Diagnosis status and genetic characteristics analysis of Fanconi anemia in China / 中华儿科杂志

Objective: To analyze the clinical and molecular diagnostic status of Fanconi anemia (FA) in China. Methods: The General situation, clinical manifestations and chromosome breakage test and genetic test results of 107 pediatric FA cases registered in the Chinese Blood and Marrow Transplantation Registry Group (CBMTRG) and the Chinese Children Blood and Marrow Transplantation Registry Group (CCBMTRG) from August 2009 to January 2022 were analyzed retrospectively. Children with FANCA gene variants were divided into mild and severe groups based on the type of variant, and Wilcoxon-test was used to compare the phenotypic differences between groups. Results: Of the 176 registered FA patients, 69 (39.2%) cases were excluded due to lack of definitive genetic diagnosis results, and the remaining 107 children from 15 hospitals were included in the study, including 70 males and 37 females. The age at transplantation treatment were 6 (4, 9) years. The enrolled children were involved in 10 pathogenic genes, including 89 cases of FANCA gene, 7 cases of FANCG gene, 3 cases of FANCB gene, 2 cases of FANCE gene and 1 case each of FANCC, FANCD1, FANCD2, FANCF, FANCJ, and FANCN gene. Compound heterozygous or homozygous of loss-of-function variants account for 69.2% (72/104). Loss-of-function variants account for 79.2% (141/178) in FANCA gene variants, and 20.8% (37/178) were large exon deletions. Fifty-five children (51.4%) had chromosome breakage test records, with a positive rate of 81.8% (45/55). There were 172 congenital malformations in 80 children.Café-au-Lait spots (16.3%, 28/172), thumb deformities (16.3%,28/172), polydactyly (13.9%, 24/172), and short stature (12.2%, 21/172) were the most common congenital malformations in Chinese children with FA. No significant difference was found in the number of congenital malformations between children with severe (50 cases) and mild FANCA variants (26 cases) (Z=-1.33, P=0.185). Conclusions: FANCA gene is the main pathogenic gene in children with FA, where the detection of its exon deletion should be strengthened clinically. There were no phenotypic differences among children with different types of FANCA variants. Chromosome break test is helpful to determine the pathogenicity of variants, but its accuracy needs to be improved.

Clinical features and prognosis of juvenile myelomonocytic leukemia: an analysis of 63 cases / 中国当代儿科杂志

OBJECTIVES@#To investigate the clinical features of juvenile myelomonocytic leukemia (JMML) and their association with prognosis.@*METHODS@#Clinical and prognosis data were collected from the children with JMML who were admitted from January 2008 to December 2016, and the influencing factors for prognosis were analyzed.@*RESULTS@#A total of 63 children with JMML were included, with a median age of onset of 25 months and a male/female ratio of 3.2∶1. JMML genetic testing was performed for 54 children, and PTPN11 mutation was the most common mutation and was observed in 23 children (43%), among whom 19 had PTPN11 mutation alone and 4 had compound PTPN11 mutation, followed by NRAS mutation observed in 14 children (26%), among whom 12 had NRAS mutation alone and 2 had compound NRAS mutation. The 5-year overall survival (OS) rate was only 22%±10% in these children with JMML. Of the 63 children, 13 (21%) underwent hematopoietic stem cell transplantation (HSCT). The HSCT group had a significantly higher 5-year OS rate than the non-HSCT group (46%±14% vs 29%±7%, P<0.05). There was no significant difference in the 5-year OS rate between the children without PTPN11 gene mutation and those with PTPN11 gene mutation (30%±14% vs 27%±10%, P>0.05). The Cox proportional-hazards regression model analysis showed that platelet count <40×109/L at diagnosis was an influencing factor for 5-year OS rate in children with JMML (P<0.05).@*CONCLUSIONS@#The PTPN11 gene was the most common mutant gene in JMML. Platelet count at diagnosis is associated with the prognosis in children with JMML. HSCT can improve the prognosis of children with JMML.

