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Objective:To investigate the efficacy and safety of Rituximab (RTX) in the treatment of children with frequently relapsing/steroid-dependent nephrotic syndrome (FRNS/SDNS) and to analyze the factors influencing the efficacy.Methods:Case series study.The clinical data of children with FRNS/SDNS who received B-cell-guided RTX (single dose: 375 mg/m 2, maximum dose: 500 mg, one additional dose when peripheral blood CD19 + B lymphocytes ≥0.01) in the First Affiliated Hospital of Zhengzhou University from September 2019 to March 2022 were retrospectively collected.The frequency of relapse and cumulative dose of glucocorticoids before and after RTX treatment were compared.The Kaplan-Meier method was used to analyze relapse-free survival rate and FRNS/SDNS-free survival rate after RTX treatment.The influencing factors of relapse were analyzed using the Cox proportional hazards regression model. Results:Totally 47 children were enrolled, including 35 males and 12 females; the age of first application of RTX was 10.2 (6.9, 13.0) years; 33 children had used one type of immunosuppressant before, and 14 children had used two or more types of immunosuppressant before; the dose of RTX treatment was 3.0 (2.0, 3.0). The frequency of relapse[0(0, 0.55) times/year vs.1.62 (1.09, 2.40) times/year] and cumulative dose of glucocorticoids[0.12 (0.05, 0.21) mg/(kg·d) vs.0.40 (0.20, 0.56) mg/(kg·d)] after RTX treatment significantly decreased compared with previous immunosuppressive treatment ( Z=-5.56, -5.54, all P<0.001). The relapse-free survival rates at 6, 12, 18 and 24 months after treatment were 80.9%, 72.3%, 68.1% and 68.1%, respectively, and the FRNS/SDNS-free survival rates were 93.6%, 89.4%, 89.4% and 89.4%, respectively.Univariate Cox regression analysis showed that the high frequency of relapse during previous immunosuppressive therapy was a risk factor for relapse after RTX treatment ( P<0.05). Of the 14 children who relapsed, 6 occurred in children whose CD19 + B lymphocytes<0.01, and the frequency of relapse after RTX treatment was significantly higher than those whose CD19 + B lymphocytes≥0.01 ( Z=-2.84, P=0.005). No severe adverse reactions occurred during RTX treatment and follow-up. Conclusions:The B-cell-guided RTX is effective and safe in the treatment of FRNS/SDNS in children.The high frequency of relapse during previous immunosuppressive therapy is a risk factor for relapse after RTX treatment, and relapse in the state of B lymphocyte depletion predicts poor outcomes of RTX treatment.
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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.
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Objective@#To investigate the clinicopathological features of IgA nephropathy(IgAN) complicated with acute kidney injury (AKI) in children.@*Methods@#The clinical and pathological data of children with biopsy-proven primary IgAN and complicated with AKI from January 2012 to December 2016 in the First Affiliated Hospital of Zhengzhou University were analyzed retrospectively, and the data of macroscopic hematuria (MH) associated AKI(15 cases) and other MH without AKI(99 cases) were compared.@*Results@#(1) Among 211 patients diagnosed with IgAN, 21 (9.95%) patients were complicated with AKI.Among the 21 cases, the average age was (9.5±2.1) years old, in which 17 cases(80.9%) were males, 19 cases with MH, and the range of the peak serum creatinine was 93-627 μmol/L.In histology, cellular/fibrocellular crescents were found in 11 cases, while high proportion of tubules filled with red blood cell(RBC) casts were observed in 8 cases, and moderate to severe acute tubular injury (ATI) were observed in 16 cases.In 2 cases, extensively mixed inflammatory cell infiltration with eosinophils was present in the interstitium.(2) According to the clinical and pathological characteristics, the 21 cases were divided into 3 groups: 15 cases with MH related AKI, 4 cases with crescentic glomerulonephritis, and 2 cases with acute interstitial nephritis.Compared with MH without AKI group(99 cases), the MH associated AKI group(15 cases) had significantly longer duration of MH, higher proteinuria level, urinary levels of β2-microglobulin (β2-MG) and N-acetyl-β-D-glucosaminidase (NAG), and greater frequency of RBC casts and ATI, and the differences were statistically significant(all P<0.05). In 2 acute interstitial nephritis cases, one had a history of taking weight loss drug with unknown ingredients, while the specific cause of the other was not clear.(3) None of the patients was on dialysis, but corticosteroid was prescribed in 17 cases (including methylprednisolone pulse therapy in 6), and 3 cases were combined with cyclophosphamide.Almost all patients achieved normal renal function except for one who had no response within 2 months after treatment.The median follow-up period was 24 months, in which 1 patient with crescentic glomerulonephritis progressed to end-stage renal disease.@*Conclusions@#AKI is commonly seen in children with IgAN, and complete recovery of renal function was seen in all patients with MH associated AKI and acute interstitial nephritis.
