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The present study aimed to investigate the composition of the terpene synthase(TPS) gene family in Gynostemma pentaphyllum and its role in abiotic stresses. The G. pentaphyllum TPS gene family was identified and analyzed at the genome-wide level using bioinformatics analysis, and the expression patterns of these family members were analyzed in different tissues of G. pentaphyllum as well as under various abiotic stresses. The results showed that there were 24 TPS gene family members in G. pentaphyllum with protein lengths ranging from 294 to 842 aa. All of them were localized in the cytoplasm or chloroplasts and unevenly distributed on the 11 chromosomes of G. pentaphyllum. The results of the phylogenetic tree showed that the G. pentaphyllum TPS gene family members could be divided into five subfamilies. As revealed by the analysis of promoter cis-acting elements, TPS gene family members in G. pentaphyllum were predicted to respond to a variety of abiotic stresses such as salt, low temperature, and dark stress. The analysis of gene expression patterns in different tissues of G. pentaphyllum revealed that nine TPS genes were tissue-specific in expression. The qPCR results showed that GpTPS16, GpTPS17, and GpTPS21 responded to a variety of abiotic stresses. This study is expected to provide references in guiding the further exploration of the biological functions of G. pentaphyllum TPS genes under abiotic stresses.
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Gynostemma , Phylogeny , Alkyl and Aryl Transferases , ChloroplastsABSTRACT
Objective:To explore the relationship between serum lactate level and early prognosis after liver transplantation (LT) in children.Methods:Between January 1, 2018 and December 31, 2020, 675 pediatric LT recipients were recruited. Clinical data were retrospectively reviewed, early postoperative serum lactate level and clearance rate recorded and receiver operating characteristic (ROC) curve plotted for determining optimal cut-off values. The inter-group differences in early postoperative complications and patient/graft survival rates were compared.Results:According to ROC, blood lactate levels >1.99 mmol/L at 12 h postoperatively were associated with early postoperative graft loss (AUC 0.73, 95% CI: 0.62-0.84, P=0.01). Age and weight of recipients in high-level group were 7.17(5.70-10.40) month and 7.00(6.00-8.60) kg and both were significantly lower than those in low-level group [7.80(6.21-13.58) month and 7.20(6.45-9.00) kg]. The inter-group differences were statistically significant ( P=0.017, P=0.034). Blood plasma transfusion volume, red blood cell transfusion volume, portal vein pressure pre-closure, postoperative intensive care unit (ICU) stay, ventilator use time, early allograft dysfunction rate, early postoperative pulmonary infection rate and recipient mortality rate in high-level group were 400 (200-400) ml, 2.00 (2.00-4.00) U, (15.71±4.44) mmHg, 2.50(2.00-3.00) day, 3.81(2.47-8.50) hour, 22.95%(42/185), 16.76%(31/185) and 6.49%(12/185) respectively. The above values were significantly higher than those in low-level group 200(100-400) ml, 2.00 (2.00-3.00) U, (14.69±4.68) mmHg, 2.00(2.00-3.00) day, 3.53(2.34-6.12) hour, 14.69%(72/490), 11.02%(54/490) and 1.43%(7/490) respectively. The inter-group differences were statistically significant ( P<0.001, P=0.014, P=0.015, P=0.037, P=0.043, P=0.011, P=0.045 & P<0.001). The incidence of early postoperative acute cellular rejection was significantly lower in high-level group than that in low-level group [11.89%(22/185) vs 22.86%(112/490)]. The inter-group difference was statistically significant ( P=0.01). The 1/3-month cumulative survival rates of patient/graft were 94.6%, 94.1% and 92.4%, 91.4% in high-level group versus 99.2%, 98.6% and 99.0%, 98.4% in low-level group. There were significant inter-group differences ( P=0, P<0.000 1). With a rising level of lactate at 12 h postoperatively, risk of early graft loss and early recipient mortality spiked markedly ( P<0.05). Conclusions:Serum lactate level post-operation is a valid predictor of early prognosis after LT in children.
