Background:Henoch-Schönlein purpura(HSP)is one of the most common vasculitides in children.It is manifested by skin purpura,arthritis,abdominal pain,renal involvement,etc.Typically,HSP is considered to be self-li...Background:Henoch-Schönlein purpura(HSP)is one of the most common vasculitides in children.It is manifested by skin purpura,arthritis,abdominal pain,renal involvement,etc.Typically,HSP is considered to be self-limiting,although renal involvement(HSP purpura nephritis,HSPN)is the principal cause of morbidity from this disease.For this reason,it is important to clarify the mechanism of onset and clinical manifestations of HSPN and to ascertain the most appropriate treatment for HSPN.In this article,we review the updated pathophysiology and treatment strategies for HSPN.Data 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 review.Results: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 severity.Conclusions:HSPN is a severe disease of childhood.To better understand this disease,detailed investigations into the pathogenesis of HSPN and prospective randomized controlled treatment studies on children with severe HSPN are needed.展开更多
Background: The aim of this retrospective study was to defi ne the clinical manifestations, pathological features and prognosis of children with membranoproliferative-like Henoch-Schönlein purpura nephritis (HSPN...Background: The aim of this retrospective study was to defi ne the clinical manifestations, pathological features and prognosis of children with membranoproliferative-like Henoch-Schönlein purpura nephritis (HSPN), representing International Study of Kidney Disease in Children (ISKDC) grade VI. Methods: Among 245 patients with HSPN treated in our hospital between 2008 and 2010, nine patients (3.7%) were diagnosed with HSPN of ISKDC grade VI (males=5, females=4, age: 9.5±2.03 years, mean±SD). The clinical features, laboratory and pathologicalfi ndings, treatment and outcome of the 9 patients were retrospectively analyzed. Results: Of the 9 patients, 7 (78%) presented with hematuria and nephrotic syndrome, and were treated with steroids (oral prednisone or intravenous methylprednisolone pulse therapy) and immunosuppressants (oral tripterygium glycosides or intravenous cyclophosphamide pulse therapy). One (11%) patient had hematuria and nephrotic range proteinuria (>50 mg/kg per 24 hours) and was treated with oral prednisone and tripterygium glycosides. Another (11%) patient presented with hematuria and moderate proteinuria (25-50 mg/kg per 24 hours) and was treated with oral tripterygium glycoside only. Histopathological examination showed diffuse glomerular mesangial and endocapillary proliferation, mesangial interposition, double-contour formation, podocyte hypertrophy, shedding, and cytoplasmic absorption droplets. The percentages of glomeruli with small cellular crescents varied from 4%-25% in 6 of 9 patients. Follow-up for 2 to 4 years showed excellent recovery in all patients. Conclusions: The main clinical feature of ISKDC grade VI HSPN in children is a nephrotic syndrome with hematuria. The excellent prognosis of the disease was probably related to early diagnosis and treatment with steroids and/or immunosuppressants, and mild degree of glomerulosclerosis and tubulointerstitial damage.展开更多