Study on the correlation between ceramic and chronic obstructive pulmonary disease in Foshan City / 中华劳动卫生职业病杂志

Objective: To study the correlation between ceramic and chronic obstructive pulmonary disease (COPD), and explore its related risk factors. Methods: In January 2021, five representative ceramic enterprises were selected from Chancheng District, Nanhai District, Gaoming District and Sanshui District of Foshan City. The ceramic workers who came to Chancheng Hospital of Foshan First People's Hospital for physical examination from January to October 2021 were selected as the research objects, and 525 people were included. Conduct questionnaire survey and pulmonary function test. Logistic regresion was performed to analyze the influencing facters of COPD among ceramic workers. Results: The subjects were (38.51±1.25) years old, 328 males and 197 females, and the detection rate of COPD was 9.52% (50/525). The incidence of respiratory symptoms such as dyspnea, chronic cough, wheezing and chest tightness, the detection rates of abnormal lung age, abnormal lung function and COPD in males were higher than those in females (P<0.05). The logistic regression analysis showed that male, age, working years, smoking status and family history of COPD were the risk factors for COPD among ceramic workers (P<0.05) . Conclusion: The ceramic workers are the high risk population of COPD. We should do a good job in health education, and do a regular physical examination to find the changes of lung function in time, and prevent the occurrence of COPD as soon as possible.

Research progress of specific pro-resolving mediators in ocular surface diseases / 国际眼科杂志(Guoji Yanke Zazhi)

@#Specific pro-resolving mediators(SPM)are a class of lipid mediators that trigger and orchestrate the resolution of inflammation, which formed in cells by the metabolism of polyunsaturated fatty acids. SPM pathway and receptors are highly expressed inocular surface, and constitute endogenous SPM networks which are important for maintaining ocular surface health and immune homeostasis. Recent evidence indicates that SPM and their analogs are essential mediators in promoting corneal wound healing, corneal nerve regeneration, and inhibiting the immune inflammatory response of corneal transplant rejection, allergic conjunctivitis and microbial keratitis. In addition, they are potential therapeutic drug targets of dry eye disease, and provide novel insight on the research and treatment of ocular surface diseases. Here, we will review and discuss evidence for SPM as important endogenous regulators of ocular surface health and disease and their therapeutic potential.

CRISPR/Cas9 Targeted Editing of Osiris24 in Drosophlia melanogaster / 中国生物化学与分子生物学报

The gene expression of Osiris is coincident with the timing of chitin deposition. Osiris gene may be involved in the developmental regulation of insect cuticle. The objective of this study is to generate the gene-edited flies with Osiris24 by CRISPR/Cas9-mediated editing system to understand the traits of Osiris24 mutant flies and the expression pattern of Osiris24. Two sgRNA targeted sequences were designed according to the sequence of exon 1 of Osiris24 and inserted into pCFD4 vector backbone. A donor vector with Gal4 protein sequence was constructed. Above two plasmids were mixed and injected into nosCas9 fly embryos to generate GO generation. The results showed that 92.8% GO flies have Gal4 protein insert in genome. Homozygous mutants of Osiris24 were lethal at the embryonic stage or first-instar stage, and no visible phenotype was observed in heterozygous mutants. Osiris24 is expressed throughout larval and pupal stages. At the larval stage, Osiris24 is mainly expressed in the integument, foregut and hind-gut, while Osiris24 is expressed in the integument and wings at the pupal stage. These results indicated that Osiris24 plays an important role in the development of Drosophila. This study provides a research model for in-depth exploration of Osiris gene function.

