ABSTRACT
Objective:To investigate the clinicopathological features, treatment and short-term prognosis of diffuse endocapillary proliferative Henoch-Schonlein purpura nephritis (DEP-HSPN) in children.Methods:Clinicopathological data of children with DEP-HSPN diagnosed by renal biopsy in the First Affiliated Hospital of Zhengzhou University from January 2012 to December 2019 were retrospectively analyzed.Children with HSPN with segmental endocapillary proliferation (non DEP-HSPN) and matched with the gender, age and pathological grade at the ratio of 1∶2 in the same period were recruited as controls.Results:(1) A total of 42 cases of DEP-HSPN were pathologically confirmed, accounting for 5.9% of the 712 children with HSPN during the same period.Thirty-nine newly treated cases were included, with the mean age of (8.9±3.2) years old, and the gender ratio was 1.79∶1.00.There were 21 cases of nephrotic syndrome, 14 cases of hematuria and albuminuria, 2 cases of acute glomerulonephritis, 1 case of rapid progressive nephritis and 1 case of isolated proteinuria.Pathological findings were accompanied by diffuse prolife-ration of mesangial and endocapillary.There were 13, 22 and 4 cases with pathological gradeⅡb, Ⅲb and Ⅳb, respectively.(2) Compared with non DEP-HSPN subjects, DEP-HSPN patients had a shorter course from renal symptoms to renal biopsy, and a higher incidence of nephrotic albuminuria, hypoalbuminemia, hypocomplementemia, hypertension and anemia.The main clinical type was nephrotic syndrome.The levels of D-dimer, 24-hour urinary protein (24 h UP) and urea nitrogen were significantly higher in DEP-HSPN group ( Z=-2.416, -2.595, -2.019, all P<0.05), while the red blood cells, hemoglobin, serum albumin, C 3 and glomerular filtration rate (eGFR) were significantly lower ( t=-2.499, -3.746, 2.836, -3.410, 3.236, all P<0.05). Besides, the glomerular C 3 deposition was higher than those in non DEP-HSPN subjects ( Z=-1.977, P<0.05). (3)The urinary protein remission rate in DEP-HSPN group was significantly reduced at 1 month follow-up [37.0%(10/27 cases) vs.62.5%(40/64 cases), P<0.05]. There was no significant difference between the 2 groups at 3 months, and the urinary protein remission was relieved at 6 months in both groups.There was no significant difference in hematuria remission between the 2 groups at the end of follow-up. Conclusions:Clinical manifestation of DEP-HSPN is severe, which is easy to be complicated with hypertension, anemia, hypocomplementemia C 3 and so on, and the hypercoagulable state is obvious.The degree of glomerular complement C 3 deposition was high in DEP-HSPN group.Urinary protein can be relieved slowly within 1 month after active treatment, but can be relieved at 6 months.
ABSTRACT
Objective:To observe the clinical efficacy of modified Zhibo Dihuangwan on henoch-schonlein purpura nephritis with deficiency of liver and kidney yin in children (HSPN) and its effect on immune inflammatory response and hypercoagulable state. Method:Totally 120 patients were randomly divided into observation group (60 cases) and control group (60 cases) by random number table. Patients in two group was orally given prednisolone acetate tablets, 1.5-2 mg·kg-1·d-1, 2 times. Four weeks later, the drug was taken orally every other day, and the dosage decreased gradually after 4 weeks. Besides, patients in control group was intravenously dripped with cyclophosphamide, 8-12 mg·kg-1·d-1, for 2 days, and stopped for 2 weeks before another treatment course. The treatment lasted for 6 months. In the control group,Dabuyin Wan was taken orally,3 g/time,3 times/d.Patients in observation group was also added with modified Zhibo Dihuang Wan, 1 doe/day. The treatment lasted for 6 months. Urine routine was tested once a month, and disappearance time and rate of hematuria and albuminuria were recorded. The 24 h urine protein quantification, levels of microalbuminuria (mAlb) and urinary β2-microglobulin (β2-MG) were assessed before and after treatment. Furthermore, deficiency of liver and kidney Yin was scored, and levels of T lymphocyte subsets (CD3+, CD4+, CD8+, CD4+/CD8+), fibrinogen (FIB), D-dimer (D-D), fibrin degradation products (FDP), interleukin-2 (IL-2), interferon-γ (IFN-γ), interleukin-4 (IL-4), interleukin-10 (IL-10) were detected. Result:The clinical efficacy in observation group was superior to that in control group (Z=2.078，P<0.05). Disappearance times of hematuria and albuminuria of children in observation group were shorter than those in control group (P<0.01). The disappearance rate of proteinuria in observation group was 90.48%（38/42）, which was higher than 69.77%（30/43） in control group (χ2=5.694，P<0.05). The 24 h urinary protein quantity, mAlb and levels of β2-MG, FIB, D-D and FDP in observation group were lower than those in control group (P<0.01). The levels of CD3+, CD4+, IL-2 and IFN-γ and the ratio of CD4+/CD8+ in observation group were higher than those in control group (P<0.05), while the CD8+, IL-4 and IL-10 were lower than those in control group (P<0.05). The efficacy in observation group was better than that in control group (Z=2.106，P<0.05). Conclusion:In addition to conventional western medicine therapy, modified Zhibo Dihuang Wan have an effect on HSPN with deficiency of liver and kidney Yin in children by promoting the disappearance of albuminuria and hematuria, shortening the course of disease, improving T lymphocyte subpopulation, reducing inflammatory reaction and correcting hypercoagulable state of blood, with better clinical efficacy and syndrome effect of traditional Chinese medicine.
