AIM: To investigate the effect of apoptosis and the change of mitochondial tansmembrane potential in A549 cells induced by thermochemotherapy of ADM. METHODS: ADM of different concentrations was directly applied to A549 cells cultivated in vitro. Cells maintained at 42.5 ℃ for 30 min. RESULTS: The inhibition of A549 cells was increased by thermochemotherapy of ADM significantly,and the cytoplasmic concentration of thermochemotherapy of ADM was significantly higher than that of chemotherapy (P

Objective To explore the value of the Accordion severity grading system (ASGS) in predicting short-term outcomes after orthotopic liver transplantation for severe hepatitis by classifying post-surgery complications.Methods The clinical documents of 159 patients were retrospectively analyzed who underwent orthotopic liver transplantation for severe hepatitis between Aug.1,2004 to Sept.1,2014 at our center.Complications were categorized according to the ASGS:grade 1 (mild),grade 2 (moderate),grade 3-5 (severe),and grade 6 (death).Outcome measures included ventilator support time,the length of ICU stay,postoperative recovery time.Spearman rank correlation analysis was used to test the correlation between the different grades with these outcome measures.1-year survival trends of different grade complication groups were demonstrated by Kaplan-Meier method and compared by Log-rank test.Results In total,43 (27.0％) patients had a grade 2 complication;41 (25.8％) grade 3;31 (19.5％) grade 4;9 (5.7％) grade 5;and 35 (22.0％) grade 6.There was no grade 1 patient.There was a significant correlation between the complication grades and the ventilator support time,the length of ICU stay and postoperative inpatient time (P＜0.01).With the increase of the complication grades,the outcome measures were even worse.Severe grade complication group had a longer ventilator support time,the length of ICU stay and postoperative inpatient time than the moderate grade complication group (P＜0.01).There was a significant downward trend in 1-year survival with the increase of the complication grade (P＜0.01).Conclusion The ASGS is helpful to assess risks and predict short-term outcomes after liver transplantation for severe hepatitis.Higher Accordion grades are correlated with even worse short-term outcomes.

Objective The present study is to investigate the distribution of endogenous sulfur dioxide (SO2) in severe acute pancreatitis (SAP) rats.Methods Thirty-two SPF male Sprague-Dawley rats were randomized (random number) into sham operation group,SAP rat 3 h group (SAP 3 h),SAP rat 6 hgroup (SAP6h),SAP rat 12 hgroup (SAP 12 h),n=8 in each group.The SAPmodel rats were induced by retrograde cholangiopancreatic infusion of 5％ sodium taurocholate.Rats were sacrified 3 h,6 h or 12 h after treatment.,then we collected pancrease,liver,lung,kidney and serum.The SO2 concentration in each tissue or serum was detected by enzyme-linked immune sorbentassay.Results The concentration of SO2 in tissues of pancreas (1.72 ± 0.14) μmol/g,liver (1.62 ± 0.11) μmol/g,lung (1.65 ± 0.11) μ.mol/g,kidney (1.12 ± 0.06) μmol/g or serum (16.80 ± 1.27) μmol/g in SAP 3 h rats was not significant compared with the sham operation group (P ＞ 0.05 in each group).The SO2 content in the pancreas (1.89 ± 0.17) μmol/g,liver (1.92 ± 0.16) μmol/g,lung (1.91 ± 0.15) μmol/g,kidney (1.30 ± 0.10) μmol /g and serum (14.93 ± 1.00) μmol /g of SAP 6 h was significantly increased compared with sham operation group (P ＜ 0.05 each group).The content SO2 in the pancreas (2.31 ± 0.23) μmol /g,liver (2.22 ± 0.15) μmol /g,lung (2.17 ± 0.07)μmol /g,kidney (1.55 ± 0.15) μmol /gand serum (18.88 ± 1.56) μmol /g of SAP rats reached the peak 12Hafter treatment and was significantly higher compared with the sham operation group (P ＜ 0.05).Conclusions The increase of SO2 concentration in SAP might be,at least in our present opinion,involved into the pathogenesis of SAP rats.

Age impact in mouse model of secondary hepatic alveolar echinococcus was investigated in this research . Twenty-nine 8-week-old ,twenty-five 18-week-old and twenty-five 28-week-old female mice were anesthetized with 20% ure-thane by intraperitoneal injection and then transhepatically injected by Echinococcus multilocularis (E .m) tissue suspension through skin incision and abdominal muscle to liver in all three groups to establish mouse model of secondary hepatic alveolar e-chinococcus .Results showed that the survival rates for the three groups of mice were 62 .1% ,84% and 68% ,respectively (P>0 .05) .The E .m infection rates in liver were 72 .2% ,71 .4% and 76 .5% ,respectively (P>0 .05) .The diameter of E .m cysts in liver were 0 .915 ± 0 .103 cm ,1 .247 ± 0 .112 cm and 1 .215 ± 0 .197 cm ,respectively (P>0 .05) .The mass of E .m cysts in liver were 0 .332 ± 0 .035 g ,0 .532 ± 0 .155 g and 0 .382 ± 0 .085 g ,respectively (P> 0 .05) .HE stain showed no difference in pathology .Results indicated that the establishment of secondary hepatic alveolar echinococcus model by using transhepatic injection through skin incision and abdominal muscle of 18-week-old mice was capable of simplifying operation and improving the survival rate of the mice .

