Objective:To investigate the diagnosis and treatment of congenital leukemia and the main factors affecting its prognosis.Methods:Clinical data of children with congenital leukemia admitted to Zhengzhou Children′s Hospital from July 2007 to December 2017 were retrospectively collected.Their clinical characteristics, chemotherapy and factors affecting their long-term survival were analyzed.Results:A total of 23 cases of congenital leukemia were collected, who mostly complained of " increased white blood cells, skin nodules and skin hemorrhage" . Among them, 10 cases abandoned the treatment, who all died according to the follow-up results.Among the 13 treated cases, 7 cases were male, and 6 were female.One of the 13 cases was a twin.Eight cases were acute myeloid leukemia, and 5 cases were acute lymphoblastic leukemia.One case did not perform the fusion gene test. MLL gene was positive in 2 cases and negative in 10 cases.No other fusion genes were detected.Karyotype analysis showed that 1 case was 21-trisomy and 12 cases had a normal karyotype.All 13 patients received symptomatic and/or reduced chemotherapy.The median follow-up time was 4 years and 11 months.Six cases recovered and 7 cases died during the follow-up.One case was diagnosed with acute lymphoblastic leukemia on the 6 th day after the birth in another hospital.He was not treated timely and came to Zhengzhou Children′s Hospital with general contract blocks at the age of 32 days.After checking, he was diagnosed with center leukemia and testicular leukemia.He developed a severe infection in the lung and brain after strong chemotherapy, and died even after the remote consultation with Lurie Children′s Hospital of Chicago.The other 6 cases died of severe infection and multiple organ injuries after chemotherapy, and no one died of leukemia relapse. Conclusion:Reduction chemotherapy may be an important treatment to children with congenital leukemia.

Objective:To investigate the manifestations of liver injury in hospitalized patients with coronavirus disease 2019 (COVID-19), to investigate the prognosis indicators of the disease, and to provide the reference for clinical diagnosis and treatment.Methods:From January 10 to February 14, 2020, at Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, the data of 333 hospitalized patients with COVID-19 were collected. The changes of alanine aminotransferase (ALT), aspartate aminotransferase (AST), total bilirubin (TBil), direct bilirubin (DBil), indirect bilirubin (IBil) and albumin of the first liver function test after admission and the reexaminations of liver function test during hospitalization period in patients with liver injury were retrospectively analyzed. Student t test and Chi-square test were used for statistical analysis. Results:Liver injury occurred in 39.6% (132/333) of COVID-19 patients. There was no statistically significant difference in the rate of liver injury between patients in intensive care unit (ICU) and in general ward (45.6%, 26/57 vs. 38.4%, 106/276; χ2=1.026, P>0.05). 67.4% (89/132) of COVID-19 patients with liver injury presented with increased ALT or AST level on admission. During hospitalization, the level of ALT was higher than that of the first examination after admission ((60.28±50.44) U/L vs. (42.25±32.21) U/L), and the difference was statistically significant ( t=-3.230, P<0.05). The levels of ALT and AST of 71.2% (94/132) patients were both <80 U/L, which indicated that most of the patients showed mild liver injury. The patients with elevated level of TBil, DBil and IBil accounted for 3.9% (13/333), 5.4% (18/333) and 2.4% (8/333) of the COVID-19 patients, respectively. The albumin level of COVID-19 patients with liver injury during hospitalization was lower than that of the first examination after admission ((31.8±5.1) g/L vs. (33.7±5.4) g/L), and the difference was statistically significant ( t=2.712, P<0.05). The albumin levels at first examination on admission and reexamination during hospitalization of patients in ICU were both significantly lower than those of patients in general ward ((29.3±3.7) g/L vs. (34.8±5.1) g/L and (27.6±2.8) g/L vs. (32.9±5.1) g/L), and the differences were statistically significant ( t=4.928 and 4.783, both P<0.05). Conclusions:The incidence of liver injury in COVID-19 patients is high. A slight increase in aminotransferase levels is particularly common. Bilirubin abnormality is relatively rare and mild. The level of albumin may be one of the indicators for the severity and prognosis of COVID-19.

