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Objective:To compare the clinical efficacy and safety of hemoperfusion (HP) and gammaglobulin on the treatment of Henoch-Sch?nlein purpura (HSP) with gastrointestinal bleeding in children.Methods:Case-control study.A total of 39 HSP children combined with gastrointestinal bleeding diagnosed in the Department of Pediatric Nephrology, Rheumatology and Immunology, Shengjing Hospital of China Medical University from January 2015 to December 2019 were retrospectively recruited.They were divided into the HP group and the gammaglobulin group according to the therapeutic strategy.Clinical data were collected, and a 6-month follow-up survey was conducted for monitoring the relapse of gastrointestinal bleeding and the occurrence of kidney injury.The differences between groups were compared by Fisher′s exact test, two independent samples t-test, Mann-Whitney U-test, Kruskal-Wallis H-test, and One-Way ANOVA. Results:(1) There were 20 cases in the HP group and 19 cases were included in the gammaglobulin group.The gammaglobulin group was younger than the HP treatment group.(2) In addition to gastrointestinal bleeding, children in both groups had other clinical symptoms, such as abdominal pain, angioneurotic edema, and hematuria.(3)Comparison of laboratory indexes: Inflammatory indexes: white blood cell count (WBC), C-creative protein (CRP) and coagulation function indexes: fibrin degradation products (FDP), D-dimer (DD) were significantly elevated before treatment in the 2 groups, and there was no difference between the 2 groups ( P>0.05); WBC, CRP and FDP, DD declined in the 2 groups after treatment compared with the former, and there was no difference between the 2 groups ( P>0.05); (4) Comparison of clinical manifestations: when HP was applied with gammaglobulin in the treatment window within 3 d, the difference in the time of abdominal pain relief in the HP group was shorter than that of the gammaglobulin group [1.00(1.00, 1.00) d vs.2.00(1.75, 6.50) d, P=0.011]; comparing the time of gastrointestinal bleeding stopping when HP was applied with gammaglobulin comparison, the difference in gastrointestinal bleeding cessation time was not statistically significant ( P>0.05); (5) Comparison of hospitalization time: within 3 d application of HP compared with other window period hospitalization time were significantly reduced [(16.89±4.99) d than (19.20±2.39) d than (34.83±8.40) d, both P<0.05]; (6) Comparison of hospitalization costs: within 3 d application of HP compared with other window period hospitalization costs were significantly reduced [25 554.03 (22 168.61, 28 527.30) yuan than 33 619.48 (32 661.18, 36 971.47) yuan than 51 290.34 (34 163.04, 64 772.66) yuan, both P<0.05]; There were no statistically significant difference in the hospitalization time and hospitalization cost between and within the gammaglobulin group (all P>0.05); (7) Comparison of hormone dosages: the difference in the results of the initial dose of hormone use, pre-treatment dose of gammaglobulin/HP, and post-treatment dose of gammaglobulin/HP between the two groups of children was not statistically significant(all P>0.05). Safety profile was comparable between groups.The difference in hormone dosage before and after treatment within the gammaglobulin and HP treatment group was statistically different ( P<0.001). Conclusions:For children with severe HSP accompanied by gastrointestinal bleeding, early treatment with blood purification can rapidly relieve clinical symptoms and reduce the number of hospital days and hospitalization costs.For cases where blood purification is not available or suitable, gammaglobulin treatment is another option.
