Objective:To retrospectively analyze the treatment status of tyrosine kinase inhibitors (TKI) in newly diagnosed patients with chronic myeloid leukemia (CML) in China.Methods:Data of chronic phase (CP) and accelerated phase (AP) CML patients diagnosed from January 2006 to December 2022 from 77 centers, ≥18 years old, and receiving initial imatinib, nilotinib, dasatinib or flumatinib-therapy within 6 months after diagnosis in China with complete data were retrospectively interrogated. The choice of initial TKI, current TKI medications, treatment switch and reasons, treatment responses and outcomes as well as the variables associated with them were analyzed.Results:6 893 patients in CP ( n=6 453, 93.6%) or AP ( n=440, 6.4%) receiving initial imatinib ( n=4 906, 71.2%), nilotinib ( n=1 157, 16.8%), dasatinib ( n=298, 4.3%) or flumatinib ( n=532, 7.2%) -therapy. With the median follow-up of 43 ( IQR 22-75) months, 1 581 (22.9%) patients switched TKI due to resistance ( n=1 055, 15.3%), intolerance ( n=248, 3.6%), pursuit of better efficacy ( n=168, 2.4%), economic or other reasons ( n=110, 1.6%). The frequency of switching TKI in AP patients was significantly-higher than that in CP patients (44.1% vs 21.5%, P<0.001), and more AP patients switched TKI due to resistance than CP patients (75.3% vs 66.1%, P=0.011). Multi-variable analyses showed that male, lower HGB concentration and ELTS intermediate/high-risk cohort were associated with lower cytogenetic and molecular responses rate and poor outcomes in CP patients; higher WBC count and initial the second-generation TKI treatment, the higher response rates; Ph + ACA at diagnosis, poor PFS. However, Sokal intermediate/high-risk cohort was only significantly-associated with lower CCyR and MMR rates and the poor PFS. Lower HGB concentration and larger spleen size were significantly-associated with the lower cytogenetic and molecular response rates in AP patients; initial the second-generation TKI treatment, the higher treatment response rates; lower PLT count, higher blasts and Ph + ACA, poorer TFS; Ph + ACA, poorer OS. Conclusion:At present, the vast majority of newly-diagnosed CML-CP or AP patients could benefit from TKI treatment in the long term with the good treatment responses and survival outcomes.

OBJECTIVE: To explore the genetic mechanism underlying a case with para-Bombay phenotype. METHODS: The ABO and Lewis phenotype were identified with serological methods. The coding regions of exons 6 and 7 of the ABO and FUT1 genes were amplified with PCR and directly sequenced. Haploid sequence analysis was carried out on the variant sites of the FUT1 gene. RESULTS: Serological analysis confirmed that the proband has a rare para-Bombay phenotype. Direct sequencing revealed that he was a B.01/O.01.02 heterozygote for the ABO gene, and had heterozygous deletion for the 768 and 881-882 sites of the FUT1 gene. Further haploid analysis showed that the c.881_882delTT deletion has occurred in one haploid while c.768delC was present in the other haploid. The proband was therefore determined as a FUT1*01N.13/01N.20 heterozygote, which have resulted in frameshift in polypeptide chain p.Phe294Cysfs*40 and p.Val257Phefs*23, respectively. CONCLUSION A rare bi-allelic heterozygous deletion of para-Bombay phenotype has been identified in a blood donor. The c.881_882delTT and c.768delC deletions may decrease the activity of α-1,2-fucosyltransferase.
Animals ; Male ; ABO Blood-Group System/genetics* ; Alleles ; Fucosyltransferases/genetics* ; Genotype ; Heterozygote ; Mutation ; Phenotype ; Humans

Transtibial tunnel reconstruction is one of the classical surgical techniques for posterior cruciate ligament (PCL) reconstruction. However, this surgical technique inevitably produces the "killer turn" effect. Specifically, during the transtibial tunnel reconstruction, there is a sharp tunnel edge at the exit of the tibial tunnel, and the graft has a large stress at this edge, which leads to the failure of transplantation due to the repeated friction between the graft and the tunnel edge. The "killer turn" effect may lead to the "residual laxity", thus resulting in postoperative knee instability, affecting the long-term efficacy of reconstructive surgery and reducing the postoperative satisfaction of patients. In recent years, many scholars have proposed a series of improved techniques for PCL reconstruction in dealing with the "killer turn" effect, including tibial inlay technique and improved transtibial tunnel technique. The authors review the formation mechanism of "killer turn" effect and methods to eliminate or reduce the effect, in order to provide a reference for improving the effect in PCL reconstruction.