目的探讨吗替麦考酚酯联合百令胶囊对小儿过敏性紫癜性肾炎(HSPN)的疗效。方法选择2021年1月至2022年1月在郑州大学第一附属医院住院治疗的82例HSPN患儿为研究对象,并随机将其分为A组(单纯吗替麦考酚酯治疗,41例)、B组(吗替麦考酚酯结...目的探讨吗替麦考酚酯联合百令胶囊对小儿过敏性紫癜性肾炎(HSPN)的疗效。方法选择2021年1月至2022年1月在郑州大学第一附属医院住院治疗的82例HSPN患儿为研究对象,并随机将其分为A组(单纯吗替麦考酚酯治疗,41例)、B组(吗替麦考酚酯结合百令胶囊治疗,41例),观察其效果、肾功能、炎症及免疫情况。结果B组总有效率(87.80%)高于A组(53.41%)(P<0.05)。治疗后,B组患儿血肌酐(Scr),尿素氮(BUN)、24 h尿蛋白(24 h Upro)、单核细胞趋化因子(MCP-1)、炎症因子水平均低于A组(P<0.05);免疫力高于A组(P<0.05)。结论吗替麦考酚酯联合百令胶囊治疗较单一应用吗替麦考酚酯治疗小儿HSPN的效果佳,有利于患儿肾功能恢复,还可减轻机体炎症,提高免疫力,值得临床应用。展开更多
目的探讨吗替麦考酚酯联合糖皮质激素对过敏性紫癜性肾炎患儿肾功能指标血肌酐(Scr)、血尿酸(BUA)、尿素氮(BUN)、24 h尿蛋白(PRO)定量及细胞、体液免疫指标的影响,为该疾病的治疗提供依据。方法以随机数字表法将2020年1月至2022年12月...目的探讨吗替麦考酚酯联合糖皮质激素对过敏性紫癜性肾炎患儿肾功能指标血肌酐(Scr)、血尿酸(BUA)、尿素氮(BUN)、24 h尿蛋白(PRO)定量及细胞、体液免疫指标的影响,为该疾病的治疗提供依据。方法以随机数字表法将2020年1月至2022年12月于河北省儿童医院就诊的42例过敏性紫癜性肾炎患儿分为对照组(21例)和观察组(21例)。所有患儿均进行对症治疗,对照组患儿进行泼尼松+环磷酰胺治疗,观察组患儿进行泼尼松+吗替麦考酚酯治疗,两组患儿均在治疗6个月后评估疗效。比较两组患儿治疗6个月后的临床疗效,治疗前及治疗6个月后肾功能、免疫功能指标,以及治疗期间不良反应发生情况。结果观察组患儿临床总有效率高于对照组;与治疗前比,治疗6个月后两组Scr、BUA、BUN、24 h PRO定量、免疫球蛋白A(IgA)、免疫球蛋白G(IgG)及CD8+百分比均降低,观察组更低;CD^(3+)、CD^(4+)百分比及CD^(4+)/CD^(8+)比值升高,观察组更高(均P<0.05);两组不良反应总发生率比较,差异均无统计学意义(均P>0.05)。结论过敏性紫癜性肾炎患儿以吗替麦考酚酯联合糖皮质激素进行治疗能够提高临床疗效,改善肾功能,抑制机体体液免疫反应,且安全性良好。展开更多
基金supported by grants from the National Natural Science Foundation of China(Grant.81270792,81470939 and 81170664)State"1025"Science and Technology Support Project(2012BAI03B02)the Research Fund for the Doctoral Program of Higher Education of China(20120101110018)
文摘Background:Henoch-Schönlein purpura(HSP)is one of the most common vasculitides in children.It is manifested by skin purpura,arthritis,abdominal pain,renal involvement,etc.Typically,HSP is considered to be self-limiting,although renal involvement(HSP purpura nephritis,HSPN)is the principal cause of morbidity from this disease.For this reason,it is important to clarify the mechanism of onset and clinical manifestations of HSPN and to ascertain the most appropriate treatment for HSPN.In this article,we review the updated pathophysiology and treatment strategies for HSPN.Data 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 review.Results: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 severity.Conclusions:HSPN is a severe disease of childhood.To better understand this disease,detailed investigations into the pathogenesis of HSPN and prospective randomized controlled treatment studies on children with severe HSPN are needed.
基金supported by grants from the 12th Five-year Plan of National Science and Technology Support(No.2013BAI02B07)Talents Support Fund for Science and Technology Innovation in Colleges and Universities of Hennan Province in 2011(No.2012HASTIT019).