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Objective:To summarize the diagnosis and treatment process of abdominal involvement in 229 children with IgA vasculitis and to provide reference for clinic treatment.Methods:A total of 229 pediatric patients, diagnosed as IgA vasculitis with abdominal involvement admitted to the Department of Pediatric Nephrology of Shengjing Hospital, China Medical University from January 1st 2018 to December 31st 2019, were retrospectively analyzed in the study and were divided into three groups according to Numerical Rating Scale to compare indexes in different degrees of abdominal pain.Results:The duration of hospitalization was related with degree of abdominal pain, as the more severe the abdominal pain was, the longer the hospitalization time was( P<0.001). The incidence of bloody stool were also proportionate to the degree of abdominal pain( P<0.001). With the aggravation of abdominal pain, the proportion of intestinal wall edema increased, as the highest proportion was severe group( P<0.001). The proportion of renal involvement in severe group was significantly higher than that in non-severe group( P<0.001). Twenty cases of intestinal wall edema with decreasing of albumin were treated by intravenous hormone therapy after albumin infusion as the results of no intestinal complications occurred.Compared with the mild and moderate groups, the white blood cells of the severe group were higher( P<0.001)and the albumin was lower( P<0.05). It was no significant difference in hemoglobin, serum amylase and serum lipase among three groups.The mean value of CRP had no difference among three groups and was higher than that of normal.Interleukin(IL)-6 in severe group was higher than that in other two groups( P<0.05), but there was no significant difference in IL-2, IL-4, IL-10, IL-17 and tumor necrosis factor.In terms of treatment, 40 cases were treated with immunoglobulin and four cases with hemoperfusion.The average duration of intravenous glucocorticoid application was related to the degree of abdominal pain among three groups.The longest duration was severe group(16.00±6.91)d and the shortest one was mild group(6.71±3.75)d. Conclusion:Pediatric patients diagnosed as IgA vasculitis with severe abdominal pain whose part of inflammatory indexes increased and albumin decreased obviously should complete imaging examinations to evaluate the extent of intestinal wall edema.If diagnosed as hypoalbuminemia and intestinal wall edema distinctly, hormone therapy should be given after albumin infusion to prevent severe complications such as intestinal perforation.For pediatric patients of IgA vasculitis with severe abdominal symptoms, on the basis of hormone therapy, immunoglobulin and hemoperfusion could be used to quickly remove abnormal immune substances to slow down the disease.
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Objective:To summarize the characteristics of methylmalonic acidemia(MMA) with thrombotic microangiopathy as the prominent manifestation.Methods:Clinical data was collected from MMA patients with thrombotic microangiopathy as the prominent manifestation, who were treated at Shengjing Hospital of China Medical University from September 2014 to December 2019.The clinical manifestations, laboratory results, imaging results, histopathological examination, treatment and prognosis were analyzed.Results:Six children aged 1 month to 7 years old were included in our study, including 5 males and 1 female.Six cases were all MMA complicated hyperhomocysteinemia.All patients had a history of prodromal infection before admission.All cases had edema.Five patients were hypertenson and 4 patients accompanied neurologic presentation.One patient had a family history.All patients were treated with Vitamin B 12, levocarnitine, folic acid and betaine.Three patients′ psychiatric symptoms and renal function were improved and hemolysis were controlled.Two of them were similar with the same age children in intelligence and motor development, with normal renal function, good blood pressure control and normal urine protein.The third one was significantly improved in intelligence development, but still slightly worse than that of the children with the same age.He had no convulsions occurred in recent one year with antiepileptic drugs in oral.His blood pressure was maintained between 135/90-160/110 mmHg(1 mmHg=0.133 kPa), 24-hour urine volume was about 2 000 mL, creatinine was maintained at about 150 μmol/L, ionic level was normal, and urinary protein was 2 + .One case discharged from hospital.Two patients died of multiple organ failure. Conclusion:An early screening of metabolic diseases should be achieved for thrombotic microangiopathy of unknown etiology.Early screening of homocysteine is important for early detection and treatment.
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Nephrotic syndrome is a common glomerular disease in childhood and easy to recur.It has been found that children with nephrotic syndrome are often accompanied by atopic manifestations and there is high co-morbidity between nephrotic syndrome and atopic diseases.This article reviewed the pathogenesis, cytokines and treatment of nephrotic syndrome and atopic diseases in children to explore the similarity between them to provide new clues and methods for the treatment of nephrotic syndrome in children.
