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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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Background Fatigue driving is an important cause of road traffic accidents in modern society, and the fatigue condition of heavy-duty commercial truck drivers has attracted widespread attention. Research on the fatigue status and influencing factors of heavy-duty commercial truck drivers in China is relatively rare at present. Objective To analyze the main characteristics of fatigue among heavy-duty commercial truck drivers and the impacts of factors such as working hours, insomnia, and occupational burnout on their fatigue status. Methods Using cluster sampling method, a cross-sectional study was conducted from July to August 2023, enrolling heavy-duty commercial truck drivers in long-distance freight logistics markets (stations) located in three administrative regions of W City, Xinjiang Uygur Autonomous Region. A self-designed questionnaire was used to collect demographic and occupational characteristics of heavy-duty commercial truck drivers, and the Chinese versions of Fatigue Scale-14 (FS-14), Athens Insomnia Scale (AIS), and Maslach Burnout Inventory General Survey (MBI-GS) were used to evaluate their fatigue, insomnia, and occupational burnout status, respectively. Mann-Whitney U test and Kruskal-Walls H test were used to compare intergroup differences, and Spearman correlation coefficient was used to analyze the correlation between variables. Hierarchical regression models were used to study the impacts of selected variables on fatigue status. Results This study obtained 311 valid questionnaires, with a valid recovery rate of 88.86% (311/350). The physical fatigue, mental fatigue, and total fatigue scores of the survey subjects in M (P25, P75) were 3.00 (2.00, 4.00), 2.00 (1.00, 3.00), and 5.00 (4.00, 6.00), respectively. The comparison results showed that, except for smoking, there were statistically significant differences in total fatigue scores between different groups of age, marital status, number of children, educational level, service length of freight transportation, average daily working time, and average monthly income (P<0.05). The difference in total fatigue score among the groups without sleep disorders, with suspected insomnia, and with insomnia was statistically significant (P<0.001). The difference in total fatigue score among the groups without occupational burnout, with moderate occupational burnout, and with severe occupational burnout was also statistically significant (P<0.001). Positive correlations were found between insomnia score and scores of physical fatigue (rs=0.507), mental fatigue (rs=0.547), and total fatigue (rs=0.618) (P<0.001). Hierarchical regression models revealed that having more children, extended daily working hours, insomnia, and increased scores of decreased personal accomplishment were negative factors affecting the fatigue status of heavy-duty commercial truck drivers (P<0.05), and the final regression equation was: total fatigue score=7.579+0.581×number of children+0.916×average daily working time+0.434×score of AIS+0.754×score of reduced personal accomplishment. Conclusion The fatigue status of heavy-duty commercial truck drivers is not optimistic. An increase in the number of children, extended daily working hours, severe insomnia symptoms, and increased scores of decreased personal accomplishment associate with their worse fatigue status.
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Objective:To analyze the clinicopathological features and prognosis of idiopathic membranous nephropathy (IMN) in children, and to investigate the factors influencing their prognosis.Methods:The clinical and pathological data of 128 children with IMN hospitalized in the First Affiliated Hospital of Zhengzhou University from January 2012 to December 2019 were retrospectively analyzed.They were divided into 2 groups according to the pathological manifestations: group A[typical membranous nephropathy(MN) group] and group B (atypical MN group), and the clinicopathological characteristics of the 2 groups were compared.Different treatment regimens and their efficacy were summarized, and the prognosis and its influencing factors were analyzed.The primary endpoint event at follow-up was the occurrence of end stage renal disease (ESRD), and the secondary endpoint event was the occurrence of renal insufficiency.Children with IMN were further divided into endpoint event group and non-endpoint event group according to the presence or absence of endpoint events at the last follow-up.Survival analysis was performed using the Kaplan-Meier survival curve method.The Cox proportional risk model method was used to analyze the factors influencing the prognosis of poor kidney outcomes in children with IMN. Results:(1)A total of 128 children were included, with the male-to-female ratio of 1.13∶1.00.The median age of onset and peak age of onset were 13.0 (10.3, 15.0) years, and 12-16 years (68.8%), respectively.Massive proteinuria was detected in 119 cases (93.0%), including 103 cases (80.5%) with massive proteinuria and hematuria, 4 cases(3.1%) with simple hematuria, and 5 cases (3.9%) with non-renal proteinuria.There were 29 cases (22.7%) in group A and 99 cases (77.3%) in group B. (2)Blood triacylglycerol level was significantly higher in group B than that of group A[2.1 (1.5, 3.0) mmol/L vs.1.7(1.1, 2.5) mmol/L], while high-density lipoprotein[1.5(1.1, 1.8) mmol/L vs.1.8(1.4, 2.1) mmol/L], serum albumin[22.0(17.0, 27.3) g/L vs.25.5 (21.0, 32.5) g/L] and complement C3[(1.1±0.2) g/L vs.