Anesthesia Management at Fuwai Hospital:Practice, Evidence and Outcomes / 中国医学科学杂志(英文版)

Fuwai Hospital was established in 1956 and the Anesthesia Department of Fuwai Hospital was one of the earliest anesthesia departments then in China. Under the leadership of several department directors and with the concerted efforts of all generations of colleagues, the Anesthesia Department of Fuwai Hospital has dramatically transformed, upgraded and modernized. For more than six decades, the Anesthesia Department has been providing high-quality peri-operative anesthesia care for cardiovascular surgeries, conducting innovative experimental and clinical researches, and offering comprehensive training on cardiovascular anesthesiology for professionals across China. Currently, Fuwai Hospital is the National Center for Cardiovascular Diseases of China and one of the largest cardiovascular centers in the world. The present review introduces the Anesthesia Department of Fuwai Hospital, summarizes its current practice of anesthesia management, the outcomes of cardiovascular surgeries at Fuwai Hospital, accumulates relevant evidence, and provides prospects for future development of cardiovascular anesthesiology.

Pharmacokinetics and pharmacodynamics of pegylated recombinant human granulocyte colony-stimulating factor in children with acute lymphoblastic leukemia: a prospective control trial / 中国当代儿科杂志

OBJECTIVE@#To study the pharmacokinetic characteristics, clinical effect, and safety of pegylated recombinant human granulocyte colony-stimulating factor (PEG-rhG-CSF) in children with acute lymphoblastic leukemia (ALL).@*METHODS@#A prospective study was performed on children with ALL who cyclophosphamide, cytarabine, and 6-mercaptopurine were used for consolidation therapy. PEG-rhG-CSF (PEG-rhG-CSF group) or rhG-CSF (rhG-CSF group) was injected after chemotherapy. The plasma concentration of PEG-rhG-CSF was measured, and clinical outcome and safety were observed for both groups.@*RESULTS@#A total of 17 children with ALL were enrolled, with 9 children in the PEG-rhG-CSF group and 8 children in the rhG-CSF group. In the PEG-rhG-CSF group, the peak concentration of PEG-rhG-CSF was 348.2 ng/mL (range 114.7-552.0 ng/mL), the time to peak was 48 hours (range 12-72 hours), and the half life was 14.1 hours (range 11.1-18.1 hours). The plasma concentration curve of PEG-rhG-CSF was consistent with the mechanism of neutrophil-mediated clearance. Compared with the rhG-CSF group, the PEG-rhG-CSF group had a significantly shorter median time to absolute neutrophil count (ANC) recovery (P0.05).@*CONCLUSIONS@#The pharmacokinetic characteristics of PEG-rhG-CSF in children with ALL receiving consolidation chemotherapy are consistent with the mechanism of neutrophil-mediated clearance, with a short half life and fast recovery of ANC, and there are no significant differences in safety between PEG-rhG-CSF and rhG-CSF.

The Correlation of Minimal Residual Disease with Prognosis in TCF3-PBX1 / 中国实验血液学杂志

OBJECTIVE@#To investigate the consistency between FCM and PCR on the detecting of MRD in TCF3-PBX1@*METHODS@#55 cases of paediatric TCF3-PBX1@*RESULTS@#Among the 55 children with TCF3-PBX1@*CONCLUSION@#The detection result of MRD in TCF3-PBX1 detect by FCM and PCR shows better consistency. MRD positivity detected by FCM at the end of induction therapy (day 33) predicts a high risk of relapse in TCF3-PBX1 ALL patients.

Clinical features and gene mutations of children with Shwachman-Diamond syndrome and malignant myeloid transformation / 中国当代儿科杂志

