Objective To discuss the correlation of Wiskott-Aldrich syndrome (WAS) with autoimmune disease of the central nervous system and its possible pathogenesis by reporting one case of the disease and reviewing related literature.Methods One case of WAS complicated with demyelinating disease of the central nervous system was reported.The patient' s clinical symptoms,laboratory examinations (such as blood tests,immune function tests,etc) and imaging features were analyzed.The patient's blood DNA was extracted and performed gene testing.And related literature was reviewed.Results The patient showed typical clinical symptoms of WAS,including eczema,thrombocytopenia and immune deficiency,complicated with demyelinating disease of the central nervous system.The DNA testing showed C400G ＞ C p.(ALa134Pro) mutation,which is a missense mutation.The 134th amino acid in protein was changed from alanine to proline.The patient also showed the symptoms of demyelinating disease of the central nervous system,which drew our attention.This was the first report on WAS complicated with demyelinating disease of the central nervous system,which was perhaps caused by a gene mutation.Conclusions WAS complicated with demyelinating disease of the central nervous system is possibly resulted from the gene mutation,which leads to the expression disorder of WAS protein.And then non-red hematopoietic cells lead to signal transduction and cytoskeleton recombination disorders in response to environment stimulus,which produces lymphocytes immigration,signal transduction and immune synaps formation disorders.

Purpose:To explore the problems in the treatment of the elderly patients suffering from colonic carcinoma.Methods:182 cases of elderly patients aged over 70 who received operation for colonic carcinoma during Jan 1994 to Dec 1998 were analyzed retrospectively. Results:176 cases underwent resections (resection rate 96.7%), of which 129 cases(70.9%)had radical resection and 47 cases(25.8%)palliative resection. 6 cases(3.3%)experienced simple colostomy or bypass. Postoperative complications were found in 67 cases (36.8%),of which 8 cases died during perioperation period(mortality rate 4.4%). Conclusions:Old age is not the major determining factor when considering surgical operation as the treatment for colonic carcinoma patients. In order to enhance resection rate especially radical resection rate and survival rate,to diminish postoperative complications and mortality, and thus to improve the quality of life for the elderly colonic carcinoma patients, a series of measures must be taken. First, diagnosis and treatment of the associated conditions must be emphasized. Second, full preoperative preparations with appropriate choice of the time and procedure for operation, as well as exact operation principles must be followed. Finally, intensive monitoring during the perioperative period and prevention and treatment of postoperative complications were also important factors to be considered.

r governments and mescal institutions in their prevention and control.

Objective To find out the significance of different radiologieal examinations in the di-agnosis of the prostate carcinoma through studying their radiological misdiagnosis rate. Methods By searching for patients having radiological examination results in the database of prostate carcinoma in Tianjin,got the patients whose examination results were different from prostate carcinoma. Analyzed the misdiagnosis rate of each kind of imaging diagnosis using x2-test retrospectively. Results In all patients searched, the misdiagnosis rate of transabdominal ultrasonography, transrectal uhrasonography (TRUS), CT , MRI was 45.1%, 10.1%, 34.5% and 7.5% respectively. Statistical analysis showed that the misdiagnosis rate of MRI was the lowest, then TRUS, and CT. Transabdominal ultrasonography had the highest misdiagnosis rate. There was not significant difference between MRI and TRUS. Conclusions As an efficient and important screening method, transabdominal ultrasonography still need to get a higher definite diagnosis rate. TRUS don't have obvious advantages over pelvis MRI in the diagnosis of prostate carcinoma except its usefulness in the biopsy of prostate. Pelvis MRI is still the most important imaging diagnosis of prostate carcinoma and should be first choice because of its noninvasive intervention, convenience and lowest misdiagnosis rate.

Objective To evaluate the therapeutic effects and complications of modified facial mask for non-invasive ventilation (NIV) in elderly patients with respiratory failure.Methods A total of 132 elderly patients(107 males and 25 female,aged 78.5±8.6 years) treated with NIV from February 2008 to May 2011 were randomized into two groups:modified facial mask(group A,n=68,56 males and 12 females,aged 78.8±22.2 years) and control facial mask(group B,n=64,64 males and 13 females,aged 76.6±20.4 years).Duration of NIV,time in RICU(respiratory intensive care unit),length of hospital stay,risk for hospital acquired pneumonia (HAP),risk for invasive ventilation,cure rates,in-hospital mortality,NIV failure rate and cost were compared between the two groups.The complications of NIV,such as oropharyngeal dryness,skin damage of face and nose,abdominal bloating,gas leakage from mask were also compared between the two groups.Results Compared with group B,duration of NIV(12.2±2.3 d vs.18.4±3.6d),time in RICU(7.3±3.2d vs.14.6t5.4d),length of in hospital stay(16.6±4.2d vs.28.2±6.2)d,and cost(2.23±0.12 ten thousand yuan vs.4.23± 0.24 ten thousand yua) in group A were significantly decreased(t=9.72,14.91,13.08,10.81 respectively,all P＜0.05).The risk for invasive ventilation [2.9％ (2 cases) vs.43.8％(28 cases)],NIV failure rate [5.9％ (4 cases) vs.12.5％ (28 cases)] were also decreased in group A compared with group B(x2 =31.26,25.74,both P＜0.05).Compared with group B,The complications of NIV such as skin damage of face and nose[4.4％ (3 cases) vs.37.5％ (24 cases)],abdominal bloating [2.9％ (2 cases) vs.28.1％ (18 cases)],gas leakage from mask [8.8 ％ (6 cases)vs.50％(32 cases)] in group A were significantly decreased(x2 =31.26,25.74,all P＜0.05).Conclusions Modified facial mask for NIV is effective in the treatment of elderly patients with respiratory failure.The complications and in-hospital mortality are reduced with the application of modified facial mask for NIV and it is highly tolerated by patients.Modified facial mask for NIV is the first choice in the treatment in elderly patients with respiratory failure.

