RA+ /CD45RO+ ratio of the kidney type group were (8.02±3.63)%, (2.19±0.33)/μL, (-0.28±0.19), respectively, which were significantly lower than those of the skin group [(15.74±7.71)%, (2.55±0.33)/μL, (0.27±0.12)], the differences were statistically significant(t=3.085, 2.709, 4.013, all P<0.05). The ratio of CD45RA+ /CD45RO+ in the abdominal group was significantly lower than that in the skin group[(-0.07±0.27)vs.(0.27±0.12), t=2.989, P<0.05].@*Conclusion@#Peripheral blood T lymphocyte subsets CD45RA+ and CD45RO+ may play a role in the generation and development of immune function changes in children with HSP, and the decrease of CD45RA+ T cells may be related to the risk of HSP nephritis.
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Objective To explore the effect of Qushi-Huaban granule for the allergic purpura and the impact on CD4+CD25+T cells. Methods A total of 80 children with allergic purpura from March 2014 to January 2016 were randomly divided into two groups, the control group and observation group, 40 in each group. The control group was given routine medicine andmontelukast chewable tablets. The observation group was treated with Qushi-Huaban granule on the basis of conventional therapy. The clinical effect, purpura recession time, recurrence rate before and after treatment were observed and compared. The serum levels of IFN-γ (interferon-γ, IFN-γ) and IL-5 were detected by ELISA. The expression level of CD4+CD25+ Treg were observed by flow cytometry before and after treatment. Results The effective rate of the treatment group [92.5% (37/40) vs. 72.5%(29/40), χ2=9.270] was significantly higher than control group (P＜0.01). After treatment, the serum IFN-γ levels in the observation group and the control group were significantly higher than those before treatment (t=3.960, 4.175, P＜0.05). The serum IL-5 levels of two groups were significantly lower than those before treatment (t=8.061, 8.776, P＜0.01).There was no significant differencein the serum INF-γ and IL-5 leves after treatment between two groups (P＞0.05). The regression time of purpura in the observation group (5.2 ± 1.1 d vs. 10.2 ± 2.4 d, t=12.460) was significantly shorter than that in the control group ( P＜0.01). The recurrence rate of the observation group was [7.4% (5/37) vs. 10.3% (3/29)] which was significantly lower than that of the control group, but the difference was not statistically significant (χ2=0.153, P=0.696). The CD4+CD25+T cells (t=7.367, 6.957, P＜0.05) and CD3+CD4+CD25+ T cells (t values were 9.080, 8.885, P＜0.05) of two groupswere significantly higher than those beforetreatment, butthere was no significant difference between the 2 groups after treatment ( P＞0.05). Conclusions The Qushi-Huaban granule combined with montelukast sodium for Henoch Schonlein purpura showed efficacy, and significantly increase the moisture level of T cell subsets.
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Objective Comparison of clinical and pathological features of Henoch-Sch(o)nlein purpura nephritis with different proportions of crescent.Methods Patients with Henoch Schonlein nephritis diagnosed by renal biopsy in our hospital from 2012 to 2017 (proportions of crescent ＜ 50％)were enrolled and grouped according to the rate of crescent formation (A group:crescent formation rate ＜ 10％;B group:10％-24％;C group:＞ 24％-50％),their epidemiological,clinical manifestations,laboratory examination,clinicopathological features were compared.Results There was no significant difference between the three groups in sex,age and incidence of diabetes(P ＞ 0.05).The morbidity of hypertension in C group is higher than others (P ＜ 0.05).There were no different in the incidence of hematemesis,abdominal pain,joint pain in the three groups (P ＞0.05),while the incidence of edema and gross hematuria was significantly higher in group C than others.There were more patients had Nephritic syndrome (38.8％) in C group than the other groups (P ＜ 0.05).There was no significant difference in 24 h urinary protein,C-reactive protein (CRP),urine red blood cell,eosinophil granulocyte and immunoglobulin E (IgE) in the 3 groups.The levels of serum creatinine,blood urea nitrogen (BUN),and low density lipoprotein (LDL) increased significantly in group C,and the level of glomerular filtration rate (GFR) and albumin decreased significantly(P ＜ 0.05).The tubulointerstitial lesions,mesangial cell proliferation and deposition of C1q levels,C group was significantly higher than the other two groups (P ＜ 0.05).Conclusions In Henoch Sch(o)nlein purpura nephritis,with the increase of the proportion of the crescent body,the renal function and pathological changes of the kidney are gradually aggravated.Therefore,the crescent is important for the prognosis of Henoch Sch(o)nlein purpura nephritis.
