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.

OBJECTIVE To study characteristics of hospital infection,risk factors and bacterial resistance in our Intensive Care Unit(ICU) to draw up effective interfering measure and supply the scientific basis.METHODS The data of 1115 inpatients in our ICU in 2008 were analysed prospectively and retrospectively.RESULTS The hospital Infection rate was 12.10%,the case-time infection rate 12.47%,the lower respiratory tract infection rate 56.12%,the G-infection rate 57.30%,the G+ infection rate 25.84%,and the fungus infection rate was 16.85%,These pathogens were resistant to antibiotics in various degree.CONCLUSIONS The ICU infection rate is obvious higher than that in all clinical departments.The resistance to most of the antibiotics are increasing which need enhancing bacterial supervi.

Currently,heart failure is one common disease causing serious damage to children's health and life which has high missed diagnosis rate and mortality.Accurate and effective evaluation of pediatric cardiac function is of great importance in the treatment,surveillance and prognosis of disease.Tei index was first presen-ted by Japanese scholars as a new comprehensive indicator of global (combined systolic and diastolic)myocar-dial function.It was defined as a ratio between the sum of isovolumic times (ICT and IRT)and ejection time (ET).This index can be measured by a simple,reproducible and non-invasive method regardless of changes in the heart rate or ventricular shape and other external factors.The sensitivity for ventricular dysfunction could be high.Tei index has been used more and more often in the clinical evaluation of cardiac function especially in children.This paper reviews the measurement methods,related factors and pediatric clinical application progress of Tei index.

Separations of neurotransmitters such as dopamine (DA), ser otonin (5-HA), norepinephrine (NE) and epinephrine (E) were performed successf ully using a homemade electric field modulated capillary electrophoretic system, which could offer both radial and axial electric fields with only one high volt age power supply. DA and 5-HT were eluted simulaneously and could not be resolv ed in 0.01 mol/L phosphate buffer at pH 2.5. Alcohol additives, such as methanol , ethanol or 1-propanol were added to the buffer to change the solvation shell of the solutes, which changed their effective sizes and electrophoretic mobiliti es of the solutes accordingly. The optimum composition was a buffer of 20% (V ／V） 1-propanol, with resulted resolutions 0.74 (DA/5-HT), 0.56(5-HT/NE) and 0.77 (NE/E). If a positive radial voltage of 6.6 kV was applied, the resolut ions were improved to 1.48, 0.71 and 1.32, respectively.

This paper introduces the perioperative nursing of 20 cases of branchial cysts in newborn with cyst extraction. The nursing care focused on body position,respiratory care,diet management and wound care,which alleviated the patients' dyspnea,prevented respiratory aspiration and promoted wound healing. All the 20 newborn recovered well without recurrence in the four years after the operation.

Objective　To investigate the effect of placental isoferritin in pathogenesis of preeclampsia and its predictive value. Methods　A prospective double-blinded study was performed. There were 120 initial normal pregnant women at earlier third trimester (from 24 to 34 weeks) in this study. Their plasma placental isoferritin and nitric oxide (NO) metabolites (nitrite/nitrate) (NO2-/NO3-) were examined by enzyme linked immunoabsorbent assay (ELISA) and Griess assay respectively. The outcomes of pregnancies were followed up. Results　In 120 initial normal pregnant women, 19 pregnant women occurred preeclampsia (preeclampsia group), and 101 cases were normal (normal group) at follow up. The levels of plasma placental isoferritin (PLF) were significantly decreased in preeclampsia group (285.31±53.73 mg/L) than that of normal group (699.05±203.03) mg/L, P＜0.01. The levels of plasma NO were significantly increased in preeclampsia group (54.57±32.71 μmol/L) than that of normal group (38.89±30.00) μmol/L, P＜0.05. The significant negative correlation between the plasma placental isoferritin and NO levels was observed（r=0.329，P<0.01）. At the cut point of 400 mg/L PLF level, the sensitivity, specificity, positive predictive value, negative predictive value and Kappa index of PLF level predicting on the outcomes of pregnancy with preeclampsia were 100%, 85.15%, 55.88%, 100%, 0.645. Conclusions　 The decrease of plasma placental isoferritin levels is associated with preeclampsia, and the endothelial cell damage may be one of its mechanism. The PLF may be an earlier predictor of preeclampsia at 24～34 gestational weeks.

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.

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.

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.