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Objective To evaluate the efficacy and safety of mycophenolate mofetil (MMF) in treatment of IgA nephropathy. Methods The Cochrane library, PubMed, EMBASE, Wanfang Data Knowledge Service Platform, CNKI and VIP were searched from the time when the databases were established to March 31, 2018. Reports on randomized controlled trials (RCTs) on treating IgAN with MMF were collected. Data were extracted and assessed independently by three reviewers and the methodological quality of included RCTs was assessed by the Cochrane collaboration's tool for assessing risk of bias. The Meta analysis of homogeneous RCTs was managed by using Stata 12.0. Results Nine RCTs, of which two RCTs were assessed as A-level studies scoring from 4 to 7 points, six RCTs were assessed as B-level studies scoring from 2 to 3 points, and one RCT was assessed as C-level study with scores less than 2 points, were enrolled in the study. Important outcomes of this systematic review were described as follows: (1) Compared to placebo plus ACEI/ARB or ACEI/ARB monotherapy, MMF plus ACEI/ARB did not reduce the incidence of increased serum creatinine and ESRD, but increased the partial remission rate of urinary protein (OR=2.59, 95%CI 1.01-6.65, P=0.049. (2) No significant difference was detected in the efficacy of reducing urinary protein for MMF monotherapy or MMF plus glucocorticoid (GC) compared to GC monotherapy. (3) MMF showed no significant difference in the efficacy of reducing urinary protein compared to LEF or CTX, but lower incidence rate of serum creatinine increasing than that of CTX group (OR=0.21, 95%CI 0.04-1.07, P=0.043. (4) Different levels of adverse reactions occurred in each treatment group with MMF, but most symptoms were mild, and recovered gradually after reducing or withdrawing MMF. Conclusions MMF monotherapy shows a superiority in curing IgAN compared to ACEI/ARB, but no significant superiority compared to GC. MMF can replace a part of the effect of GC when used in combination with GC and can reduce the dosage of GC compared to GC monotherapy. Additionally, MMF displays no better short-term efficacy than LEF or CTX, but a better long-term efficacy and fewer side effects than CTX. And the side effects occurred in the treatment groups with MMF are mostly mild, and disappear gradually after reducing or stopping the use of the drug. MMF is a safe and effective drug for the treatment of IgAN.