OBJECTIVE@#To study the clinical features and genetic mutations of children with Shwachman-Diamond syndrome (SDS) and malignant myeloid transformation.@*METHODS@#Next-generation sequencing was used to analyze the gene mutations in 11 SDS children with malignant myeloid transformation, and their clinical features and genetic mutations were analyzed.@*RESULTS@#Of the 11 children with SDS, 9 (82%) presented with refractory cytopenia of childhood (RCC), 1 (9%) had myelodysplastic syndrome with excess blasts (MDS-EB), and 1 (9%) had acute myeloid leukemia with myelodysplasia-related changes (AML-MRC). The median age of onset of malignant myeloid transformation was 48 months (ranged 7 months to 14 years). Of the 11 children, 45% had abnormalities in the hematological system alone. Mutations of the SBDS gene were detected in all 11 children, among whom 5 (45%) had c.258+2T>C homozygous mutation and 3 (27%) had c.184A>T+c.258+2T>C compound heterozygous mutation. The new mutations of the SBDS gene, c.634_635insAACATACCTGT+c.637_638delGA and c.8T>C, were rated as "pathogenic" and "possibly pathogenic" respectively. The 3-year predicted overall survival rates of children transformed to RCC and MDS-EB/AML-MRC were 100% and 0% respectively (P=0.001).@*CONCLUSIONS@#SDS children may have hematological system symptoms as the only manifestation, which needs to be taken seriously in clinical practice. The type of malignant transformation is associated with prognosis.

Quality Evaluation of Chrysanthemum indicum Based on Fingerprint, Pattern Recognition and Simultaneous Determination of Multi-Component / 中国药学杂志

OBJECTIVE: To establish a method based on HPLC fingerprint, pattern recognition and simultaneous determination of multi-component for quality evaluation of Chrysanthemum indicum, and investigate its feasibility and accuracy. METHODS: HPLC fingerprints of 21 batches of Chrysanthemum indicum were established, similarity analysis, principal component analysis and cluster analysis were performed on fingerprints of Chrysanthemum indicum by TCMYS fingerprint software, and 12 common chromatographic peaks in fingerprints were identified, and 12 components were quantified by external standard method. RESULTS: The HPLC fingerprints of 21 batches of Chrysanthemum indicum were similar, and their classification trend was found. Seventeen common peaks were found and 12 of them were identified as neochlorogenic acid, chlorogenic acid, caffeic acid, 1,3-dicaffeoylquinic acid, cynaroside, isochlorogenic acid C, isochlorogenic acid A, (1S, 3R, 4R, 5R)-3, 4-bis[[(E)-3-(3, 4-dihydroxyphenyl)prop-2-enoyl]oxy]-1, 5-dihydroxycyclohexane-1-carboxylic acid, linarin, luteolin, apigenin and acacetin. Good linear relationship (r≥0.999 6) was shown for the external standard method, the average recoveries were between 95.84% and 102.46%, and the RSDs were all below 2.72%. CONCLUSION: Combination of HPLC fingerprint, pattern recognition and simultaneous determination of multi-component are accurate and reliable for quality evaluation of Chrysanthemum indicum, which can provide important information for the overall quality evaluation of Chrysanthemum indicum.

Effect of Mangiferin on mRNA Expressions of Hepatic PRPS, PRPPAT and Cerebral HGPRT in Hyperuricemic Mice Induced by Potassium Oxonate / 中国实验方剂学杂志

Objective:To investigate the effect of mangiferin on the mRNA expression of phosphoribosylpyrohoosphate synthetase (PRPS), phosphate ribose pyrophosphate amide transferase (PRPPAT) in liver and hypoxanthine-guanine phosphate transfer enzyme (HGPRT) in brain of hyperuricemic mice induced by potassium oxonate. Method:Hyperuricemic mice were induced through intraperitoneal injection with uricase inhibitor potassium oxonate. The serum uric acid level was determined by the phosphotungstic acid method. The mRNA expression levels of PRPS and PRPPAT in liver as well as HGPRT in brain of hyperuricemic mice were measured by reverse transcriptase polymerase chain reaction (RT-PCR). Result:An intraperitoneal injection with potassium oxonate caused a marked increase in serum uric acid level, compared with normal control group (P-1 was able to significantly reduce serum uric acid levels, compared with hyperuricemic control group (PPConclusion:The hyporuricemic effect of mangiferin might not be related with PRPS, PRPPAT and HGPRT in therapeutic dose.

