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Henoch-Sch(o)nlein purpura nephritis (HSPN) is one of the most common types of secondary glomerulonephritis in children.Patients with severe HSPN are at distinctively higher risk of poor prognosis.Currently,no unified therapeutic method is adopted to treat severe HSPN,and disagreements exist in prognostic factors.This paper reviews the treatment measures including steroid,multiple immunosuppressive agents,rituximab and plasmapheresis for severe HSPN.Relevant factors for long-term prognosis of HSPN are discussed,which provide reference for clinical treatment and management.
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Objective To analyze the relationship of clinical manifestations and pathological characteristics of Henoch-Sch(o)nlein purpura nephritis combined with hyperuricemia in children.Methods A retrospective study was conducted in 50 children with Henoch-Sch(o)nlein purpura nephritis who hospitalized at Department of Nephrology,Affiliated Children's Hospital,Capital Institute of Pediatrics from January 2014 to May 2018.The differences between the hyperuricemia group(19 cases)and the normal uric acid group (31 cases),were compared in age,sex,blood pressure,serum albumin,24-hour urinary protein,serum creatinine,triglyceride,cholesterol,high density lipoprotein,low density lipoprotein,serum uric acid,estimated glomerular filtration rate,and renal pathological characteristics,and the short-term prognosis was analyzed.Results (1) The average urinary protein in the hyperuricemia group and the normal uric acid group was (91.67 ±90.37) mg/(kg · d) and (64.62 ±43.28) mg/(kg · d),respectively and the difference was statistically significant between the both groups(t =2.04,P =0.047);and the morbidity with massive proteinuria in hyperuricemia group and normal uric acid group was 18/19 cases (94.7％)and 17/31 cases (54.8％),respectively and the difference was statistically significant between the both groups (x2 =8.930,P =0.003).(2)In all cases,there were 4 cases of glomerular pathological grade Ⅱ,43 cases of grade Ⅲ and 3 cases of grade Ⅳ.The pathological grading of hyperuricemia group and normal uric acid group was mainly grade Ⅲ,including 16/19 cases (84.2％) in hyperuricemia group and 27/31 cases (87.1％) in normal uric acid group,4 cases of grade Ⅱ in normal uric acid group and 3 cases of grade Ⅳ in hyperuricemia group,the pathological grade of hyperuricemia group was relatively severe (x2 =7.358,P =0.025).There was no significant difference about the degree of global sclerosis and mesangial proliferation between hyperuricemia group and normal uric acid group(x2 =2.426,P =0.119,x2 =0.043,P =0.836,respectively);7/19 cases (36.8％) had severe foot process lesions in hyperuricemia group,which was significantly higher than that in normal uric acid group [4/31 cases(12.9％)] (x2 =3.934,P =0.047).In hyperuricemia group,tubulointerstitial lesions were found in 9/19 cases (47.4％) of (+) grade and 10/19 cases (52.6％) of (+ +) grade,and 12/31 cases (38.7％) had normal tubulointerstitium in normal uric acid group,(+) and (+ +) grade lesions were also less than those in the hyperuricemia group (x2 =10.694,P =0.005).The mean scores of tubular atrophy and interstitial fibrosis were significantly higher in hyperuricemia group than that in normal uric acid group(t =2.36,P =0.001).(3) The interval from renal biopsy to final visit was 10.0 months and 10.5 monthsin hyperuricemia group and normal uric acid group respectively (P =0.85).In hyperuricemia group,complete remission was found in 5/19 cases (26.3％),slight abnormality in 10/19 cases (52.6％),severe abnormality in 4/19 cases (21.1％).Howe-ver,in normal uric acid group,complete remission was found in 19/31 cases (61.3 ％),10/31 cases (32.3 ％) of slight abnormalities and 2/31 cases (6.5％)of severe abnormalities.The non-remission cases in the hyperuricemia group were significantly higher than those in the normal uric acid group (x2 =7.878,P =0.042).Conclusions Urinary protein was higher in children with Henoch-Sch(o)nlein purpura nephritis complicated with hyperuricemia,the pathological of renal tubulointerstitium and glomerulus and the foot process change are more serious than those of patients with normal uric acid.Therefore,hyperuricemia may be used as a risk factor for poor prognosis.
