The single nucleotide polymorphism rs1814521 in long non-coding RNA ADGRG3 associates with the susceptibility to silicosis: a multi-stage study

BACKGROUND@#This study aimed to evaluate the correlation between long non-coding RNA (lncRNA)-related single nucleotide polymorphisms (SNPs) and susceptibility to silicosis.@*METHODS@#First, RNA-sequencing (RNA-seq) data were comprehensively analyzed in the peripheral blood lymphocytes of eight participants (four silicosis cases and four healthy controls) exposed to silica dust to identify differentially expressed lncRNAs (DE-lncRNAs). The functional SNPs in the identified DE-lncRNAs were then identified using several databases. Finally, the association between functional SNPs and susceptibility to silicosis was evaluated by a two-stage case-control study. The SNPs of 155 silicosis cases and 141 healthy silica-exposed controls were screened by genome-wide association study (GWAS), and the candidate SNPs of 194 silicosis cases and 235 healthy silica-exposed controls were validated by genotyping using the improved Mutiligase Detection Reaction (iMLDR) system.@*RESULTS@#A total of 76 DE-lncRNAs were identified by RNA-seq data analysis (cut-offs: fold change > 2 or fold change < 0.5, P < 0.05), while 127 functional SNPs among those 76 DE-lncRNAs were identified through multiple public databases. Furthermore, five SNPs were found to be significantly correlated with the risk of silicosis by GWAS screening (P < 0.05), while the results of GWAS and iMLDR validation indicated that the variant A allele of rs1814521 was associated with a reduced risk of silicosis (OR = 0.76, 95% CI = 0.62-0.94, P = 0.011).@*CONCLUSION@#The presence of the SNP rs1814521 in the lncRNA ADGRG3 is associated with susceptibility to silicosis. Moreover, ADGRG3 was found to be lowly expressed in silicosis cases. The underlying biological mechanisms by which lncRNA ADGRG3 and rs1814521 regulate the development of silicosis need further study.

Clinical and prognostic analysis of rhabdomyosarcoma in infants: a single-center report / 中华实用儿科临床杂志

Objective:To analyze the clinical characteristics and prognosis of infants with rhabdomyosarcoma (RMS), thus enhancing the understanding of this disease.Methods:Clinical data of all RMS patients younger than 12 months treated in the Hematology Oncology Center, Beijing Children′s Hospital, Capital Medical University from May 2006 to June 2019 were retrospectively analyzed, including the age, gender, histological type, tumor primary site, tumor size, and the prognosis.Patients were followed up until December 31, 2019.The 3-year event free survival (EFS) rate of children was performed by plotting the Kaplan-Meier survival curves.Results:A total of 15 RMS children younger than 12 months were enrolled, accounting for 4.9% of all RMS cases in the same period, including 6 males and 9 females.The median age at diagnosis was 7.0 months (3.0-11.5 months). Classified by the primary site, 40.0% (6 cases) located in the head and neck, followed by 26.7% (4 cases) located in the limbs, 26.7% (4 cases) located in other parts, and 6.7% (1 case) located in the urogenital system.Embryonal RMS, alveolar RMS and spindle cell RMS accounted for 46.6% (7 cases), 26.7% (4 cases), and 26.7% (4 cases), respectively.Ten cases (66.7%) were stage Intergroup Rhabdomyosarcoma Study (IRS)-Ⅲ and 1 case (6.7%) was in stage Ⅳ.There were 10 cases (66.6%) in the middle-risk group, 4 cases (26.7%) in the low-risk group, and 1 case (6.7%) in the high-risk group.Two cases had a larger than 5 cm primary tumor; lymph node involvement was confirmed in 3 cases, and pulmonary metastasis occurred in 1 case at the time of diagnosis.All children were treated with chemotherapy, and 13 cases received postoperative chemotherapy and 1 case received preoperative chemotherapy.One case were not operated.Only 3 children underwent radiotherapy, including 1 case underwent particle implantation and 2 cases received external radiotherapy.Among the 15 children with RMS, 5 cases had relapse and disease progression with the 3-year EFS rate of (59.1±14.5)%, and 2 died with the 3-year overall survival rate of (80.8±12.6)%.Conclusions:The median age of diagnosis of RMS in single-center infants is 7 months.Head and neck are the most common primary sites of RMS.Nearly 50% of the children have the primary site of RMS with poor prognosis.More than a quarter of the pathological subtypes are the spindle cell type.Local treatment significantly influences the local progression or recurrence of RMS.