Objective To investigate the expressions of programmed death-ligand 1 (PD-L1) and PD-L2 and phosphorylated protein kinase B (p-AKT) in diffuse large B-cell lymphoma (DLBCL) patients and their correlations with clinicopathological features and prognosis. Methods A total of 68 paraffin-embedded specimens of DLBCL patients diagnosed in Shanxi Provincial Cancer Hospital with detailed follow-up record from January 2010 to December 2012 were included in the study. The expressions of PD-L1, PD-L2 and p-AKT proteins in DLBCL were detected by using immunohistochemistry (IHC). Results The positive rate of PD-L1 protein in DLBCL patients was 22.1％ (15/68), which was related to germinal center B-cell (GCB) subtype or not (χ2= 5.591, P= 0.018), clinical stage (χ2= 3.969, P= 0.046), international prognostic index (IPI) grades (χ2=4.178, P=0.041) and treatment remission rate (χ2=6.587, P=0.010). The positive rate of PD-L2 protein in DLBCL patients was 14.7％ (10/68), which was related to extranodal metastasis or not (χ2=6.772, P= 0.009). The positive rate of p-AKT for DLBCL patients was 61.8％ (42/68), which was correlated with age (≥60 years old) or not (χ2=6.227, P=0.013), Eastern Cooperative Oncology Group (ECOG) grades (χ2=4.005, P=0.045), B symptoms (χ2=10.187, P=0.001) and treatment remission rate (χ2=4.096, P=0.043). Univariate survival analysis showed that the overall survival (OS) rate and progression free survival (PFS) rate of PD-L1 protein positive expression group were lower than those of PD-L1 protein negative expression group (both P< 0.05). In the patients with non-GCB subtype, OS rate and PFS rate of PD-L1 protein positive expression group were lower than those of PD-L1 protein negative expression group (both P<0.05). p-AKT protein positive expression group had poorer OS rate and PFS rate compared to p-AKT negative expression group (both P< 0.05). Correlation analysis showed that PD-L1 protein expression was correlated with PD-L2 and p-AKT proteins expressions (r= 0.380, P= 0.001;r= 0.273, P= 0.025). The prognosis was worse when p-AKT and PD-L1 proteins was co-expressed (P< 0.05). Multivariate analysis suggested high expressions of PD-L1 and p-AKT proteins were independent prognosis risk factors in DLBCL (both P<0.05). Conclusions The expressions of PD-L1 and p-AKT proteins may be involved in the occurrence and development of DLBCL. Blocking PD-1 and PD-L1 access or combined blocking could provide a promising future for the clinical therapy.

To analyze the outcomes of early Fontan failure after Fontan takedown. Methods A retrospective study of early Fontan Failure(EFF) children undergoing Fontan takedown from November 2013 to December 2017 was performed. Fontan takedown was defined as takedown back to an intermediate circulation, superior cavopulmonary connection. Fontan takedown was performed in 14 patients. There were 9 boys and 5 girls. Children were on average aged(4. 3 ± 1. 4) years when they had Fontan procedure. The mean weight was(14. 3 ±2. 7)kg. Operative procedure was extra-cardiac Fontan in 5 children, 9 had intra-cardiac Fontan. Fenestration was used in 11/14 patients. The outcomes were summarized with statistics, and risk factors for mortality after Fontan takedown were identified. Results The mortality after Fontan takedown was 4/ 14(28. 6％). In two patients(14. 3％), ECMO was followed after takedown, one of them died after two days. The time of ICU stay and hospital stay was relatively long(17. 0 ±11. 2)days and(33. 8 ±19. 4)days. The interval time between the Fontan procedure and the takedown operation is the risk fact after Fontan takedown. Conclusion Fontan takedown can be used as an effective management for the early Fontan failure, still with a high risk of mortality. Early diagnosis and takedown is recommended for EFF.