文摘Background: The aim of this retrospective study was to defi ne the clinical manifestations, pathological features and prognosis of children with membranoproliferative-like Henoch-Schönlein purpura nephritis (HSPN), representing International Study of Kidney Disease in Children (ISKDC) grade VI. Methods: Among 245 patients with HSPN treated in our hospital between 2008 and 2010, nine patients (3.7%) were diagnosed with HSPN of ISKDC grade VI (males=5, females=4, age: 9.5±2.03 years, mean±SD). The clinical features, laboratory and pathologicalfi ndings, treatment and outcome of the 9 patients were retrospectively analyzed. Results: Of the 9 patients, 7 (78%) presented with hematuria and nephrotic syndrome, and were treated with steroids (oral prednisone or intravenous methylprednisolone pulse therapy) and immunosuppressants (oral tripterygium glycosides or intravenous cyclophosphamide pulse therapy). One (11%) patient had hematuria and nephrotic range proteinuria (>50 mg/kg per 24 hours) and was treated with oral prednisone and tripterygium glycosides. Another (11%) patient presented with hematuria and moderate proteinuria (25-50 mg/kg per 24 hours) and was treated with oral tripterygium glycoside only. Histopathological examination showed diffuse glomerular mesangial and endocapillary proliferation, mesangial interposition, double-contour formation, podocyte hypertrophy, shedding, and cytoplasmic absorption droplets. The percentages of glomeruli with small cellular crescents varied from 4%-25% in 6 of 9 patients. Follow-up for 2 to 4 years showed excellent recovery in all patients. Conclusions: The main clinical feature of ISKDC grade VI HSPN in children is a nephrotic syndrome with hematuria. The excellent prognosis of the disease was probably related to early diagnosis and treatment with steroids and/or immunosuppressants, and mild degree of glomerulosclerosis and tubulointerstitial damage.
文摘目的探讨吗替麦考酚酯联合百令胶囊对小儿过敏性紫癜性肾炎(HSPN)的疗效。方法选择2021年1月至2022年1月在郑州大学第一附属医院住院治疗的82例HSPN患儿为研究对象,并随机将其分为A组(单纯吗替麦考酚酯治疗,41例)、B组(吗替麦考酚酯结合百令胶囊治疗,41例),观察其效果、肾功能、炎症及免疫情况。结果B组总有效率(87.80%)高于A组(53.41%)(P<0.05)。治疗后,B组患儿血肌酐(Scr),尿素氮(BUN)、24 h尿蛋白(24 h Upro)、单核细胞趋化因子(MCP-1)、炎症因子水平均低于A组(P<0.05);免疫力高于A组(P<0.05)。结论吗替麦考酚酯联合百令胶囊治疗较单一应用吗替麦考酚酯治疗小儿HSPN的效果佳,有利于患儿肾功能恢复,还可减轻机体炎症,提高免疫力,值得临床应用。
文摘目的探讨吗替麦考酚酯联合糖皮质激素对过敏性紫癜性肾炎患儿肾功能指标血肌酐(Scr)、血尿酸(BUA)、尿素氮(BUN)、24 h尿蛋白(PRO)定量及细胞、体液免疫指标的影响,为该疾病的治疗提供依据。方法以随机数字表法将2020年1月至2022年12月于河北省儿童医院就诊的42例过敏性紫癜性肾炎患儿分为对照组(21例)和观察组(21例)。所有患儿均进行对症治疗,对照组患儿进行泼尼松+环磷酰胺治疗,观察组患儿进行泼尼松+吗替麦考酚酯治疗,两组患儿均在治疗6个月后评估疗效。比较两组患儿治疗6个月后的临床疗效,治疗前及治疗6个月后肾功能、免疫功能指标,以及治疗期间不良反应发生情况。结果观察组患儿临床总有效率高于对照组;与治疗前比,治疗6个月后两组Scr、BUA、BUN、24 h PRO定量、免疫球蛋白A(IgA)、免疫球蛋白G(IgG)及CD8+百分比均降低,观察组更低;CD^(3+)、CD^(4+)百分比及CD^(4+)/CD^(8+)比值升高,观察组更高(均P<0.05);两组不良反应总发生率比较,差异均无统计学意义(均P>0.05)。结论过敏性紫癜性肾炎患儿以吗替麦考酚酯联合糖皮质激素进行治疗能够提高临床疗效,改善肾功能,抑制机体体液免疫反应,且安全性良好。
