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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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Henoch-Sch?nlein purpura(HSP) is a common small vessel inflammation in childhood, and most of them have good prognosis.Due to too many inflammatory factors, the body injury will persist in some severe cases of HSP that hormone alone is difficult to improve symptoms in a short time.Recent studies have found that gamma globulin or blood purification combined with hormone can relieve clinical symptoms more quickly.Plasma exchange and hemoperfusion are commomly used.The purpose of this paper is to review the status of gamma globulin and blood purification treatment in severe HSP.
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Hemophagocytic lymphohistiocytosis(HLH)is a life-threatening inflammatory response syndrome, which progresses rapidly.Its etiology and clinical manifestations are diverse, and its diagnosis is difficult, and the rescue treatment has not been unified at home and abrord.The paper reviews the classification, pathogenesis, clinical manifestations, laboratory indexs, diagnosis and treatment of HLH, in order to provide reference for timely diagnosis and individualized treatment.
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Systemic lupus erythematosus (SLE) is a serious multisystem autoimmune disease with different clinical manifestations.Childhood-onset SLE (cSLE) is similar to adult-onset SLE, while its morbidity and mortality are higher than adults, and it is prone to damage important organs.Therefore, early diagnosis and treatment are very important.With the in-depth exploration of the pathogenesis and the development of cell and molecular biology, the progress of drug therapy for SLE has been promoted.Immunosuppression still remains the cornerstone of treatment, and glucocorticoids still plays a leading role.Biologics bring the gospel to SLE patients, and non-specific immunotherapy gains treatment time for refractory and severe SLE patients.Treatment options are led by the level of disease severity.It is of great significance to understand the treatment progress of cSLE and combine theory with practice together to control the disease activity and improve the prognosis.This article reviews recent advances regarding the update on the treatment of cSLE in recent years.
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Autoimmune hemolytic anemia is a disorder of immune function caused by various reasons, by produceing autoantibody or complement which can react with erythrocyte autoantigen, increasing the destruction of red blood cell and beyond the compensatory ability of bone marrow hematopoiesis.Children usually have acute onset and the clinical manifestations are related to the pathogenesis.As the first-line treatment of glucocorticoids, Most children respond well to glucocorticoids.Some children suffer from steroid dependence and resistance; or recurrence due to different types of antibodies, often requiring second-line treatment, such as splenectomy, immunosuppressive, etc.This article reviews the recent progress in the treatment of children with autoimmune hemolytic anemia.
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Chronic kidney disease has a high mortality but limited treatment.Although the primary causes of chronic kidney disease are varied, the final pathological outcome is renal fibrosis.Preventing the progress of renal fibrosis will help to delay the progress of chronic renal disease.In recent years, more and more studies have shown that non-coding RNAs are involved in the occurrence and development of renal fibrosis.This paper summarized the role of non-coding RNAs in the process of renal fibrosis, in order to provide a new possible target for diagnosis and treatment of chronic renal diseases.
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Vav-family proteins are guanosine nucleotide exchange factors(GEFs)and one of RhoGEFs family numbers.Vav proteins can influence cell signaling transduction, cytoskeleton recombination and adherence migration in special cells by RhoA, RhoG and Rac-1 pathway.There are 80 kinds of RhoGEFs in mammalian cell.Vav proteins play an important role in hematological system, nervous system, cardiovascular system and immune system.So the function of vav protein in immune system was reviewed in this paper.
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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.
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Cathepsin S(Cat S) is an important member of the cysteine cathepsin protease family.It is a lysosomal protease, which has been shown to be expressed in inflammatory conditions and autoimmune diseases.It has more specific roles such as antigen presentation, tissue repair, cell proliferation and apoptosis.It contributes to a variety of clinical diseases.In this review, we summarize the structure, characteristics and functions of cathepsin S in kidney and rheumatic diseases.
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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.
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Objective To investigate the dynamic changes of neutrophil gelatinase-associated lipoca-lin(NGAL) and kidney injury molecule 1(KIM-1) in children after contrast media administration and evalu-ate the effect of hydration therapy. Methods A total of 58 patients with urinary system diseases who were admitted to Shengjing Hospital of China Medical University from March 2012 to March 2014 for intravenous pyelography(IVP) in pediatric department were enrolled. The 58 patients were randomly divided into hydra-tion group of 28 patients and non-hydration group of 30 patients. Contemporaneous 24 patients received respiratory system enhanced CT examination without urinary tract diseases and hydration were enrolled as control group. Urine NGAL and KIM-1 of the three groups at 0 h,24 h,48 h,72 h,96 h after using intravenous contrast media were detected by ELISA. Serum creatinine of the three groups at 0 h,48 h,96 h after using intravenous contrast media were detected. Results All of the 82 subjects in this study didn′t occur contrast- induced acute kidney injury. The urinary NGAL of non-hydrated group significantly increased at 24 h and 48 h after contrast media administration ( P < 0. 05 ) and the urinary NGAL of hydrated group significantly increased at 48 h and 72 h(P<0. 05). But the urinary NGAL at 24 h and 48 h of the hydration group were lower than these of the non-hydrated group,there were statistically significant differences(P<0. 05). At 24 h,48 h and 72 h after contrast media administration,the level of urine KIM-1 in the non-hydration group sig-nificantly increased(P<0. 05). Urine KIM-1 at 48 h and 72 h in the hydration group significantly increased (P<0. 05). But the urine KIM-1 at 24 h,48 h and 72 h of the hydration group were lower than these of the non-hydration group,the differences were statistically significant(P<0. 05). Comparison of urine NGAL and KIM-1 at different times before and after contrast media administration in children receiving enhanced CT examination who without urinary tract disease showed no statistically significant differences ( P >0. 05 ). Conclusion The urine NGAL and KIM-1 of children with urinary system diseases increase after contrast media administration and there is a trend of spontaneous recovery. Hydration intervention can alleviate the up-ward trend of urine NGAL and KIM-1. For children receiving enhanced CT examination but without urinary system diseases,the change of urine NGAL and KIM-1 are not significant.
