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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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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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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 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.
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Objective To investigate the risk factors for occult pneumonia(OP) in children with primary nephrotic syndrome(PNS).Methods The clinical data of 115 children with PNS and findings of chest CT from July 2010 to June 2016 at the First Affiliated Hospital of Zhengzhou University were retrospectively analyzed.Based on the findings of chest CT,the subjects were divided into 2 groups:OP group and unoccult pneumonia (UOP) group.The comparisons were made between 2 groups,including gender,age,season,course of disease before admitting to hospital,formation of ascites,white blood cells,C-reactive protein,erythrocyte sedimentation rate,total protein (TP),albumin (ALB),total cholesterol,immunoglobulin G (IgG),immunoglobulin E,urine N-acetyl-beta-D-glucosaminidase (NAG) and 24 h urinary protein quantity/body weight.The single factor analysis was performed to analyze above indicators between 2 groups,and the indicators which had statistical significance were analyzed by single factor analysis were analyzed by the multifactor Logistic regression.The receiver operator characteristic (ROC) curve was drawn to evaluate the predicting ability of the indicators for PNS combined with OP.Results Among 1 15 cases,68 (59.1％) PNS patients were complicated with OP.The result of single factor analysis indicated that the risk factors were the formation of ascites,TP,ALB,IgG and NAG (all P ＜0.05).The multifactor Logistic regression showed that ascites,TP and ALB were the risk factors for OP in children with PNS(P =0.003,0.004,0.003).The area under curve (AUC) of ALB was 0.709,and the critical value was 18.55 g/L(P =0.000);the AUC of TP was 0.658,and the critical value was 39.15 g/L(P =0.004).Conclusion The incidence rate of PNS combined with OP was high.With the presence of formation of ascites,TP ＜39.15 g/L and ALB ＜ 18.55 g/L,it may indicate OP for the PNS children which require special consideration clinically and earlier chest CT examination.
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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 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 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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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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Kinesio Taping therapy is commonly used in sports injury, and has been focused in central neurological rehabilitation in re-cent years. This article reviewed the efficacy, mechanism of Kinesio Taping in central neurological rehabilitation.
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Objective To investigate the correlation between food intolerance and Henoch-Sch(o)nlein purpura (HSP) in children and the efficacy of food forbidden or alternative therapy. Methods The levels of IgG against several common food in serum obtained from 40 children with HSP were measured by ELISA. The efficacy of food forbidden or alternative food therapy was assessed after 3 months. Results Total positive rate of serum food-intolerant IgG antibodies in HSP children was 92.5%. Among these 14 intolerant foods, the positive percentage of egg was the highest (33.8%), followed by tomato (14.9%), milk (13.5%) and morrhua (12.2%). Significant differences of the sort and degree of food intolerance existed among different age groups (t=2.257, P=0.045), but not between boys and girls (t=1.053, P=0.315), city and countryside (t= 1.388, P=0.193). There was no linear correlation between total food intolerance and serum IgG level (t=0.793, P=0.445). Food intolerance had no direct relation to immune complex deposition in kidneys of HSP nephritis (r =-0.262, P =0.387). The efficacy of adjusting diet was 95% . Conclusions HSP is closely related to food intolerance. Egg is the most common intolerant food. Food forbidden or alternative therapy shows acceptable efficacy in the treatment of most Henoch-Sch(o)nlein purpura children.