Objective: To investigate the relationship of PD-L1 protein expression and gene amplification in gastric cancer and their correlation with clinicopathologic factors. Methods: The cohort included 247 gastric cancer specimens with follow-up data and clinicopathologic data obtained from Shanxi Cancer Hospital in 2011. PD-L1 expression was detected by immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH). Results: PD-L1 protein was expressed in 25.9% (64/247) of the tumor cells and 26.7% (66/247) of the tumor infiltrating immune cells (IC). There was a correlation between the two (P<0.01). The expression of PD-L1 in tumor cells correlated with the degree of differentiation and tumor diameter(P<0.05). The PD-L1 expression in IC correlated with vascular tumor thrombi(P<0.05). The amplification rate of PD-L1 gene detected by FISH was 19.0% (47/247), and was associated with age, large/small curvature of the stomach, tumor location, tumor diameter, and lymph node metastasis(P<0.05). The positive coincidence rate of the two methods was 25.0% (16/64), negative coincidence rate was 83.0% (152/183), and total coincidence rate was 68.0% (168/247), suggesting that the coincidence of IHC and FISH was poor (P=0.157). There was a negative correlation between PD-L1 protein expression on tumor cells and prognosis in gastric cancer. There was no significant correlation between PD-L1 protein expression on IC and PD-L1 gene amplification with prognosis. Vascular tumor thrombi, tumor diameter, depth of invasion, and lymph node metastasis were all poor prognostic factors of gastric cancer(P<0.05). Multivariate Cox regression analysis showed that PD-L1 protein expression, depth of invasion and lymph node metastasis were all independent prognostic risk factors for gastric cancer. Conclusions Concordance between PD-L1 protein expression and gene amplification is poor. PD-L1 protein expression may signify poor prognosis. There is no significant correlation between PD-L1 gene amplification and prognosis of patients with gastric cancer.

Objective To observe the clinical characteristics of bacterial pneumonia children,and the changes of the serum levels of white blood cells(WBC),C-reactive protein(CRP) and procalcitonin(PCT) before and after anti-bacterial therapy,and to explore the predictive value in early diagnosis and therapy.Methods 130 bacterial pneumonia children were enrolled prospectively as pneumonia group.The clinical data were collected and the serum CRP,PCT and WBC were detected before anti-bacterial therapy (within 24h after admission) and after anti-bacterial therapy (the seventh day after admission).34 healthy children were enrolled as control group.The general clinical characteristics of the children in the pneumonia group were observed.The levels of serum CRP,PCT and WBC between the pneumonia group and the control group were compared.The levels of serum CRP,PCT and WBC before and after anti-bacterial therapy in the bacterial pneumonia children were compared.The clinical value of PCT,CRP and WBC in early predicting bacterial pneumonia was identified.Results Compared with the control group,the sex and age of the bacterial pneumonia children demonstrated no statistically significant differences (t =1.012,P =0.395;x2 =0.003,P =0.959).The mean course of the disease before admission was (5.34 ± 1.27) d,with mean temperature of (38.27 ± 0.96) ℃,and hospital days of (8.92 ± 3.93) d.35 cases were cured,and 95 cases were improved,with no death.The serum levels of CRP,PCT and WBC in pneumonia children on admission were (12.24 ±6.35) mg/L,(0.18 ± 0.15) ng/mL and (14.25 ± 7.59) 109/L,respectively,which were higher than those of the control group,the differences were statistically significant (t =4.650,5.867,2.548,all P ＜ 0.05).The serum levels of CRP,PCT and WBC in pneumonia children before anti-bacterial therapy were higher than after bacterial therapy,showed statistically significant differences(t =8.165,7.232,5.112,all P ＜ 0.05).The area under the curve (AUC)of the PCT,CRP and WBC in early predicting bacterial pneumonia were 0.928,0.834 and 0.718 respectively by the relative operating characteristic (ROC) curve analysis (P ＜ 0.05).The sensitivity and specificity of PCT in predicting bacterial pneumonia were higher than CRP and WBC.Conclusion The statistics efficacy of PCT in early predicting children bacterial infection was obviously higher than CRP and PCT.The combined detection of CRP,PCT and WBC was benefit to improve the diagnostic sensitivity and specificity of children with bacterial pneumonia.

