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Objective:To analyze the clinicopathologic features and prognosis of children with Henoch-Sch?nlein purpura nephritis (HSPN).Methods:The clinicopathological data of children with HSPN who were followed up for more than 5 years and underwent renal biopsy in Jinling Hospital affiliated to Medical School of Nanjing University from January 2001 to June 2015 were retrospectively analyzed. The follow-up endpoint event was defined as estimated glomerular filtration rate (eGFR)<90 ml·min -1·(1.73 m 2) -1. Participants were divided into two groups according to whether the children had reached the primary endpoint event or not. Cox proportional hazards model was used to analyze the influencing factors of renal poor prognosis in children with HSPN. Kaplan-Meier survival curve method was used for survival analysis, and log-rank test was used to compare the difference of renal cumulative survival rate between segmental sclerosis/adhesion (S1) group and non-segmental sclerosis/adhesion (S0) group. Receiver operating characteristic curve (ROC curve) and area under the curve ( AUC) were used to evaluate the diagnostic value. Results:A total of 130 children with HSPN were enrolled in the study. The median onset age was 11.7(8.6, 13.3) years old, of whom 71 cases were males (54.6%). At a median follow-up time of 100.0(75.8, 119.0) months, 12 cases (9.23%) with HSPN reached the primary endpoint event. Compared with the non-endpoint event group, the endpoint event group had higher proportion of hypertension, higher levels of 24-hour urinary protein, serum cholesterol, serum uric acid, and serum creatinine, and lower levels of serum albumin (all P<0.05). There was no statistical difference in treatment between the two groups (all P>0.05). In terms of pathological features, compared with the non-endpoint event group, the endpoint event group had higher proportion of mesangial hyperplasia (M1), S1, tubular atrophy/interstitial fibrosis (T1/T2) and Glomerulus-Bowman's capsule adhesion (all P<0.05). Multivariate Cox regression model showed that S1 was significantly correlated with renal poor prognosis ( HR=7.739, 95% CI 1.422-42.114, P=0.018). As was revealed in a Kaplan-Meier plot, renal cumulative survival rate in the S1 group was significantly lower than that in the S0 group (log-rank χ2=17.069, P<0.001). The ROC curve showed S1 accurately predicted the outcome ( AUC=0.710, 95% CI 0.549-0.872) with specificity of 0.667(95% CI 0.349-0.901) and specificity of 0.754(95% CI 0.667-0.829). Conclusions:S1 is an independent risk factor affecting renal poor prognosis and has a diagnostic value.
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IgA vasculitis is a common autoimmune disease mediated by IgA in childhood, which can involve many systems.Henoch-Sch?nlein purpura nephritis(HSPN)is one of the main complications.Both HSPN and IgA nephropathy(IgAN)are common glomerulonephritis in children, but the former is the most common secondary glomerulonephritis and the latter is one of the most persistent diseases in primary glomerulonephritis.There are differences in clinical phenotype and prognosis.This article reviews the relevant literature, and summarizes the similarities and differences in the pathogenesis of HSPN and IgAN, so as to better understand the two diseases.
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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 compare the efficacy and safety of leflunomide(LEF)to mycophenolate mofetil(MMF)and cyclophosphamide(CTX)in the treatment of Henoch-Sch?nlein purpura nephritis(HSPN)in children with nephrotic proteinuria.Methods:Thirty-nine children who were diagnosed as HSPN with nephrotic proteinuria were randomly divided into three groups: LEF group, MMF group and CTX group.Each group had 13 children.Proteinuria, hematuria, adverse effect and cost were followed up at 1, 3, 6 and 9 months of medication.Results:Proteinuria and hematuria were significantly decreased in each group at 1, 3, 6 and 9 months of medication.Compared to CTX group, proteinuria and hematuria in LEF group and MMF group were lower at 9 months.It was probably associated with one child whose proteinuria did not completely return to normal at 9 months and another child who suffered from a relapse of proteinuria and hematuria at 6.5 months in CTX group.The results suggested that the efficacy of LEF and MMF was slightly better than CTX.During the observation period, all children were well tolerated and no serious adverse reactions occurred.One case showed a slight increase of alanine aminotransferase at 1 month, and returned to normal range at 3 months without any medication in MMF group.The cost of LEF group was(8 231±665)RMB and CTX group was(11 523±469)RMB which was significantly lower than(19 953±386)RMB in MMF group.Conclusion:LEF is as effective as MMF and CTX in the treatment of HSPN with nephrotic proteinuria in children.The adverse reactions of LEF are mild, and the cost is cheaper.LEF is worth popularizing in clinical practice.
