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henoch-schönlein purpura nephritis in children:incidence,pathogenesis and management

World Journal of Pediatrics 2015年第1期11卷 29-34页

作者： Jun-Yi Chen Jian-Hua Mao Department of Nephrology Children's HospitalZhejiang University School of MedicineHangzhou 310003China

Background:henoch-schönlein purpura(HSP)is one of the most common vasculitides in *** is manifested by skin purpura,arthritis,abdominal pain,renal involvement,***,HSP is considered to be self-limiting,although renal involvement(HSP purpura nephritis,HSPN)is the principal cause of morbidity from this *** this reason,it is important to clarify the mechanism of onset and clinical manifestations of HSPN and to ascertain the most appropriate treatment for *** this article,we review the updated pathophysiology and treatment strategies for *** sources:We searched databases including PubMed,Elsevier and Wanfang for the folowing key words:henoch-schönlein purpura,nephritis,mechanism and treatment,and we selected those publications written in English that we judged to be relevant to the topic of this ***:Based on the data present in the literature,we reviewed the following topics:1)the possible pathogenesis of HSPN:several studies suggest that immunoglobulin A immune complexes deposit in the mesangium and induce renal injury;2)multiple-drug treatment for HSPN:although there have been few evidence-based treatment strategies for HSPN,several studies have suggested that immunosuppressive drugs and multiple drug combination therapy were effective in ameliorating proteinuria and histological ***:HSPN is a severe disease of *** better understand this disease,detailed investigations into the pathogenesis of HSPN and prospective randomized controlled treatment studies on children with severe HSPN are needed.

关键词： henoch-schönlein purpura immunosuppressive drug nephritis pathogenesis treatment

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Heat shock protein 70-2 and tumor necrosis factor-α gene polymorphisms in Chinese children with henoch- schönlein purpura

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世界儿科杂志:英文版 2016年第1期12卷 49-54页

作者： Gui-Xia Ding Chen-Hu Wang Ruo-Chen Che Wan-Zhen Guan Yang-Gang Yuan Min Su Ai-Hua Zhang Song-Ming Huang Department of Nephrology Nanjing Children's HospitalNanjing Medical UniversityNanjingChina Institute of Pediatrics Nanjing Medical UniversityNanjingChina

Background:henoch-schönlein purpura(HSP)or IgAassociated vasculitis is related to immune *** of the heat shock protein 70-2 gene(HSP70-2)and the tumor necrosis factor-αgene(TNF-α)are known to be associated with immune *** purpose of this study was to investigate the likely association of HSP70-2(+1267A/G)and TNF-α(+308A/G)gene polymorphisms with HSP in ***:The polymerase chain reaction restriction fragment length polymorphism method was used to detect the HSP70-2 and TNF-αpolymorphisms in 205 cases of children with HSP and 53 controls;and the association of these polymorphisms with HSP and HSP nephritis(HSPN)was ***:The G/G genotypic frequencies at the+1267A/G position of HSP70-2 in the HSP group(22.9%)were signifi cantly higher than those in the healthy control group(9.4%)(χ^(2)=4.764,P<0.05).The frequencies of the A/A,A/G and G/G genotypes of HSP70-2 in patients in the nephritis-free group and the HSPN group showed no statistically significant *** A/A genotype frequency at the+308G/A position of TNF-αin the HSP group was 8.3%,which was higher than that in the control group(χ^(2)=6.447,P<0.05).The A allele frequency of TNF-αin the HSP group was higher than that in the control group,with a statistically significant difference(χ^(2)=7.241,P<0.05).Conclusions:The HSP70-2(+1267A/G)and TNF-α(+308G/A)gene polymorphisms were associated with HSP in *** G/G homozygosity of HSP70-2 and the A/A homozygosity of TNF-αmay be genetic predisposing factors for HSP.

关键词： gene polymorphism heat shock protein 70-2 henoch-schönlein purpura henoch-schönlein purpura nephritis tumor necrosis factor-α

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Risk factors for intussusception in children with henoch-schönlein purpura:A case-control study

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World Journal of Clinical Cases 2021年第22期9卷 6244-6253页

作者： Qian Zhao Yan Yang Song-Wei He Xin-Tai Wang Chang Liu Traditional Chinese Medicine Department National Children's Medical CenterBeijing Children's Hospital Affiliated to Capital Medical UniversityBeijing 100045China

BACKGROUND The etiology of henoch-schönlein purpura(HSP)with intussusception remains *** To investigate the risk factors for intussusception in children with HSP and gastrointestinal(GI)*** Sixty children with HSP and concomitant intussusception admitted to the Beijing Children’s Hospital of Capital Medical University between January 2006 and December 2018 were enrolled in this *** hundred pediatric patients with HSP and GI involvement but without intussusception,admitted to the same hospital during the same period,were randomly selected as a control *** baseline clinical characteristics of all patients,including sex,age of onset,duration of disease,clinical manifestations,laboratory test results,and treatments provided,were *** and multiple logistic regression analyses were performed to identify possible risk *** The 60 children in the intussusception group comprised 27 girls(45%)and 33 boys(55%)and the 100 children in the non-intussusception group comprised 62 girls(62%)and 38 boys(38%).The median age of all patients were 6 years and 5 *** and multiple regression analyses revealed age at onset,not receiving glucocorticoid therapy within 72 h of emergence of GI symptoms,hematochezia,and D-dimer levels as independent risk factors for intussusception in children with HSP(P<0.05).CONCLUSION The four independent risk factors for intussusception in pediatric HSP with GI involvement would be a reference for early prevention and treatment of this potentially fatal disease.

关键词： henoch-schönlein purpura Intussusception Gastrointestinal Risk factors Children Logistic regression analysis

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Three-Month-Induction Therapy with Prednisone and Mycophenolate Followed by Maintenance Therapy with Mycophenolate Alone for 2 Years: An Effective and Safe Autoimmune Treatment for Triggering Factors Adults with Acute Non-Crescentic Nephritis Associated with henoch-schönlein purpura

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Open Journal of Nephrology 2022年第1期12卷 154-161页

作者： Kamel El-Reshaid Shaikha Al-Bader John Madda Department of Medicine Faculty of Medicine Kuwait University Kuwait City Kuwait Department of Medicine Nephrology Unit Amiri Hospital Ministry of Health Kuwait City Kuwait Department of Histopathology Amiri Hospital Kuwait City Kuwait

Background: henoch-schönlein purpura (HSP) is an acute systemic disorder characterized by IgA associated vasculitis. The available data indicate an inherited predisposition to disease with triggering autoimmune phenomena. Hence, we evaluated prospectively the role of a new autoimmune regimen in treatment of its severe nephrotic/nephritic flares associated with non-crescentic nephritis in adult patients. Patients and methods: The regimen consisted of an initial induction phase of 3-month Prednisone and Mycophenolate followed by a maintenance phase of Mycophenolate alone for 2 years. Results: They were satisfactory with complete remission in 5 of 7 patients and partial in 2. Creatinine clearance was normalized in patients with complete remission and remained stable in the partially-responsive ones. Conclusion: Our study has shown the short- and long-term safety and efficacy of such autoimmune regimen directed towards the autoimmune triggering factors in severe forms of non-crescentic HSP.

关键词： Acute henoch-schönlein purpura Treatment Nephrotic Syndrome Nephritis Mycophenolate Mofetil

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