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Cystic kidney disease is the main disease of cystic kidney change in children. It may be caused by non_genetic fetal malformations or genetic diseases,or may be acquired rarely. Most renal cysts are usually isolated oraspart of a syndrome. However,fatal renal cystic diseases can develop from these space occupying lesions. Although renalcystic diseases are similar in presentation,they possess distinct features and variable prognosis later in life. In order to correctly diagnose this kind of disease in the early stage,it claim to accurately grasp its pathogenesis,pathology,clini_cal characteristics and radiological findings. A comprehensive analysis of common cystic kidney disease in children is carried out to help clinicians to aid in early distinction and appropriate treatment.
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Cystic kidney disease is a major disease which can cause kidney cystic change in children. Cystic kidney disease refers to a series of congenital or acquired diseases with one or multiple cysts in the kidney due to different causes. With the development and wide application of ultrasound technology,it is better than CT and magnetic resonance imaging in reflecting the renal cystic disease,and more and more recognized by pediatricians. Now,the ultrasonographic findings of common cystic kidney disease in children were summarized and analyzed,in order to provide help for clinical diagnosis.
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Objective To investigate the changes of B cell-activating factor (BAFF) and a proliferation-inducing ligand (APRIL) in serum of children with Hen(o)ch-Sch(O)nlein 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 Hen(O)ch-Sch(O)nlein 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.
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Objective To investigate the expression of N-myc downstream regulated gene-2 (NDRG2) on renal fibrosis in unilateral ureteral obstruction(UUO) rat model and the mechanism of renal fibrosis.Methods Forty-eight male Wistar rats (120-150g)were randomly divided into two groups:the sham-operation group (n =24)underwent the left ureteral dissection,the UUO group (n =24)underwent the left ureteral ligation.At the 3,7,14,21day after the operation,6 rats from each of the group were sacrificed and the obstructive kidneys were collected.The histopathological changes were observed through HE and Masson staining.E-cadherin and α-SMA were detected by Western blot and immunohistochemistry.NDRG2 were detected by Western blot,immunohistochemistry and real-time PCR.Results There is not diffenrence in the sham group at anytime.Compared with the sham-group,the fibrosis was obvious in UUO group.The protein of E-cadherin decreased(P ＜ 0.05) and α-SMA increased(P ＜0.05).The expression of NDRG2 in UUO group was lower than that in the sham group.The OD of immunohistochemistry was detected by ImagePro Plus 6.0.OD of NDRG2 was the lowest[(14.33 ± 2.45) x 10-3] in 21d group.The western blot showed that the ratio of NDRG2 to β-actin was the lowest(0.03 ± 0.01) in 21d group.The mRNA of NDRG2 decreased obverously in UUO group (P ＜ 0.05).Conclusion NDRG2 decreases in UUO group and NDRG2 might be involved in the pathologic process of renal fibrosis.
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Objective To explore the application of urodynamics in the diagnosis and treatment of nonmonosymp-tomatic nocturnal enuresis(NMNE)in children,and to provide some evidence for its clinical treatment. Methods Patients who were clinically diagnosed as NMNE at Department of Pediatrics Kidney Rheumatism and Pediatric Urology of Shengjing Hospital of China Medical University from January 2015 to August 2016 were collected. Sixty - six children with NMNE were included in the urodynamic study,and the different treatments were selected according to the results of the urodynamics and the efficacy was evaluated. Results The urodynamic results of 61 patients(92. 43％)were abnor-mal,and 5 cases(7. 57％)had no abnormalities. There were 35 cases with overactive bladder(OAB)in abnormal re-sults,with a remarkable decrease in bladder pressure capacity in 7 cases,detrusorsphincter discoordination(DSD)in 1 case,and OAB with other abnormal results in 18 cases. OAB was found in 53 cases,and the detection rate was 86. 89％(53 / 61 cases),accounting for 80. 3％(53 / 66 cases)of the total samples. The desmopressin acetate combined with Tolterodine tartrate was used to treat OAB patients,and its total effective rate was 94. 34％(50 / 53 cases). The biofeed-back treatment had effect on DSD child. The patients with a marked decrease in bladder pressure capacity and with the normal urodynamics received desmopressin acetate joint behavior training treatment,and the efficiency of the therapy was 71. 43％(5 / 7 cases)and 80. 00％(4 / 5 cases),respectively. Conclusions The urodynamics study can uncover the pathophysiological mechanism of NMNE,so this examination is essential for NMNE children to improve the treat-ment. Since the rate of OAB in NMNE is very high,it is suggested that anticholinergic medications combined with desmopressin can be used in the initial treatment of the NMNE patients to improve the rate of cure and prognosis.