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Objective To explore the activation of renin-angiotensin system (RAS),efficiency and safety of Captopril,and the predictor of therapeutic activity for Henoch-Sch(o)nlein purpura nephritis (HSPN) characterized by mild proteinuria.Methods A total of 71 children who were hospitalized in Children's Hospital Affiliated to Capital Institute of Pediatrics from July 2014 to January 2017 were involved,with the diagnosis of HSPN and the characteristic of mild proteinuria.The cases were divided into 2 groups,one as Captopril group,the other as case control group.The patients were followed up for 6 months.Forty healthy children were assigned as healthy control group.Blood pressure,urinary protein excretion,levels of urinary angiotensinogen (AGT) and transforming growth factor β1 (TGF-β1),and the side effects of Captopril were surveyed.The therapeutic effects of these groups were analyzed by Kaplan-Meier survival curve.Results (1) Clinical characteristics:in the 71 cases,43 cases were male,28 cases were female,aged from 3 years to 14 years and 7 months.A total of 32 cases (45.1％) had manifested with isolated proteinuria,39 cases (54.9％) were with hematuria and proteinuria.The volume of 24 hours' urinary protein was 4.2-23.5 mg/(m2 · h) [median 9.6(7,12) mg/(m2 · h)] at the beginning.(2) The level of urinary AGT:the levels of urinary AGT in the children with HSPN were significantly higher than those of the healthy control group(Z =-3.010,P =0.003).(3) Curative effect:there was no significant difference in age,disease staging,mean arterial pressure(MAP),levels of urinary of proteinuria and estimated glomerular filtration rate (eGFR) between the patients with or without Captopril.The proteinuria was relieved in 88.57％ cases of Captopril group(35 cases),and the proportion was 80.55％ in the case control group(36 cases),and there was no significant difference between the 2 groups.The levels of proteinuria were decreased significantly in the children of Captopfil group 2 months after the enrollment,and there was a statistical significance (Z =2.010,P =0.044).But in the patients of each group,the levels of urinary protein excretion (Z =-2.127,P=0.030;Z=-2.639,P=0.010),TGF-β1(Z=-2.126,P=0.030;Z=-2.058,P=0.040) at theonset were significantly higher in the children with persistent proteinuria compared to those with remission of proteinuria completely,and there was a statistical significance.(4)Side effect:among 35 cases with therapy of Captopril,4 cases (11.42％) were verified to have adverse reaction (hypotension,dry cough and abnormal renal function),with mild symptom.Conclusion The overall prognosis of children of HSPN presenting as mild proteinuria are not improved completely by Captopril.The occurrence of adverse effects for Captopril is seldom and less severe.The level of urinary protein excretion,TGF-31 and AGT at the onset have some relevance with the prognosis of the patients of HSPN.
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Objective To analyze the clinical feature and prognosis of Henoch-Sch(o)nlein purpura nephritis (HSPN) with mild proteinuria in children.Methods The data of 78 HSPN children with mild proteinuria who were hospitalized in Children's Hospital Affiliated to Capital Institute of Pediatrics from September 2013 to September 2016 were retrospectively analyzed.All the cases were followed up.The clinical manifestation,histologic characteristics,treatment and outcome were analyzed,and the prognosis was compared between groups of different levels of proteinuria.Results (1) In the 78 cases,45 cases were male and 33 cases were female.The attack age range was from 3 years and 8 months to 14 years and 3 months,and the renal involvement occurred from 1 day to 60 months in courses of Hen(o)chSch(o)nlein purpura.(2)Twenty-seven cases were manifested with isolated proteinuria,and 51 cases were with hematuria and proteinuria.All the cases had normal kidney function while 2 cases had hypertension.Renal biopsy was performed in 9 cases,in which 6 cases were Ⅲ b,the other 3 cases were Ⅱ b.(3) Twelve cases were treated with regimen of immunosuppression and/or glucocorticoid.By the end of follow-up,the urinary analysis showed 46 cases (50.9％) had completely recovered,29 cases (37.2％)just had microscopic hematuria but no proteinuria,and 3 cases (3.8％) had obvious proteinuria.The renal involvement of 7 cases (9％) had been recurrent during the follow-up.(4) The time of recovery of proteinuria in the group with urinary protein ≤ 15 mg/kg(median:1 month) was less than that of the group with urinary protein ＞ 15 mg/kg(median:3 months),and the difference was significant (Z =-4.12,P =0.001),and 6 cases were found recurrent in the former group (10.9％) but 1 case(4.3％) in the latter group,and the difference was not significant (x2 =0.24,P =0.624).Conclusions The clinical characteristics of children with HSPN with mild proteinuria are not serious,and most of the cases had a favorable prognosis.However,some cases have protracted and recurrent courses which have serious pathological grade.So,urine analysis,long term follow-up,timely kidney biopsy and appropriate treatment are significant for HSPN with mild proteinuria.