ABSTRACT
Objective • To evaluate the changes of urinary angiotensinogen (UAGT), fibroblast-specific protein-1 (FSP-1) and thrombin in the children with Henoch-Schonlein purpura nephritis (HSPN). Methods • Fourteen children with HSPN (HSPN group), 28 children with Henoch-Schonlein purpura (HSP) but without renal injury (HSP group) and 23 children with normal urinalysis (control group) were included in the study. Ten HSPN children before treatment (untreated group), 9 HSPN children after glucocorticoid (GC) pulse therapy (GC group) and 8 HSPN children after GC and cyclophosphamide (CTX) double pulse therapy (GC+CTX group) were also selected in the study. Clinical information and fresh morning urine samples were collected from all the children. UAGT, FSP-1 and thrombin in urine were measured by kits. Urine creatinine (Ucr) was also measured for correction. Results • UAGT/ Ucr and FSP-1 in HSPN group were significantly higher than those in HSP group and control group (P0.05), but thrombin levels in HSPN group and HSP group were both significantly higher than that in control group (P<0.05). UAGT/Ucr in HSPN untreated group had no significant difference, compared with GC group, while it was significantly higher than that in GC+CTX group (P=0.000). FSP-1 in untreated group was significantly higher than that in GC group, but had no significant difference, compared with GC+CTX group. There was no significant difference in thrombin among the 3 HSPN groups. Conclusion • UAGT/Ucr, FSP-1 and thrombin all increase in the urine of HSPN children, and UAGT/Ucr and FSP-1 may reflect the treatment effect to some extent. [Key words]
ABSTRACT
Monoclonal gammopathy of renal significance (MGRS) can present with different morphologic features and lead to kidney failure. The Henoch-Schönlein purpura nephritis (HSPN) that cannot be relieved by treatment with glucocorticoid and immunosuppressive agents suggests the presence of monoclonal gammopathy in adult patients. The present study reports on a single case of HSPN associated with IgA-κMGRS. The patient who suffered from recurrent skin purpura for 6 months and nephrotic syndrome for 2 months was admitted to our hospital. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy indicated a Henoch-Schönlein purpura nephritis (HSPN, ISKDC classified as type III) with positive staining with κ-light chain in the glomeruli and renal tubular epithelial cells. Furthermore, skin biopsy showed leukocytoclastic vasculitis and negative staining for Congo red and light chain. Given both the renal and cutaneous involvement, the patient was considered to have HSPN associated with IgA-κMGRS. The patient experienced an exacerbation in his purpura-like lesions and clinical status after treatment with glucocorticoid and immunosuppressive agents. Consequently, the patient was put on a regimen that included dexamethasone (20 mg on the 1st, 4th, 8th, and 11th days of each month, iv) and bortezomib (2.4 mg on the 1st, 4th, 8th, and 11th days of each month, iv). Eight weeks after treatment, he had complete resolution of his cutaneous purpura and his biochemical parameters improved. The latent presence of MGRS in cases of HSPN should be considered in adult patients. Increased cognizance and correct treatment options could improve patient outcomes.