Effect of HE4 and PAX8 gene knockdown on malignant biological behaviors of epithelial ovarian cancer cells treated with TC regimen / 中国肿瘤生物治疗杂志

@#Objective: To investigate the effect of human epididymal protein 4 (HE4) and paired box gene 8 (PAX8) gene knockdown on proliferation, migration, invasion and apoptosis of human epithelial ovarian cancer OVCAR3 cells treated with TC regimen (paclitaxel+carboplatin). Methods: Sequences of single-target siRNA (HE4-siRNA or PAX8-siRNA) and double-target siRNA (HE4+PAX8siRNA) as well as negative siRNAwere respectively designed and synthesized, and then linked with plasmid vector pGCsi-H1 to obtain the recombinant plasmids. The obtained recombinant plasmids were then transfected into human epithelial ovarian cancer OVCAR3 cells, namely HE4-siRNA group, PAX8-siRNA group, HE4+PAX8-siRNA group and siRNA-NC group, respectively. The blank control group was also set up (without any treatment). The cells in above five groups were treated with TC regimen (paclitaxel 3.13 g/ml+carboplatin 2.82 µg/ml), and the changes in proliferation, migration, invasion and apoptosis of the cells were detected by MTT, wound-healing assay, Transwell chamber assay, and flow cytometry, respectively. Results: After knocking down the HE4 and PAX8 genes, compared with siRNA-NC group and blank control group, the proliferation, migration and invasion abilities of OVCAR3 cells in HE4-siRNA group, PAX8-siRNA group and HE4+PAX8-siRNA group significantly decreased (all P<0.01), and the apoptosis rate significantly increased (P<0.01), especially in HE4+PAX8-siRNA group. Conclusion: Knockout of either HE4 or PAX8 can enhance the effect of TC regimen on inhibiting proliferation, migration and invasion as well as promoting apoptosis of epithelial ovarian cancer cells, and the effect of simultaneous down-regulation of HE4 together with PAX8 is better.

The risk factors and preventive measures of hospital infections on preoperative children with cleft lips and palates / 华西口腔医学杂志

OBJECTIVE@#To analyze the risk factors of preoperative upper respiratory infections in children with cleft lips and palate (CLP) and investigate preventive measures to reduce infections and improve the quality of treatments.@*METHODS@#A total of 510 children with CLP of ages 3 years old or younger were selected from hospital cases from June to December 2017. The test group comprised 50 children with upper respiratory infections, whereas the control group comprised 460 children without upper respiratory infections. A t-test and a multivariate logistic analysis were utilized to analyze the risk factors and to investigate the preventive measures.@*RESULTS@#Feeding patterns, the presence of infected companions during hospitalization, and ventilation at night were statistically significant. The feeding patterns and the presence of infected companions during hospitalization were independent risk factors for upper respiratory infections in children with CLP.@*CONCLUSIONS@#Bottle feeding, infected companions during hospitalization, and the absence of window ventilation at night are risk factors for preoperative upper respiratory infections in children 3 years old or younger with CLP. Among the risk factors identified, feeding patterns and the presence of infected companion during hospitalization were the most influential. Medical staff members should streng-then corresponding health education and nursing measures to control the risk factors.

Clinical features of Wiskott-Aldrich syndrome: an analysis of 13 cases / 中国当代儿科杂志