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Henoch-Sch(o)nlein purpura (HSP) is the common form of systemic vasculitis in children,which characterized by the deposition of immunoglobulin E within the small vessels.The long-term prognosis of HSP depends on the severity of renal involvement.Renal involvement in HSP affects 10％-100％ patients and the clinical manifestations vary from microscopic hematuria to nephritic/nephritic syndrome even renal dysfunction.Early diagnosis,early treatment and long-term follow-up affect the prognosis of HSP patients.As for the treatment of HSP nephritis (HSPN),there have been many reports dealing with the immunosuppressive agents.Now glucocorticoid is the first-line treatment for HSPN,and other immunosuppressive agents offer more choices to treat HSPN especially for severe HSPN.But,the use of immunosuppressive agents still needs large sample multicenter randomized controlled trials and evidence-based study.
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Objective To analyze the clinical feature and prognosis of Henoch-Sch(o)nlein purpura nephritis (HSPN) with mild proteinuria in children.Methods The data of 78 HSPN children with mild proteinuria who were hospitalized in Children's Hospital Affiliated to Capital Institute of Pediatrics from September 2013 to September 2016 were retrospectively analyzed.All the cases were followed up.The clinical manifestation,histologic characteristics,treatment and outcome were analyzed,and the prognosis was compared between groups of different levels of proteinuria.Results (1) In the 78 cases,45 cases were male and 33 cases were female.The attack age range was from 3 years and 8 months to 14 years and 3 months,and the renal involvement occurred from 1 day to 60 months in courses of Hen(o)chSch(o)nlein purpura.(2)Twenty-seven cases were manifested with isolated proteinuria,and 51 cases were with hematuria and proteinuria.All the cases had normal kidney function while 2 cases had hypertension.Renal biopsy was performed in 9 cases,in which 6 cases were Ⅲ b,the other 3 cases were Ⅱ b.(3) Twelve cases were treated with regimen of immunosuppression and/or glucocorticoid.By the end of follow-up,the urinary analysis showed 46 cases (50.9％) had completely recovered,29 cases (37.2％)just had microscopic hematuria but no proteinuria,and 3 cases (3.8％) had obvious proteinuria.The renal involvement of 7 cases (9％) had been recurrent during the follow-up.(4) The time of recovery of proteinuria in the group with urinary protein ≤ 15 mg/kg(median:1 month) was less than that of the group with urinary protein ＞ 15 mg/kg(median:3 months),and the difference was significant (Z =-4.12,P =0.001),and 6 cases were found recurrent in the former group (10.9％) but 1 case(4.3％) in the latter group,and the difference was not significant (x2 =0.24,P =0.624).Conclusions The clinical characteristics of children with HSPN with mild proteinuria are not serious,and most of the cases had a favorable prognosis.However,some cases have protracted and recurrent courses which have serious pathological grade.So,urine analysis,long term follow-up,timely kidney biopsy and appropriate treatment are significant for HSPN with mild proteinuria.