Subject(s)
Humans , Male , Middle Aged , Paraproteinemias/etiology , IgA Vasculitis/complications , Nephritis/complications , Paraproteinemias/pathology , Paraproteinemias/drug therapy , IgA Vasculitis/pathology , IgA Vasculitis/drug therapy , Glucocorticoids/administration & dosage , Immunosuppressive Agents/administration & dosage , Nephritis/pathology , Nephritis/drug therapyABSTRACT
Objective@#To investigate the changes of B cell-activating factor (BAFF) and a proliferation- inducing ligand (APRIL) in serum of children with Henöch- Schönlein purpura nephritis (HSPN), and to explore their role in the pathogenesis of children HSPN.@*Methods@#A total of 28 children with HSPN who were before treatment were selected in Department of Pediatrics Nephrology and Rheumatology, Shengjing Hospital of China Medical University from November 2017 to August 2018.Sixteen children with Henöch-Schönlein purpura were selected as HSP group, and 20 healthy children were selected as healthy control group.Followed the HSPN guideline to cure the patients for 6-8 weeks.The clinical data were collected.Serum levels of BAFF and APRIL were measured by adopting enzyme-linked immunosorbent assay (ELISA).@*Results@#(1)Changes of serum BAFF level: the serum levels of BAFF in HSPN children were significantly lower than those in the HSP group and the healthy control group[ HSPN group (0.652±0.360) μg/L, HSP group (1.276±0.459) μg/L, healthy control group (1.285±0.299) μg/L, F=17.519, P=0.000]. Moreover, the serum levels of BAFF in before treatment were significantly lower than those in after treatment [before treatment (0.652±0.360) μg/L, after treatment (0.860±0.262) μg/L, P<0.05). However, there were no significant di-fferences in the serum levels of BAFF between HSP group and healthy control group (P>0.05). (2)Changes of serum APRIL level: the serum levels of APRIL in HSPN and HSP children were both significantly higher than those in healthy control group, but there were no marked differences between the 2 groups [HSPN group (2.285±1.015) μg/L, HSP group (2.609±1.264) μg/L, healthy control group (1.677±0.118) μg/L, F=3.647, P=0.016]. There were no significant differences in the serum levels of APRIL between before treatment and after treatment [ before treatment (2.285±1.015) μg/L, after treatment (2.042±0.695) μg/L, P>0.05]. (3)Pearson correlation analysis results showed that the serum levels of BAFF were negatively correlated with 24 h urinary protein, urinary microalbumin, and urine red blood cell count (r=-0.587, -0.608, -0.515, all P<0.05). The serum levels of APRIL were positively correlated with serum IgA(r=0.588, P<0.05).@*Conclusions@#The level of serum BAFF decreased and APRIL increased in children with HSPN, which was related to the degree of renal involvement.It suggests that BAFF and APRIL may be related to the pathogenesis of HSPN in children.
ABSTRACT
Background@#Henoch-Schonlein purpura nephritis (HSPN) is a very common secondary kidney disease of childhood. Its pathogenesis and the treatment mechanism of glucocorticoid have not been fully elucidated. The aim of this study was to determine the relationship between p300 and the pathogenesis, glucocorticoid therapy in mice with HSPN, respectively.@*Methods@#Forty-eight C57BL/6N male mice, weighing 18 to 20 g, were selected (3–4 weeks old, n = 8 per group). The mice in the normal control group (Group I) were given normal solvent and the HSPN model group (Group II) were given sensitizing drugs. The mice in Group III were injected intraperitoneally with dexamethasone after being given sensitizing drugs. Meanwhile, mice in Groups IV, V and VI with conditional knockout of p300 were also given normal solvent, sensitizing drugs and dexamethasone. The levels of serum IgA, creatinine, and circulating immune complex (CIC) concentrations, 24 h urinary protein and urinary erythrocyte in C57 wild mice, and p300 conditional knockout mice in each group were measured. The expression of p300 in renal tissues and the expression of glucocorticoid receptor (GR) α and β, transforming growth factor (TGF)-β1, and activator protein (AP)-1 after dexamethasone treatment were determined by real-time polymerase chain reaction and Western blotting.@*Results@#Compared with the normal solvent control group (Group I), the expression of p300 mRNA in the model group (Group II) was significantly up-regulated. Western blotting further confirmed the result. Urinary erythrocyte count, 24 h urinary protein quantification, serum IgA, CIC, and renal pathologic score in Group V were distinctly decreased compared with non-knockout mice in Group II (9.7 ± 3.8 per high-power field [/HP] vs. 18.7 ± 6.2/HP, t = 1.828, P = 0.043; 0.18 ± 0.06 g/24 h vs. 0.36 ± 0.08 g/24 h, t = 1.837, P = 0.042; 18.78 ± 0.85 mg/mL vs. 38.46 ± 0.46 mg/mL, t = 1.925, P = 0.038; 0.80 ± 0.27 μg/mL vs. 1.64 ± 0.47 μg/mL, t = 1.892, P = 0.041; 7.0 ± 0.5 vs. 18.0 ± 0.5, t = 1.908, P = 0.039). Compared with non-knockout mice (Group III), the level of urinary erythrocyte count and serum IgA in knockout mice (Group VI) increased significantly after treatment with dexamethasone (3.7 ± 0.6/HP vs. 9.2 ± 3.5/HP, t = 2.186, P = 0.024; 12.38 ± 0.26 mg/mL vs. 27.85 ± 0.65 mg/mL, t = 1.852, P = 0.041). The expression level of GRα was considerably increased in the knockout group after dexamethasone treatment compared with non-knockout mice in mRNA and protein level (t = 2.085, P = 0.026; t = 1.928, P = 0.035), but there was no statistically significant difference in the expression level of GRβ between condition knockout and non-knockout mice (t = 0.059, P = 0.087; t = 0.038, P = 1.12). Furthermore, the expression levels of glucocorticoid resistance genes (AP-1 and TGF-β1) were notably increased after p300 knockout compared with non-knockout mice in mRNA and protein level (TGF-β1: t = 1.945, P = 0.034; t = 1.902, P = 0.039; AP-1: t = 1.914, P = 0.038; t = 1.802, P = 0.041).@*Conclusions@#p300 plays a crucial role in the pathogenesis of HSPN. p300 can down-regulate the expression of resistance genes (AP-1 and TGF-β1) by binding with GRα to prevent further renal injury and glucocorticoid resistance. Therefore, p300 is a promising new target in glucocorticoid therapy in HSPN.