ObjectiveTo induce human peripheral blood mononuclear cells differentiate into hepatocyte-like cells by hepatocyte growth factor (HGF) and fibroblast growth factor-4 (FGF-4) in vitro and determine whether PKH26 could be used to serve as an effective tracer for the cells,and observe the ability of transplanted hepatocyte-like cells differentiate into hepatic cells in nude mice.MethodsGroup A and B were set up respectively.In Group A,mononuclear cells were cultivated without hepatocyte growth factor (HGF) and fibroblast growth factor-4 (FGF-4) in cell culture.They were used as negative control group.In Group B,mononuclear cells were cultured with the administration of both HGF and FGF-4 to induce the differentiation into liver hepatocyte-like cells.The changes in cell morphology were observed and the expressions of AFP and CK 19 were detected by immunocytochemical staining in two groups at different times after induction.The hepatocyte-like cells differentiated from human peripheral blood mononuclear cells labeled by the fluorescent dye PKH26 injected into caudal vein in nude mice is experimental group.The nude mice injected with equal amount of normal saline in control group.The migration of the labeled cells into the liver are observed by the fluorescence microscope in the hepatic tissue sections of nude mice and the expressions of ALB were detected by immunocytochemical staining two weeks after the cells transplantation.ResultsCells in group B have a strong proliferative activity.It becomes large and oval,grows in colonies following induction.Cells in group A that showed spherical shape when peripheral blood mononuclear cells were just isolated are gradually becoming inconformity in morphology,spindle or fibroid,and a few cells are round:cells developed apoptosis and cracked following incubation.The expressions of AFP and CK19 were positive after induction in group B as detected by immunocytochemicat staining.Inversely,the expressions of AFP and CK19 were negative in group A after incubation.The experimental group showed numerous PKH26 labeled cells in the hepatic tissue sections of nude mice.But the control group did not show PKH26 labeled cells.The expressions of ALB were positive in the experimental group as detected by immunocytochemical staining after two weeks of the cells transplantation.ConclusionHuman peripheral blood mononuclear cells have the potential to differentiate into hepatocyte-like cells under the induction of HGF and FGF-4.Additionally,PKH26 is an effective tracer in hepatocyte-like cell transplantation.The hepatocyte-like cells settled in hepatic tissue begin to differentiate into mature hepatocyte after two weeks of the cells transplantation.It plays hepatic cells function and expresses alhnmin.

Objective To understand the development history and current situation of healthcare-associated infection(HAI)management in children’s hospitals in China during the past 30 years.Methods A retrospective survey was conducted in 20 children’s hospitals,the investigated contents included:basic situation of hospitals, HAI management organizations and professionals,the support of hospital leaders for the management of HAI, training on HAI knowledge,monitoring on HAI,and so on.Results Up to 2015,20 hospitals all set up independent HAI management departments and HAI management committees,46.15%(6/13)of hospitals with more than 1 000 beds were understaffed in HAI management.There were a total of 88 professionals in HAI management in 20 children’s hospitals in 2015,53.41% (n=47)of professionals had bachelor degree,34.09% (n=30)were with intermediate professional title,46.59%(n=41)were nursing staff.Up to 2015,incidences and missing report rates of HAI,antimicrobial usage rates,incidences of HAI in neonates all decreased compared with 2006.In 2015,20 (100.00%)hospitals all carried out monitoring on antimicrobial application,multidrug-resistant organisms,disin-fection and sterilization,and provided personnel protective equipment.In 2015,9(45.00%)hospitals used non-tou-ch faucets in all wards,19(95.00%)hospitals supplied hand-drying paper towel and alcohol-based hand rub in all wards.None of professionals in these hospitals participated in national-level training on HAI for ≥3 times in 1996, professionals in 11 hospitals (55.00%)participated in national-level training for ≥3 times in 2015.Conclusion In the past 30 years,the management of HAI in children’s hospitals in China has basically met the requirement,moni-toring on HAI gradually improved,but allocation of HAI professionals and personnel quality ability still need to be strengthened.

