Relationship between serum lactate and early prognosis after liver transplantation in children / 中华器官移植杂志

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.

Identification of terpene synthase gene family in Gynostemma pentaphyllum and expression pattern analysis under abiotic stresses / 中国中药杂志

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.

Delineation of a mosaicism fetal supernumerary marker chromosome with combined genetic techniques / 中华医学遗传学杂志

OBJECTIVE@#To delineate the origin and content of a mosaicism small supernumerary marker chromosome (sSMC) in a fetus with combined chromosomal karyotyping, chromosomal microarray analysis (CMA) and fluorescence in situ hybridization (FISH).@*METHODS@#The fetus of a 31-year-old pregnant woman who had presented at the Maternal and Child Health Care Hospital of Longhua District of Shenzhen City in 2022 was selected as the study subject. Non-invasive prenatal testing suggested that the fetus has harbored a 8.75 Mb duplication in 4q12q13.1. With informed consent, amniotic fluid and peripheral blood samples were taken from the couple for chromosomal karyotyping analysis. The origin and content of a sSMC was identified by CMA, and its proportion in amniotic fluid was determined with a FISH assay.@*RESULTS@#The karyotypes of the pregnant woman, her husband and the fetus were respectively determined as 46,XX, 46,XY,inv(9)(p12q12), and 47,XY,inv(9)(p12q12)pat,+mar[75]/ 46,XY,inv(9)(p12q12)pat[25]. CMA test of the amniotic fluid sample was arr[hg19]4p11q13.1(48978053_63145931)×3, which revealed no mosaicism. However, FISH analysis showed that 59% of interphase cells from the cultured amniotic fluid sample had contained three signals for the centromere of chromosome 4, whilst 65% of interphase cells from the re-sampled amniotic fluid had three such signals, which confirmed the existence of trisomy 8 mosaicism.@*CONCLUSION@#Chromosomal structural abnormality combined with mosaicism can be delineated with combined chromosomal karyotyping and molecular techniques such as FISH and CMA, which has enabled more accurate counseling for the family.

Risk factors of biliary complications after pediatric living donor liver transplantation / 中华器官移植杂志

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.

Effect of allograft type on the prognosis of pediatric liver transplantation in low-body-weight recipients / 中华器官移植杂志

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.

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.

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 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.

Research progress of miRNA involvement in plant polyphenols in regulating lipid metabolism / 中国药理学通报

Lipid metabolism disorder is an important risk factor for obesity,type 2 diabetes mellitus,non-alcoholic fatty liver disease and other chronic metabolic diseases.Micro RNAs(miRNAs)are short stranded RNA molecules with the size of 19 to 25 nucleotides that regulate lipid metabolism,within a number of physiological functions.Studies have shown that miRNAs can participate in the regulation of lipid metabolism by polyphenolic phytochemicals.In this paper,the mechanism of plant polyphenols improving lipid metabolism disorders and maintaining lipid balance through miRNA is summarized from the synthesis of triglyceride,fatty acid β oxidation,cholesterol efflux and cholesterol esterification,aiming to provide a new thought for the application and development of plant polyphenols to improve the diseases related to lipid metabolism disorders,and offer theoretical basis for the research and development of miRNA as a potential target for the prevention and treatment of diseases related to lipid metabolism disorders.

Impact of graft/recipient weight ratio on the prognosis of infants with whole liver transplantation / 中华器官移植杂志

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.

Impact of left hepatic vein classification on hepatic vein reconstruction and prognosis after pediatric living-donor liver transplantation using left lateral liver segments / 中华肝胆外科杂志

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.

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.

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.

The Relationship between Mitochondrial Quality Control and Beige Adipocytes / 中国生物化学与分子生物学报

The prevalence of obesity has been rising in recent years. Obesity has become one of the global public health problems‚ which can increase the risk of hypertension‚ coronary heart disease and other cardiovascular diseases‚ and prevention and treatment of obesity has become an urgent social problem to be solved. Beige adipocytes are new kind of heat-producing adipocytes‚ which can be transformed by white adipocytes stimulated by chronic cold exposure‚ some medicine and exercise. The morphology and function of beige adipocytes are different from white adipocytes‚ but are similar to brown adipocytes. Beige adipocytes have abundant mitochondria and can produce heat to consumer energy‚ which are new target for the prevention and treatment of obesity. Mitochondria is the main part of energy metabolism in eukaryotic cells‚ which can provide energy to the body. Mitochondrial quality control regulates mitochondrial quantity and quality in beige adipocytes‚ which can affect the conversion and thermogenesis of beige adipocytes. Mitochondrial quality control is regulated by several coordinated mechanisms including mitochondrial biogenesis‚ mitophagy and fission / fusion. The mitochondrial biogenesis and mitophagy can regulate mitochondrial renewal and degradation‚ and the mitochondrial fusion / division is responsible for mitochondrial repairing. Some studies have found that the change of mitochondrial quantity‚ morphology and function can affect the beige adipocytes formation‚ beige adipocytes maintenance and thermogenesis. However‚ the relationship between mitochondrial quality control and beige adipocytes remains unclear. In this review‚ we discuss the effect of mitochondrial biogenesis‚mitophagy and mitochondrial fission / fusion on beige adipocytes‚ and illustrate the relationship between mitochondrial quality control and beige adipocytes‚ which may provide new insights into the prevention and treatment of obesity.

Research progress on regulation of PGC-lα in obesity-related metabolic diseases / 中国药理学通报

Peroxisome proliferator-activated receptor γ coactivator 1-alpha (PGC-1α) is a member of superfamily of transcriptional co-regulators. It was first discovered to be related to the adaptive thermogenesis of brown adipose tissue. Later, it has been discovered that PGC-la can also regulate glucose and lipid metabolism and vascular endothelial function, thus influence the occurrence and development of obesity-related metabolic diseases such as hyperlipidemia, type II diabetes and hypertension. Drugs targeting PGC-1α are promising for the prevention and treatment of these diseases. Here we review the research progress on regulation of PGC-1α in obesity related metabolic diseases, aiming to provide theoretical basis for the development of drugs targeting PGC-1α.

Research progress on mechanisms of AMPK in diabetes prevention and control / 中国药理学通报

AMP-activated protein kinase (AMPK) is a serine/threonine protein kinase that plays a key role in the maintenance and regulation of energy homeostasis in body. It is an important regulator of energy metabolism. Numerous studies have shown that AMPK plays an important role in the prevention and control of diabetes. AMPK can activate or inhibit a variety of downstream effectors, thereby regulating a variety of physiological activities, including regulating glucose and lipid metabolism, promoting browning of white adipose, anti-inflammatory, antioxidant stress, etc. In this review, the mechanisms of AMPK preventing the occurrence and development of diabetes were reviewed, aiming to provide a reference for the development of safe and effective new AMPK targeting drugs.

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.