ABSTRACT
Objective@#To analyze the relationship of clinical manifestations and pathological characteristics of Henoch-Schönlein purpura nephritis combined with hyperuricemia in children.@*Methods@#A retrospective study was conducted in 50 children with Henoch-Schönlein 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(χ2=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 (χ2=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(χ2=2.426, P=0.119, χ2=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%)](χ2=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(χ2=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(χ2=7.878, P=0.042).@*Conclusions@#Urinary protein was higher in children with Henoch-Schönlein 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.
ABSTRACT
Objective@#To establish the pathological grades of Henoch-Schönlein purpura nephritis(HSPN) in children with diagnostic prediction models by stepwise Fisher discriminant in children.@*Methods@#Based on the investigation 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 clinical manifestations with different pathological grades of HSPN.In children with pathological grade Ⅱ, Ⅲ and Ⅳ, 5 indicators 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 grade Ⅲa 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.
ABSTRACT
Objective@#To investigate the role of midkine(MK)in the pathogenesis of Henoch-Schonlein purpura(HSP) and Henoch-Schonlein purpura nephritis(HSPN).@*Methods@#In the case group, 35 cases were hospitalized in the pediatric kidney rheumatism immunology ward of Shengjing hospital affiliated to China Medical University from December 2016 to January 2018.Among them, 10 cases were HSP, 25 were HSPN.According to quantitative level of 24-hour urine protein, HSPN group was divided into HSPN(nephrotic level of proteinuria)group of 15 cases and HSPN(non-nephrotic level of proteinuria)group of 10 cases.The control group consisted of 12 healthy cases who underwent physical examination at outpatient department in the same period in the developmental pediatric clinic of our hospital.Blood samples were collected to detect MK.The other clinical datas including renal function, 24-hour urine protein quantitative, immunoglobulin, etc were collected.The serum MK and renal function indexes were compared among groups.The correlation between MK and various clinical indicators was analyzed, and the receiver operating characteristic(ROC) curve was used to analyze the diagnostic significance of MK for HSP and HSPN.@*Results@#MK level of case group was higher than that of healthy control group[(289.34±160.70)pg/ml vs.(100.03±56.75)pg/ml, P<0.05]. Moreover, the difference of MK concentration among the HSPN(nephrotic proteinuria)group, the HSPN(non-nephrotic proteinuria)group and the HSP group was still statistically significant[(449.91±141.91)pg/ml vs.(244.04±89.15)pg/ml vs.(175.94±46.30)pg/ml, P<0.05]. MK was positively correlated with urine microalbumin(r=0.54), IgA(r=0.132), IgE(r=0.304), urine β2 microglobulin(r=0.483), 24-hour urine protein /body weight(r=0.503), and urine transferrin level(r=0.509)in the case group(P<0.05). According to the ROC curve, the area under ROC of MK for predicting the diagnosis of HSP was 0.908(95%CI 0.828-0.988). The optimal value in predicting the diagnosis of HSP was 182.762 pg/ml, with sensitivity and specificity of 81.4% and 91.7%.The area under ROC of MK in predicting HSPN was 0.947(95%CI 0.888-1.000), and the optimal value of predicting HSPN was 218.186 pg/ml, with sensitivity and specificity of 84.0% and 95.5%.@*Conclusion@#MK may be involved in the pathogenesis of HSP and HSPN.It can provide the basis for clinical diagnosis of HSP and HSPN, and has significance in evaluating the degree of renal damage of HSPN.