The effect of steatotic donor livers on the prognosis of donors and recipients after pediatric living donor liver transplantation / 中华外科杂志

Objectives: To evaluate the effects of steatotic donor livers on the safety of donors and the prognosis of donors and recipients in pediatric living donor liver transplantation. Methods: A total of 814 pediatric living donor liver transplantations were performed between January 2013 and December 2020 at Department of Pediatric Organ Transplantation,Tianjin First Central Hospital.The clinical data were collected and a retrospective study was conducted.The recipients and the donors were divided into non-steatotic donor liver group(n=733) and steatotic donor liver group(n=81) according to whether the donor graft had steatosis. The recipients and the donors in the steatotic donor liver group were further divided into mild and moderate steatosis groups based on the degree of liver steatosis.Among the donors of non-steatosis donor group,there were 307 males and 426 females,with a median age of 30 years(range:18 to 57 years);the recipients included 351 males and 382 females,with a median age of 7 months(range:4 month to 14 years).Among the donors of steatosis donor group,there were 41 males and 40 females,with a median age of 31 years(range:22 to 51 years);the recipients included 34 males and 47 females,with a median age of 8 months(range:5 months to 11 years).The donors and the recipients were followed up regularly by means of outpatient reexamination and questionnaire survey after operation.Statistical analysis of data between groups was performed using t-test,Wilcoxon rank-sum test,repeated measures ANOVA,χ2 test,or Fisher's exact test,respectively.The survival curves of recipients and grafts in different groups were created by Kaplan-Meier method,and the survival rates of the steatotic donor liver group and the non-steatotic donor liver group were compared by Log-rank method. Results: There was no significant difference in the gender of donors in both groups (P=0.132).There were significant differences in the age and blood type distribution as well as body weight and body mass index(all P<0.05) between the two groups.No significant difference was seen in the recovery of liver function markers ALT and AST at 1,2,5 days and 1 month after operation (all P>0.05) between the two groups.The steatotic donor liver group showed longer operation time ((294±75) minutes vs. (264±81) minutes; t=3.149,P=0.002),increased incidence of postoperative biliary leakage (3.7%(3/81) vs. 0.5% (4/733); P=0.025) and delayed incision healing (7.4%(6/81) vs. 2.0%(15/733); P=0.013).There were no significant differences in gender,age,blood type distribution,height,weight and pediatric end-stage liver disease score of recipients between the two groups (all P>0.05).As compared to the non-steatotic donor liver group,the steatotic donor liver group showed similar levels of ALT, AST and total bilirubin within 2 weeks after operation(all P>0.05). The cumulative recipient survival rates in both groups were both 96.3%,the cumulative graft survival rates were 96.3% and 95.5%,respectively,without significant difference(both P>0.05). No statistical difference was observed in the incidence of major complications between the two groups (all P>0.05). There was no significant difference in the recovery of liver function markers of donors and recipients between mild and moderate steatosis groups(all P>0.05).The cumulative recipient survival rates were both 95.9% and the cumulative graft survival rates were both 100% in mild and moderate steatosis groups,without significant difference(P=0.592). Conclusions: The application of mild to moderate steatotic donor livers in pediatric living donor liver transplantation may prolong the operation time of donors,increase the incidence of complications such as biliary leakage and delayed incision healing. But there is no significant impact of mild to moderate steatotic donor livers on the overall postoperative recovery of donors and recipients,and the prognosis is ideal.

CD36 gene deletion reduces muscle insulin sensitivity in mice by up-regulating PTP1B expression / 南方医科大学学报

OBJECTIVE@#To investigate the effect CD36 deficiency on muscle insulin signaling in mice fed a normal-fat diet and explore the possible mechanism.@*METHODS@#Wild-type (WT) mice and systemic CD36 knockout (CD36-/-) mice with normal feeding for 14 weeks (n=12) were subjected to insulin tolerance test (ITT) after intraperitoneal injection with insulin (1 U/kg). Real-time PCR was used to detect the mRNA expressions of insulin receptor (IR), insulin receptor substrate 1/2 (IRS1/2) and protein tyrosine phosphatase 1B (PTP1B), and Western blotting was performed to detect the protein expressions of AKT, IR, IRS1/2 and PTP1B in the muscle tissues of the mice. Tyrosine phosphorylation of IR and IRS1 and histone acetylation of PTP1B promoter in muscle tissues were detected using co-immunoprecipitation (Co-IP) and chromatin immunoprecipitation (ChIP), respectively.@*RESULTS@#CD36-/- mice showed significantly lowered insulin sensitivity with obviously decreased area under the insulin tolerance curve in comparison with the WT mice (P < 0.05). CD36-/- mice also had significantly higher serum insulin concentration and HOMA-IR than WT mice (P < 0.05). Western blotting showed that the p-AKT/AKT ratio in the muscle tissues was significantly decreased in CD36-/- mice as compared with the WT mice (P < 0.01). No significant differences were found in mRNA and protein levels of IR, IRS1 and IRS2 in the muscle tissues between WT and CD36-/- mice (P>0.05). In the muscle tissue of CD36-/- mice, tyrosine phosphorylation levels of IR and IRS1 were significantly decreased (P < 0.05), and the mRNA and protein levels of PTP1B (P < 0.05) and histone acetylation level of PTP1B promoters (P < 0.01) were significantly increased as compared with those in the WT mice. Intraperitoneal injection of claramine, a PTP1B inhibitor, effectively improved the impairment of insulin sensitivity in CD36-/- mice.@*CONCLUSION@#CD36 is essential for maintaining muscle insulin sensitivity under physiological conditions, and CD36 gene deletion in mice causes impaired insulin sensitivity by up-regulating muscle PTP1B expression, which results in detyrosine phosphorylation of IR and IRS1.