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Nephrotic syndrome is a common glomerular disease in childhood and easy to recur.It has been found that children with nephrotic syndrome are often accompanied by atopic manifestations and there is high co-morbidity between nephrotic syndrome and atopic diseases.This article reviewed the pathogenesis, cytokines and treatment of nephrotic syndrome and atopic diseases in children to explore the similarity between them to provide new clues and methods for the treatment of nephrotic syndrome in children.
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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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Objective To examine the clinical features and long - term outcome of pediatric IgA nephropathy and to explore the clinical effect of Mycophenolate Mofetil(MMF)and Cyclophosphomide(CTX)in children with IgA nephropathy with nephrotic syndrome(NS). Methods A single - centre,retrospective,observational study of 115 chil-dren with IgA nephropathy from 2004 to 2013 in Pediatric Nephrology of Shengjing Hospital of China Medical University was conducted. Demographic and clinical data were reviewed retrospectively for age,sex,medical history,presenting symptoms,medications,follow - up duration and the responsiveness to treatment. Results In all children,NS occurred in 20(17. 4% ). There were 35 patients(30. 4% )with non - NS and 60 patients(52. 2% )with isolated hematuria. No special treatment in patients with IgA nephropathy with isolated hematuria. Among patients with proteinuria less than 20 mg/(kg·d),12 patients were treated with angiotensin - converting - enzyme - inhibitor(ACEI),8 patients were trea-ted with ACEI and corticosteroid. At all time points,mean proteinuria was significantly decreased in ACEI and cortico-steroid group compared with ACEI group(P ﹤ 0. 001). Patients with 20 - 49 mg/(kg·d)proteinuria were treated with ACEI and corticosteroid. At all time points,mean proteinuria was significantly decreased compared with the prior time point. Patients with NS were treated with MMF and corticosteroid or CTX and corticosteroid. Eleven patients were trea-ted with MMF,9 patients were treated with CTX. A significantly difference was seen after 3 months in proteinuria greater decrease from pretreatment in CTX group than those in MMF group(P ﹤ 0. 001). No significant difference in proteinuria was observed at other time point. No significant change in white blood cell count was observed in MMF group and CTX group. No serious complication developed in any patient during treatment. During the median follow - up of 35. 2 months (range 4. 0 - 124. 6 months),no patient progressed to end stage renal disease. Conclusions IgA nephropathy patients with isolated hematuria should be long - term followed up. Children with non - nephrotic - range proteinuria should be treated with ACEI or corticosteroid. Patients with NS should be treated with corticosteroid and MMF or CTX. The long -term prognosis within 3 - 5 years should be good if proteinuria within normal range in pediatric IgA nephropathy pa-tients.
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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.
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Objective To identify uropathogens responsible for urinary tract infection in children less than 5 years of age and determine the antibiograms.Methods The data of 523 children(2 months to 5 years old) admitted at the Shengjing Hospital of China Medical University from January 2008 to December 2013 were studied retrospectively.Results Out of 523 children suffering from urinary tract infection,54 (10.3％) were complicated urinary tract infection,including 24 vesicoureteral reflux,8 ureter-pelvic junction stenosis,5 hydronephrosis,4 double kidneys,2 renal dysplasia,2 bladder diverticula,2 bladder ear,2 neurogenic bladder,1 urethral vaginal fistula,1 congenital megaureter,1 horseshoe kidney,and 1 Ureteral cyst and stone.A total of 487 cases underwent urine culture,207 (42.5 ％) had positive bacterial growth,the gramnegative bacteria accounted for 94.69％,gram-positive bacteria 5.31％.E coli was the most common uropathogens in gram-negative bacteria (79.23 ％),the second was Klebsiella (5.31％),the third was Proteus mirabilis(2.90％).Gram-positive bacteria was almost Enterococcus (4.35％).Twenty one strains were extended-spectrum beta-lactamase enzyme positive(ESBLs +),and they were sensitive to imipenem,amikacin and piperacillin/tazobactam.Conclusion The clinical features were atypical in children with urinary tract infection,we should investigate the underlying causes such as urinary anomalies or stones.E coli was still the most common uropathogens in children with urinary tract infection,the empirical therapy should according to the patient's conditions while awaiting the culture and sensitivity results.