ABSTRACT
Objective:To observe the clinical effect of Jiawei Xijiao Dihuang Tang on Henoch-schonlein purpura nephritis of children with blood heat and stasis syndrome and the inflammatory factors and coagulation function. Method:One hundred and twenty-two patients were divided into control group (60 cases) and observation group (62 cases) by random number table. Patients in control group got prednisolone acetate tablets every day, 1.5-2 mg·kg-1·d-1, after 4 weeks, of the treatment, and the 4 weeks dosage decreased progressively. Patients in control group also got dipyridamole tablets, 3-5 mg·kg-1·d-1, and compound rutin tablets, 1 tablet/day, 3 times/days. In addition to the therapy of control group, patients in observation group were also given Jiawei Xijiao Dihuang Tang, 1 dose/day. And one course of treatment was 12 weeks. Before and after treatment, blood heat and stasis syndrome were graded. And levels of urinary β2 microglobulin (β2-MG), 24-hour urine protein quantitative (24 h UmAlb), microalbuminuria (mAlb), cystatin C (CysC), platelet count (PLT), plasma prothrombin time (PLT), plasma prothrombin time (PT), activated partial thromboplastin time (APTT), interleukins-2 (IL-2), IL-4, IL-10, tumor necrosis factor-α (TNF-α), von willebrand factor (vWF), platelet activating factor (PAF), thrombomodulin (TM), plasma fibrinogen (FIB), urine RBC and urine protein (PRO) were detected. Result:The total clinical effect in observation group was 95.16%, which was higher than 81.54% in control group (χ2=5.466, Pχ2=12.052, Pβ2-MG, 24 h UmAlb, mAlb and CysC were higher than those in control groups (PPPα and vWF, PAF and TM were lower than those in control groups, but level of IL-2 was higher than that in control groups (PConclusion:In addition to the hormone therapy, Jiawei Xijiao Dihuang Tang can protect renal function, relieve symptoms of proteinuria and hematuria, with the effects of resisting inflammation and alleviating coagulation function.
ABSTRACT
Objective To investigate the efficacy of total glucosides of paeony ( TGP ) in the treatment of hematuria and proteinuria purpura nephritis ( HSPN ) and serum interleukin?6 ( IL?6 ), interleukin?1 beta (IL?1 beta) and interleukin?18 (IL?18).Methods From June 2017 to December 2018, 64 cases of children with primary hematuria and proteinuria purpura nephritis admitted to the department of pediatrics affiliated hospital of Xuzhou medical university were selected as study subjects,and were divided into control group (33 cases) and observation group (31 cases) according to random number method.The children in the control group were given oral administration of poniasone acetate,and the observation group was treated with TGP on the basis of the control group.Both groups received continuous treatment for 4 weeks.Two groups′ before and after treatment expression level of serum interleukin?6, interleukin?1β and interleukin?18,and level of clinic indicator such as Urinary red biood cell (URBC),24 hour urinary protein quantity, urinary immune globulin G ( IGU ), micro?albuminuria ( urinary microalbumin, MAU ), α?Microglobulin (α1?MG) and urinary transferrin ( TFRU) is made a comparison.Results After 4weeks of treatment,the total clinical effective rate of the observation group (93.5%(29／31)) was significantly higher than that of the control group (72.7%(24／33)),and the difference between the two groups was statistically significant (χ2＝4.868,P＜0.05).Before treatment,difference between serum interleukin?6,interleukin?1β and interleukin?18 from two groups and each clinic indicators level has no statistic significance (all P＞0.05) .After treatment,in the observation group,Serum IL?6 ( (16.68±6.83) ng／L and (32.24±6.99) ng／L,t＝12.373),IL?1β((63.83 ±8.97)ng／L and (85.59±9.42) ng／L,t＝9.758),IL?18((64.52±5.46) ng／L and (88.50±5.54) ng／Ll,t＝18.899),24 h urinary protein quantification ( 0.3 (0.21,0.36) g／24 h and 1.0 (0.65,1.23) g／24 h,Z＝－4.861),URBC (15.30 (3.80,36.80)×106／L and 168.9 (58.4, 324.0)×106／L,Z＝－4.840),were lower than before treatment ( all P＜0.05); After treatment, in the control group,Serum IL?6 ((23.62±5.95) pg／ml and (33.44±4.68) pg／ml,t＝9.149),IL?1β ((68.67 ±6.31) pg／ml and (86.59±8.71) pg／ml,t＝10.617),IL?18((71.25±9.69) pg／ml and (89.87±6.68) pg／ml,t＝11.506),24 h urinary protein quantification (0.42 (0.33,0.56) g／24 h and 0.94 (0.74,1.25) g／24 h,Z＝－5.013),URBC ( 57.00 ( 39.25,77.50)×106／L and 145.60 ( 58.20,360.85)×106／L,Z＝－4.762),were lower than before treatment ( all P＜0.05).The difference between the two groups was statistically significant (P＜0.05).Conclusion The adjuvant effect of total glucoside of white peony root on hematuria and proteinuria purpura nephritis is significant, which can reduce the level of hematuria and proteinuria and improve the renal symptoms of children.Its mechanism of action may be to reduce renal inflammation and protect the kidney by down?regulating the expression of serum IL?6,IL?1 and IL?18.