Prevalence and risk factors of pterygium in Zhuang and Miao nationality adults aged 40 and over in Wenshan prefecture, Yunnan Province / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM: To investigate the prevalence and risk factors of pterygium in Zhuang and Miao nationality adults aged 40 and over in Wenshan prefecture, Yunnan Province.METHODS: An epidemiological survey of Zhuang and Miao nationality in Yunnan Province was conducted by using the portable slit-lamp and Keratograph-D eye surface analyzer. The Logistic regression analysis was operated to determine the impact of gender, age, occupation, outdoor activities and nationality on pterygium.RESULTS: Totally 1 239 participants were included from March to November 2019, 437 people had pterygium in one or both eyes, and the total prevalence was 35.27%. Pterygium of both eyes accounted for 59.27% while pterygium of single eye accounted for 40.73%. The prevalence of pterygium was 34.84%(224 people)in Zhuang population and 35.74%(213 people)in Miao population, there was no significant difference between the two groups(<i>P</i>=0.740). According to the result of Logistic regression analysis, opening of meibomian is the influencing factor of pterygium(<i>P</i>=0.019), with <i>OR</i>=1.348. Gender, age, education, outdoor activities, hypertension and eating habits were not the influencing factors of pterygium. Logistic regression was used to analyze the invasion degree of pterygium. The results showed that the height of lacrimal river was the influencing factor of pterygium(<i>P</i>=0.048). The lower the lacrimal river height, the greater the degree of invasion.CONCLUSION: The prevalence of pterygium in Zhuang and Miao nationality aged 40 and over in Wenshan prefecture, Yunnan Province is high, with no significant difference between the two nationalities. The function of meibomian gland, the quality and quantity of tears are related to pterygium.

Uptake and transport of Laportea bulbifera extract in Caco-2 cell model / 中国中药杂志

Laportea bulbifera extract is effective in resisting inflammation and shows a good therapeutic effect on rheumatoid arthritis in rats. However, the absorption characteristics of active components in L. bulbifera extract in Caco-2 cells are still unclear, which limits the in-depth development of L. bulbifera resources. The purpose of this study was to investigate the absorption and transport mechanism of the active components of L. bulbifera extract in the Caco-2 cell model and explore the effects of different factors(concentration, time, pH value, temperature, and efflux transporter inhibitor) on its uptake and transport. The results showed that L. bulbifera extract at the concentration of 2.0-8.0 mg·mL~(-1) showed no toxicity to Caco-2 cells. The uptake and transport of L. bulbifera extract in the Caco-2 cell model were concentration-dependent and time-dependent. The main absorption mechanism was passive diffusion, and acidic condition(pH 5.0-6.0) and 37 ℃ were more favorable for drug absorption. P_(app)>1.0×10~(-6 )cm·s~(-1) of each component indicated that L. bulbifera was a moderately absorbed drug. P-gp, MRP2, and BCRP were not involved in its uptake and transport.

Clinical characteristics and long-term survival quality in single-center children with low and intermediate-risk neuroblastoma / 中华实用儿科临床杂志

Objective:To summarize the clinical characteristics of single-center children with low and intermediate-risk neuroblastoma (NB), report the long-term follow-up results of the growth and survival quality, and provide a basis for further clinical research.Methods:Clinical characteristics, including the sex, age, stage, risk of disease, and metastatic site of 370 newly treated children with low and intermediate-risk NB admitted to Hematology Oncology Center, Beijing Children′s Hospital from March 2007 to June 2019 were retrospectively analyzed.Kaplan-Meier method was used for survival analysis.WHO Anthro Plus was used for calculating Z score.Results:A total of 370 eligible children with low and intermediate-risk NB were included, with the mean age at onset of 16.8 months (1-191 months). Among them, 148 cases (40%) were younger than 12 months old.Mediastinal region was the most common primary site of NB (47.8%, 177 cases), followed by retroperitoneum/adrenal gland (41.4%, 153 cases). The median follow-up time of 370 patients was 31 months (0.3-157.0 months), the 5-years event free survival (EFS) and 5-year overall survival (OS) were 86.2% and 96.9%, respectively.Thirty-seven cases had growth and deve-lopment problems, of which 22 cases had stunted growth, 6 cases had low body mass, 9 cases had wasting, and 7.3%(27/370 cases) had scoliosis.5.5% of them had heart damage and 5.0%(18/357 cases) had kidney damage, involving 12 cases related to the primary tumor and 6 cases were surgically related.30.2%(95/315 cases) of them had hair changed after chemotherapy, and curly hair was the most common change.Compared with before treatment, 14.9% of the children had a personality change, with an impatient being the most common.Conclusions:The 5-year overall survival rate of the single-center large sample of low and intermediate-risk NB was high, mediastinal was the most common primary site of tumor, and the long-term quality of life is good, but there were still treatment-related side effects, and further clinical monitoring and long-term follow-up were needed.