(1.2±0.2) g/L] were significantly lower in group B than those of group A (all P<0.05). (3)Complete clinical data during hospitalization and follow-up data were obtained from 91 children with IMN, with a median follow-up time of 87.0 (49.0, 104.5) months.Among them, 5 cases (5.5%) progressed to ESRD, involving 3 cases received renal transplantation, and 9 cases (9.9%) had secondary endpoints.Cumulative renal survival rate for ESRD at 5 and 10 years were 96.2% and 92.9%, respectively, which, for the secondary endpoints at 5 and 10 years were 95.2% and 84.8%, respectively.(4)Kaplan-Meier survival analysis showed no significant difference in the cumulative renal survival between group A and group B ( P>0.05). Multifactorial Cox regression analysis showed that tubular atrophy/interstitial fibrosis was an independent risk factor for renal insufficiency in children with IMN ( HR=0.102, 95% CI: 0.011-0.940, P<0.05). Conclusions:Massive proteinuria combined with hematuria is the major clinical manifestation of IMN in children, and atypical MN is the major pathological manifestation.Tubular atrophy/interstitial fibrosis is an independent risk factor for renal insufficiency in children with IMN.
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Atypical hemolytic uremic syndrome (aHUS) is characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. IgA nephropathy (IgAN) is a common primary glomerular disease, while aHUS combined with IgAN is rare, and has been reported rarely in the past. This paper reported a case of children with aHUS combined with IgAN. In the acute stage, she was treated with plasma therapy and glucocorticoid. After remission, she was treated with glucocorticoid combined with immunosuppressants. During the follow-up period, aHUS did not recur, the renal function was normal, and urinary protein decreased to weakly positive.
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Objective:To summarize the clinical features and gene phenotype of children with spondyloenchondrodysplasia with immune dysregulation (SPENCDI) caused by ACP5 gene mutation. Methods:The medical data and genetic phenotype of a child diagnosed with SPENCDI in the Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University in February 23, 2017 were analyzed retrospectively.Besides, " spondyloenchondrodysplasia" were taken as the search terms to perform the retrieval in CNKI, Wanfang Data, and PubMed, in an attempt to conduct the literature review.χ 2 test was used to compare the factors among children with different mutations. Results:The 4.5-year-old girl was admitted to hospital for complaint of " fever and chilblain-like rash" when she was 2 years old.She was diagnosed with systemic lupus erythematosus (SLE) concomitant with lupus nephritis.Methylprednisolone combined with cyclophosphamide, mycophenolate mofetil was used for the treatment.However, she experienced multiple infections, thrombocytopenia, limp, and growth retardation during the treatment.Genetic detection identified ACP5 gene compound hybrid mutation: c.779C>A and c. 770T>C.She was diagnosed with SPENCDI, and was subjected to follow-up.A total of 78 SPENCDI patients were retrieved from the databases, with various clinical manifestations of SPENCDI, commonly with skeletal involvement and immune phenotypes; 73.08% of the cases were positive for antinuclear antibodies, 57.69% of cases were positive for anti-double stranded-DNA antibodies and 34.62% of cases had neurological symptoms.In 58 cases, ACP5 gene mutations were detected, including 44 homozygous mutations and 14 compound heterozygous mutations.Patients with ACP5 gene homozygous mutation had a higher probability of consanguineous marriage in parents [56.82% (25/44 cases) vs.14.29% (2/14 cases)]; patients with ACP5 gene heterozygous mutation were more likely to develop SLE [64.29% (9/14 cases) vs.34.09% (15/44 cases)]( χ2=7.722, 3.992; all P<0.05). Conclusions:The majority of the ACP5 gene mutations are homozygous mutations in patients with SPENCDI, and heterozygous mutations are rare.The clinical manifestations of SPENCDI are various and complex, it is prone to develop autoimmune diseases, and there was no clear correlation between clinical features and gene phenotype in SPENCDI patients.
