Chromosome 22qll deletion syndrome(22q11DS) is a common chromosomal microdeletion syndrome. Its clinical manifestation is complex, comprising congenital heart disease, dysmorphic facial, immunodeficiency, endocrine dysfunction and so on. The syndrome has a population prevalence of approximately 1/2500-1/4000. There have been many recent advances in understanding of the clinical manifestation, behavior and psychiatric problems and the mechanisms leading to the specific phenotypic features in chromosome 22q11 deletion syndrome. Asymmetric recombination of homologous low copy repetitives in the deletion region causes the deletion of 22q11. TBX1 is the dominant gene contributing to the phenotype.

The immune microenvironment of hepatocellular carcinoma (HCC) is mainly composed of tumor-associated macrophages, myeloid-derived suppressor cells and other cellular components, as well as extracellular components, such as cytokines, growth factors and extracellular matrix, etc. In China, most liver cancer patients are complicated with chronic hepatitis B and cirrhosis. Immune microenvironment promotes the incidence and progression of HCC, immune escape and treatment resistance, and exerts immunosuppressive effect. In recent years, significant progress has been made in immunotherapy for systemic treatment of HCC, such as immune checkpoint inhibitors (ICIs). However, in the KEYNOTE-240 and CheckMate 459 trials, anti-PD-1 therapy with nivolumab or pembrolizumab as a single drug failed to reach the expected overall survival endpoint. At present, it is urgent to deepen the understanding of immune microenvironment of HCC and explore novel therapies to improve clinical efficacy of ICIs. Currently, the combination of ICIs with other therapies (such as tyrosine kinase inhibitors, monoclonal antibodies or local therapy) has been proven to improve the efficiency of single ICIs. In this article, research progress in immune microenvironment, immunotherapy and immune combined with targeted therapy for HCC was reviewed.

Animals ; Brain ; drug effects ; pathology ; physiology ; Child ; Child Development ; drug effects ; Female ; Fetus ; drug effects ; Humans ; Hypothalamo-Hypophyseal System ; physiology ; Metals ; toxicity ; Pituitary-Adrenal System ; physiology ; Pregnancy ; Stress, Psychological ; complications

Objective Delivery of oxygen in tissues is limited in the space where oxygen must diffuse between the vascular and the surrounding tissues. Normal capillaries are relatively straight and well spaced, in contrast, the tumor vascular networks usually display more irregularity and the vessel wall shows higher permeability and less elasticity. The purpose of this study was to investigate the effect of capillary elasticity and tortuosity on the oxygen distribution and make further investigation on the mechanism of the formation of hypoxic regions in tumor. Method One-dimensional capillary model was coupled with the oxygen diffusion model. Oxygen transport was investigated in a Krogh and tortuous tissue model. The capillarygeometrywas obtained bythe one-dimensional model and transferred to the tissue model. Finite element method was employed in the analysis. Result The capillary radii along the flow direction under pressures were obtained for different initial radii and the oxygen distribution in the Krogh cylinder tissue model and the model with a tortuous capillary were computed. Conclusions when the capillary radius is small, the effect of vessel elasticity may have not significant effect on the oxygen distribution. However, with the capillary radius increasing, the effect on the oxygen transport becomes obvious. Moreover, with the tortuosity of the capillary increasing, the oxygen distribution becomes more heterogeneous, which is in agreement with the result in available reference. This work will be helpful to the investigation of oxygen transport within tumor.

BACKGROUND:Daclizumab can be special y combined with the inerleukin-2 receptor on the surface of activated T cells in human body, and this method can reflect the close of interleukin-2 receptor thus inferring the effect of induction therapy. At present, the daclizumab has been widely used in renal transplantation, but there is no consensus on its clinical application in liver transplantation. OBJECTIVE:To investigate the expression of serum CD25+T cells and soluble interleukin-2 receptor in the patients receiving daclizumab for liver transplantation during perioperative period. METHODS:A total of 58 patients received orthotopic liver transplant for the first time were included and then the patients were randomly divided into two groups:control group (n=28) and treatment group (n=30). The patients in the two groups were treated with tacrolimus, mycophenolate mofetil and corticosteroids triple immunosuppressive regimen. The patients in the treatment group received immune induction therapy with daclizumab, and the patients in the control group did not receive daclizumab. RESULTS AND CONCLUSION:Flow cytometry and enzyme-linked immunosorbent assay showed the expression levels of CD25+T cells in the treatment group were significantly lower than those in the control group at different time points after liver transplantation (P<0.01);and the expression levels of soluble interleukin-2 receptor in the treatment group were lower than those in the control group during transplantation and at the first day after transplantation (P<0.05, P<0.01). At 6 months after transplantation, the incidence of acute rejection was decreased in the treatment group (P<0.01). The results indicate that daclizumab can effectively suppress the expression level of CD25+T cells, as wel as the expression level of soluble interleukin-2 receptor in the peripheral blood in the early stage of liver transplantation, thus effectively reducing the rate of acute rejection.

Because of insufficient intake,synthesis disorder,metabolic disorder and excessive consumption,patients with cirrhosis often lead to malnutrition. Providing scientific nutritional support and guiding the patients to a reasonable diet can effectively improve the liver metabolism and reduce the complication of cirrhosis such as hepatic encephalopathy,and improve the quality of life and survival rate. This article described the nutritional metabolism disorder in patients with cirrhosis and nutritional treatment in hepatic encephalopathy.