Analysis of early death in children with neuroblastoma / 中华实用儿科临床杂志

Objective:To summarize the causes of death and severe complication in the early diagnosis of children with neuroblastoma (NB), and to analyze the relative factors of early death of children with NB, so as to raise awareness and reduce early mortality by early detection and early intervention.Methods:Patients with newly diagnosed NB in the Hematology Oncology Center of Beijing Children′s Hospital from April 2007 to December 2017 were included consecutively, and those died within 1 month after diagnosis were retrospectively analyzed.The general data of patients, immediate causes of death, complications, time elapsed between death and diagnosis, whether to receive chemotherapy and other information were collected.Results:A total of 654 cases were included for diagnosis, treatment and follow-up, 31 cases of which died in early stage, accounting for 4.7% of the total.The major complication were pulmonary infection in 18 cases (58.1%) and bone marrow suppression after chemotherapy in 17 cases (54.8%), tumor rupture hemorrhage in 16 cases (51.6%), multiple organ failure in 8 cases (25.8%). Risk factor analysis of the 31 early death cases with NB was conducted.Single factor analysis: there were statistical differences between early death group and non-early death group in risk grouping ( P=0.006 6), bone marrow invasion ( P=0.020 7), site of primary tumor ( P=0.016 7), age ( P=0.003 3), lactate dehydrogenase (LDH) level ( P<0.000 1), neuron-specific enolase (NSE) level ( P<0.000 1), serum ferritin level ( P=0.016 0), D dimer level ( P<0.000 1), fibrinogen level ( P=0.002 7), diameter of tumor ( P<0.000 1), hemoglobin ( P<0.000 1), platelet level ( P<0.000 1), serum albumin level ( P<0.000 1). Multiple-factor analysis: age younger than 30 months, OR=2.824 (95% CI: 1.084-7.359), LDH level greater than 1 004 IU/L, OR=6.991 (95% CI: 2.135-22.887), albumin level less than 36 g/L, OR= 65.237 (95% CI: 2.024-13.545), hemoglobin level less than 92 g/L, OR=5.358 (95% CI: 2.024-13.545), platelet level less than 192×10 9/L, OR=3.554 (95% CI: 1.267-9.965). Conclusions:Strengthening vital signs detection after admission, identifying severe life-threatening complications such as rupture of tumors as early as possible, implementing symptomatic interventions such as appropriate sedation and active transfusion of blood products as early as possible after invasive operation, and transferring to intensive care unit for respiratory support when necessary are important means to avoid early death.

Clinical characteristics and prognosis of neuroblastoma in the pelvic and sacral regions / 中华实用儿科临床杂志

Objective:To summarize the clinical characteristics, treatment response and long-term postoperative complications in children with neuroblastoma (NB) in the pelvic and sacral regions as the primary site.Methods:The clinical characteristics of 16 NB children (8 males and 8 females) with primary pelvic and sacral admitted to the Department of Hematology Oncology Center in Beijing Children′s Hospital, Capital Medical University from March 2007 to June 2019 were analyzed retrospectively with respect to the age at first diagnosis, primary tumor site, tumor size, clinical stage, risk grouping, and other clinical characteristics.The clinical characteristics of the patients who were followed up for regular treatment were analyzed, and the postoperative complications of the patients were summarized, and the Kaplan-Meier method was used for survival analysis.Results:The median age at diagnosis of these 16 children was 23.0 months (5.7-102.0 months), of which 6 cases (37.5%) were younger than 12 months old.All these children received chemotherapy, with a median of 6 (1-8) courses of chemotherapy.Fifteen children received surgical resection of the pelvic tumor, with complete resection in 12 cases (80%). The surgical approach was mainly transabdominal (86.7%, 13/16 cases). The median follow-up time of these children was 33.5 (8-136) months.The patella was absent in 3 patients (18.8%) after the operation, and no permanent neurological damage occurred in all patients.Five-year overall survival (OS) rate was 100%.Conclusions:A single-center summary showed a high survival rate for NB patients in the pelvic and sacral regions.Complete tumor resections combined with chemotherapy could be effective measures and rare cases occurred permanent postoperative neurological complications.

Analysis of the clinical factors related to fibrosis after pediatric liver transplantation / 中华器官移植杂志