Vorapaxar,a novel antagonist of the protease-activated receptor 1 (PAR-1 ),can inhibit the clotting process. Deuterium-labeled vorapaxar was required for the analysis of clinical sample as an internal stand-ard. Starting for unlabeled vorapaxar,four-step reactions including hydrolysis,condensation,transesterification and hydrogen-deuterium exchange were carried out to synthesize [D8]vorapaxar effectively for the first time. All intermediates and final products were confirmed by NMR and high resolution mass spectrometry (HRMS).Impor-tantly,the prepared [D8]vorapaxar could meet the requirements of sample analysis as the internal standard.

Objective To evaluate the effects,safety and economic cost of long-term home noninvasive ventilation (NIV) therapy in elderly patients with chronic hypercapnic respiratory failure.Methods A total of 128 elderly patients with chronic hypercapnic respiratory failure were randomly assigned to two groups:the NIV group (n=66) with conventional therapy in addition to long-term home NIV therapy,and the control group (n=62) with conventional therapy alone.Compared were parameters before and after two year follow up,which included dyspnea grade,scale for accessory muscle use,scoring for emotional disorders,mean pulmonary pressure (mPAP) by electrocardiography,arterial blood gas,the times of pulmonary infection and hospitalization rates,the duration of hospitalization invasive ventilation,the duration of in RICU and in hospital stay,tracheal intubation rates and mortality.The medical cost was calculated.Results After two years,the differences in the dyspnea grade,scale for accessory muscle use,anxiety scores,depression scores,mPAP,arterial PaCO2 and PaO2,hospitalization rates,the times of pulmonary infection,the days of hospitalization for exacerbation in the home NIV group [2.2± 0.2,2.4 ± 0.3,4 ± 1,5.3 ± 1.2,(36.6±5.2)mm Hg,(50.2±4.5)mm Hg,(63.5±4.2)mm Hg,(1.3±0.2) times/year,(2.4±0.2) times/year,(15.8 ± 4.4) days/times] were statistically significant compared to the control group [4.1±0.2,4.9±0.5,12±3,11.3±1.6,(45.2±5.2)mm Hg,(67.3±4.5) mm Hg,(48.3±4.3)mm Hg,(5.4±0.4)times/year,(8.9 ±0.3) times/year,(38.5± 6.3) days/times] (all P＜0.01).The duration of invasive ventilation,the days in RICU and in hospital stay,tracheal intubation rates on admission to the hospital were significantly decreased in the home NIV group [(8.2 ± 2.2)days,(9.6±3.1) days,(15.8±4.4) days,(2±0.2) times/two years],as compared with the control group [(15.8±3.4) days,(18.6±4.4)days,(38.5±6.3)days,(8.0±0.8) times/two years].The mortality was decreased significantly in the home NIV group (3.0 ％)compared with the control group (29.0％) (P＜0.05).The medical cost in two years was significant lower in the home NIV group [(6.4 ± 0.5) thousand yuan],compared with the control group (18.4 ±0.6) thousand yuan (P＜0.01).Conclusions Long-term home NIV therapy in patients with chronic hypercapinc respiratory failure is effective,safe and can decrease the mortality and medical cost.

Objective To describe the characteristics of radiation induced mucositis in patients withhead and neck cancer ( HNC) during radiotherapy, and analyze the effect of radiation induced mucositis on diet patterns and weight change and the influencing factors for radiation induced mucositis. Methods Patients with HNC treated with radiotherapy in one cancer hospital were recruited. Data were collected before, during and at the end of the radiotherapy, which included radiation induced oral and pharyngeal mucositis, pain during eat-ing, diet patterns and weight. Results Two hundred and two patients were completely investigated and 43.5％and 34. 2％ of the patients suffered from moderate to severe (≥grade 2) oral mucositis and pharyngeal mucosi-tis, respectively during the radiotherapy. At the end of radiotherapy, 53. 5％ and 51. 5％ of the patients suffered from moderate to severe oral mucositis and pharyngeal mucositis (≥grade 2 ) , respectively. Oral and pharyngeal mucositis were significantly correlated with pain during eating, diet patterns and weight ( P<0. 05) . Tumor site was the main reason that affected the severity of mucositis ( Wald χ2 =26. 033, 14. 216;P<0.001). Conclusion Radiation induced mucositis was gradually aggravated with radiotherapy progress, which is closely related to pain during eating, change of diet patterns and weight loss. The severity of mucositis is re-lated to the tumor site. Measures should be taken to strengthen the management of adverse reactions and nutri-tional status of patients.