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IgA nephropathy(IgAN)is a common primary glomerular disease.The abnormal increase of IgA molecules in serum can be regarded as the initiation stage of IgAN development, and the abnormal IgA antibody class switch process in B cells leads to the overproduction of IgA.In order to provide new ideas for the prevention and treatment of IgAN, this paper reviews the role of IgA antibody class switch in the pathogenesis of IgAN from the aspects of mucosal immunity, T cells, cytokines and signaling pathways, as well as possible therapeutic targets.
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Primary nephrotic syndrome (PNS) is one of the common glomerular diseases in children.Glomerular disease treatment by anti-CD 20 monoclonal antibodies is currently a hot topic.There are three generations of anti-CD 20 monoclonal antibodies.As a representative of the first generation of anti-CD 20 monoclonal antibodies, Rituximab (RTX) has been proven effective in treating children with steroid-dependent/frequent-relapsing nephrotic syndrome (SDNS/FRNS). Ofatumumab (OFA) and Obinutuzumab (OBI) represent the second-and the third-generation anti-CD 20 monoclonal antibodies, respectively.OFA and OBI have showed good efficacy and safety in pediatric PNS.In this paper, clinical studies and applications of anti-CD 20 monoclonal antibodies in the treatment of children with PNS were reviewed, so as to provide a reference for the treatment of PNS in children.
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Objective:To investigate the efficacy and safety of plasma exchange(PE) in the treatment of autoimmune hemolytic anemia in children.Methods:The data from 8 hospitals in China during November 2014 to April 2017 were collected, and the clinical characteristics of PE in children with AHA were analyzed retrospectively.Results:A total of 21 children with AHA were included in the study, including 17 cases from PICU and 4 cases from pediatric kidney ward, with 11 boys and 10 girls, and the median age was 3.64(0.25, 11.10)years old, and median hospital stay was 12(4, 45)days.There were 15 cases(71.4%) with infection, 2 cases(9.5%)with autoimmune diseases, 4 cases(19.0%) with unknown.Consciousness disturbance occurred in 4 patients before replacement and recovered to normal after PE.The volume of blood decreased in two cases(9.5%) and completely relieved.There were 20 cases of anemia (95.2%), 15 cases were normal after PE, and 5 cases were improved.Jaundice occurred in 18 cases (85.7%), 12 cases were normal after PE, 6 cases were improved.Hepatosplenomegaly was found in 11 cases, 10 cases were normal after PE, 1 case was improved.After PE, the hemoglobin and red blood cell count increased, while the total bilirubin, indirect bilirubin, urea nitrogen and lactate dehydrogenase decreased, there were significant differences between pre-and post-replacement ( P<0.05). Only 1 case had allergic reaction, which was improved after symptomatic treatment, and PE was continued.After PE, 2 cases (9.5%) had complete remission, 16 cases (76.2%) had partial remission and 3 cases (14.3%) had been discharged. Conclusion:PE therapy can obviously improve the clinical symptoms and laboratory indexes of children with AHA who have failed to respond to conservative treatment.It can be used as a treatment measure for children with severe AHA and has a good safety.
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Objective:To investigate the efficacy and safety of plasma exchange(PE) in the treatment of autoimmune hemolytic anemia in children.Methods:The data from 8 hospitals in China during November 2014 to April 2017 were collected, and the clinical characteristics of PE in children with AHA were analyzed retrospectively.Results:A total of 21 children with AHA were included in the study, including 17 cases from PICU and 4 cases from pediatric kidney ward, with 11 boys and 10 girls, and the median age was 3.64(0.25, 11.10)years old, and median hospital stay was 12(4, 45)days.There were 15 cases(71.4%) with infection, 2 cases(9.5%)with autoimmune diseases, 4 cases(19.0%) with unknown.Consciousness disturbance occurred in 4 patients before replacement and recovered to normal after PE.The volume of blood decreased in two cases(9.5%) and completely relieved.There were 20 cases of anemia (95.2%), 15 cases were normal after PE, and 5 cases were improved.Jaundice occurred in 18 cases (85.7%), 12 cases were normal after PE, 6 cases were improved.Hepatosplenomegaly was found in 11 cases, 10 cases were normal after PE, 1 case was improved.After PE, the hemoglobin and red blood cell count increased, while the total bilirubin, indirect bilirubin, urea nitrogen and lactate dehydrogenase decreased, there were significant differences between pre-and post-replacement ( P<0.05). Only 1 case had allergic reaction, which was improved after symptomatic treatment, and PE was continued.After PE, 2 cases (9.5%) had complete remission, 16 cases (76.2%) had partial remission and 3 cases (14.3%) had been discharged. Conclusion:PE therapy can obviously improve the clinical symptoms and laboratory indexes of children with AHA who have failed to respond to conservative treatment.It can be used as a treatment measure for children with severe AHA and has a good safety.