Objective To investigate the correlation between AS160 hyperphosphorylation and 18Ffluorodeoxyglucose (18F-FDG) uptaking in invasive breast cancer.Methods Thirty-nine primary breast caner patients received PET/CT scan before operation and their maximum standard uptake values (SUVmax) were recorded.The levels of p-AS160 (Thr642) were detected using immunohistochemical method in breast cancer tissues.The association between p-AS160 (Thr642) levels and SUVmax was assessed using the Spearman nonparametric test.Results The 18F-FDG uptake of tumors of the 39 patients with primary breast caner was 6.1 ± 2.7 (SUVmax).There was one false negative case in PET/CT scan imaging,compared with postoperative pathologic result.The positive rate of p-AS160 (Thr642) was 76.9％,and it was positively correlated with SUVmax (r =0.672,P ＜ 0.001).Conclusion AS160 plays a significant role in the glucose metablism of breast cancer tissues and its hyperphosphorylation may contribute to the increased uptaking of glucose in patients with breast cancer.

Objective To explore the protective effects of 10-HDA combined with AA-2G on UVA-induced photodamage in fibroblasts. Methods The primary cultured human skin fibroblasts were divided into three groups: blank control group, UVA irradiation group and 10-HDA+AA-2G group. The cell viability and cellular senescent state were analyzed using CCK-8 and senescence associated-β-galactosidase (SA-β-gal) staining, respectively. Fluorometric assays were performed to detect the formation of reactive oxygen species (ROS) in the cells. Results Compared with those in control group, the cell viability was decreased (P < 0.05), and the SA-β-gal positive cells and ROS level were significantly increased (P<0.01 for both) in UVA-irradiated group. Compared with those in UVA-irradiated group, the cell viability was significantly increased (P < 0.01), and the SA-β-gal positive cells and ROS level were significantly reduced (P < 0.05, P < 0.01, respectively) in the 10-HDA combined with AA-2G group. Conclusion Fibroblasts treated with 10-HDA and AA-2G are significantly protected from UVA-induced cytotoxicity, cellular senescence and ROS, indicating that 10-HDA combined with AA-2G has photoprotective effects. Therefore, 10-HDA combined with AA-2G may be a potential combination for the prevention and treatment of skin photoaging.

OBJECTIVETo review the experience of left ventricular assistance device (LVAD) using for anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in perioperative period.

METHODSThere were 29 patients with ALCAPA underwent surgical repair from May 2006 to May 2013. The mean age was 6.5 months (ranging from 3.3 to 12.1 months). The mean weight was 6.2 kg (ranging from 4.1 to 9.5 kg). Diagnosis was established by echocardiography in all patients. There were clinical symptomatic of the severe heart dysfunction and ejection fraction were 23% to 45%. Mitral insufficiency was moderate to severe in 8 patients and less than moderate in others. Surgical methods included the intrapulmonary tunnel (Takeuchi procedure) of 4 cases, direct reimplantation of the left coronary artery onto the aorta of 6 cases and reimplantation by pericardiac patch enlarge of 19 cases. Valvuloplasty were performed in 5 patients with mitral severe insufficiency. Twenty-two patients were treated only by medicine therapy. LVAD was used in 7 patients: there were 3 patients with low blood pressure at the end of surgical repair and 4 patients with low cardio output within 24 hours postoperatively.

RESULTSPostoperatively, transesophageal echocardiography demonstrated that blood flow of the left coronary artery is fluently but left ventricular is also largement. The hemodynamic of 18 patients was stable in medicine group but 3 patients were sudden died of low cardiao output and ventricular fibrillation respectively. One patient was died of diffuse intravascular coagulation at the time of 72 hours after operation. The hemodynamic was stable in 6 patients in LVAD group and the devices after using time from 72 to 108 hours was taken down except one patient died of multi-organ dysfunction. The hospital mortality was 5/29 (17.2%). Nineteen survival (19/24) was followed up of 3.5 years (ranging from 1 to 7 years). Reoperations was performed for one patient with the supravalvar pulmonary stenosis due to the Takeuchi procedure 4 years postoperatively. Echocardiographic demonstrated that the blood flow of the left coronary artery are fluently. Mitral insufficiency was moderate in 2 cases, mild to moderate in 9 cases and mild in 8 cases. The ejection fraction value were 43% to 55% and apparent arrhythmia didn't occur.