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Objective:To study the impact of donor left hepatic vein classification and the reconstruction methods on hepatic venous outflow obstruction (HVOO) after pediatric living-donor liver transplantation using left lateral liver segments.Methods:A retrospective study was performed on the clinical data of 653 children recipients who underwent living-donor liver transplantation with left lateral liver segments from January 2014 to December 2020 at Tianjin First Central Hospital. There were 309 males and 344 females, aged 7.0 (6.0, 10.0) months, with an age range of 3-121 months. Based on the left hepatic vein on preoperative donor enhancement CT as well as the intraoperative reconstruction methods, the recipients were divided into 3 groups: type Ⅰ group ( n=514), anastomosis using a single opening was performed directly between the donor and the recipient; type Ⅱ group ( n=118), angioplasty was performed on two adjacent recipient venous orifices before anastomosis, and type Ⅲ group ( n=21), an interposition vessel was anastomosed to two widely spaced openings or the two veins were anastomosed separately. The preoperative general status of the patient, postoperative HVOO incidences, and graft and recipient survival rates were compared among the three groups. The patients were followed up by outpatient reexamination or telephone. Results:Graft to recipient weight ratio in the type Ⅲ group was smaller than that in the type Ⅰ group and the type Ⅱ group ( P<0.05). For all the 653 patients, the incidence of postoperative HVOO was 4.59% (30/653), with the incidences of HVOO in the 3 groups of patients were 4.1% for the type Ⅰ group (21/514), 5.1% for the type Ⅱ group (6/118), and 14.3% for the type Ⅲ group (3/21), respectively. There was no significant difference among the groups ( P>0.05). The recipient cumulative survival rates at 1 and 3 years after surgery in the type I group were 97.8% and 97.0%, and the corresponding rates in the type Ⅱ group were 96.5% and 94.2%, and in the type Ⅲ group were 94.1% and 86.9%, respectively. There was a significant difference between the type Ⅰ and type Ⅲ groups ( P=0.048). The graft cumulative survival rates at 1 and 3 years in the type Ⅰ group were 97.4% and 96.9%, and the corresponding rates in the type Ⅱ group were 94.9% and 92.5%, and in the type Ⅲ group were 94.1% and 86.9%, respectively. The difference in the postoperative graft cumulative survival rates between the type Ⅰ group and type Ⅱ group was significant ( P=0.044). Conclusions:The anatomy of the left hepatic vein supplying the left lateral liver segment was highly variable, and the majority of the variations could be reconstructed. A reasonable reconstructive method could reduce the incidence of postoperative HVOO and improved the outcomes of the graft.
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@#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.
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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.
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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.
Subject(s)
Humans , Case-Control Studies , Genetic Predisposition to Disease , Genome-Wide Association Study , Polymorphism, Single Nucleotide , RNA, Long Noncoding/genetics , Silicosis/geneticsABSTRACT
Objective:To explore the risk factors of biliary complications(BCS)after pediatric living donor liver transplantation(LDLT).Methods:From January 2016 to December 2020, retrospective review of clinical data was performed for 681 children aged <18 years undergoing LDLT.There were 324 boys and 357 girls with a median age of 7.4 months and a median weight of 7.0 kg.Among 61 BCS patients(9.0%), there were biliary stricture(n=34, 5.0%), bile leakage(n=21, 3.1%)and bile leakage combined with biliary stricture(n=6, 0.9%). According to the absence or presence of BCS after LT, the recipients were divided into two groups of BCS(n=61)and non-BCS(n=620). The incidence and risk factors of BCS were analyzed.T-test, Wilcoxon rank sum test, Chi square or Fisher exact test was employed for univariate statistical analysis and Logistic regression for multivariate statistical analysis.Results:The median follow-up period was 35.5 months.Univariate analysis revealed statistically significant inter-group differences( P=0.005, 0.046, 0.009, 0.011, 0.024, 0.023, 0.004, 0.038, 0.002, 0.029, 0.023, 0.002, 0.011)in donor age[(31.4±5.7)vs.(34.3±7.5)years], time of anhepatic phase[43(37.0, 53.0)vs.47(38.8, 56.0)min], time from portal vein opening to hepatic artery opening[35(30.0, 41.0)vs. 38(30.8, 47.8)min], type of perfusion fluid, number of donor bile ducts, intestinal loop length[40(30.0, 40.0)vs.40(25.0, 40.0)cm], mode of biliary reconstruction, whether or not placing a support tube, incidence of hepatic artery thrombosis[1.6%(10/620)vs.9.8%(6/61)], incidence of abdominal infection[4.5%(28/620)vs.11.5%(7/61)], incidence of cytomegalovirus(CMV)infection[55.3%(343/620)vs.70.5%(43/61)], incidence of portal vein thrombosis[1.1%(7/620)vs.8.2%(5/61)]and incidence of pulmonary infection[19.0%(118/620)vs.32.8%(20/61)]. Multivariate analysis indicated that independent risk factors of BCS included donor age( P=0.023), number of donor bile ducts( P=0.017), time from portal vein opening to hepatic artery opening( P=0.010), hepatic artery thrombosis( P=0.004), abdominal infection( P=0.019), CMV infection( P=0.022), portal vein thrombosis( P=0.003), pulmonary infection( P=0.021)and short intestinal loop length( P=0.012). Conclusions:Biliary complications are common after pediatric LDLT.Independent risk factors are donor age, number of donor bile ducts, time from portal vein opening to hepatic artery opening, hepatic artery thrombosis, abdominal infection, CMV infection, portal vein thrombosis, pulmonary infection and short length of intestinal loop.