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Objective To compare the effectiveness,safety and related clinical indicators between simple drainage treatment and drainage treatment combined with intrathoracic urokinase for children with parapneumonic pleural effusion(PPE).Methods Twenty-nine in patients with PPE given pleural effusion drainage in the First Affiliated Hospital of Zhengzhou University from January 2013 to December 2015 were selected as research subjects,who were divided into a simple group and an urokinase group based on whether intrathoracic urokinase was injected or not.The total number of hospital stay,the total drainage volume,the total number of catheter days,the total cost,the days with fever,efficient rate,operation rate and security of the patients were retrospectively analyzed between two groups.Results The intrathoracic days of hospital stay [M(P25,P75)] of urokinase group[19(11,30) days]were less than those of simple group[30(21,38) days],and the difference was significant (Z =-2.545,P =0.011);the total drainage volume[M(P25,P75)] of the urokinase group [430 (175,1 308) mL] was more than that of the simple group [110 (10,325)mL],and the difference was significant (Z =-2.811,P =0.005);the total number of catheter days [M (P25,P75)] of urokinase group [9 (7,19) days] was less than that of the simple group [20 (10,30) days],and the difference was significant (Z =-2.020,P =0.043);the total cost [M(P25,P75)] of the urokinase group [20 000(10 000,30 000)RMB] was less than that of the simple group [40 000 (30 000,50 000) RMB],and the difference was significant (Z =-2.631,P =0.009);the days with fever between urokinase group and the simple group was not significant (Z =-0.820,P =0.412).The urokinase group had a higher cure rate[76.9% (10/13 cases)] and a lower surgical rate [23.1% (3/13 cases)] compared with those of the simple group[18.7% (3/16 cases),81.3% (3/16 cases)],and the difference was significant (x2 =9.814,P =0.003).Conclusions Intrapleural urokinase therapy as an adjuvant treatment of PPE is simple and convenient,economic,higher efficiency,lower risk,which can be used as an effective clinical solution such disease.
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Objective To discuss the clinical characteristics and prognosis of lipoprotein glomerulopathy (LPG) in chil-dren. Method Clinical data of one pediatric LPG patient were retrospectively analyzed. The clinical features and prognosis of childhood LPG were summarized based on literature review. Results A nine years old girl presented with frequent urination. The ifrst urine test revealed hematuria and proteinuria. After one week anti-infection treatment, the hematuria and proteinuria were continued. The serum albumin was slightly reduced. The hyperlipidemia and mild anemia were emerged. Kidney biopsy showed that enlarged glomeruli, with dilated capillary loops and weak eosinophilic lipoprotein thrombi in the capillary lumina under the light microscope;layered or tuftedemboluscontaining particulated lipid vacuoles under electron microscope. Gene sequencing identified APOE Tokyo (Leu141-Lys143→0). The diagnosis of LPG was confirmed. The lipid-lowering therapy was administrated and the disease was alleviated. Conclusion LPG is a rare disease in children. The level of blood lipid was signiifcantly increased, and the hormone therapy was ineffective. Kidney biopsy is the main basis for diagnosis. The genetic testing can prompt the genetic background. Lipid lowering therapy can relieve the progress of the disease.
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Objective To investigate the changes in serum cortisol and adrenocorticotrophic hormone(ACTH) levels in children with steroid-sensitive nephritic syndrome (SSNS) and the clinical significance of intervention.Methods According to whether a child with SSNS would relapse,48 children with SSNS were divided into non-recurrence group(n =19) and recurrence group(n =29),at the same time 14 age-matched healthy children were enrolled as the healthy control group.Serum cortisol and ACTH were measured in patients with SSNS by using electrochemiluminesence methods.In recurrence group,methylpredisolone or methylprednisolone combined with ACTH injection therapy was used on 15 patients with their serum cortisol and ACTH below normal.Results Before standard glucocorticoid treatment,the serum levels of cortisol and ACTH had no difference among 3 groups (all P > 0.05).By the end of remission phase,the serum levels of cortisol and ACTH in recurrence group were statistically lower than those of the non-recurrence group[(113.03 ± 80.02) μg/L vs (251.54 ± 185.05) μg/L,t =-2.925,P < 0.05 ; (12.81 ± 10.14) ng/L vs (23.53 ± 12.05) ng/L,t =-0.885,P < 0.05].Eight to 12 weeks after being adjusted,the serum level of cortisol and the average monthly time of recurrent were both significantly improved in 15 abnormal children in recurrence group [(168.90 ± 133.43) μg/L vs (73.62 ± 58.04) μg/L,t =3.016,P < 0.05 ; (0.09 ± 0.08) times vs (0.35 ± 0.11) times,t =-7.560,P <0.05],but as to the serum level of ACTH,there was no significant difference in abnormal children in recurrence group [(14.05 ± 10.99) ng/L vs (8.72 ± 4.11) ng/L,t =1.991,P > 0.05].Conclusions The concentrations of serum cortisol and ACTH can reveal the risk of recurrence for children with SSNS to some extent,and effective intervention can reduce the recurrence rate and shorten the course of disease.