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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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Primary nephrotic syndrome refers to one of the common glomerular diseases in children.Glucocorticoid is the first choice in the treatment of nephrotic syndrome, and the using cycle is long.Long term application of glucocorticoids can cause side effects, including Cushing′s disease, hypertension, decreased immune function, growth retardation and eye adverse reactions.Among eye adverse reactions, glaucoma and cataract are more common.Glucocorticoid-induced glaucoma and glucocorticoid-induced cataract are often concealed, and children often cannot accurately express their discomforts.At the same time, some clinicians lack the understanding of the adverse reaction, which is easy to cause missed diagnosis and misdiagnosis.Eventually, irreversible visual impairment occurred.Here, the etiology, pathogenesis, clinical diagnosis, and treatment of glucocorticoid-induced glaucoma and glucocorticoid-induced cataract are elaborated, thus aiming to improve clinicians′ understanding of glucocorticoid ophthalmopathy and the level of clinical diagnosis and treatment.
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Objective:To report 1 case of mushroom poisoning complicated with multiple organ dysfunction syndrome treated by liver transplantation, so as to explore surgical effectiveness and prognosis of liver transplantation on children mushroom poisoning.Methods:Retrospectively analyzed a child with mushroom poisoning who was admitted to the First Affiliated Hospital of Zhengzhou University in June 2017.Conventional treatment was not ideal, the child was conducted orthotopic liver transplantation.The effectiveness was evaluated and the feasibility of liver transplantation in treating children mushroom poisoning with literatures was reviewed.Results:A male 2-year old child obtained, preo-perative Child-pugh class for C. This child recovered well postoperatively, the jaundice disappeared, and liver enzyme and blood coagulation function improved significantly within 1 week after liver transplantation.However, the head magnetic resonance imaging presented brain atrophy and ventricular expansion, with intelligence and athletic ability being significantly behind his peers through postoperative regular follow-up for 2 years.Conclusion:Fulminant hepatic failure that was caused by mushroom poisoning is an indication for liver transplantation, and orthotopic liver transplantation is an effective method for the treatment of mushroom poisoning, which can effectively improve liver function, metabolic disorders and blood coagulation function for children with mushroom poisoning, but nervous system damage that was caused by mushroom poisoning is irreversible.Although liver transplantation technology successfully cured liver failure, it still left severe neurological sequelae.
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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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Adrenocorticotrophic hormone(ACTH)is a pituitary neuroimmunoendocrine polypeptide that belongs to the melanocortin system.Glomerular disease is common in children with kidney disease and may progress to renal insufficiency or even renal failure.The application of ACTH in the treatment of glomerular disease is a hot topic.Studies have shown that ACTH has a significant protective effect on podocytes, and plays a role in anti-urine protein and lipid-lowering.ACTH may play these roles through steroid and non- steroid mechanisms.This article reviews the types, mechanism and study progress of ACTH in the treatment of glomerular disease.
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Adrenocorticotrophic hormone (ACTH) is a pituitary neuroimmunoendocrine polypeptide that belongs to the melanocortin system.Glomerular disease is common in children with kidney disease and may progress to renal insufficiency or even renal failure.The application of ACTH in the treatment of glomerular disease is a hot topic.Studies have shown that ACTH has a significant protective effect on podocytes,and plays a role in anti-urine protein and lipid-lowering.ACTH may play these roles through steroid and non-steroid mechanisms.This article reviews the types,mechanism and study progress of ACTH in the treatment of glomerular disease.
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Drug-related acute kidney injury(AKI)is a common problem in clinical medicine.It is more prone to occur in children,especially in children with severe infection,low circulating blood volume,congestive heart failure,diabetes and chronic kidney disease clinically.Mechanisms include altered intra-glomerular haemodynamics, oxidative damage,hypersensitivity reactions,renal obstruction,tubular inflammatory changes,interstitial nephritis,rhab-domyolysis,thrombotic microvascular disease and metabolic disorders. Therefore,the early detection of adverse drug reactions to prevent its progression to end-stage renal disease is important.Despite many efforts to improve the early assessment of AKI in patients,effective markers are still lacking.Preventive measures requires knowledge of mecha-nisms of drug-induced AKI,improve the prognosis by correcting risk factors,assessing baseline renal function before initiation of therapy,adjusting the drug dosage and avoiding use of nephrotoxic drug combinations.