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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+ CD24hiCD38hi) and naïve B cells (CD19+CD24intCD38int) 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.
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Objective To investigate the role of neutrophils and their IgA Fc receptor CD89 in the occurrence of Henoch-Sch（o）nlein purpura (HSP),to evaluate their effects on vascular endothelial cell apoptosis,and to explore their mechanisms.Methods Peripheral blood neutrophils were isolated from 30 children with acute HSP and 9 age-matched healthy controls separately.After isolation of serum IgA by Jacalin affinity chromatography,IgA was purified by polypropylene dextran gel chromatography.Real-time fluorescence-based quantitative PCR (qPCR) and Western blot analysis were performed to determine the mRNA and protein expression of CD89 on neutrophils respectively,and flow cytometry was conducted to measure the expression of neutrophil activation marker CD11b.Human umbilical vein endothelial cells (HUVEC) were co-cultured with neutrophils isolated from patients with HSP (HSP group) and healthy controls (healthy control group) separately.Moreover,the HSP group were divided into 3 subgroups to be treated with serum IgA isolated from the HSP patients (HSP IgA group),monomeric IgA (mIgA group) and phosphate-buffered saline (blank control group) respectively.Then,flow cytometry was conducted to detect apoptosis of co-cultured HUVEC,and enzyme-linked immunosorbent assay (ELISA)to measure levels of interleukin-8 (IL-8) and tumor necrosis factor-alpha (TNF-α) in the supernatant of co-cultured cells.Results There was no significant difference in the mRNA expression of CD89 on neutrophils between the patients with HSP and healthy controls (P =0.98),but the protein expression of CD89 was significantly lower in the patients with HSP than in the healthy controls (0.60 ± 0.16 vs.0.83 ± 0.24,P =0.03).The expression of CD1 1b on neutrophils was significantly higher in the patients with HSP than in the healthy controls (1 880.25 ± 388.29 vs.1 109.25 ± 364.25,P ＜ 0.01).The apoptosis rate of co-cultured HUVEC was also significantly higher in the HSP group than in the healthy control group (37.44％ ± 5.49％ vs.17.14％ ± 4.45％,P ＜ 0.01).In addition,the H SP IgA group showed significantly higher apoptosis rate of cocultured HUVEC and levels of IL-8 and TNF-cα in the supematant compared with the mIgA group (all P ＜0.01) and blank control group (P ＜ 0.01,=0.01,=0.02,respectively).Conclusions Peripheral blood neutrophils in patients with HSP are activated,which can induce the apoptosis of vascular endothelial cells.HSP IgA can promote the neutrophil-mediated apoptosis of vascular endothelial cells and secretion of IL-8 and TNF-α,while mIgA may show a certain inhibitory effects.
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Objective@#To explore age-based clinical and immune parameters in Henoch-Schönlein purpura (HSP) to determine clinically useful markers reflecting disease characteristic.@*Methods@#A cohort of 502 patients with HSP were enrolled into this retrospective study to evaluate their clinical and immune data.@*Results@#Majority HSP cases occurred at age ≤14 years and showed significant immune imbalances of ESR, CD3+ cells, CD4+ cells, CD3-CD16+CD56+ cells, CD4+/CD8+ cells, IgG, IgA, IgM, IgE, complements C3/C4 and ASO in the acute phase. Compared to patients aged >14 years, symptoms of joint were more frequent at disease onset in patients aged ≤14 years (20.8% vs 7.6%, χ2=13.547, P<0.001) , and involvement of digestive tract and joint were also more frequent (57.4% vs 33.8%, χ2=24.106, P<0.001; 55.9% vs 32.5%, χ2=23.768, P<0.001, respectively) , but not for involvement of kidney (21.4% vs 51.3%, χ2=42.440, P<0.001) . The patients aged ≤14 years had distinct immune state, reductions of CD3+ cells, CD4+ cells and IgG were more frequent than patients aged >14 years, also increase of ASO (33.1% vs 20.0%, χ2=6.656, P=0.010) , but not increase of IgA (2.6% vs 39.4%, χ2=15.582, P<0.001) . In addition, reduction of IgG and increase of IgE were positively associated with digestive tract involvement (P<0.001, P=0.001, respectively) , reduction of CD3+CD4+ cells and normal IgM were positively associated with joint involvement (P=0.004, P=0.003, respectively) , increase of CD3+CD8+ cells and normal CD3+ cells were positively associated with kidney involvement (P=0.032, P=0.014, respectively) .@*Conclusion@#HSP showed significant immune imbalance in the acute phase, patients between aged ≤14 and >14 years had distinct clinical and immune characteristic, and abnormal immune parameters were significantly associated with organ involvements.