OBJECTIVE@#To study the clinical features of Wiskott-Aldrich syndrome (WAS) in children.@*METHODS@#A retrospective analysis was performed for the clinical data of 13 children with WAS.@*RESULTS@#All 13 children were boys, with a median age of onset of 3 months (range 1-48 months) and a median age of 24 months (range 1-60 months) at the time of diagnosis. Of the 13 children, only 3 had typical WAS and the remaining 10 children had X-linked thrombocytopenia (XLT). The mean WAS score was 2 (range 1-3), the mean platelet count was 20.5×10/L [range (13-46)×10/L], and the mean platelet volume was 8.1 fl (range 6.7-12.1 fl). Lymphocyte subsets and immunoglobulins were measured for 4 children, among whom 1 (25%) had a reduction in both the percentage of CD3T cells per lymphocyte and lymphocyte per nuclear cells, 1(25%) had a reduction in CD3CD56 NK cells. Among these 4 children, 1 (25%) had an increase in IgG, 2 (50%) had a reduction in IgM, 1 (25%) had a reduction in IgA, and 4 (100%) had an increase in IgE. A total of 14 gene mutations belonging to 13 types were found in 13 children, among which there were 9 missense mutations (65%), 2 splicing mutations (14%), 2 nonsense mutation (14%), and 1 frameshift mutation (7%). The median follow-up time was 39 months (range 3-62 months), and all 13 children survived.@*CONCLUSIONS@#Children with WAS often have a young age of onset, and most of them are boys. Major clinical features include thrombocytopenia with a reduction in platelet volume. Missense mutation is the main type of gene mutation.

New mutation site of SEC23B gene in type Ⅱ congenital erythrocythememia anemia: one case report and literatures review / 中华血液学杂志

Objective: To enrich the gene mutation sites and accumulate treatment experience of congenital dyserythropoietic anemia (CDA) type Ⅱ by reporting one case of CDA patient with new mutation site of SEC23B and was successfully treated by homozygous allogeneic hematopoietic stem cell transplantation (allo-HSCT) . Methods: The mutation within SEC23B gene in a child case with the reduced hemoglobin for more than 3 months, and his family were analyzed in combination with literatures review. Results: A 3-day 5-month female child was admitted due to "decreasing hemoglobin for more than 3 months" , blood routine test showed HGB 44 g/L, positive for acid hemolysis test (Ham test) . Bone marrow showed that the proportion of erythroid line was 69%, mainly middle and late juvenile erythrocytes, binuclear and odd nucleated erythrocytes could be observed, and nuclear fragmentation and nuclear budding could be seen occasionally in nucleated erythrocytes, transmission electron microscopy disclosed that bone marrow harbored the typical double-layer membrane structure of nuclear erythrocytes. There were two unreported new mutation sites in the SEC23B gene, including 1504 G>C/wt and c. 2254-2255 insert A/wt. The two mutations were derived from the father and mother of the child respectively. At the late stage, the child was successfully treated with allo-HSCT, the original mutation turned negative. Conclusion: This study reported the mutation type of SEC23B gene insertion for the first time in China. Allo-HSCT could be utilized as a treatment for CDA.

Postoperative local incision analgesia for acute pain treatment in patients with hepatocellular carcinoma

Summary Objective: The present study aimed to investigate the analgesic effect and safety of using local incision analgesia to treat acute postoperative pain in patients with hepatocellular carcinoma (HCC). Method: A cohort of 60 patients undergoing liver cancer resection was randomly divided into three groups (n=20 per group): local incision analgesia (LIA) group, which received local infiltration with ropivacaine combined with a postoperative analgesia pump; intravenous patient-controlled analgesia (PCA) group, which received fentanyl intravenous analgesia postoperatively; and the control group, which received tramadol hydrochloride injection postoperatively according to the NRS scoring system. The postoperative analgesic effect in each group was compared and tumor recurrence (survival) was analyzed using the Kaplan-Meier method. Results: NRS scores, rate of analgesic usage, ambulation time (h) and intestinal function recovery time (h) were significantly reduced in LIA group compared with the control group at each postoperative time point (6, 12, 24 and 48 hours; p<0.05). Additionally, the NRS scores of LIA patients at 12 hours post-surgery was significantly reduced compared with PCA group (p<0.05), and the occurrence of postoperative adverse events in LIA group was significantly lower than that in PCA group (p<0.05). Survival analysis demonstrated that the mean survival time (tumor recurrence) was significantly increased in LIA group compared with the control group (χ2=4.749; p=0.029). Conclusion: Local incision analgesia improves the analgesic effect, causes fewer adverse reactions and increases postoperative survival time. Our study demonstrated that local incision analgesia is a safe and effective method of postoperative pain management following hepatectomy.

