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.

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.

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 24-hour ambulatory blood pressure of patients with cervical spinal cord injury. Methods 42 patients with cervical spinal cord injury and 45 patients with thoracic-lumbar spinal cord injury were included. Their 24-hour ambulatory blood pressures were monitored. Their day systolic blood pressure (dSBP) and day diastolic blood pressure (dDBP) when seating and standing, night SBP and DBP, and SBP descending rate at night were compared. Results There were significant differences in dSBP and dDBP when standing, and SBP descending rate at night between 2 groups (P<0.01). There was significant difference in dSBP between seating and standing position in patients with cervical spinal cord injury (P<0.01). Conclusion There is orthostatic hypotension in cervical spinal cord injury patients and their SBP descending rate at night is upside down.

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.

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.

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.

Angioplasty, Balloon, Coronary ; Drug Delivery Systems ; Humans ; Stents

Adenocarcinoma ; metabolism ; pathology ; Aged ; Carcinoid Tumor ; metabolism ; pathology ; Chromogranin A ; metabolism ; Humans ; Immunohistochemistry ; Male ; Neoplasms, Multiple Primary ; metabolism ; pathology ; Phosphopyruvate Hydratase ; metabolism ; Rectal Neoplasms ; metabolism ; pathology ; S100 Proteins ; metabolism