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Objective To explore the relationship between serum 25-hydroxy vitamin D [25(OH)D] concentrations and the risk of Henoch-Schonlein purpura (HSP).Methods A case control study was designed.Serum 25 (OH)D concentrations were measured by radioimmunoassay in 214 participants,including 53 H SP patients and 161 status-matched healthy controls.Information concerning demographic data,genetic,background,and environmental exposures was collected using questionnaire.The study participants were divided into four groups according to quartile range of 25(OH)D concentration and logistic regression modeling was used to evaluate the relation with HSP risk by estimating odds ratios(OR)and 95％confidence intervals(CI).Results The HSP group had a significantly lower concentration of 25(OH)D than the control group (the median in the HSP group was 11.4 ng/mL;controls:15.36 ng/mL,P＜0.05).When the first interval was set as the reference level,the OR (95 ％ CI) of the second,third,and fourth intervals were:0.468(0.341-0.771),0.442(0.302-0.627),0.339 (0.199-0.501).After adjusting the analysis for the presence of pathogenic related confounding fact OR,the OR(95％CI)of the second,third,and fourth intervals were:0.459(0.333-0.741),0.408(0.317-0.611),0.387 (0.221-0.517).The 25 (OH) D level was inversely correlated with the risk of HSP(P＜ 0.05).Conclusion The risk of HSP was decreased with the increase of serum 25 (OH) D concentration,25 (OH) D may be a protection factor in the pathogenesis of HSP.
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Abstract: The Henoch-Schönlein purpura is the vasculitis associated with deposits of immunoglobulin A in small vessels. Its association with cytoplasmic antineutrophil cytoplasmic antibodies is possible, but rare. This vasculitis is uncommon in adults and the main clinic manifestations are purpuric lesions in lower limbs with gastrointestinal symptoms and renal involvement. The present work describes a rare case of Henoch-Schönlein purpura in an adult with cytoplasmic antineutrophil cytoplasmic antibodies.
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Humans , Male , Young Adult , IgA Vasculitis/diagnosis , Antibodies, Antineutrophil Cytoplasmic/analysis , Fluorescent Antibody Technique, DirectABSTRACT
Objective To explore the relationship of neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) with gastrointestinal bleeding in adult patients with Henoch-Sch(o)nlein purpura (HSP).Methods Laboratory data including routine blood test results,C-reactive protein (CRP) levels and erythrocyte sedimentation rate (ESR) were collected from 115 adult patients with HSP and 100 healthy controls,and retrospectively analyzed.NLR and PLR were calculated and compared.Logistic regression analysis was used to determine variables associated with gastrointestinal bleeding in adult patients with HSP,and the receiver operating characteristic (ROC) curve was drawn to estimate the predictive efficacy of these variables.Results NLR and PLR were both significantly higher in adult patients with HSP than in healthy controls (both P ＜ 0.05),and NLR,PLR and CRP levels were all significantly higher in adult HSP patients with gastrointestinal bleeding than in those without (all P ＜ 0.05).Logistic regression analysis showed that NLR was an independent risk factor for gastrointestinal bleeding in adult HSP patients (P ＜ 0.05).The ROC curve revealed that the optimal cut-off point of NLR for predicting gastrointestinal bleeding was 3.24 in adult patients with HSP,with the sensitivity and specificity being 85.1％ and 71.3％ respectively.Conclusions Both NLR and PLR evidently increase in adult patients with HSP,especially in those with gastrointestinal bleeding.NLR is a potential predictor for gastrointestinal bleeding in adult patients with HSP.