ObjectiveTo explore the clinical value on the detection of urinary podocyte marker protein podocalyxin(PCX) in children with nephritic syndrome(NS) and Schonlein-Henoch purpura nephritis(HSPN).MethodsUrinary samples voided in the morning were obtained from 14 healthy children and 75 children with NS or HSPN or Schonlein-Henoch purpura(HSP),including 21 children with NS in the acute phrase,14 children with NS in the catabasis,16 children with HSPN in the acute phrase,14 children with HSPN in the catabasis,10 children with common HSP,and 14 healthy children for control group.And urinary PCX content of the first morning urine was quantified by enzyme-linked immunosorbent assay(ELISA).SPSS 13.0 software was used to analyze the data.Results1.The levels of PCX content were significantly higher in the urine from children with any case of NS and HSPN compared with those in the control group(P≤0.009),but there was no obvious difference between common HSP children and children in the control group(P=0.754).2.The level of urinary PCX content in acute phrase of NS was(0.593?0.271) ?g/L,in the catabasis of NS was(0.162?0.093) ?g/L,there were significant difference(P=0).The level of urinary PCX content in acute phrase of HSPN was(1.822?1.342) ?g/L,in the catabasis of HSPN it was(0.236?0.141) ?g/L,which was significantly different(P=0.004).The level of urinary PCX content in common HSP was(0.089?0.061) ?g/L,there were significant difference in any case of HSPN(Pa

Objective: To explore the clinical effect of modified preflush on the treatment of CRRT without heparin in patients received dialysis with complication of cerebral hemorrhage. Methods: 44 patients received dialysis with complication of cerebral hemorrhage were divided into control group (28 cases) and observation group (16 cases) according to the implemented time of modified preflush. A retrospective analysis was adopted to compare the effect of filter in blood coagulation grade and service life between conventional preflush (control group)and modified preflush (observation group). Results: The differences of I, II, III grade of blood coagulation between the two methods were significant (x2=12.020, P<0.05), and the differences of average service time of filter, transmembrane pressure(TMP), pressure of filter and value of pressure drawdown (ΔP) between the two methods also were significant (t=3.535, t=4.024, t=6.071, t=3.524, P<0.01). Besides, the difference of venous pressure (PV) between them was significant (t=2.166, P<0.05), while the differences of BFR, UFR and Pa between the two methods were not significant (t=0.263, t=1.162, t=0.258, P>0.05). Conclusion: Modified preflush method can make the fiber of filter to obtain more full stretch and soak, and it also can prolong the service of time, and decrease incidence of blood coagulation, and ensure curative effect and smooth process.

Objective Severe sepsis, especially complicated multiple organ dysfunction syndrome (MODS) ,remains a serious problem in pediatric intensive care unit (PICU). This study aims to explore the clinical characteristics of sepsis and the cause of death for septic patients. Methods A cohort of severe sepsis admitted to PICU between Jan 2008 and Dec 2008 was enrolled in the study. Forty six variables were included in the univariate analysis as potential risk factors for mortality in severe sepsis, followed by logistic regression analysis. Results Forty five children were enrolled with a mortality of 32. 1%. The risk factors of mortality were assessed using the PRISM Ⅲ score ( OR 1. 502;95% CI 1. 131 ～ 1.995) and maximum platelet count during hospitalization ( OR 0. 991 ;95% CI 0.982 ～ 1.000). The mortality of severe sepsis complicated with 1,2,3,4 or more organ dysfunction was 10.0%, 11.1%, 44. 4% and 68. 8 %, respectively ( P ＜ 0. 001 ). The organ dysfunction most frequently appeared in the cardiovascular system (75.6%) and respiratory system (66.7%). In severely septic children with MODS, respiratory failure (OR 23. 179; 95% CI 2.095 ～256. 522) and renal failure( OR 9. 637 ;95% CI 1. 698 ～ 54. 703) were the main risk factors of death. Conclusion The risk factors of death in severe sepsis were PRISM Ⅲ score and maximum platelet count during hospitalization. Severe sepsis combined with MODS had a poor prognosis, with a positive correlation between the number of dysfunctional organs and mortality rate. Respiratory and renal failure rate was associated with death in severe sepsis with MODS.

Objective To assess the effect of lung protective ventilation on outcome of children with acute respiratory distress syndrome(ARDS).Methods Between January 1999 and December 2007,43 children with ARDS were enrolled from PICU of Shanghai Children's Medical Center and assigned to the protective-ventilation group(group A) or the conventional-ventilation group(group B).The patients in group A (from January 2004 to December 2007)received lower tidal volume(6～7 ml/kg) and high levels of positive end-expiratory pressure(PEEP),and optimal oxygenation was achieved by adjusting FiO2 and PEEP.The patients in group B(from January 1999 to December 2003) received relatively higher tidal volume(8～12 mL/kg) with lower PEEP(2～6 cm H2O),and optimal oxygenation was achieved by adjusting FiO2.Tidal volume,PEEP,arterial blood gas,mortality and the number of ventilator-free days were compared between the two groups.Results Since protective ventilation was adopted after 2004,tidal volume was significantly lower in group A[(7.09±1.66)ml/kg]as compared with that in group B[(9.82±2.31) ml/kg](P=0.001).PEEP was significantly higher in group A[(7.15±2.08) cm H2O]as compared with that of group B[(5.40 + 1.84) cm H2O](P=0.021).The mortality was 30.3% in group A and 60.0% in group B.The number of ventilator-free days were(10.88±8.84) d in group A and(8.40±10.86) d in group B.Although mortality was lower and number of ventilator-free days was greater in group A,no significant differences were found between the two groups(P＞0.05).Conclusion Lung protective ventilation may improve the outcome for pediatric patients with ARDS,however,larger trials are required before a definite conclusion can be reached.