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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 explore the effect and safety of mycophenolate mofetil (MMF) and glucocorticoid on Henoch-Schonlein purpura nephritis in children and compared with cyclophosphamide (CTX).Methods The data of 48 patients diagnosed as Henoch-Schonlein purpura by renal biopsy were retrospectively analyzed.Median follow-up time was 22(7,48) months.The subjects were divided into 2 groups.34 cases were in the MMF group:MMF (15-20 mg · kg-1 · d-1 or 800-1200 mg/m2)+prednisone,and 14 cases in the CTX group:CTX (8-12 mg · kg-1 · d-1) + prednisone.Clinical and laboratory data were collected at baseline and 1,3,6 months after treatment.During follow-up,cumulative retreatment rate and adverse reactions after treatment were recorded.Results In two groups after treatment for 1,3,6 months,24 hours urinary protein quantitative was significantly lower than the baseline value,serum albumin (sAlb) was significantly higher than the baseline value,and serum creatinine (Scr) indicated no statistically significant difference during the follow-up period.After the treatment of 1 month,the efficient rate of MMF group was higher than the CTX group (MMF 73.5 ％ vs 42.9％,P=0.046),the effective treatment of 3,6 months at the end of the follow-up,no statistically significant difference were observed in the accumulative remission rate.The total rate of retreatment was 10.4％ (5/48),where MMF group was lower that of the than CTX group (3.0％ vs 28.6％,P＜0.001).The retreatment often occurred after discontinuation of prednisone and CTX,MMF reduction process.Eleven children received IMPDH2 gene polymorphisms test in MMF group,9 AA children had shorter time for drugs to be effective than that of the AG and GG children.The incidence of adverse reactions of MMF group was obviously lower than CTX group at the end of the follow-up (8.8％ vs 35.7％,P=0.025),where two groups developed fungal infection.Conclusions The short-term effect of both groups are the same,but the recurrent rate and incidence of adverse reactions of MMF group are lower than those of the CTX group.The preliminary study shows that IMPDH2 gene polymorphisms is associated with MMF curative effect and adverse reactions.
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Objective To investigate the clinical effect of fluticasone propionate combined with noninvasive positive pressure ventilation in the treatment of chronic obstructive pulmonary disease (COPD).Methods 88 cases of COPD patients from October 2014 to October 2016 in our hospital were selected and randomly divided into two groups of the control group observation group with 44 cases in each group.The control group was treated with conventional treatment, the observation group was treated with fluticasone propionate plus noninvasive positive pressure ventilation, and then the curative effect of the two groups was compared.Results The total effective rate of observation group was 100% higher than that of the control group 70.5%, the difference was statistically significant (P<0.05), after treatment, the observation group of FVC (2.50 +0.32) L, FEV1 (1.36 +0.20) L, FEV1%(51.23+4.32), PaO2(10.51+2.10) kPa, PaCO2(5.15 +1.19) kPa, compared with the control group, the difference was statistically significant (P<0.05), the observation group recurrence rate 4.5% was lower than the control group 13.6%, the difference was statistically significant (P <0.05).Conclusion COPD patients with fluticasone propionate combined noninvasive positive pressure ventilation, can effectively improve lung function, reduce disease recurrence rate, a significant effect.
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Objective To explore the clinical characteristics of Henoch-Sch(o)nlein purpura complicated with acute necrotizing acute pancreatitis,in order to provide information for the diagnosis and differentiation of Henoch-Scht(o)nlein purpura complicated with acute abdominal disease.Methods There was a case present with purpura,abdominal pain,massive ascites and poor spirit in Department of Pediatrics,the First Affiliated Hospital of Zhengzhou University.The clinical manifestations and physical examination results were summarized and discussed,some pediatric specialists from nephrology department,surgery department,digestive system department were invited to discuss the case.The treatment was adjusted according to result of discussion,the final diagnosis was tracked.Results The case was firstly diagnosed with Henoch-Sch(o)nlein purpura,acute diffuse peritonitis with cause in dispute.After the discussion,the intestinal necrosis,perforation could not be excluded,acute pancreatitis required further identification.Exploratory laparotomy was received because of the changing condition after discussion.The intraoperative diagnosis was acute necrotizing pancreatitis.The patient recovered well with medical treatment.Conclusion When children with Henoch-Sch(o)nlein purpura had an acute abdominal disease,acute necrotizing pancreatitis should be considered in addition to intestinal necrosis,perforation.