ABSTRACT
Henoch-schonlein purpura nephritis (HSPN) is a pathological renal manifestation of systemic allergic vasculitis caused by the deposition of immune complexes in small blood vessels. It occurs most frequently in children and the main manifestation is hematuria seen under microscope. At present, western medical doctors have not reached a consensus on the therapeutic regimen of HSPN, but they all have agreed that early diagnosis and early treatment in time are of great significance to the prognosis of this disease, especially the children with persistent proteinuria should be actively and properly treated. In recent years, with the deepening understanding of HSPN in traditional Chinese medicine, it is considered that the blood stasis is the key factor of the pathogenesis, therefore, the treatment of promoting blood circulation and removing blood stasis is applied through the whole therapeutic course. This study summarizes the various aspects of HSPN treatment based on the blood stasis as the key factor of the pathogenesis.
ABSTRACT
Objective To investigate the clinical efficacy of Huoxuehuayu liquid in treating children with Henoch-Schonlein purpura nephritis (HSPN). Methods Sixty children with HSPN admitted to the Department of Traditional Chinese Medicine (TCM) of Tianjin Children's Hospital were enrolled, and they were randomly divided into a Huoxuehuayu liquid group and a western medicine control group, 30 cases in each group. In Huoxuehuayu liquid group, modified Huoxuehuayu prescription was given, modification means the ingredients of prescription can be added and subtracted according to patients TCM syndrome manifestations (ingredients of prescription: Faeces Togopteri, carbonized Pollen Typhae, Radix Angelicae Sinensis, Herba Cirsii, Cortex Moutan Radicis, carbonized Nodus Nelumbinis Rhizomatis, each 10 g, Poria, Polyporus Umbellatus, Rhizoma Alismatis, carbonized Herba Schizonepetae, each 6 g, Radix Notoginseng 3 g, in patients accompanied by heat, Rhizoma Imperatae and Herba Lophatheri were added, in patients accompanied by dampness Semen Phaseoli and Semen Euryales were added, and in patients accompanied by deficiency, Radix Astragali seu Hedysari, Rhizoma Polygonati, Radix Polygoni Multiflori were added),1 dose per day, 2 times a day; in western medicine treatment group, vitamin C, aminopeptin, loratadine syrup were given; after 30 days of treatment in two groups, the changes of clinical signs, laboratory indexes and clinical curative effect were observed. Results After treatment of TCM method as above, the numbers of patients with skin purpura subsided (13 cases vs. 10 cases), partially subsided (12 cases vs. 9 cases), with occult blood <+ (13 cases vs. 10 cases), with 24 hours urinary protein transferring to normal (25 cases vs. 20 cases), with urine erythrocyte in sediment < 3 cells/HP (11 cases vs. 9 cases), with urinary streaming type red blood cells < 30 cells/μL (11 cases vs. 8 cases) in Huoxuehuayu liquid group were higher significantly than those in western medicine control group (all P < 0.05). After treatment, there were 13 cases with marked therapeutic effect, 12 cases with effect and 5 cases without effect, the total therapeutic effect being 83.33% (25 cases) in Huoxuehuayu liquid group; while in western medicine control group, there were 10 cases with marked effect, 9 cases with effect and 11 cases without effect, the total therapeutic effect being 63.33% (19 cases); total therapeutic effect rate in Huoxuehuayu liquid group was obviously higher than that in western medicine control group, the difference was statistically significant (P < 0.05). Conclusions Huoxuehuayu liquid can obviously improve the laboratory indexes and clinical signs, and elevate the clinical total therapeutic effective rate in treatment of children with HSPN; since the curative effect is definite, it is worthwhile to be used extensively in clinical practice.
ABSTRACT
Objective To analyze the clinical features of isolated hematuria Henoch-Schonlein purpura nephritis (HSPN). Methods The clinical data of children with isolated hematuria HSPN diagnosed in the First Affiliated Hospital of Henan University of Chinese Medicine from November 2003 to December 2014 was analyzed retrospectively. Results One hundred and thirty-five isolated hematuria HSPN patients were rolled in the study. Eighty cases were male and fifty-five cases were female. Male to female was 1. 45:1,average age was(9. 24 ± 5. 32)years old. Thirty one cases had pure skin purpura, thirty three cases had purpura accompany with abdominal pain,twenty eight cases had purpura accompany with arthralgia,forty three purpura accompany with abdominal pain and arthralgia. The pathogenesis was (8. 62 ± 7. 28)months. Seven cases were given nephridial tissue biopsy. In the 135 cases,there were 31 cases accepted general therapy,and 104 cases accepted the ther-apy of immunodepressant. In the patients with general therapy,there were 19 cases with recovery,8 cases with isolated hematu-ria,3 cases with mild proteinuria,1 case with moderate proteinuria. In the patients with therapy of immunodepressant,there were 72 cases with recovery,23 cases with isolated hematuria,7 cases with mild proteinuria,one case with moderate proteinuria,one case severe proteinuria. There was no significant difference in the prognosis between the general therapy patients and immu-nodepressant therapy patients(z = 0. 65,P = 0. 516). Conclusion The prognosis of the HSPN in children accompany with iso-lated haematuria is favourable,but a few patients may progress to moderate and severe proteinuria.
