Objective:To analyze the clinical characteristics,treatment,and prognosis of adult patients with early T-cell precursor acute lymphoblastic leukemia/lymphoma(ETP-ALL/LBL).Methods:Clinical data of 113 T lymphoblastic leukemia/lymphoma(T-ALL/LBL)patients from January 2006 to January 2019 were collected from three hematology research centers,including Peking University Third Hospital,the First Medical Center of Chinese PLA General Hospital and Institute of Hematology and Blood Diseases Hospital,Chinese Medical University.The clinical characteristics and prognosis of ETP-ALL/LBL patients were analyzed compared with non-ETP-ALL/LBL patients.Results:In 113 T-ALL/LBL patients,13 cases(11.5％)were diagnosed as ETP-ALL/LBL,including 11 males,with a median age of 28(18-53)years.Compared with non-ETP-ALL/LBL patients,there were no significant differences in age,sex,incidence of large mediastinal mass,clinical stage,international prognostic index(IPI)score,white blood cell(WBC)count and lactate dehydrogenase(LDH)level among ETP-ALL/LBL patients.Among 13 ETP-ALL/LBL patients,9 cases(69.2％)achieved complete remission(CR),and there was no statistically significant difference in response rate induced by chemotherapy between ETP-ALL/LBL patients and non-ETP-ALL/LBL patients.Among patients who received chemotherapy without allogeneic hematopoietic stem cell transplantation(allo-HSCT),ETP-ALL/LBL group had a worse 5-year overall survival(OS)rate compared with non-ETP-ALL/LBL group(0 vs 7.1％,P=0.008),while in patients with allo-HSCT,there was no significant difference for 5-year OS rate between the two group(37.5％vs 40.2％,P＞0.05).Multivariate Cox regression analysis showed that CR after induction therapy,allo-HSCT,and LDH level were independent prognostic factors affecting T-ALL/LBL patients.Conclusion:No significant difference in response rate induced by chemotherapy is observed between ETP-ALL/LBL and non-ETP-ALL/LBL patients.Allo-HSCT consolidation after induction of remission therapy may have significant favorable influence on OS for patients with ETP-ALL/LBL.

Objective:To establish and evaluate a nomogram for long-term survival of patients with intrahepatic cholangiocarcinoma (ICC) after radical resection.Methods:The data of ICC patients who underwent radical resection for the first time at Zhongshan Hospital, Fudan University from January 2014 to December 2017 were retrospectively analyzed. Of 167 patients who were enrolled, there were 104 males and 63 females, with the age of (60.3±10.9) years. Tumor tissues were collected for immunohistochemical staining and interpretation. Univariate Cox regression, LASSO regression and multivariate Cox regression were used to analyze influencing factors of postoperative long-term survival after ICC. R software was used to construct a nomogram in predicting ICC prognosis.Results:Cox regression analysis showed that TNM staging, poorly differentiated tumor, positive resection margin, positive mucin 5 expression and abnormal P53 expression to be independent risk factors associated with poor long-term survival after radical resection. The prognostic nomogram model of ICC was constructed based on these factors. The C-index was 0.821. The nomogram model consistency index had a high degree of prognostic differentiation. The 45° diagonal of the 3-year postoperative calibration curve which represented the actual survival fitted well with the segmented line which represented the predicted survival of the nomogram. The area under the receiver operating characteristic curve of the nomogram model was higher than that of AJCC TNM staging (0.894 vs. 0.803, z=4.10, P<0.001). The nomogram model was more effective in predicting postoperative survival of ICC patients than the TNM staging. Conclusion:TNM staging, poorly differentiated tumor, positive resection margin, positive mucin 5 expression and abnormal P53 expression were independent risk factors for postoperative survival of ICC. The nomogram model could better evaluate long-term prognosis of ICC patients after radical resection than the traditional TNM staging system.