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Objective To establish the pathological grades of Henoch-Sch?nlein purpura nephriti(s HSPN) in children with diagnostic prediction models by stepwise Fisher discriminant in children. Methods Based on the in-vestigation of 28 clinical indicators from 144 cases with HSPN came from Children′s Hospital of Chongqing Medical University,the sensitive indicators were found and stepwise Fisher discriminant model was established and its accuracy in predicting the pathological classification of HSPN was tested. Results There were 5 laboratory indicators and clini-cal manifestations with different pathological grades of HSPN. In children with pathological gradeⅡ,ⅢandⅣ,5 indi-cators were screened(P<0. 05)and stepwise Fisher discriminant models were established. And the correct rate of comprehensive diagnosis was(61. 371 ± 8. 740)% in 100 random sampling diagnostic simulations;in children with pathological gradeⅢa and Ⅲb,5 indicators were also screened(P<0. 05)and stepwise Fisher discriminant models were established. And the correct rate of comprehensive diagnosis was(68. 015 ± 5. 736)% in 100 random sampling diagnostic simulations. Conclusions The stepwise Fisher discriminant models established in this research have a better diagnostic accuracy in forecasting for pathological grade of HSPN,and have a certain guiding value on early treatment and prognosis evaluation of children with newly diagnosed HSPN.
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Objective To explore the significance of glomerular fibrinogen (Fib) deposition in Henoch-Sch?nlein purpura nephritis (HSPN). Methods The clinical and pathological data of 82 children with HSPN diagnosed by renal biopsy were retrospectively analyzed. The clinical and pathological characteristics of each group were compared according to whether there were glomerular Fib deposition and deposition intensity in the kidney. Results Glomerular Fib deposition was observed in 63 cases (76.83%) in 82 cases, including Fib+23 cases (28.05%), Fib++37 cases (45.12%) and Fib+++3 cases (3.66%), and no deposition had been found in renal tubules. The levels of high sensitivity C reactive protein (hs-CRP), D-Dimer (DD), CD19+CD23+lymphocyte subsets, and urinary albumin to creatinine ratio (UMA/Cr) were significantly different among non-deposition group, mild deposition group and severe deposition group (P<0.01). The levels of hs-CRP and CD19+CD23+in severe deposition groups were significantly higher than those in the mild and non-deposition groups (P<0.05). The level of D-D in the severe and mild deposition group was significantly higher than that in the non-deposition group (P<0.05). The UMA/Cr in the severe deposition group was significantly higher than that in the non-deposition group (P<0.05). Glomerular Fib deposition is positively correlated with IgA deposition (r=0.64, P<0.001). The proportion of glomerular IgG deposition among three groups was significantly different (P<0.05), and the proportion of IgG deposition in the severe group was the highest. Conclusions When children had glomerular Fib deposition, especially in the case of severe Fib deposition and immune dysfunction, inflammatory response and hypercoagulability are more serious and renal function damage may be more serious.
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Henoch-Sch?nlein purpura ( HSP ) is a well-known systemic vasculitis disease mediated by IgA1 immune complex in children, and the pathogenesis remains unclear. Henoch-Sch?nlein purpura nephritis ( HSPN) is the most serious complication when the kidneys are damaged. It is also the key to determine the prog-nosis of the disease. Due to a combination of genetic,environmental,and infectious factors,IgA1 is abnormally glycosylated during the immune response. Galactose-deficient immunoglobulin A1(Gd-IgA1)is easy to self-ag-gregate and recognized by the antibody to form an immune complex deposited in the glomerular mesangial, which lead to kidney damage. This article reviews the progress of a series of pathogenic mechanisms of IgA1 glycosylation abnormality in HSPN.
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Group A streptococcus ( GAS) is the most common pathogens of human streptococcal infec-tion. It is considered that GAS infection is one of the important factors leading to Henoch-Sch?nlein purpura (HSP). It can promote the occurrence of Henoch-Sch?nlein purpura nephritis(HSPN),and aggravate HSPN. Hemolysin,protease and superantigen involved in the pathogenesis of GAS. Research progress of the relationship between streptococcal infection and HSP and HSPN are summarized as follows.
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Scholars have focused on the relationship between Henoch-Sch?nlein purpura nephritis and IgA nephropathy in children, when they gradually recognizes the similarities and differences between Henoch-Sch?nlein purpura nephritis and IgA nephropathy in their incentives, genetic factors, renal immunopathology, therapy and so on,with aberrantly glycosylated IgA1 involved in the pathogenesis of Henoch-Sch?nlein purpura nephritis and IgA nephropathy. This paper will review their differences and similarities,along with their relation, according to the research progress.
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Objectives To analysis clinical pathology of organ speciifc IgA vasculitis (IgA nephropathy) and systemic IgA vasculitis (allergic purpura) of purpura nephritis in children. Methods Clinical and pathological data of hospitalized pediatric patients of IgA nephropathy and purpura nephritis were retrospectively analyzed from June 1993 to November 2014. Results There were 405 patients of IgA nephropathy (256 males and 149 females). The ratio of male to female was 1.7:1. The average age was 10.2±2.8 years. The nephrotic syndrome (31.6%) was the most common clinical type, followed by hematuria and proteinuria (27.9%). There were 548 patients of purpura nephritis, 329 males and 219 females. The ratio of male to female was 1.5:1. The average age was 10.2±3.1 years. The hematuria and proteinuria (61.6%) was the most common clinical type, followed by nephrotic syndrome (21.4%). None of the IgA nephropathy progressed to systemic vasculitis (allergic purpura). Conclusions The causes, onset ages and clinical manifestations of IgA nephropathy and allergic purpura may be consistent or overlap, but none of IgA nephropathy (organ speciifcity IgA vasculitis) progressed to allergic purpura (systemic IgA vasculi-tis). IgA nephropathy might have more renal immune disorder mechanisms involved in its pathogenesis.