Objective:To explore the clinicalfactors related to allograft fibrosis after pediatric liver transplantation.Methods:The clinical data were respectively analyzed for 94 pediatric recipients from January 2013 to December 2016 at Tianjin First Central Hospital.The Patients were assigned into fibrotic and non-fibrotic groups based upon the results of protocol liver biopsies. Univariate and multivariate Logistic regression analyses were performed for examining the risk factors of fibrosis after pediatric livertransplantation. Then Logistic regression model was established to obtain the predicted value of combined predictive factors.Thereceiver operating characteristic curve (ROC) was conducted to evaluate the predictive value of combined predictive factors.Results:A total number of 54(57.5%) patients occurred fibrosis among the 94 patients. There weresignificant differences in cold ischemia time (Z=2.094), warm ischemia time (Z=2.421), biliary stricture( χ2=4.560), drug-induced liver injury ( χ2=7.389), hepatic artery thrombosis and rejection ( χ2=6.955)between two groups ( P<0.05). Logistic regression analysis showed that cold ischemia time (OR=1.003, 95%CI: 1.000~1.007, P=0.044), biliary stricture(OR=6.451, 95%CI: 1.205~33.295), rejection(OR=2.735, 95%CI: 1.057~7.077)and drug-induced liver injury (OR=4.977, 95%CI: 1.207~20.522, P=0.026) were independent risk factors for fibrosis 5 years after liver transplantation. The area under the ROC curve was 0.786(95%CI: 0.691~0.881), for predicting patient outcome.If using 0.311as a cutoff Value, the sensitivity was 90.70%, and the specificity was 60.00%. However, through the ROC curve comparison, there was statistical significance between combined predictive factors and the other independent risk factors ( P>0.05). Conclusions:The incidence of fibrosis 5 years after pediatricliver transplantation is 57.5%. Prolonged cold ischemia time, biliarystricture, rejectionand drug-induced liver injury after liver transplantation are independent risk factors for fibrosis 5 years after pediatric liver transplantation.And the combined predictive factors have a high predictive value forallograftfibrosis.

Efficacy analysis of reduced left lateral segment graft during pediatric living donor liver transplantation / 中华器官移植杂志

Objective:To explore the efficacy of reduced left lateral segment graft during pediatric living donor liver transplantation.Methods:From January 2014 to December 2019, 67 children aged under 1 year underwent living donor liver transplantation with reduced left lateral segment graft (RLLS group). Clinical data were analyzed retrospectively and compared with those of left lateral segmentgraft living donor liver transplantation (LLS group). The differences in basic profiles, postoperative complications and postoperative patient/graft survival rate were compared.They were divided into two groups according to whether graft/recipient weight ratio (GRWR) was more than 4%.And major postoperative complications and graft/recipient survival rates were compared.Results:Age, height and weight of recipients were significantly lower in RLLS group than those in control group ( P<0.05). However, donor weight, donor body mass index (BMI), estimated graft volume and proportion of fatty liver from donor were significantly higher than those in control group ( P<0.05). Operative duration, intraoperative blood loss and erythrocyte transfusion were significantly higher than those in control group ( P<0.05). No significant inter-group differences existed in average postoperative hospital stay, intensive care unit (ICU) stay duration or postoperative ventilator use time ( P>0.05); no significant inter-group difference existed in the incidence of such major surgical complications as hepatic artery thrombosis, portal vein stenosis and bile duct complications ( P>0.05). The 1/3-year cumulative survival rates of postoperative patients and grafts were 92.5%, 91.2% and 92.5%, 91.2% in RLLS group and 96.3%, 95.3% and 95.9%, 95.1% in LLS group respectively.There was no significant inter-group difference ( P<0.05). The rate of postoperative hepatic vein stenosis was significantly higher in GRWR>4% group than that in control group ( P<0.05). Conclusions:Due to a rapid progress of technology, living donor liver transplantation has achieved satisfactory outcomes in children with reduced left lateral segment graft.Whether or not performing reduction surgery should be judged comprehensively according to the matching of donors and recipients and blood flow of liver during operations.And GRWR>4% is not an implementation criterion.

Factors and outcomes of lymphatic leakage after pediatric liver transplantation / 中华器官移植杂志

Objective:To examine the incidence of lymphatic leakage after pediatric liver transplantation and explore the diagnosis and treatment of lymphatic leakage.Methods:From January 1, 2016 to December 31, 2019, clinical data were analyzed retrospectively for 805 pediatric liver transplant recipients. Based upon the diagnosis of lymphatic leakage, they were divided into two groups of lymphatic leakage ( n=271) and lymphatic non-leakage ( n=534). Analyzing the incidence of lymphatic leakage after liver transplantation in children, evaluating the treatment plan, comparing survival rate and the incidence of postoperative complications between two groups. Results:The incidence of lymphatic leakage was 33.7%(271/805); the proportion of partial liver donors was 14.8% in lymphatic leakage group and 25.8% in lymphatic non-leakage group ( P<0.001). Other basic profiles of two groups were not statistically different. The median follow-up period was 32 months in lymphatic leakage group and 30.6 months in lymphatic non-leakage group. No significant inter-group difference existed in cumulative survival rate, vascular complications, bile leakage, acute cell rejection or intestinal obstruction. The area-under-curve (AUC) of ascites to serum triglyceride (TG) ratio for predicting lymphatic leakage was 0.741, optimal cut-off value 0.54, sensitivity 59.2% and specificity 80.1%. Conclusions:Lymphatic leakage is a common complication after liver transplantation in children. With no significant correlation with the morbidity or mortality, it prolongs postoperative hospital stay. The ratio of ascites to serum TG may be utilized as an effective reference index for diagnosing lymphatic leakage. And lymphatic leakage can be improved by taking a low-fat diet.