Objective:To investigate the role of umbilical cord mesenchymal stem cell-derived exosomes (ucMSC-exos) in acute skin wound healing in mice.Methods:ucMSC-exos were extracted by ultracentrifugation, and identified by transmission electron microscopy, Western blot analysis of exosome surface markers CD63 and TSG101, and particle size analysis. Firstly, in vitro cultured third- to fifth-passage human skin fibroblasts (HSF) were incubated with high-glucose Dulbecco′s modified Eagle′s medium (DMEM) containing 0, 1 and 2 μg/ml exosome suspension for 24 hours (negative control group, 1- and 2-μg/ml groups, respectively) , and cell counting kit-8 (CCK8) assay was performed to evaluate the effect of ucMSC-exos on the proliferative activity of HSF. Secondly, 24 male BALB/c mice aged 8 weeks were selected to construct a mouse model of full-thickness skin wound, and then divided into ucMSC-exos group and phosphate-buffered saline (PBS) group by using a random number table to be subcutaneously injected with exosome suspension and PBS respectively at multiple equidistant sites located about 1 mm apart from the wound edge. On days 0, 4, 7, 10 and 14 after operation, the wounds in mice were observed, and the percentage of residual wound area was calculated in the above two groups. On days 7 and 14 after operation, wound tissues were resected and subjected to hematoxylin and eosin (HE) and Masson staining to observe structural changes of skin tissues. On day 14 after operation, wound tissues were collected in the two groups, and real-time quantitative PCR (qRT-PCR) and Western blot analysis were performed to determine the mRNA and protein expression of type Ⅰ collagen, fibronectin and vascular endothelial growth factor, respectively. Statistical analysis was carried out by using one-way analysis of variance, least significant difference- t test, two-way repeated measures analysis of variance and unpaired t-test. Results:Under the transmission electron microscope, the ucMSC-exos were oval in shape with a diameter of about 100 nm; Western blot analysis showed positive expression of ucMSC-exos surface proteins CD63 and TSG101; particle size analysis showed that 96.2 % of the ucMSC-exos had diameters of 30 - 150 nm. CCK8 assay showed that the relative proliferative activity of HSF was significantly higher in the 1- and 2-μg/ml groups (0.97 ± 0.05, 1.08 ± 0.07, respectively) than in the negative control group (0.71 ± 0.04; t = 2.00, 7.05, respectively, both P < 0.05) , and significantly higher in the 2-μg/ml group than in the 1-μg/ml group ( t = 5.09, P < 0.05) . On days 4, 7, 10 and 14 after operation, the percentage of residual wound area was significantly lower in the ucMSC-exos group than in the PBS group (all P < 0.05) . HE and Masson staining showed increased numbers of hair follicles, glands and granulation tissues, more neovascularization, and neater arrangement of collagens in neonatal skin tissues of the mice in the ucMSC-exos group compared with the PBS group. qRT-PCR and Western blot analysis showed significantly increased mRNA and protein expression of type Ⅰ collagen, fibronectin and vascular endothelial growth factor in the ucMSC-exos group compared with the PBS group (all P < 0.01) . Conclusion:Subcutaneous injections of ucMSC-exos can promote acute skin wound healing in mice, likely by promoting the synthesis of extracellular matrix and vascular endothelial growth factor in wound tissues of mice and proliferation of HSF.

Objective ， To investigate the role of serum chemokines and oxidative and antioxidant biomarkers in occupational （ silicosis） Methods silicosis hereinafter referred to as . A total of 58 patients with stage Ⅰ silicosis were selected as the - （ ）， research subjects using convenient sampling method. The serum levels of nuclear factor erythroid 2 related factor 2 Nrf2 -（ - ） - （ - - ） - heme oxygenase 1 HO 1 and 8 isoprstaglandin F2α 8 iso PGF2α were determined by enzyme linked immunesorbent assay. （ ） （ - ） The serum levels of lipid peroxide LPO and total antioxidant capacity TAOC were determined by chemistry colorimetric method. - - （ - ）， Luminex flow fluorescence technology was used to detect the serum levels of interferon γ inducible protein10 IP10 macrophage （ ）- ， - - （ ） inflammatory protein MIP 1α MIP1β and macrophagederived chemokine MDC . The above indicators were analyzed by factor Results - analysis. The information extraction rate of the original indicators of the nine biomarkers was 58.5%96.5%. Four common ， ， （ ） ， factors were extracted including Nrf2 antioxidant signaling pathway helper T cell Th 1 dominant chemotaxis the total ， ， ， ， ， oxidation/antioxidant balance and Th2 dominant chemotaxis whose variance contribution rates were 32.2% 19.1% 16.4% ， ， Conclusion - and 11.8% respectively and the cumulative variance contribution rate was 79.5%. Both the oxidant antioxidant ， disturbance and the dominance chemotaxis are involved in the occurrence and development of silicosis and the Nrf2 antioxidant signaling pathway plays the most critical role.