To investigate risk factors of death in newborns with congenital diaphragmatic hernia (CDH). A total of 126 newborns with CDH from June 2012 to September 2018 were enrolled. Concomitant malformations were recorded by descriptive analysis. Newborns received surgical treatment (=120) for CDH were divided into survival group and fatal group. The risk factors of death were analyzed by univariate and multivariate logistic regression and the ROC curve with generated with relevant variables. There were 55 CDH newborns with concomitant malformations (43.7%), including 20 cases (15.9%) with multi-malformation. Logistic regression analysis showed that premature rupture of membranes (PROM), postoperative atelectasis, long duration of postoperative mechanical ventilation, postoperative high oxygenation index (OI) were related to death (all <0.05), and the delayed surgery was a protective factor (<0.05). In ROC analysis of postoperative OI in predicting death, the area under the curve (AUC) was 0.841, with the cutoff value of 5.74, the sensibility and specificity of OI was 81.0% and 75.0%, respectively(<0.01). Newborns with CDH have a high rate of malformations. The risk factors of death were PROM, postoperative atelectasis, postoperative long duration of mechanical ventilation and higher postoperative OI, and delayed surgery may reduce mortality.
Hernias, Diaphragmatic, Congenital ; diagnosis ; mortality ; surgery ; Humans ; Infant ; Infant Death ; Infant, Newborn ; Logistic Models ; ROC Curve ; Retrospective Studies ; Risk Factors ; Sensitivity and Specificity

OBJECTIVETo review the application of thoracoscopic repair for treatment of congenital diaphragmatic hernia in neonates, so as to improve the cure rate.

METHODSClinical data of 47 neonates with congenital diaphragmatic hernia receiving thoracoscopic repair from June 2012 to June 2017 were reviewed. The admission age, gestational age, birth weight, timing of diagnosis, hernia location, clinical manifestation, surgical timing, surgical method, operation time, postoperative mechanical ventilation time of patients were analyzed.

RESULTSThere were 42 cases of left diaphragmatic hernia and 5 cases of right diaphragmatic hernia. Thirteen cases were diagnosed prenatally. Primary diaphragmatic repair was successfully accomplished under thoracoscope in 45 neonates without perioperative complications, while 2 patients were converted to open surgery. The average operation time was (63±13) min (42-150 min), the average blood loss was (3.0±1.7) mL (1.0-9.0 mL), and the average postoperative mechanical ventilation time was (3.9±1.4) d (2.0-11.0 d). Two patients died and the treatment was withdrawn in 3 patients with an overall cure rate of 89.4% (42/47).

CONCLUSIONSThoracoscopic repair is effective and can be used as first-choice treatment of diaphragmatic hernia in neonates.

Objective To evaluate a new type of adult scapular anatomical locking plate which was designed for fixation of the major fracture fragment based on the characteristics of major fracture location and posterior surgical approach.Methods The Computed Tomography (CT) data of the scapula were collected for this studv of the 80 normal adults who had undergone CT scanning from January 2015 to January 2018 at Department of Orthopedics,Hanzhong Central Hospital.They were 43 males and 37 females.3D models of the scapula were reconstructed using the CT data by computer.After scapular neck (CD),scapular lateral border (D)E),angulus scapulae (EF),scapular spine (HJJ2 and HKK2),and angles of the scapula (∠ CD)E,∠ DEF,∠ HKK2 and ∠ HJJ2) were measured,our new type of adult scapular anatomical locking plate was designed to meet the requirements of operation and fixation.Results The main parameters of the scapula at the major fixation region were CD of 10.27 ±0.99 mm,DE of 73.03 ±6.27 mm,EF of 52.03 ±6.85mm,HJ of 36.27 ± 2.10 mm,HKof41.44±2.58mm,∠ CDE of128.61° ± 6.30°,∠DEF1of 167.11 °±3.83°,∠DEF2 of 173.01 °±4.45°,∠HJJ2 of 133.43°±7.47°,and ∠HKK2 of 123.55°± 7.84°.The new type of claw-shaped scapular locking plate we had designed conformed to the segmental lengths and the angles between the adjacent segments of the scapula.Conclusions Our new type of adult scapular anatomical locking plate mav be used to fix the major scapular fracture fragment,providing a new choice to solve the problem in fixation of complex scapular fractures.However,its clinical practicality and effectiveness needs further clinical research.

Objective To study the relevance of 1iver fibrosis at Kasai procedure with age, degree of pathological changes of liver tissue, header bile ductuler proliferation and pathological feature of portal fibrous tissue in biliary atresia patients. Methods From January 2013 to June 2016, 48 biliary atresia patients accepted Kasai procedure, and liver tissue biopsy and portal fibrous tissue excision was done. Level of liver fibrosis was evaluated under microscope using Masson trichrome staining and HE staining, and extent of ductular reaction was evaluated using CKl9 immunohistochemistry staining with image analysis technology. Based on classification of Okamoto severity degree of liver fibrosis, forty eight patients were divided into two groups:low-grade liver fibrosis group (23 patients)and severe liver fibrosis group (25 patients). The relevance of age at Kasai procedure, liver pathological changes, header bile ductuler proliferation, portal fibrous tissue in volume and bile ductuler proliferation, inflammation reaction with severity degree of liver fibrosis was analyzed with SPSSl6.0 software statistically. Results Age of severe 1iver fibrosis group at Kasai procedure were 38- 89 d, and the mean age was (62.64 ± 14.92) d. Age of low-grade 1iver fibrosis group were 18- 92 d, and the mean age was (53.13 ± 16.89) d. There was significant difference (t=2.071, P=0.044). Mean number of patients with header bile ductuler proliferation at Kasai procedure in severe 1iver fibrosis group and low-grade 1iver fibrosis group were (17.28 ± 4.31) cases and (11.39 ± 4.00) cases, and there was significant difference (t=4.900, P=0.000). There was significant difference in degree of hepatocellular pathological changes at Kasai procedure between groups (χ2 =4.691, P=0.030). There were no significant differences in the volume of portal fibrous tissue in volume (t=0.276, P=0.784), portal bile ducts proliferation (t=0.53, P=0.60) and inflammation reaction (χ2=3.074, P=0.080) between groups. Conclusions There is significant relevance between the degree of 1iver fibrosis in biliary atresia patients at Kasai procedure and age, level of hepatocellular pathological change and degree of header bile ductuler proliferation , The more older the age, the more sever the hepatocellular pathological change and the bile ductuler proliferation, the more sever the liver fibrous.