Analysis of de novo non-alcoholic fatty liver disease in early period after pediatric liver transplantation: a report of 8 cases / 中华器官移植杂志

Objective:To summarize the clinical characteristics of de novo non-alcoholic fatty liver disease(NAFLD)in pediatric recipients in early stage post liver transplantation(LT)to enhance our understanding of this rare complication.Methods:The clinical data of 8 recipients who underwent liver transplantation in the children's organ transplantation Department of Tianjin first central hospital from January 2014 to December 2019 and developed NAFLD within 3 months after operation were retrospectively analyzed. Taking liver biopsy as the standard for the diagnosis of NAFLD, the clinical and histological characteristics of early NAFLD after transplantation were summarized and analyzed.The median time from LT to NAFLD was 1.55(0.63, 2.93)months and the median follow-up period 23.60(8.74, 32.58)months.Results:NAFLD was all pathologically confirmed by liver biopsy. Seven cases had abnormal liver function and 1 case of steatosis was detected by ultrasound pre-biopsy. There were acute cellular rejection(2 cases)and drug-induced graft injury(1 case). The median period of recovery for graft function was 32.0(12.0, 34.0)days. Macrovesicular graft steatosis predominated.Conclusions:Occurring earlier in children after LT, NAFLD is frequently accompanied by abnormal graft function. Liver biopsy is required for making a definite diagnosis. Abnormal graft function persists a long time. However, prognosis is generally decent.

Simultaneous determination of four components of Shuganning Injection in rat plasma by UPLC-MS/MS and its application to a pharmacokinetic study / 中国中药杂志

This study aims to reveal the pharmacokinetics of Shuganning Injection in normal rats. In this experiment,ultra-high performance liquid chromatography-electrospray-tandem mass spectrometry( UPLC-ESI-MS/MS) was used to establish an analytical method for simultaneous determination of chlorogenic acid,gardenioside,oroxylin A and baicalin in rat plasma. Then,the non-compartmental model( NCA) in Phoenix WinN onL in 6. 4 software was used to fit pharmacokinetic parameters. The methodological validation showed that the linear relationship of the components in rat plasma samples were good( r>0. 995). The recovery rate and matrix effect of plasma samples with low,middle and high concentration were 79. 14%-101. 4%. The intra-day and inter-day precision,accuracy and stability meet the requirements of biological sample analysis. The half-life( t1/2) of chlorogenic acid,gardenioside,oroxylin A did not change significantly and the area under blood concentration-time curve( AUC0-t) is proportional to the dose,which suggested that three components showed a linear kinetic characteristics,but baicalin showed nonlinear kinetic characteristics. Moreover,the retention time of each component in rats was short. The established UPLC-MS/MS quantitative analysis method is rapid,sensitive and accurate,which can be used for the determination of chlorogenic acid,gardenioside,oroxylin A and baicalin in rat plasma and pharmacokinetic study of Shuganning Injection.

Clinicopathological characteristics and treatment of hepatoblastoma with macrotrabecular structures / 中华肝胆外科杂志

Objective:To analyze the clinicopathological characteristics and treatment strategies of hepatoblastoma with macrotrabecular structures.Methods:To retrospectively analyze the data of children with hepatoblastoma treated in the Department of Oncology at Beijing Children's Hospital affiliated to Capital Medical University amd Baoding Children's Hospital from January 1, 2011 to December 31, 2019. The study structure consisted of collecting clinical data and formulating treatment plan, including clinical stage, alpha-fetoprotein (AFP), chemotherapy plan, surgical margin, pathological classification and follow-up data to study the long-term prognosis of these patients.Results:Among 17 patients, 13 males and 4 females, age 5 to 134 months; 5 patients had the macrotrabecular type, 10 patients had the epithelial type with macrotrabecular, 2 patients had the mixed epithelial and mesenchymal with macrotrabecular type. For the PRETEXT staging, 1 patient had stage I, 2 patients had stage II, 14 patients had stage III and IV. A total of 15 patients received preoperative chemotherapy, and 6 achieved partial response. Among 17 patients, 10 had negative resection margins. The AFP of 7 patients was normal after chemotherapy, and 10 patients relapsed after surgery. The 2-year event-free survival was 26.18%.Conclusions:Patients with hepatoblastoma containing giant trabecular components are very rare. Among them, the recurrence rate of patients with giant trabecular and epithelial and giant trabecular components was high. Preoperative neoadjuvant chemotherapy (vincristine + irinotecan), transcatheter arterial chemoembolization and liver transplantation are treatment options for this type of hepatoblastoma.