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Objective To evaluate the efficacy and safety of tacrolimus (TAC) for treatment of Henoch-SchSnlein purpura nephritis in children.Methods Forty children with Henoch-SchSnlein purpura nephritis (pathological grade Ⅲ-Ⅵ) in Children's Hospital Affiliated to Soochow University from January 2013 to June 2016 were selected and divided into TAC group (n =19) and cyclophosphamide (CTX) group (n =21).The children in TAC group were given TAC orally;the children in CTX group were given CTX pulse therapy intravenously.The 24 h urine protein,urine red blood cell count,serum albumin,serum creatinine and blood urea nitrogen of children before treatment and after 6 months treatment were observed and compared between the two groups.The treatment effects and adverse reactions of patients were observed and compared between the two groups.Results There was no statistic difference in the 24 h urine protein,urine red blood cells count,serum albumin,blood creatinine and blood urea nitrogen levels of children between the two groups before treatment (P ＞ 0.05).The 24 h urinary protein and urine red blood cells count of children after 6 months of treatment in the two groups were significantly lower than those before treatment (P ＜ 0.05);there was no statistic difference in the serum albumin,serum creatinine and blood urea nitrogen levels of children in the two groups before treatment and after 6 months of treatment (P ＞ 0.05).Mter 6 months of treatment,the 24 h urine protein and urine red blood cells count of children in TAC group were significantly lower than those in the CTX group (P ＜ 0.05);there was no statistic difference in the serum albumin,serum creatinine and blood urea nitrogen levels of children between the two groups (P ＞ 0.05).After 6 months treatment,the effective rate of children in the TAC group was significantly higher than that in the CTX group (x2 =4.607,P ＜ 0.05).The incidence of adverse reactions of children in the TAC group was significantly lower than that in the CTX group (x2 =4.043,P ＜ 0.05).Conclusion TAC is effective in treatment of Henoch-Sch(o)nlein purpura nephritis in children.It is easy to take,and has less adverse reactions.
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Henoch-Sch(o)nlein purpura(HSP) is one of the most common vasculitides in children with many manifestations including skin purpura,arthritis,abdominal pain and renal involvement.The renal involvement(Henoch-Sch(o)nlein purpura nephritis,HSPN) is the principal cause of morbidity and major risk factor for prognosis in HSP.The paper reviewed the updated treatment strategy for HSPN.Accordingly,steroids,cyclophosphamide,mycophenolate mofetil,mizoribine,cyclosporine,triptolide,angiotensin converting enzyme inhibitors (ACEI) / angiotensin receptor blockers(ARB),plasma apheresis and tonsillectomy are possible indicator for the further therapy of HSPN.Furthermore,prospective randomized controlled treatment studies on children with severe HSPN would be needed.
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Objective To study the feature of clinical and pathological type of Henoch-Sch(o)nlein purpura nephritis (HSPN) in children.Methods Clinical and pathological data of HSPN in 279 children were collected and retrospectively analyzed.Results Clinical manifestation of HSPN in 279 children with haematuria and proteinuria (107 cases,38.4％) was the most common type,followed by nephritic syndrome type (69 cases,24.7％),isolated proteinuria type (40 cases,14.3％),isolated hematuria type (29 cases,10.4 ％),acute glomerulonephritis type (21 cases,9.3 ％),rapidly progressive glomerulonephritis type (8 cases,2.9％),chronic glomerulonephritis type (5 cases,1.8％).According to the International Study of Kidney Disease in Children,the majority renal pathological type of HSPN were grade Ⅱ (133 cases,47.7％)and grade Ⅲ (109 cases,39.1％).The pathological changes of hematuria and proteinuria type were mainly grade Ⅱ (61 cases,57.0％) and grade Ⅲ (35 cases,32.7％),and the pathological change of nephritic syndrome type was grade Ⅲ (41 cases,59.4％).All of renal pathological changes,38.7％ (108/279) had codeposition of immunoglobulins A and M,30.8％ (86/279) had co-deposition of immunoglobulins A,G and M.The pathological change of nephritic syndrome type was more serious (x2 =35.989,P ＜ 0.05).Immune complex deposition was not correlated with renal pathologic classification (P ＞ 0.05).Conclusion The HSPN patients mainly show the type of hematuria and proteinuria and the type of nephritic syndrome.The majority renal pathological type of HSPN are grade Ⅱ and grade Ⅲ.In children with HSPN,the severity of the clinic symptoms is not completely consistent with the pathological changes.The pathological changes of nephritic syndrome type are more serious.To improve theprognosis of HSPN,we should make the treatment planning according to the clinical types and pathologic classification.