Effects of bradykinin on the proliferation of rabbit corneal endothelial cells and the expression of tight junction-related proteins ZO-1 and ZONAB / 眼科新进展

Objective To investigate the effects of bradykinin (BK) on the proliferation of rabbit corneal endothehal ceils (RCECs) and the expression of tight junction-related proteins zonula occludens-1 (ZO-1) and zonula occludens-1-associated nucleic-acid-binding protein (ZONAB),and to explore the underlying mechanisms of BK on cell proliferation in corneal endothelium.Methods RCECs at logarithmic growth phase were treated with different concentrations of BK (0.01,0.1,1,10 μmol · L-1) BK group,with the controls left untreated.Morphological changes of cells in each group were examined under phase-contrast microscope,and MTT assays were used to detect cell proliferation at 24 h,48 h,72 h,96 h after BK treatment.And,at 72 h,the expression levels of ZO-1 and ZONAB protein were determined by Western blot.Results After 72 h of treatment,the cells in each group were fused into pieces and closely linked into a monolayer;but after 96 h,the growth of the cells was restricted,with the intercellular space become larger and the cells exfoliated.Compared with the control group,BK induced a significant increase of absorbance value and cell viability,and the differences were statistically significant (all P ＜ 0.001),and the promoting effects showed a concentration-dependent manner,and 1 μmol · L-1 BK demonstrated the strongest regulative effect (P ＜ 0.001).Western blot results showed that BK upregulated the expression of ZO-1 and ZONAB protein in a concentration-dependent manner.Conclusion BK can stimulate the proliferation of RCECs in a time-and concentration-dependent manner,and the mechanisms are probably associated with ZO-1/ZONAB-mediated signaling pathway.

The Effect of PCSA with Parecoxib Sodium and Sufentanil on the Postoperative Delirium in Patients after the Spinal Surgery / 昆明医科大学学报

Objective To investigate the effect of patient controlled subcutaneous analgesia (PCSA) of Sufentamil and Parecoxib sodium on the postoperative delirium in patients after the spinal surgery. Methods 240 patients with ASA Ⅱ- Ⅲ age 18-64 yrs after the spinal surgery were divided into two groups: group NO-PCSA (analgesic management: patients were accepted pethidine 25-50 mg intramuscular injection n = 120 );group PCSA (analgesic management: patient controlled subcutaneous analgesia was started since skin suture with the following composition:Sufentanil 0.9 ug/(kg.d)+Parecoxib sodium 120 mg+Tropisetron 10 mg+normal saline 150 mL. The PCSA setting was as follows:background infusion at 2 mL/h, a bolus dose of 0.5 mL, lockout interval 15 min. If the VAS was greater than 5, patient was accepted pethidine 25-50 mg intramuscular injection n=120) . The effect of analgesia was assessed by visual analogue scale (VAS) . The delirium was assessed by the confusion assessment, VAS and delirium were recorded with in 2, 24, 48, 72 hours postoperatively. Results During the analgesia period, the VAS and the incidence of postoperative delirium were significantly lower in group PCSA than those in group NO-PCSA ( <0.05) . Conclusion PCSA of Sufentamil and Parecoxib sodium have a good postoperative analgesic effect in patients after the spinal surgery. It is an effective measure and the incidence of postoperative delirium can be decreased by reliefing postoperative pain.