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Objective To investigate the expression of β-arrestin2 and microtubule-associated pro-tein light chain(LC)3 in renal of rat with acute renal ischemia reperfusion injury,and to analyze the relation-ship between them and renal injury. Methods Fifty-four male SD rat(3-4 weeks old) were randomly divid-ed into three groups:control group,sham group,acute ischemic reperfusion injury group. We established the acute renal ischemia reperfusion injury model through removing the right kidney and clamping the left renal for 45 minutes with noninvasive arterial clip. We obtained the kidney and blood samples respectively at 12 h, 24 h,36 h,48 h,72 h,96 h after the surgery. Expressions ofβ-arrestin2 and LC3 protein were detected by the immunohistochemistry method and Western blot method. The renal function and morphological changes were assessed. Results Compared with control group and sham group,the serum creatinine and kidney pathologi-cal grading of acute ischemia reperfusion injury group obviously rised. The kidney injury was the most serious at the 24 h after acute ischemic reperfusion injury. The expressions of β-arrestin2 and LC3 were little in the control group and sham group. However,the expressions of these two indicators were obviously higher and reached the peak at the 12 h after acute ischemia reperfusion injury. All these results suggested that the chan-ges of these two indicators were anterior to the histopathological changes. The expressions ofβ-arrestin 2 and LC3 protein were in positive correlation with the kidney injury(r=0. 821,P<0. 05;r=0. 913,P<0. 05). Conclusion In the acute renal ischemia-reperfusion injury,β-arrestin2 may be as a kind of upstream regula-tory protein involving in the kidney pathological process through the regulation of the autophagy.
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Objective To summarize the clinical and pathogenic characteristics of urinary tract infection(UTI)in infants,and to provide reference for clinical diagnosis and treatment.Methods One hundred and eighty-eight cases of hospitalized infants with UTI diagnosis standard in Department of Pediatric Renal Rheumatism Immunology,Shengjing Hospital Affiliated to China Medical University from January 2012 to January 2015 were collected,and on the basis of the imaging they were divided into complex group(complicated UTIs) and non-complex group(non-complicated UTIs),the differences between 2 groups in the general condition,clinical manifestations,serological examination,urine culture and distribution of pathogenic bacteria and drug sensitivity results were analyzed.Results Among 188 UTI infants,148 were male and 40 were female,50 cases were in complex group (26.6％),and 138 cases were in non-complex group (73.4％).In complex group,the prevalence of prenatal ultrasound abnormalities (34.0％),the incidence of recurrent infections(24.0％),the number of fever incidence(78.0％),and peripheral WBC count [(16.4 ± 4.3) × 109/L] were higher than those in non-complex group[1.5％,10.9％,58.7％,(14.6 ± 3.5) × 109/L] (all P ＜ 0.05),and the naked eye hematuria incidence was lower than that of the non-complex group (14.0％ vs 34.8％,P =0.006).In complex group,pneumonia klebsiella bacteria positive rate was higher than that of non-complex group (22.0％ vs 2.9％,P =0.000),and the positive rate of Escherichia coli was lower than that of non-complex group(26.0％ vs 46.4％,P =0.000).There was no difference in the drug sensitivity and resistance of the pathogenic bacteria in the urine culture of 2 groups.Conclusions For fever,increased peripheral WBC,repeated infection and urine culture for Klebsiella pneumoniae UTI infants,in particular,should be alert to the prese-nce of urinary tract abnormalities,timely improve the urinary system ultrasound and urinary tract contrast is very necessary.
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Paired box2 ( PAX2 ) is a transciption factor which mainly expressed in the developing kid-ney. Researches indicate that PAX2 promote the transcription through interactions with the adaptor PAX transac-tivation domain interacting protein(PTIP). Otherwise,PAX2 protein can lead to chromatin compaction and gene silencing through interactions with Grg4. PAX2 reexpressed in acute kidney injury and involved in promoting cell proliferation. Congenital PAX2 gene mutation is closely related to congenital abnormalies of the kidney and uri-nary tract. In chronic kidney disease,PAX2 promote proliferation and cyst formation. Here,the recent researches on the function of PAX2 and its role in acute kidney injury and chronic kidney disease are reviewed.