Objective To evaluate the expression of transient receptor potential melastatin 7(TRPM7)in cholangiocarcinoma and its correlation with prognosis.Methods The expressions of TRPM7 were detected by SP immunohistochemical in 49 cases of cholangiocarcinoma,7 cases of benign bile duct lesions and 36 cases of adjacent histologically noncancerous bile duct tissues,and to analysis its relationship with the clinical pathological characteristics of cholangiocarcinoma.Results The positive expression rate of TRPM7 in cholangiocarcinoma was 77.6%(38/49),which was higher than that in benign bile duct lesions(0,0/7)and adjacent his-tologically noncancerous bile duct tissues(2.8%,1/36),the difference was statistically significant(P <0.05).The positive expres-sion of TRPM7 in cholangiocarcinoma was correlated with the TNM stage of tumor,lymph node metastasis and organ metastasis (P <0.05),but not related to patients′age,gender,site,differentiation and hepatitis(P >0.05).Kaplan-Meier analysis showed that increased expression of TRPM7 was associated with shorted overall survival (P <0.05).Cox regression analysis showed that the expression level of TRPM7 was significantly associated with prognosis and an independent risk factor for prognosis(P <0.05 ). Conclusion TRPM7 plays an important role in the tumorigenesis progression,invasion,and metastasis of cholangiocarcinoma,and is an important factor for prognosis in patients with cholangiocarcinoma.

Objective To study the impact of splenectomy and devascularization on liver function and liver fibrosis in patients with non-cirrhotic portal hypertension (NCPH). Methods The clinical data of patients with NCPH who were treated in Beijing You'an Hospital of Capital Medical University from April 2008 to December 2017 were retrospectively analyzed. The patients were divided into the observation group (n=16 ) and the control group ( n =30 ) according to their treatment methods. The observation group underwent splenectomy combined with devascularization, while the control group received conservative treatment. The changes in liver function, ascites, varicose vein rupture and bleeding before and after treat-ment, and the changes in liver blood flow before and after treatment in the observation group were compared. Results The before and after treatment of the 2 groups in ALT, AST, total bilirubin, albumin showed no statistically significant difference (P＞0. 05). Six months after treatment, the rates of ascites and bleeding were significantly higher than the observation group, (P＜0. 05). Ten patients who had a history of preoper-ative variceal hemorrhage in the observation group did not bleed within 6 months after treatment. Further-more, in the observation group after treatment when compared with the control group, the PC-Ⅲ [(32. 3 ± 12. 1) g/L vs. (56. 7 ± 15. 3)g/L],Ⅵ-C [(46. 6 ± 35. 0) g/L vs. (121. 3 ± 30. 4)g/L], LN [(32. 5 ± 10. 5) g/L vs. (65. 8 ± 11. 1) g/L] were significantly lower than the control group ( P ＜0. 05). The preoperative portal venous blood flow in the observation group was significantly higher than that after surgery [(1 056. 8 ± 679. 8) ml/min vs. (481. 0 ± 227. 6) ml/min, P＜0. 05]. Conclusion Splenectomy and devascularization effectively stopped variceal bleeding and delayed liver fibrosis in NCPH patients without affecting the liver function.

OBJECTIVE: To summarize the clinical phenotype and genotype of a Chinese child affected with Mowat-Wilson syndrome (MWS). METHODS: Clinical data of the patient were collected. The patient was analyzed by whole-exome sequencing (WES) as well as Sanger sequencing. RESULTS: The patient was a male infant with recurrent fever and slow growth. He also had characteristic facies, recurrent spasm, and growth retardation. WES revealed that he has carried a heterozygous nonsense c.2609C>G (p.Ser870X) variant of the ZEB2 gene (30% mosaicism). Based on the American College of Medical Genetics and Genomics standards and guidelines, the variant was predicted to be pathogenic (PVS1+PS1+PS2+PM2). CONCLUSION The c.2609C>G variant of the ZEB2 gene probably underlay the MWS in this child. The mosaicism of the variant may explain his mild symptoms.
Child ; Facies ; Hirschsprung Disease/genetics* ; Humans ; Infant ; Intellectual Disability/genetics* ; Male ; Microcephaly/genetics* ; Mutation