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Objective To investigate the clinical and pathological features of Henoch-Sch?nlein purpura nephritis (HSPN) in children. Methods The clinical and pathological data of 242 children with HSPN admitted from January 2009 to January 2014 were retrospectively analyzed. Results The most common clinical type was proteinuria and hematuria (135 cases, 55.79%). The mainly pathological types were typeⅡandⅢ. The distribution of pathological types was statistically signiifcant between different clinical types (P=0.000). The patients with normal urine test had the lowest pathological grade. The patients with isolated hematuria and proteinuria mainly had pathological typeⅡ. The patients with hematuria and proteinuria mainly had pathological typeⅢa. The patients with nephrotic syndrome mainly had pathological typeⅢb. The length of the courses at renal biopsy was not statistically signiifcant among different clinical types (P>0.05) and pathological types (P>0.05). The deposition of immune complex in kidney was not statistically signiifcant among different pathological types (P>0.05). The levels of IgA, C3, and platelet count were not statistically signiifcant among different clinical and pathological types (P>0.05). Conclusion The clin-ical classiifcation is related to the pathological grade in children with HSPN.
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Objective To analyze the relationship between clinical and pathological features in children with Henoche-Sch?nelin purpura nephritis (HSPN). Methods Clinical and pathological data of 89 children with HSPN from April 2009 to April 2013 in our hospital were retrospectively analyzed. Results A majority of patients (94.83%) suffered nephritis within two month after purpura. Clinically, hematuria and proteinuria (41.6%), nephrotic syndrome (27.0%), and isolated protein-uria (21.3%) are the most common type in children with HSPN. The majority of pathological changes of these three types HSPN are gradeⅢ, which accounts for 67.6%, 70.8%, and 73.7%, respectively. The clinical manifestations are closely associated with pathologic classiifcation (H=26.88, P=0.000). IgA plus IgM deposit was the most common type (40.45%) in histological and immunopathologic changes.There was no statistical difference between the pathologic classification and immunopathologic types. Gastrointestinal bleeding was associated with pathologic classiifcation. Conclusions The pathological changes of HPSN patients are mainly of gradesⅡandⅢ, and the clinical manifestations, gastrointestinal bleeding in particular, are closely associated with pathological classiifcation.
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Objective Few reports are seen about the effects of cooling blood and removing stasis on nephrin and podocin . This study was to evaluate the therapy of cooling blood and removing stasis for Henoch -Sch?nlein purpura nephritis ( HSPN) and its ac-tion mechanisms by observing the effects of Danshao Granular Ⅲ on hematuresis , proteinuria and the expressions of nephridial nephrin and podocin in HSPN rats . Methods Twenty-one SD male rats were e-qually randomized to a blank control , an HSPN model , and a Dan-shao group.At 13 weeks after modeling , the animals in the model group were treated intragastrically with distilled water , while those in the Danshao group with Danshao Granular Ⅲtwice daily for 4 weeks. Then, the urinary red blood cell ( RBC) count was examined , the 24 h urinary protein quantity determined , the glomerular mesangial changes observed under the light microscope , the protein expres-sions and distributions of nephrin and podocin detected by indirect immunofluorescence , and their mRNA expressions determined by re-al-time PCR. Results The urinary RBC count and 24 h urine protein quantity were significantly higher in the HSPN model than in the blank control group (26.5/HP vs 0.3/HP and [2.214 ±1.090]g/24 h vs [0.624 ±0.354]g/24 h, both P0.05).The distributions of nephrin and podocin were improved after Danshao treatment .The mRNA expressions of nephrin and podocin were markedly higher in the Danshao group than in the HSPN models (0.530 ±0.089 vs 0.117 ±0.021 and 0.490 ±0.160 vs 0.033 ±0.025, P<0.05). Conclusion Danshao Granular Ⅲ, with its main action mechanisms of cooling blood and removing stasis , can effectively reduce urinary RBC count and urinary protein quantity and improve the symptoms of HSPN in rats .
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Henoch Sch?nlein purpura( HSP) is a vasculitis of primary systemic vascular lesions of aller-gic diseases in children. Henoch Schonlein purpura nephritis( HSPN) is the most common allergic purpura sec-ondary renal disease,hematuria and(or)proteinuria of allergic in purpura the course(the most six months) can make diagnosis. The clinical manifestations varyies in severity,and the incidence has increased year by year,the incidence may be related to infections, allergies, immune abnormalities, immune damage and so on. So raising awareness of children nephritis,early diagnosis,early treatment has a great impact on the prognosis of children.