Background: Esophageal cancer is one of the leading causes of cancer deaths worldwide. T-cell immunoglobulin and mucin domain-containing protein 3 (Tim-3) has been shown to be implicated in the tumor immune escape, and associated with tumor progression and poor prognosis in a variety of malignancies. Aims: To investigate the expression of Tim-3 in esophageal tumorigenesis and its clinical significance. Methods: Paraffin-embedded tissues from 103 esophageal cancer, 21 high-grade esophageal intraepithelial neoplasia, 16 low-grade esophageal intraepithelial neoplasia, and 20 chronic esophagitis were collected in this study. Using immunohistochemistry, the expression level of Tim-3 was evaluated; and the correlation of Tim-3 expression in cancerous tissue with the clinicopathological parameters of esophageal cancer was analyzed. Results: Tim-3 expression was increased from chronic esophagitis, low-grade intraepithelial neoplasia, high-grade intraepithelial neoplasia to esophageal cancer (0.271±0.138, 0.368±0.198, 0.443±0.147, and 0.639±0.119, P<0.05). Moreover, Tim-3 expression was significantly correlated with depth of tumor invasion, regional lymph node metastasis and TNM staging of esophageal cancer (P<0.05), whereas no relationship was found between Tim-3 expression and gender, age and tumor location of esophageal cancer (P>0.05). Conclusions: Tim-3 expression is increased in esophageal tumorigenesis; overexpression of Tim-3 in esophageal cancer is closely correlated with tumor progression. Tim-3 might be served as a biomarker for development, progression and prognosis of esophageal cancer.

Objective: To discuss the application of plane-shaped flaps for reconstruction of the web space in polysyndactyly of the fifth toe fused with the fourth toe. Methods: A total of 62 cases (81 feet) with polysyndactyly of the fifth toe fused with the fourth toe were involved in the study, 53 of which were incomplete and 9 were complete fusion of the fourth and fifth toe. The fifth toe showed various degrees of fibular clinodactyly and minor deformities. Excision of polydactyly and then using arthroplasty or osteotomy correction deviation was for fibular clinodactyly. All plane-shaped flaps were used for reconstruction of the web space deeply, and the lateral sides of toes were closed with flaps and the distal soft tissues of polydactyly were used lengthening the reconstructed fifth toe. Brachydactylias were improved, and nail folds were reconstructed. Thus, the appearances reached the normal level. Results: All the webs were reconstructed primarily without skingraft. After 12 months of follow-up, these reconstructed web space were satisfied with good appearances of width, depth and gradient. The fibular clinodactyly was completely corrected, and brachydactylias were corrected in various degrees. Nail folds reached the normal ones. Conclusions Plane-shaped flap in polysyndactyly of the fifth toe fused with the fourth toe for reconstruction of the web space is satisfactory. The reconstructed webs not only achieves good appearance, but also avoids skin grafts. It is one of the best choices of the polysyndactyly treatment.

Objective: To investigate the treatment of nasal deformity in patients with unicoronal craniosynostosis. Methods: In patients over 6 months old, the nasal bones were wedge-removed without fixation. The management of all patients with unicoronal craniosynostosis was distraction osteogenesis of pedicled unilateral frontal bone flap. Results: Postoperative extended distance of the frontal bone was 28—41 mm (mean, 35.4 mm). After extension, three-dimensional reconstruction of cranial CT was carried out, which showed that CVAI was 0.8—1.2 (mean, 0.98), tending to normal. After discharge, dynamic cranial braces were put on for 1 year. Postoperatively, the children were followed up for 8—36 months (mean, 28 months). The shape and nasal deformity of all children were improved compared with those before surgery. Conclusions Nasal wedge resection should be used to correct nasal deformity in children over 6 months with unicoronal craniosynostosis.