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Objective To explore the expressions and clinical significance of Toll-like receptor (TLR) 3 and TLR4 in peripheral blood mononuclear cells in Henoch-Schsnlein purpura nephritis (HSPN) children.Methods According to their 24-hour urinary albumin and whether children with HSP had renal damage or not,105 cases were divided into group A,B and C.Group A were children only with HSP but without renal damage,while group B were children only with HSPN not proteinuria and group C were children with both HSPN and proteinuria.Thirty healthy children were in healthy control group(group N).Flow cytometry (FCM) and real-time PCR detected the mRNA and protein expressions of TLR3 and TLR4 in peripheral blood mononuclear cells.Results 1.The mRNA and protein expressions of TLR4 in peripheral blood mononuclear cells were significantly higher in group A,B,C than those in group N (F =37.33,24.01,all P ＜ 0.05).The mRNA and protein expressions of TLR4 in group C were much higher than those in group A and B (all P ＜ 0.05).Meanwhile,there was no significant difference between group A and B(all P ＞0.05).2.Moreover,there was a positive relationship between protein expression of TLR4 and 24-hour urinary albumin in group C(r =0.69,P ＜ 0.01).3.Expression of TLR3 was of no significant difference in all groups(F =0.86,1.78,all P ＞ 0.05).4.The expression of TLR4 mRNA had a positive correlation with protein expression of TLR4(r =0.61,P ＜ 0.0 1).Conclusions Expressions of TLR4 in peripheral blood mononuclear cells significantly increased and had a positive correlation with urinary protein excretion from HSPN in children.This implied that aberrant activation of TLR4might be relevant to the development of HSPN.
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Objective:To analyze the podocalyxin (PCX) expression in the kidney and the number of urinary podocytes in different pathological grades of Henoch-Sch(o)nlein purpura nephritis (HSPN),and to determine whether the number of urinary podocytes reflects the renal damage in HSPN.Methods:Fifty-six children diagnosed with HSPN in our hospital were enrolled in the study and classified into 4 groups by renal pathology:grade Ⅱ (Ⅱa+Ⅱb) (n=10),grade Ⅲ (Ⅲa+Ⅲb) (n=21),grade Ⅳ (n=16),and grade Ⅴ (n=9).Four kidney autopsy specimens without histomorphologic lesions and 8 urine samples from healthy children served as controls.With immunofluorescence assay,the PCX expression in 4 normal renal tissues and in the renal tissues of the 56 HSPN children was detected and quantitatively analyzed.Positive rate and the number of urinary podocytes were detected in the 8 healthy children and 56 HSPN children.Results:In the renal tissues of the normal control group and grade Ⅱ (Ⅱa+Ⅱb) HSPN group,the PCX expression was complete.The percentage of the PCX positive area out of the total glomerular area in the renal tissues of 2 groups had no significant difference (P＞0.05).In the renal tissues of grade Ⅲ (Ⅲa+Ⅲb),Ⅳ,and Ⅴ HSPN groups,the PCX expression showed various degrees of loss,decreasing in turn from grade Ⅱ (Ⅱa+Ⅱb),Ⅲ (Ⅲa+Ⅲb),Ⅳ to Ⅴ,with significant differences between each group (P＜0.01).For HSPN with grade Ⅲ (Ⅲa+Ⅲb) or higher,positive PCX expression was found in the urine,suggesting the presence of enough podocytes in the urine.The percentage of fluorescence positive area out of the total glomerular area of PCX in the renal tissues was negatively correlated with the total number of urinary podocytes (r=-0.637,P＜0.01).Conclusion:Podocyte injury plays a certain role in the pathological progression of HSPN.The urinary detection ofpodocytes can reflect the degrees of pathological damage in HSPN.
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Henoch-Sch(o)nlein purpura nephritis (HSPN)is primarily treated with medication. Especially, glucocorticoid and immunosuppressants can control the clinical features and improve its prognosis effectively.Overall prognosis of HSPN is relatively good, but patients with severe clinical presentations and renal insufficiency will be worse.Better interventions of HSPN can be provided through extensive analysis of the treatment and prognosis.