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.

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

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 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 To establish a high performance liquid chromatography method for simultaneous determination of 10 kinds of phthalates in cosmetics. Methods 10 kinds of phthalates were separated on a C18 column using methanol-water as mobile phase at a flow rate of 1.0 ml/min, 25℃ column temperature and 280 nm detection wave. Results The detection limit of 10 kinds of phthalates were 0.1-7 mg/L. The precision was less than 3.95% and recovery rates varied from 98.59% to 108.07%. Conclusion The experimental results show that the method is simple, precise and accurate and suitable to simultaneous determination of 10 kinds of phthalates in cosmetics.

Objective To explore the operation methods,intraoperative treatment,measures for prevention and treatment of complications of laparoscopic cholecystectomy(LC) for acute cholecystitis.Methods Two hundred and serenty-two patients with acute cholecystitis from March 2003 to April 2007 were analyzed retrospectively.We performed total laparoscopic cholecystectomy in 260 cases and subtotal laparoscopic cholecystectomy with cholecystomucoclasis of remnant gallbladder in 12 cases.Results None of 272 cases was converted to open operation.There was no mortality,and no bile duct injury or major bleeding.Conclusions Laparoscopic cholecystectomy is a safe and effective method for acute cholecystitis and it has the advantages of minimal trauma,quick recovery and less blood loss.

OBJECTIVE:To provide reference for the standardization of the instruction of levonorgestrel emergency contraception. METHODS:Instruction of levonorgestrel emergency contraception in the market was compared with the national regulations and foreign instruction of the same kind of productions in order to discover the problems in domestic specification. RESULTS:The major problems of domestic instruction of levonorgestrel emergency contraception included instructions without population limits, unclear frequency and interval of drug use,without attention to ADR of levonorgestrel emergency contraception,lack of pharmacy research data. CONCLUSIONS:The problems existing in domestic instruction of levonorgestrel emergency contraception are shortage of items and warnings of drug safety. Related administration departments and enterprises are suggested to modify and perfect the instruction of levonorgestrel emergency contraception.

Objective To discuss the features and prevention of central venous catheter infection in patients undergoing digestive tract operation.Methods The clinical data of 145 patients with central venous catheters after G1 surgery in our hospital from March 2004 to March 2009 were retrospectively analyzed.Results Among the 145 cases with central venous catheters,40 cases(27.6%)presented with defite catheter related infection and 56 strains of pathogens were isolated and cultured.Of the 56 isolates,28(50%) were of gram-negative bacteria,22(39%) of gram-postive bacteria and 6(11%) of fungi.Six cases suffered from multiple catheter infection(15%),39patients were cured and 1 died.Conclusions Gram-negative bacteria are the most common pathogens for central venous catheter infection in GI operated patients.It is crucial that standardized card of intravenous infusion should be implemented and prophylactic antibiotics should properly be selected for control of enterogenous infection.

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

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.