Clinical characteristics and prognosis of pediatric non-rhabdomyosarcoma soft tissue sarcomas / 中华实用儿科临床杂志

Objective:To analyze the clinical features and prognosis of pediatric non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), and further understand these tumors.Methods:Twenty-nine NRSTS children were admitted to the Hematological Oncology Ward of Beijing Children′s Hospital from June 2011 to May 2018.The clinical and pathological data of these children were collected, and the relationships of the prognosis with clinical characteristics Intergroup Rhabdomyosarcoma Study(IRS) stage, Children′s Oncology Group(COG) risk grouping were analyzed.All the patients were followed up until October 31, 2018.The survival analysis was performed by Kaplan-Meier method.Results:There were 14 boys and 15 girls in the enrolled 29 children, aging from 7 to 169 months, with a median age of 59.5 months.There were 10 pathological types, including synovial sarcoma, infantile fibrosarcoma and malignant rhabdomyoid tumors in 5 cases, and other pathological types in 14 cases.Tumors originated from the head and neck in 10 cases, limbs or trunk in 6 cases, visceral sites in 13 cases.Ten cases showed distant metastasis.There were 12 cases at IRS stage Ⅰand Ⅱ, and 17 cases of stage Ⅲ and Ⅳ.All the children were treated with surgery and chemotherapy with or without radiotherapy.Nine cases received preoperative chemotherapy, 17 cases received postoperative chemotherapy, 3 cases could not be resected surgically.The follow-up period ranged from 4 to 89 months, with a median follow-up of 16 months.Six of 29 children died, including 3 cases of malignant rhabdomyoid sarcoma.The 2-year overall survival(OS) rate of all the children was 77.4% and the 2-year event-free survival (EFS) rate of all the children was 53.2%.By analyzing the clinical factors, it was found that the IRS stage, COG risk group, primary sites were related to prognosis.The 2-year EFS rate of children with IRS Ⅰ-Ⅱ and Ⅲ-Ⅳ were 75.0% and 35.9%, respectively ( χ2=7.303, P=0.007), the 2-year OS rate was 100% and 61.8%, respectively( χ2=4.81, P=0.028); The 2-year EFS of children in COG low-risk group and median/high-risk group were 66.7% and 44.7%, respectively( χ2=4.155, P=0.042), the 2-year OS rate of children in COG low and median/high-risk was 100% and 66.3%, respectively( χ2=3.383, P=0.066); the 2-year OS rate of children in visceral and non-visceral sites were 59.3% and 92.9%, respectively ( χ2=4.202, P=0.04). Conclusions:NRSTS in children is heterogeneous, and surgery is the main treatment. Children with primary tumors located in visceral sites and at IRS Ⅲ-Ⅳ had poor outcomes.

Clinical features and survival analysis of neuroblastoma with cervical lymph node metastasis in children: su-mmary of 10 years in single center / 中华实用儿科临床杂志

Objective:To improve the awareness of neuroblastoma (NB) with cervical lymph node metastasis in children patients by summarizing the clinical features of such patients and analyzing their survival situation.Methods:The research analyzed the medical records of 225 patients with cervical lymph node metastasis of NB from April 1, 2007 to May 31, 2017 in Beijing Children′s Hospital, Capital Medical University.The treatment were divided into 2 phases according to treatment time (one from April 1, 2007 to December 31, 2011 and the other from January 1, 2012 to May 31, 2017). The survival situation and treatment effect in each phase were analyzed.The following up time ended at Jan 1, 2018.Results:(1)Clinical features: total 225 cases admitted in the research with about 37.3% of all the NB patients.One hundred girls(44.4%) and 125 boys(55.6%). The age from 2 months to 147 months, the me-dian age of patients was 37 months.The age under 18 months were 27 cases(12.1%). The majority primary site of tumor was located in retroperitoneal(174 cases, 77.3%) and mediastinum(48 cases, 21.3%). There were 33 cases which metastasic site confined to cervical lymph node(4N stage, 14.7%); 139 cases (62.1%) with bone marrow metastasis, 159 cases (71.0%) with bone metastasis.The number of patients in high-risk group was 208 cases(91.2%), while the low-risk and the medium-risk group was 17 cases(7.5%). (2)Treatment effect and survival analysis: a total of 200 cases received systemic treatment and followed-up in our hospital.The follow up time from 2 months to 123 months, the median follow up time was 23 months.The 3 year overall survival (OS) rate of all the patients was 56.4%, the 3 year event free survival (EFS) rate was 52.0%.While the 5-year OS was 46.5%, and the 5-year EFS was 39.7%.According to the first phase of treatment(April 1, 2007 to December 31, 2011), the 3-year EFS was 49.6%, and the 3-year OS was 54.4%, the 5-year EFS was 39.5%, and the 5-year OS was 44.7%.According to the second phase of treatment(January 1, 2012 to May 31, 2017), the 3-year EFS was 58.1%, and the 3-year OS was 57.3%.The 5-year EFS was 48.7%, and the 5-year OS was 46.8%.About the 4N patients, the 3-year EFS was 71.2%, and the 3-year OS was 82.2%.The 5-year EFS was 61.5%, and the 5-year OS was 76.7%.Conclusions:Cervical lymph node is one of the most common distant metastasis of NB.More than half of the patient with bone or bone marrow metastasis.The prognosis of patients′ metastatic disease limited to cervical lymph nodes(4N stage), but still worse than the foreigner haver reported.The probable reason is the proportion of high-risk patients in the center is higher than the foreign research.