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Objective:To investigate the effects of different donor types on the prognosis of pediatric liver transplant recipients with low-body-weight (≤6 kg).Methods:The clinical data of low-body-weight pediatric liver transplant recipients from the Department of Pediatric Organ Transplantation, Tianjin First Central Hospital from January 2013 to June 2021 were retrospectively analyzed.The recipients were divided into living donor group, split donor group and whole liver group according to the donor type.The basic information of donors and grafts, preoperative and intraoperative information of recipients, major postoperative complications and survival rates of recipients and grafts were compared.Results:A total of 244 recipients were enrolled in this study, including 183 cases in the living donor group, 18 cases in the split donor group and 43 cases in the whole liver group.There were no statistical differences in the preoperative data of the three groups, including gender, age, body weight, blood type matching, primary disease, Child-pugh grading, and pediatric end-stage liver disease score (PELD). The incidence of hepatic artery thrombosis (HAT) in the three groups was 2.2%, 16.7% and 25.6%, respectively, the difference was statistically significant between the living donor group and the split donor group ( P=0.017) as well as the whole liver group ( P<0.001). There was no significant difference between the latter two groups ( P=0.525). The median follow-up time was 37, 31 and 47 months, respectively.The 1-year and 3-year cumulative graft survival rates were 92.9%, 91.3%, 83.3% and 83.3% 76.7%, 76.7% ( P=0.016), respectively.There was statistical difference between the living donor group and the whole liver group ( P=0.004), and no statistical difference between the split donor group and the living donor group ( P=0.212) as well as the whole liver group ( P=0.610). The 1-year and 3-year cumulative recipient survival rates in the three groups were 92.9%, 91.3%, 94.4% and 94.4%, 86.0%, 86.0%, respectively, and there was no statistical difference among the three groups ( P=0.463). Multivariate analysis suggested that donor age and anhepatic phase were independent risk factors for HAT.Cold ischemia time, volume of intraoperative blood transfusion and HAT were independent risk factors for early graft loss (within 3 months). The volume of intraoperative blood transfusion and the duration of anhepatic phase were independent risk factors for recipient death. Conclusions:Living donor liver transplantation is more effective than whole liver transplantation for children with low body weight (≤6 kg). Due to the small sample size and the early exploration stage of split liver transplantation in children, the efficacy of split liver transplantation remains to be explored in clinical practice.
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Objective:To explore the impact of graft recipient weight ratio(GRWR)on pediatric whole liver transplantation in infants aged under 1 year.Methods:From January 2014 to December 2019, clinical data were retrospectively reviewed for 140 children aged under 1 year with whole liver transplantation.They were divided into 3 groups of low GRWR(GRWR<2.5%, 48 cases), middle GRWR(2.5%≤GRWR<5%, 73 cases)and high GRWR(GRWR≥5%, 19 cases). Basic profiles, major postoperative complications and survival rate of graft/recipient were compared.Results:There were 62 males and 78 females with an average age of (7.34±1.81)months and an average weight of(6.81±1.09)kg.The median GRWR was 3.27%(1.33%~8.12%). The higher level of GRWR, the greater age, weight and graft weight of donor in three groups and there was statistical difference ( P<0.05); operative duration, postoperative ICU stay and hospital stay were longer in low GRWR group than those in middle GRWR group and there was statistical difference( P<0.05); The incidence of postoperative hepatic artery thrombosis was higher in low GRWR group than that in middle GRWR group(31.3%vs 8.2%)and there was statistical difference( P<0.05); 4 cases of small-for-size syndrome occurred in low GRWR group, it was significantly different from the other two groups and there was statistical difference( P<0.05); the median follow-up period was(50.7±23.4)months.The survival rates of grafts at 3-month and 1/5-year were 89.6%, 91.8%, 100%; 87.5%, 87.7%, 100%; 87.5%, 87.7%, 100%and there was no inter-group difference( P>0.05). The survival rates of recipients at 3 months, 1 year and 5 years post-operation were 93.8%, 91.8%, 100%; 91.7%, 87.7%, 100%; 91.7%, 87.7%, 100%and there was no inter-group difference( P>0.05). Conclusions:Different from pediatric living donor transplantation, GRWR≥5%does not affect the survival rate of recipient/graft during whole liver transplantation.And GRWR<2.5%may boost the postoperative incidence of hepatic artery thrombosis and small liver syndrome.
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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.
Subject(s)
Animals , Mice , Gene Deletion , Histones/genetics , Insulin , Insulin Receptor Substrate Proteins/metabolism , Insulin Resistance/genetics , Membrane Cofactor Protein/genetics , Mice, Knockout , Muscles/metabolism , Phosphoric Monoester Hydrolases/metabolism , Protein Tyrosine Phosphatase, Non-Receptor Type 1/metabolism , Proto-Oncogene Proteins c-akt/metabolism , RNA, Messenger/metabolism , Receptor, Insulin/metabolism , Tyrosine/genetics , Up-RegulationABSTRACT
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.
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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.
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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.
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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.
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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.
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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.
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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.
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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.
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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.
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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.