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Objective To compare the treatment response of plasma exchange and immunoadsorption for children suffering from severe systemic lupus erythematosus (SLE),and then find the more advantageous treatment method.Methods Between March 2007 and March 2013,27 children with severe SLE were collected from the Department of Pediatric Nephrology and Rheumatology of Shengjing Hospital of China Medical University.Part of them about 11 children accepted plasma exchange treatment (plasma exchang group) and the others accepted immunoadsorption(immunoadsorption group).The clinical features,ANA,IgG,serum ions,the cost of treatment and the hospitalization time were reviewed,and the comparative analysis were performed in two groups.Results There were comparabilities between plasma exchange group and immunoadsorption group in age,gender,couse of disease and systemic lupus erythematosus disease activity index(SLEDAI) score before treatment.(1)In plasma exchang group,11 children were conducted 26 times treatment.The SLEDAI score significantly decreased after plasma exchange (19.00 ± 3.77 vs 5.34 ± 4.35,P ＜0.05),and the ANA and IgG significantly decreased as well[2 439.58 ± 1 430.56 vs 303.54 ± 169.32; (8.35 ± 5.67) g/L vs (4.04 ± 2.23) g/L,P ＜ 0.05].(2) There were 16 children in immunoadsorption group,they accepted immunoadsorption treatment about 44 times.The SLEDAI score after immunoadsorption significantly decreased (18.25 ± 4.62 vs 4.25 ± 2.23,P ＜ 0.05),and the ANA and IgG significantly decreased as well [2 560.39 ± 1 563.78 vs 289.62 ± 137.62 ; (9.98 ± 6.03) g/L vs (3.23 ± 1.37) g/L,P ＜0.05].(3) There were no statistical differences in the value of SLEDAI score,ANA and IgG after the treatment between two groups.(4) The concentrations of serum potassium,sodium and chlorine and calcium in the children treated by plasma exchange or immunoadsorption were consistent with the original.(5) The hospitalization expense in plasma exchange group was distinctly higher than that of immunoadsorption group (P ＜ 0.05).(6) There was no significant difference in the length of hospitalization between two groups [(33.6 ± 8.60) d vs (31.9 ± 14.6) d,P ＞ 0.05].Conclusion The treatment both plasma exchange and immunoadsorption were effective methods for the children with severe SLE.However,the cost of plasma exchange was much higher and had a great influence on the concentration of antihypertensive drugs.
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Biological agents has opened a new chapter in the targeted therapy of rheumatism,and provides a new choice for the rheumatism children who is refractory to traditional disease-modifying antirheumatic drugs.This article described the classification,application,efficency,side effects and precautions of the cytokines antagonists and the cell targeting biological agents,to provide a reference for rational application in clinical work.
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Objective To investigate the mechanism of antioxygen reaction of epigallocatechin-3-gallate (EGCG) in renal tubular epithelial cells of rats with oxidative stress induced by H2O2. Methods Cultured cells were divided into control group, H2O2 group and EGCG group. Cell survival was observed with MTT. The expressions of Nrf2 mRNA and -γ-GCS mRNA in cultured cells were examined by real time quantitative PCR. Immunohistochemistry and western blotting were used to detecte the expressions of Nrf2 and γ-GCS protein. Results The survival rate of tubular cells was 97. 61 ± 6.33 in control group. There was a significant decrease in H2 O2 group (56. 38 ± 5.57) (P < 0.01), while increased when the EGCG concentration were 5,10,20 mg/L(77.42 ±5.31,83.27 ±5.94,90.24 ±5.72) (P <0.05,P <0.01). EGCG up-regulated the expressions of Nrf2 and γ-GCS mRNA and protein in renal tubular epithelial cells with dose depen-dentment. Conclusion The expressions of Nrf2 and-γ-GCS increase in renal tubular epithelial cells with oxidative stress. Resulting from suppression of oxidative stress,EGCG exerts protective effect on NRK,and this antioxidative effect may be partly induced by activating the Nrf2 signal pathway.