Clinical efficacy analysis of pediatric blood type incompatible living donor liver transplantation / 器官移植

Objective To explore the clinical efficacy of pediatric blood type incompatible living donor liver transplantation. Methods The clinical data from 242 cases of pediatric living donor liver transplantation recipients were retrospectively analyzed. Recipients were assigned to group A (ABO-identical group, n=165), group B (ABO-compatible group, n=42) and group C (ABO-incompatible group, n=35) according to the blood type compatibility between the recipients and the donors. The occurrence of postoperative complications and development of postoperative donor specific antibody (DSA) among the 3 groups were observed and compared. And the blood type distribution of donors and recipients and development of erythrocyte antibodies in group C were analyzed. The survival situation of recipients after liver transplantation was compared among the 3 groups. Results There was no significant difference in the incidence of complications among the 3 groups(all P > 0.05). DSA was dominated by human leukocyte antigen (HLA) Ⅱ antibodies after liver transplantation, mostly anti-HLA-DR and anti-HLA-DQ. The postoperative erythrocyte antibodies for liver transplant recipients in group C were dominated by IgM, with titers ≤1:2 for all. The differences in postoperative survival rates were not statistically significant among 3 groups(all P > 0.05). Conclusions Pediatric blood type incompatible living donor liver transplantation is a safe and effective treatment, which can effectively expand the source of liver transplant donors and save the children's lives.

Efficacy of short-course ganciclovir in preventing cytomegalovirus infection after pediatric liver transplantation / 中华器官移植杂志

Objective:To explore the preventive efficacy of 2-week ganciclovir intravenous injection for CMV infection after pediatric liver transplantation(LT).Methods:Clinical data were retrospectively analyzed for 404 pediatric LT recipients from January 1, 2015 to December 31, 2017. According to whether or not ganciclovir was intravenously administered for preventing CMV infection, they were divided into two groups of prevention(235 cases)and non-prevention(169 cases). The preoperative, intraoperative and postoperative follow-up data of two groups were recorded. Survival rate, incidence of CMV infection and time of initial CMV infection were compared between two groups.Results:The median follow-up time of 404 pediatric liver transplantation recipients was 856 days and the incidence of CMV infection 39.1%. No inter-group statistical difference existed in such basic clinical data as gender, age, primary disease, preoperative PELD score, CHILD grade, operative duration, intraoperative blood loss, immunosuppressive regimen or rejection rate. The median follow-up time of two groups was 1014 and 731 days; The incidence of CMV infection 37.4%(88/235)and 41.4%(70/169); The average postoperative time of initial CMV infection 75.5 and 110.2 days; The rate of CMV re-infection after initial CMV infection 26.1%(23/88)and 18.6%(13/70)respectively. No significant inter-group differences existed( P>0.05). Conclusions:Early postoperative 2-week intravenous ganciclovir injection fails to reduce the incidence of CMV infection after pediatric LT, nor delay the occurrence time of CMV infection. It is not recommended as a preventive program for CMV infection after pediatric LT.

Diagnostic and therapeutic experience of portal vein stenosis after pediatric liver transplantation from donation after citizen's death graft:a report of 30 cases / 中华器官移植杂志

Objective To summarize the experiences of diagnosing and treating portal vein stenosis (PVS) after pediatric liver transplantation from China donation after citizen 's death (CDCD) grafts .Methods Retrospective analysis was performed for 30 cases of pediatric CDCD liver transplantation recipients with PVS .The screening ,diagnosis ,treatment and prognosis of PVS were analyzed .Results Among 218 pediatric liver transplantation recipients with CDCD grafts ,PVS was diagnosed in 30 cases with an incidence rate of 13 .8％ (30/218) .The initial diagnosis of PVS ranged from 5 days to 27 months post-operation with a median age of 2 .9 months .Ultrasonography indicated that stenotic rate of anastomotic site diameter was (41 .28 ± 12 .93)％ and blood flow velocity ratio (358 .77 ± 117 .82)％ .Intervention examination showed average pressure gradient was (9 .06 ± 5 .34) mmHg between both sides of stenosis . All cases underwent percutaneous intrahepatic balloon dilatation .The recipients were followed up for a median follow-up time of 23(3-63) months .For three cases of restenosis ,percutaneous intrahepatic balloon dilatation was repeated .Two cases underwent stent implantation due to ineffective balloon dilation .After treatment ,the stenotic rate of anastomotic site diameter was (34 .69 ± 8 .82) and blood flow velocity ratio (61 .18 ± 63 .11)％ on ultrasound while the average pressure gradient was (1 .03 ± 0 .85) mmHg .Conclusions PVS is a common vascular complication after pediatric CDCD liver transplantation .Portal vein balloon dilation is both safe and efficacious .However ,some cases require repeated balloon dilation and stent implantation serves as the last option for intractable PVS .Color ultrasound is both convenient and effective for making a primary diagnosis and evaluating outcomes .