ABSTRACT
Henoch-schonlein purpura nephritis(HSPN) is the most common secondary glomerular disease in children.The pathogenesis of HSPN is unclear.In recent years,there have been some reports on B lymphocyte activating factor(BAFF) and a proliferation inducing ligand (APRIL),new members of the tumor necrosis factor family,as well as their association with HSPN.This paper reviews the related research from the following aspects:structure and biological functions of BAFF and APRIL,the role of BAFF/APRIL in the pathogenesis of HSPN,the prospect of BAFF and APRIL targeted biological agents in the treatment of HSPN.It provides a reference for further research on BAFF/APRIL system and HSPN.
ABSTRACT
To evaluate the clinical efficacy and safety of tripterygium glycosides (TG) in the treatment of henoch-schonlein purpura nephritis(HSPN). Seven English and Chinese databases (up to Nov. 9, 2017), were searched to collect the RCTs on TG for HSPN. Two researchers independently screened the literature according to inclusion criteria and exclusion criteria, extracted data, and evaluated the quality of the literature. After completion, cross-checking was performed and Meta-analysis was performed using RevMan 5.3 software. At the same time, different outcomes of the interventions were analyzed subgroupically. A total of 46 RCTs were included, with 1 659 in the experimental group and 1 596 in the control group. All the clinical studies showed a low quality. In terms of complete remission rate, the group with TG performed better than the group with conventional therapy or GC（RR=1.82，95%CI[1.39，2.39];RR=2.03，95%CI[1.37，2.99]），the group with TG+GC performed better than the group with GC（RR=1.46，95%CI[1.32，1.60]），and the group with CTX+GC performed better than the group with TG+GC（RR=0.35，95%CI[0.16，0.75]）. In terms of total effective rate, the group with TG performed better than the group with conventional therapy or GC（RR=1.44，95%CI[1.19,1.74];RR=1.30，95%CI[1.16，1.46]），the group with TG+GC performed better than the group with GC（RR=1.27，95%CI[1.21，1.34]），and the group with CTX+GC performed better than the group with TG+GC（RR=0.60，95%CI[0.43，0.85]）. No significant difference was found between the group with TG+GC and LEF+GC（RR=0.68，95%CI[0.30，1.53]）. In terms of urinary protein, urine occult blood negative time，the group with TG performed better than the group with conventional therapy（MD=-9.00，95% CI[-11.99，-6.01];MD=-12.00，95%CI[-16.13，-7.87]），the group with TG+GC performed better than the group with GC（MD=-8.86，95%CI[-10.08，-7.64];MD=-16.24，95%CI[-23.80，-8.67]）. In terms of recurrence rate, the group with TG+GC was lower than the group with GC（RR=0.13，95%CI[0.06，0.25]）, but there were no significant difference between the group with TG and conventional therapy（RR=0.43，95%CI[0.15，1.19]）. In adverse reactions, the common adverse effects of TG were gastrointestinal discomfort, liver damage and leucopenia. TG for the treatment of HSPN can improve clinical efficacy, reduce recurrence, and the adverse reactions are relatively safe. Due to the generally low methodological quality of the included studies, which affected the accuracy and reliability of the result. Therefore, more high-quality, large samples and multi-center randomized controlled trials are necessary for further evidence.
ABSTRACT
OBJECTIVES: Henoch-Schönlein purpura nephritis and immunoglobulin A nephropathy are two diseases with similar clinical presentations but very different prognoses. Transforming growth factor β1 and monocyte chemoattractant protein-1 have been associated with the development of tissue fibrosis. We examined the development of tubulointerstitial fibrosis and its relationship with Transforming growth factor β1 and monocyte chemoattractant protein-1 expression in these patients. METHODS: Renal tissue samples were collected by renal biopsy from 50 children with Henoch-Schönlein purpura nephritis and 50 children with immunoglobulin A nephropathy. Hematoxylin and eosin and Masson's trichrome-stained tissues were examined using light microscopy. Tubulointerstitial fibrosis was graded using the method described by Bohle et al. (1). The immunohistochemical detection of Transforming growth factor β1 and monocyte chemoattractant protein-1 expression was correlated with the tubulointerstitial fibrosis grade. Clinical Trial registration number: ZJCH-2012-0105. RESULTS: Transforming growth factor β1 and monocyte chemoattractant protein-1 expression in the renal tissues was significantly greater in the patients with immunoglobulin A nephropathy than in the patients with Henoch-Schönlein purpura nephritis (both p<0.001). The immunoglobulin A nephropathy patients had a higher tubulointerstitial fibrosis grade than the Henoch-Schönlein purpura nephritis patients (p<0.001). The tubulointerstitial fibrosis grade was in accordance with the Transforming growth factor β1 and monocyte chemoattractant protein-1 expression levels in both diseases (both p<0.001). CONCLUSION: Transforming growth factor β1 and monocyte chemoattractant protein-1 expression was associated with the development of immunoglobulin A nephropathy and Henoch-Schönlein purpura nephritis. Further studies are needed to better evaluate this association.