CONCLUSIONSAlthough late results are satisfactory and left ventricular function always recovery, early mortality is higher even though the protective methods are carried out during the whole cardiopulmonary bypass procedure. In order to decrease the early mortality, heart function evaluation and LVAD should be used as an effective cardiac support technique to prevent heart failure in time.

Aged ; Aorta ; Bland White Garland Syndrome ; Cardiopulmonary Bypass ; Coronary Vessel Anomalies ; surgery ; Heart ; Heart Failure ; Hospital Mortality ; Humans ; Mitral Valve Insufficiency ; Perioperative Period ; Postoperative Care ; Prostheses and Implants ; Pulmonary Artery ; abnormalities ; Reoperation ; Treatment Outcome ; Ventricular Function, Left

OBJECTIVETo study expression of CD68, cyclin D1 protein and rearrangement of bcl-6 gene impact on the prognosis of diffuse large B-cell lymphoma (DLBCL).

METHODSGets paraffin samples of the 105 cases DLBCL with the detailed follow-up information, and were studied by using immunohistochemical EnVision method for CD3, CD10, CD20, CD68, cyclin D1, bcl-6, MUM 1, SOX-11 immunolabeling. The DLBCL were classified into germinal center B cell-like (GCB) subtypes and non-germinal center B cell-like (non-GCB) subtypes according to Hans'algorithm. Application of fluorescence in situ hybridization (FISH) technique to detect the bcl-6 gene rearrangement. The relationship between CD68, cyclin D1 protein, the bcl-6 gene and the curative effect of chemotherapy and survival was analyzed using statistical software. Respectively by GCB type, non-GCB type immune phenotype and CHOP, R-CHOP chemotherapy group, compare the curative effects.

RESULTS105 patients had GCB 19 cases (18.1%), non-GCB 86 cases (81.9%), CD68 expression was 18 cases (17.1%), cyclin D1 high expression 36 cases (34.3%), bcl-6 gene rearrangement in 21 cases (21.9%), there is no correlation among the three (P > 0.05). One-way analysis of variance showed that age ≤ 60 years, clinical stage I-II, IPI score 0 to 2 points, LDH (U/L) < 245 IU/L,GCB subtypes, R-CHOP therapy, the prognosis of patients with better (P < 0.05), But gender, primary site no correlation with prognosis (P > 0.05). CD68, cyclin D1 high expression, bcl-6 rearrangement had poor prognosis (P < 0.05). Stratification analysis results show GCB-type or non-GCB type with high expression of CD68 contrast alloimmune phenotype groups had a poor prognosis, non-GCB type with high expression of cyclin D1 and rearrangement of bcl-6 gene had a poor prognosis (P < 0.001, P = 0.02). Treatment scheme of layered display, the CHOP treatment, significantly correlated with overall survival with high expression of CD68, cyclin D1 (P < 0.05), the R-CHOP treatment, there was no statistically significant difference between CD68, cyclin D1 high expression and overall survival (P = 0.428 and 0.168). Multivariate COX model analysis showed that high expression of CD68 (P = 0.026), high expression of cyclin D1 (P = 0.003) and high levels of LDH (P = 0.005) were adverse prognostic factors independent.

CONCLUSIONShigh expression of CD68, cyclin D1 and rearrangement of bcl-6 gene suggests poor prognosis, CD68, cyclin D1 protein and bcl-6 gene can be used as a prognostic indicator in patients with DLBCL.

Antibodies, Monoclonal, Murine-Derived ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Antineoplastic Combined Chemotherapy Protocols ; B-Lymphocytes ; classification ; Cyclin D1 ; metabolism ; Cyclophosphamide ; DNA-Binding Proteins ; genetics ; Doxorubicin ; Gene Rearrangement ; Germinal Center ; cytology ; Humans ; In Situ Hybridization, Fluorescence ; Lymphoma, Large B-Cell, Diffuse ; diagnosis ; Prednisone ; Prognosis ; Proto-Oncogene Proteins c-bcl-6 ; Vincristine