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Objective To evaluate the efficacy and safety of intrapleural fibrinolytic agents in the treatment of childhood empyema.Methods The data from China National Knowledge Infrastructure (CNKI),Chinese BioMedical (CBM),PubMed,VIP Database and Cochrane library were reviewed.Randomized control trials by using intrapleural fibrinolytic agents to treat childhood empyema were included.Two reviewers independently extracted the data from the eligible studies and evaluated the quality of the included studies.Meta-analysis was performed for the results of homogeneous studies using RevMan 5.2 software,while others analyzed descriptively.Results Five randomized control trials involving 248 children with empyema were included.Meta-analysis was not made in all of the indexes because of apparent heterogeneity and limited data.Two trials were compared which involved 98 patients receiving fibrinolytic agents or 9 g/L saline.The results suggested that fibrinolytic agents shortened the length of hospital stay and reduced complication(all P < 0.05),but no significant differences were found between the 2 groups in the effective rate,duration of fever and pleural effusion amount(OR =3.61,95% CI 0.84-15.49,P > 0.05).Three trials involving 150 patients compared fibrinolytic agents and video-assisted thoracoscopic surgery (VATS).The data suggested that no significant differences were found between 2 groups in the effective rate (OR =0.70,95 % CI 0.30-0.61,P > 0.05),but fibrinolytic agents cost less (P < 0.05).The adverse drug reactions to intrapleural fibrinolytic agents were mild to moderate,and well tolerated.Conclusions The findings suggest that intrapleural fibrinolytic agents in the treatment of childhood empyema is safe and effective,especially in encapsulated effusion.Compared to 9 g/L saline,it can shorten the length of hospital stay and reduce complication.Compared with VATS,the curative effect is similar,while intrapleural fibrinolytic agents are economic.
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<p><b>OBJECTIVE</b>To study the efficacy and safety of brucea javanica oil emulsion (BJOE) combining platinum-contained first-line chemotherapy in treating non small cell lung cancer (NSCLC).</p><p><b>METHOD</b>Cochrane library, PubMed, EMBASE, VIP, CBM and CNKI were searched through computers. The search was finished in February, 2011. Randomized controlled trials (RCTs) of BJOE combining platinum-contained first-line chemotherapy were included. Two researchers extracted data and assess literature quality separately,and made a meta-analyses by RevMan 5.1.2 software.</p><p><b>RESULT</b>Totally 22 RCTs involving 1512 patients were included. The Meta-analysis showed that compared with the pure application of platinum-contained first-line chemotherapy,the combination of BJOE and chemotherapy can enhance the near-term curative effect (RR = 1. 31, 95% CI: 1.18-1.45, P < 0. 000 01), improve the quality of life (RR = 1.78, 95% CI: 1.51-2. 09, P < 0.00001) and reduce the suppression of bone marrow (OR = 0.37, 95% CI: 0. 27-0. 51, P < 0.00001) and the gastrointestinal reactions (OR = 0.59, 95% CI: 0.44-0.80, P = 0.0007) ,with an improvement in organism immunity.</p><p><b>CONCLUSION</b>The current evidence indicates that BJOE can enhance the chemotherapeutic effect on NSCLC patients, improve the quality of life and reduce adverse effect of platinum-contained chemotherapeutics and thus it is worth referring in clinic.</p>