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , IgA Vasculitis/metabolism , Chemokine CCL2/metabolism , Transforming Growth Factor beta1/metabolism , Glomerulonephritis, IGA/metabolism , Kidney Tubules/metabolism , Prognosis , IgA Vasculitis/pathology , Fibrosis , Glomerulonephritis, IGA/pathology , Kidney Tubules/pathologyABSTRACT
OBJECTIVE: To investigate the outcomes of childhood diffuse endocapillary proliferation Henoch-Schönlein purpura nephritis (DEP-HSPN) in response to early diagnosis and prompt treatment. METHODS: Eleven cases of DEP-HSPN in children were investigated in comparison to HSPN without diffuse endocapillary proliferation (non-DEP-HSPN). RESULTS: DEP-HSPN had a higher prevalence of nephrotic syndrome but a lower prevalence of hematuria compared to non-DEP-HSPN. IgA, IgG and IgM antibody deposition was found in DEP-HSPN by histopathological examination. Proteinuria cleared in all 11 cases through treatment with steroids and/or immunosuppressive drugs. However, half of the DEP-HSPN patients continuously had hematuria after treatment. CONCLUSION: The early diagnosis and prompt initiation of immunosuppressive treatment based on renal biopsy are important for achieving favorable outcomes.
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Nephritis/drug therapy , Nephritis/pathology , IgA Vasculitis/drug therapy , IgA Vasculitis/pathology , Biopsy , Glucocorticoids/therapeutic use , Hematuria , Immunosuppressive Agents/therapeutic use , Kidney/pathology , Prednisone/therapeutic use , Proteinuria , Treatment OutcomeABSTRACT
Objective:To observe the effect of Bailing capsules adjuvant therapy on urinary monocyte chemoattractant protein-1( MCP-1), microalbuminuria(mAlb) and 24h urinary protein(Up)in the children with henoch-schonlein purpura nephritis(HSPN). Methods:Totally 64 cases of HSPN children were randomly divided into two groups with 32 cases in each .The normal group was given the conventional hormone , dipyridamole and loratadine etc .The Bailing group was orally treated with Bailing capsules additionally .The therapy course was 4 weeks, the changes of MCP-1,mAlb and 24hUp in the groups before and after the treatment were observed .Re-sults:Before the treatment, the levels of MCP-1,mAlb and 24hUp were similar between the groups (P>0.05).After the 4-week treatment, the levels of MCP-1,mAlb and 24hUp were lower than those before the treatment (P<0.05),and the decrease in Bailing group was more significant than that in the normal group (P<0.05).Conclusion:Bailing capsules adjuvant treatment for HSPN can significantly reduce albuminuria and improve renal function .
ABSTRACT
We aimed to investigate the differences in renal histopathological changes and laboratory parameters between adult and pediatric patients with Henoch-Schönlein purpura nephritis (HSPN), and to analyze the correlation between laboratory parameters and renal histopathological grading. A total of 139 patients diagnosed with HSPN between September 2010 and December 2014 at the First Hospital of Jilin University, China, were retrospectively reviewed. The clinical and pathological characteristics were examined and compared between the adult and the pediatric patients. A majority of adult (75.0%) and pediatric (66.2%) patients were categorized as pathological grade III HSPN. Adults having crescent lesions, interstitial fibrosis and renal artery involvement significantly outnumbered child counterparts (all P<0.05). Pathological grading showed a positive correlation with 24-h urine protein (r=0.307, P=0.009), microalbuminuria (r=0.266, P=0.000) and serum globulin (r=0.307, P=0.014), and a negative correlation with serum albumin (r=0.249, P=0.037) in pediatric patients with HSPN. Among adult patients with HSPN, histopathological grading showed a positive correlation with 24-h urine protein (r=0.294, P=0.015), microalbuminuria (r=0.352, P=0.006), α1-microglobulin (r=0.311, P=0.019) and immunoglobulin G (r=0.301, P=0.023) in urine, and serum creatinine (r=0.292, P=0.018). Further, a negative correlation between serum albumin and pathological grading was also observed (r=0.291, P=0.018). In conclusion, the severity of renal pathological lesions in HSPN patients is well reflected by the levels of proteinuria. Adult patients have more severe renal histopathological changes than pediatric patients.