[ ABSTRACT] AIM:To investigate the effects of Chaihu Shugan powder ( CSP) on lipid metabolism and the pro-teins involved in adenosine 5’-monophosphate-activated protein kinase (AMPK)/sirtuin 1 (SIRT1) pathway in the liver tissues of the rats with non-alcoholic fatty liver disease (NAFLD).METHODS: Sprague-Dawley rats were randomly di-vided into normal control ( NC) group, with HFD ( HFD) group and CSP group.The NAFLD models were established by feeding with HFD for 16 weeks in the rats.The rats in CSP group were intragastrically administered with CSP extracts (9.6 g· kg-1 · d-1 ) , and blood and liver samples were collected 16 weeks later.Serum and liver levels of total cholesterol ( TC) and triglyceride ( TG) , and serum levels of alanine aminotransferase ( ALT) and aspartate aminotransferase ( AST) were measured using an automatic biochemical analyzer.The histological changes of liver tissues were observed with HE staining, while the lipid deposition was observed with Oil Red O staining.The ultrastructural changes of the liver tissues were observed under transmission electron microscope.Moreover, the protein levels of AMPK, phosphorylated AMPK (pAMPK), SIRT1 and uncoupling protein 2 (UCP2) in the liver were detected by Western blot.RESULTS:The results of HE staining, Oil Red O staining and electron microscopy demonstrated that NAFLD rat model was successfully estab-lished.Compared with NC group, the serum and liver levels of TC and TG, and serum level of AST in model group were markedly elevated ( P<0.01) .Moreover, the protein levels of pAMPK and SIRT1 in HFD group were markedly reduced (P<0.01), whereas UCP2 level was elevated (P<0.01).Furthermore, liver levels of TC and TG, and serum level of AST in GSP group were markedly reduced as compared with HFD group ( P<0.05 ) .The protein levels of pAMPK and SIRT1 were elevated ( P<0.05 ) , whereas the UCP2 level was reduced as compared with HFD group ( P<0.01 ) .The protein level of AMPK between the 3 groups had no significant difference.CONCLUSION: CSP attenuates hepatic lipid disorder and hepatic lipid deposition in NAFLD rats induced by feeding with HFD for 16 weeks, which is associated with the activation of AMPK/SIRT1 pathway.

Objective To explore the expression of Speckle-type POZ protein-like(SPOPL)in human glioma tis?sues and its relationship with clinical pathological parameters and prognosis. Methods Immunohistochemical and west?ern blotting method were used to detect SPOPL expression in glioma tissues and tumor peripheral tissues in 68 cases of glioma patients including 32 cases of low grade gliomas (WHO I- II), and 36 cases of high grade gliomas (WHO III-IV). T test,χ2 test, Kaplan-Meier method and the Cox regression model were used to analyze the relationship between the expression and clinical indicators. Results The expression of SPOPL was significantly lower in gliomas than in tumor pe?ripheral tissues (t=-8.754, P<0.05), the expression of SPOPL was lower in high pathological grade tissues than in low grade of glioma tissues (t=-13.552, P<0.05). SPOPL expression was associated with pathological grade ( χ2=4.023, P<0.05). Cox regression model showed that the tumor pathological grade and different SPOPL protein expression were inde?pendent risk factors for the prognosis of patients with glioma. Conclusions SPOPL may be a biomarkers of human brain gliomas and can help to evaluate the prognosis of brain glioma.

Objective To explore the unique relationship between Willis circle and cerebral watershed infarction in a Chinese population. Methods A retrospective analysis of cerebral CT angiography was conducted in 471 non-cere?bral watershed infarctions and 93 MRI-diagnosed cerebral watershed infarctions (CWI)(including External CWI and In?ternal CWI)in Department of Neurology of our hospital and compare the related variations and types of Willis circle be?tween these groups. Results Compared with non-cerebral watershed infarctions, the prevalence of Uni-FTP (short for“fetal type of the Posterior cerebral artery”) in E-CWI (36.4%, P<0.05) and Bi-FTP in I-CWI (0%, P<0.05) was signifi?cantly higher in cerebral watershed infarctions. Conclusion FTP is probably an unique risk factor in Chinese patients with CWI.