HER2 Expression in Childhood ETV6/RUNX1 Acute Lymphoblastic Leukemia and Its Correlation with Clinical Features / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To explore the HER22 expression in children with ETV6/RUNX1 (E/R)-positive acute lymphoblastic leukemia(ALL) and to investigate the relationship between the HER2 expression and clinical features.</p><p><b>METHODS</b>Thirty-seven newly diagnosed E/R-positive ALL children and 6 controls (4 cases of ITP and 2 healthy children) were selected in Institute of hematology and blood disease hospital. The 37 patients were divided into standard risk (SR), intermediate risk(IR), high risk(HR) groups according to risk stratification; and they were divided into relapse and non-relapse groups according to follow-up result. The CD10CD19 cells were sorted by flow cytometry. The mRNA was extracted from these cells. Real-time fluorescent quantitative PCR was used to detect the expression level of HER2.</p><p><b>RESULTS</b>Among the 37 cases, 51.35% (n=19) were boys and 48.65% (n=18) were girls and their median age was 4.72 (1.72-11.99) years old. Among the 6 controls, 50% (n=3) were boys and 50% (n=3) were girls and the median age was 5.24 (1.53-13.17) years old. The expression level of HER2 in E/R-positive ALL patients were lower than that in controls (P<0.05). Although the difference of HER2 expression level between the 2 groups failed to achieve statistical significance, the expression level of HER2 in relapse patients were significantly lower than that in non-relapse patients, and the HER2 expression in HR group patients were lower than that in SR and IR groups. In addition, there was no significant correlation between the expression level of HER2 and the sex, age, initial white blood cell count, blast cell percentage and the level of LDH (P>0.05).</p><p><b>CONCLUSION</b>The expression level of HER2 in E/R ALL patients is lower than that in controls, and in relapse group lower than that in non-relapse patient. Thus, HER2 may play important roles in the pathogenesis and relapse mechanism of pediatric E/R-positive ALL patients.</p>

Value of multiparameter flow cytometry in the diagnosis and prognostic evaluation of childhood myelodysplastic syndrome / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate the value of multiparameter flow cytometry (MFC) and flow cytometric scoring system (FCSS) in the diagnosis and prognostic evaluation of childhood myelodysplastic syndrome (MDS).</p><p><b>METHODS</b>A retrospective analysis was performed for the clinical data of 42 children who were diagnosed with MDS. MFC was performed to investigate the phenotype and proportion of each lineage of bone marrow cells. The correlations of FCSS score with MDS type, International Prognostic Scoring System (IPSS) score, and revised IPSS (IPSS-R) score were analyzed.</p><p><b>RESULTS</b>Of all the 42 children, 20 (48%) had an increase in abnormal marrow blasts, 19 (45%) had a lymphoid/myeloid ratio of >1, 14 (33%) had abnormal cross-lineage expression of lymphoid antigens in myeloid cells, 8 (19%) had abnormal CD13/CD16 differentiation antigens, 5 (12%) had abnormal expression of CD56, 3 (7%) had reduced or increased side scatter of granulocytes, 3 (7%) had reduced expression of CD36 in nucleated red blood cells, 2 (5%) had reduced expression of CD71 in nucleated red blood cells, 1 (2%) had absent expression of CD33 in myeloid cells, 1 (2%) had reduced or absent expression of CD11b in granulocytes, and 1 (2%) had absent expression of CD56 and CD14 in monocytes. There were significant differences in the median overall survival time and event-free survival time among the low-, medium-, and high-risk FCSS groups (P<0.05). Among the low-, medium-, and high-risk FCSS groups, the low-risk FCSS group had the highest 2-year overall survival rate, while there was no significant difference between the medium- and high-risk FCSS groups (P>0.05). The three groups had a 2-year event-free survival rate of 95%, 60%, and 46% respectively (P<0.05). FCSS score was positively correlated with MDS type, IPSS score, and IPSS-R score (P<0.05).</p><p><b>CONCLUSIONS</b>MFC and FCSS help with the diagnosis and prognostic evaluation of childhood MDS.</p>