We treated a neonate who was diagnosed as rapidly involuting congenital hemangioma (RICH). With a review on the clinical manifestation, differential diagnosis, and treatment of RICH that associated with thrombocytopenia(TP)and coagulopathy(CP)and received platelet transfusions and embolization therapy including absolute ethanol and polidocanol in our hospital in March of 2015. The platelets and coagulation function soon returned to normal, the tumor involuted significantly. Surgical excision was proceeded subsequently. The platelets returned to normal level after a one-time platelet transfusion, meanwhile, multiple reexaminations of blood coagulation function were normal. Postoperatively, incision wound healed well. There was no recurrence, and the functional recovery of upper limb was satisfactory. RICH is a rare type of vascular tumor which may present with TP and CP similar to KHE-KMP in the neonatal period. More attention should be addressed to identify these two diseases, because the treatment and prognosis of which have significant differences. Consumption of coagulation factors with milder platelet decrease may also be a complication of slow flow venous or venolymphatic malformations.

Objective: To explore the feasibility of mandibular distraction for the treatment of airway obstruction in neonates with Braddock-Carey syndrome. Methods: From August 2007 to November 2017, 6 neonates with Braddock-Carey syndrome were treated in our center. All patients experienced the combined thrombopoietic treatment before operation. Bilateral oblique mandibular osteotomy was performed by extraoral approaches and the distraction was initiated on postoperative day 3 at a rate of 1.2 mm/d. It usually took around 2 weeks to get required length of distraction. Distractor was removed after 3-months fixation at second stage. Results: The distraction distance of 6 cases was from 12 to 20 mm. The CT scan showed a widening gap of the airway by 6-10 mm, and the average width was around 7 mm. 5 patients had no bleeding and infection after the operation, while one patient experienced bleeding for 4 times treated by regional compression. All neonates felt painful during distraction, and got relieved after reduction of the frequency and pace. With mandibular advancement, all patients′ face type improved, while paroxysmal bruising, inspiratory dyspnea and difficulty in breastfeeding disappeared. After 6 months of follow-up, all patients′ weight and face type were basically the same as normal children. Conclusions With the treatment of thrombocytopenia before operation, complete hematischesis during the operation, and postoperative treatment of thrombocytopenia, mandibular distraction osteogenesis was a feasible and safe management of upper airway obstruction in neonates with Braddock-Carey syndrome.

Objective To investigate the common initial clinical presentations of primary Sj(o)gren's syndrome (pSS) with pulmonary complications,and to explore the differences between patients with extraglandular manifestations at disease onset (EGM) and those with glandular manifestations at disease onset (GM).Methods A total of 1 341 hospitalized SS patients from 2003 to 2012 were retrospectively reviewed.Of them,102 hospitalized patients with pSS'associated lung disease were analyzed and included.Case control study was performed to explore the differences between the EGM group and the GM group.Results Fifty-one percent of patients were presented with EGM at onset,with significantly shorter disease duration [36 (12,156) m vs 102 (48,159) m,x2=-2.41,P=0.016].Although the mean diagnose time was similiar,only 4％ of the EGM group could be confirmed the pSS diagnose at onset,which was significantly less frequently than that of the GM group (34％,22=15.29,P＜0.01).Case control study revealed that hyperglobulinemia,elevated RF titers and anti-SSA and/or anti-SSB test positive were less predominant in the EGM group [IgG 16(12,21) g/L vs 21 (15,28) g/L,x2=-2.15,P=0.032;22 (20,171) U/ml vs 104 (20,238) U/ml,x2=-l.98,P=0.048;33％ vs 72％,x2=15.78,P＜0.01].The predicted value of TLC and FVC were lower [(87±23)％ vs (97±20)％,x2=-1.96,P=0.050;(8±28)％ vs (100±27)％,x2=-1.70,P=0.089] and HRCT score was higher in EMG group [12(88,15) vs 8(5,13),x2=-1.82,P=0.070].Conclusion EMG at onset is the common initial manifestation of pSS'associated lung involvement.Pulmonary complication is more progressively and severe than those with MG at onset.Anti'SSA positive,elevated RF titer and hyperglobulinemia are not predominant for patients with EMG at onset.