OBJECTIVE: To explore the clinical feature, diagnosis and phenotype of Majeed syndrome. METHODS: Clinical manifestation, diagnostic process, imaging feature and genetic testing of an ethnic Han Chinese patient with Majeed syndrome were reviewed. RESULTS: The patient, a 3-year-9-month-old boy, had featured psychomotor retardation and developed bone pain from 8 month on. The child had tenderness of the lower limbs and presented with repeatedly joint swelling and pain accompanied by fever. Physical signs included limb muscle weakening, slightly decreased muscle tone, reduced muscle volume and positive Gower sign. High-throughput sequencing revealed that the child has carried compound heterozygous variants of the LPIN2 gene, including c.1966A>G and c.2534delG. MRI showed multiple lesions in bilateral knee joints and distal middle tibia presenting as patchy SPAIR high signals with unclear edge, in addition with edema of soft tissue surrounding the right distal femur. CONCLUSION Majeed syndrome is characterized by chronic and recurrent multifocal osteomyelitis, congenital dyserythropoietic anemia, and growth retardation. Surrounding muscle tissue of osteomyelitis may also be involved. The syndrome may also affect the central nervous system, resulting in delayed language and motor development. Discovery of multiple pathological variants of the LPIN2 gene suggested that the clinical phenotype of this syndrome may vary between patients to some extent.
Anemia, Dyserythropoietic, Congenital/genetics* ; Child ; Genetic Testing ; Humans ; Immunologic Deficiency Syndromes/genetics* ; Infant ; Male ; Osteomyelitis/genetics*

Objective:To compare the prognostic accuracy of 16 pre-transplant scoring models in predicting the post-transplant short-term outcome of patients with hepatitis B-related acute-on-chronic liver failure (HBACLF), and to explore an efficient predictive model.Methods:A retrospective analysis of the clinical data of HBACLF patients who underwent liver transplantation at the Liver Transplant Center of Beijing Youan Hospital from August 2004 to September 2014. Score of 16 models (CTP, UNOS-MELD, Updated-MELD, Integrated-MELD, MELD-Na, MLED Na, CLIF-SOFA, CLIF-OFs, CLIF-C ACLFs, CLIF-C ADs, Refit MELD, Refit MELD Na, MELD-AS, Zheng's Risk, UKELD, MESO) was based on time-dependent operation characteristic curve, and the area under the curve (AUC) was calculated to evaluate the prediction accuracy of 3-month survival after transplantation. Selection of univariate factors associated with postoperative short-term mortality was performed, and then 16 scoring models one by one with statistically significant mortality-related factors were entered into LASSO regression (Least Absolute Shrinkage and Selection Operator regression) to confirm the independent variables. Finally, a predictive model was constructed by Cox regression.Results:A total of 135 patients were included in this study, including 106 males and 29 females, aged (45.0±10.5) years old. Among the 16 scoring models, the AUC of MELD-Na and CLIF-SOFA were more than 0.7 in early survival prediction after liver transplant. The MELD-Na was confirmed as an independent predictive variable in the final model with univariate and LASSO regression multivariate selection analysis ( HR=1.0481, 95% CI: 1.0136-1.0838, P<0.05). The model was constructed by MELD-Na and combined with other clinical parameters (female, systemic infection, placement of T tube during operation) could better predict the early survival after liver transplant. The overall C-index of the final model was 0.886, and the C-index at 3-month after liver transplant was 0.844 through internal validation (Bootstrap). Conclusion:Compared with other scoring models, MELD-Na and CLIF-SOFA were better for early survival prediction after liver transplantation for patients with HBACLF. The constructed predictive model based on MELD-Na was superior than single MELD-Na or CLIF-SOFA in prognostic assessment and case selection.

Objective To study the value of preoperative MELD-Na score (Model for End-Stage Liver Disease-Sodium) in predicting complication severity grades after liver transplantation (LT) for severe hepatitis.Methods Patients who underwent LT for severe hepatitis between August 1,2004,and September 1,2014 were retrospectively studied.The Accordion severity grading system was used to classify the complication severity grades after LT.The grades were classified as grade 1 (mild),grade 2 (moderate),grade 3-5 (severe),and grade 6 (death).The area under the curve (AUC) was calculated by plotting the receiver operating characteristic curve (ROC) to evaluate the predictive accuracy of the MELD-Na score for the severe and mortality grades after LT.The correlation between the MELD-Na score with the complication severity grade after LT was studied by the Spearman correlation and by multivariate analysis.Results The incidences of postoperative complications for the 159 patients in this study were:grade 2 in 43 patients (27.0％,MELD-Na score 27.3 ±7.4),grade 3 in 41 patients (25.8％,MELD-Na score 32.7 ± 12.4),grade 4 in 31 patients (19.5％,MELD-Na score 34.3 ± 12.1),grade 5 in 9 patients (5.7％,MELD-Na score 30.7 ± 12.3),grade 6 in 35 patients (22％,MELD-Na score 37.1 ± 10.4).There was no grade 1 patient.The AUC of the MELD-Na score for the severe and death groups were 0.631 (P ＜ 0.05;95 ％ CI,0.533 ～ 0.728) and 0.670 (P ＜ 0.05;95 ％ CI,0.574 ～ 0.766) respectively.The MELD-Na score was significantly correlated with the Accordion severity grade (rho 0.297,P ＜ 0.01) on Spearman correlation analysis.Multivariate analysis showed that a MELD-Na score ≥25 was a risk factor of postoperative severe grade complication (P ＜ 0.05,OR =4.35),a MELD-Na score ≥35 was a risk factor of postoperative mortality (P ＜0.01,HR =4.72).Conclusion The MELD-Na score was significantly correlated with the Accordion severity grade,which efficaciously predicted the complication severity grades after liver transplantation.