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.

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.

Objective:To evaluate the clinical efficacy of multi-disciplinary single center's CCCG-HB-2016 regimen in the treatment of hepatoblastoma (HB) in children.Methods:Clinical data of 36 HB patients treated with CCCG-HB-2016 program from Aug 2016 to March 2020 were analyzed.Results:These 36 patients included 20 boys and 16 girls. The serum AFP was all higher than 2 792 ng/ml,there was a correlation between AFP and tumor risk stratification ( H=14.973, P<0.05). Twenty eight cases (77.78%) were epithelial type and 8 cases (22.22%) were mixed epithelial mesenchymal type.All children were treated by tumor resection combined with chemotherapy, and there was a correlation between tumor risk stratification and surgical resection of liver lobe ( H=8.847, P<0.05). The probability of bone marrow suppression in the low-risk group was 58.33% (35/60),that in the intermediate-risk group was 73.49% (61/83) and in the high-risk group was 80.23% (69/86).All 36 cases were followed up to March 31, 2020,with an average follow-up of 21.9 months and the median survival was 22.5 months.The overall survival rate (OS) and event-free survival rate (EFS) were 97.2% and 83.3% respectively. Conclusions:The multidisciplinary CCCG-HB-2016 regimen was with a high success rate and along with a high incidence of bone marrow suppression.

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.

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.

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.

Objective To investigated the effect of procyanidin (PC) on the expression of cysteine proteinase -3 (Caspase -3) in type 2 diabetes mellitus SD rats with focal cerebral ischemia. Methods Following the random principle, 40 healthy Sprague - Dawley (SD) rats were numbered sequentially and randomly divided to normal rats with focal cerebral ischemia group,type 2 diabetes mellitus SD rats with focal cerebral ischemia group,PC low/ middle/ high -dose groups,with 8 rats in each group. The type 2 diabetes mellitus - MCAO model was set up. The doses of PC for low,middle and high - dose groups were 50 mg/ kg,100 mg/ kg,200 mg/ kg. Immunohistochemistry method was used to measure the activity of Caspase - 3. Results Compared with that in the normal rats with focal cerebral ischemia group[(11. 42 ±2. 52)],the expression of Caspase -3 increased in the type 2 diabetes with ischemia group[(15. 00 ± 2. 38)](t = 2. 17,P < 0. 01). Compared with that in the type 2 diabetes with ischemia group,the expression of Caspase - 3 decreased in the PC groups[(9. 38 ± 2. 00),(7. 71 ± 1. 55),(6. 96 ± 1. 57)](t = 2. 86,3. 13,3. 36,all P < 0. 01),whereby the middle and high - dose groups showed more significant decrease (t = 1. 92,2. 03,all P <0. 01) and with no statistically significant difference between the two groups(t = 1. 13,P > 0. 05). Conclusion PC can decrease the expression of Caspase - 3 protein in type 2 diabetes mellitus SD rats with focal cerebral ischemia, finally may inhibit the apoptosis.

Objective To compare the clinical efficacy of single utility port and multiple utility ports thoracoscopic lobectomy in the treatment of peripheral lung cancer, and to study the operation skills, relative merit and feasibility of the single utility port thoracoscopic lobectomy. Methods The clinical data was analyzed retrospectively for 223 cases with stage Ⅰ orⅡ of peripheral lung cancer who underwent thoracoscopic lobectomy from July 2011 to November 2014 in Shanxi Provincial Cancer Hospital. Among 223 cases, 78 cases received single utility port thoracoscopic lobectomy (single utility port group), 145 cases received 2 or 3 utility ports thoracoscopic lobectomy (multiple utility ports group). The clinical outcomes involved time of operation, intraoperative blood loss,chest drainage, postoperative hospital stay, stations of lymph node dissection, numbers of lymph node dissection, rate of turn to open, postoperative complications, 2-year survival rate and disease free survival rate. Results No perioperative death occurred in both groups. There was no statistical difference between single utility port group and multiple utility ports group in operation time [(157.4 ±13.6) min vs. (151.3 ±23.2) min], intraoperative blood loss [(180.77 ±59.97) ml vs.(171.31 ±77.51) ml],chest drainage [(370.26 ±146.09) ml vs. (351.17 ±159.07) ml], lymph node dissection stations (4.29±0.65 vs. 4.21±0.73), lymph node dissection number (11.50±2.30 vs. 11.04±2.29), rate of turn to open [(5.13 ％ (4/78) vs. 4.83 ％ (7/145)], incidence of postoperative complications [17.95 ％ (14/78) vs. 15.86％(23/145)], postoperative hospital stay [(8.74±0.51) d vs. (9.48±0.63) d], 2-year survival rate [96.15 ％(75/78) vs. 93.79％(136/145)] and 2-year disease free survival rate [80.77 ％ (63/78) vs. 82.07 ％ (119/145)] (all P>0.05). Conclusions The single utility port thoracoscopic lobectomy could achieve the same clinical results as the multiple utility ports. The single utility port thoracoscopic lobectomy is a safe, effective and feasible surgical procedure.

Objective To analyze the 10-year survival outcome and failure patterns for patients with nasopharyngeal carcinoma (NPC) after intensity-modulated radiotherapy (IMRT),aiming to provide reference for optimized treatment for NPC.Methods Clinical data of 866 patients with NPC receiving IMRT from January 2001 to December 2008 were retrospectively analyzed.Survival analysis was performed using the Kaplan-Meier estimator.Univariate analysis was carried out by log-rank test and multivariate analysis was performed using Cox proportional hazards model.Results The median follow-up time was 132 months.The 10-year local recurrence-free survival (LRFS),distant metastasis-free survival (DMFS),progression-free survival (PFS) and disease specific survival (DSS) were 92.0％,83.4％,75.7％ and 78.6％,respectively.A total of 210 patients died including 124 patients (59.0％) from distant metastasis,which was the primary cause of death,and 47 (22.3％) from local regional recurrence.Independent negative factors of DSS included age＞50 years (P=0.00),LDH ≥ 245 IU/L (P=0.00),Hb＜ 120 g/L (P=0.01),T2-T4 staging (P=0.00),N1-N3 staging (P=0.00) and GTV-nx＞20 cm3(P=0.00).The 10-year LRFS,DMFS and DSS of stage Ⅱ NPC patients did not significantly differ after IMRT alone and chemoradiotherapy (P=0.83,0.22,0.23).For patients with stage Ⅲ NPC,the 10-year LRFS and DSS in the chemoradiotherapy arm were significantly higher than those in the IMRT alone (P=0.01,0.01),whereas no statistical significance was observed in the DMFS between two groups (P=0.14).The overall survival of stage Ⅳa+Ⅳb NPC patients is relatively poor.Conclusions IMRT can improve the long-term survival of NPC patients.Distant metastasis is the primary failure pattern.Patients with stage Ⅰ-Ⅱ NPC can obtain satisfactory survival outcomes after IMRT alone.The addition of chemotherapy can further enhance the LRFS and DSS of stage Ⅲ NPC patients.However,the optimal therapeutic strategy remains to be urgently investigated for stage a+ Ⅳb NPC patients.