Objective To determine the clinical features and gene expression of neonatal chronic granulomatous disease(CGD) in newborns,in order to provide the evidence for early diagnosis,treatment and infection prophylaxis and so as to improve prognosis.Methods The clinical data of 6 neonates with CGD who hospitalized in Beijing Children's Hospital,Capital University of Medical Sciences,from Jan.to Dec.2013 were retrospectively analyzed,to summarize their clinical features and gene expression.Results All patients were male,mean age of onset in 6 patients was 19 days old.Six patients were onset with cough and fever,3 cases with skin abscesses.All patients had special lung images and their respiratory burst test were all positive.Six patients were all X-linked recessive heredity with mutation in CYBB gene,4 cases carried missense mutation,1 case carried frameshit mutation,1 case had shear point disappear.All patients were improved during hospitalization with anti-infective therapy including antifungal and antibiotic drugs.After their discharge,the patients were followed up for 1 year,among them 5 patients taking antibiotics according to doctor's advise hadn't got any severe infections or other complications,1 patient did not receive antibiotics according to doctor's advise,caught respiratory infection was occured once per 1-2 months and died of multiple organ failure in 5 months due to severe infection.Conclusions For newborn with respiratory symptoms such as cough and fever,with apparently nodular and massive radiologic infiltrates,CGD cases should be highly suspected.Long term prognosis is good in children with prophylactic medication and they may have a longer infection interval.

Umbilical venous catheterization (UVC) is associated with many complications, such as displacement of the catheter, infection, exudation, thrombosis, pericardial effusion/pleural effusion, of which central line-associated bloodstream infections has been of long-standing interest. There is currently no optimal method for estimating the depth of UVC insertion. Ultrasound examination can be used to evaluate the position of the end of the tube after catheterization to avoid organ damage and complications caused by displacement and dislocation. However, whilst it is known that there is a correlation between the duration of UVC and central line-associated bloodstream infection, a consensus is yet to be reached regarding the optimal duration of UVC retention. More, high quality evidence through multi-center, prospective randomized controlled study is needed.

Humans ; Infant, Newborn ; Infant, Premature ; Propranolol ; therapeutic use ; Retinopathy of Prematurity ; drug therapy

Enterovirus 71 (EV71) is a member of the Entero-virus genus of the Picornaviridae family and is the major cause of Hand,foot,and mouth disease (HFMD) in children.Different strains from Gansu were cloned and the P1 protein was sequenced and analysed.Results indicate that there are three kinds of EV71 infections prevalent in Gansu.The VP1 protein from one of these strains,55F,was expressed.The recombinant protein was expressed with high level and reacted specifically with the EV71 patient antibody,the recombinant protein was also applied to raise antiserum in rabbits and after the fourth injection a high titer of antiserum was detected by ELISA assay.These data are useful for further clarification of prevalent EV71 strains in the north of China at the molecular level and provide a basis for EV71 diagnosis.

Endothelial dysfunction was closely related with AS , NO bioavailability ( production and utilization of endothelial NO ) was decreased by oxidative stress , lipid infiltration , inflammatory factor expression , vascular tone alteration and so on , which play an important role in endothelial dysfunction .Enhanced arginine , activityand asym-metric dimethylarginine together with increased hyperhomocysteinemia all promote AS by intervening NO bioavail -ability.Diabetes mellitus, obesity, chronic kidney disease , smoking and so on also involved in AS via influencing NO bioavailability and NO level .

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ObjectiveTo investigate the efficacy and toxicity of postoperative radiochemotherapy compared with chemotherapy alone in the treatment of locally advanced gastric cancer.MethodsA total of 83 patients with resected adenocarcinoma of the stomach were randomly assigned to postoperative radiochemotherapy group (RCT) ( n =43 ) or chemotherapy alone group (CT) ( n =40 ).Patients in RCT group received radiotherapy concurrent with capecitabine chemotherapy then followed by 4 - 6 cycles of FOLFOX4 chemotherapy.The total dose of radiation was 45 Gy.The dose of capecitabine was 1600 mg/m2per day.In the CT group,patients received 6 - 8 cycles FOLFOX4 chemotherapy.Survival was analyzed using Kaplan-Meier method and Logrank test. Results The follow-up rate was 96％. The number of patients who had a minimum of 2-,3-year follow-up time were 37,12 in the RCT group and 31,10 in the CT group.The 1-,2-,3-year local control rates for RCT and CT groups were 100％,97％,94％ and 95％,87％,73％ (x2 =4.54,P =0.033),respectively.The 1-,2-,3-year survival rates were 98％,86％,81％ in the RCT group,with 93％,80％,64％ in the CT group ( x2 =3.96,P =0.047 ).The incidence of grade 3hematological toxicity in the RCT and CT group was 23％ vs 15％ ( x2 =0.93,P =0.630 ),and grade 3gastrointestinal toxicity was 16％ vs 10％ ( x2 =0.95,P =0.624 ). Conclusions Compared with chemotherapy alone,postoperative radiochemotherapy can improve survival of locally advanced gastric cancer patients with acceptable toxicities.

Objective To investigate the effects of microRNA-29a (miR-29a) on lipopolysaccharide (LPS)-induced apoptosis in human monocytes THP-1 cells in order to understand the molecular mechanisms.Methods Human monocytes THP-1 cell line were exposed to LPS after transfected with miR-29a inhibitors (100 nmol/L) or just transfected with miR-29a mimic (100 nmol/L) by lipofectamine RNAiMAX.Flow cytometry (FCM) was used to detect the cell apoptosis.Real-time RT-PCR was employed to measure expressive levels of the gene Bcl-2 and Mcl-1.The luciferase assay was performed in HEK293T cells,which were co-transfected with plasmid DNA and miRNA by using Lipofectamine 2000.Statistical analysis carried out by using SPSS 13.0 software for One-way ANOVA and Student' s t test.Results Transfection with miR-29a mimics for 48 h increased apoptosis rate and significantly reduced the expressions of Bcl-2 and Mcl-1 in THP-1 cells in comparsion with the control group.The apoptosis rate also raised in THP-1 cell stimulated by LPS for 24 h followed by LPS stimulation for 24 h,the apoptosis rate was decreased in comparison with the LPS group.In addition,our luciferase assay data showed that HEK293T cells cotransfected with miR-29a mimics and Bcl-2 3 ' UTR-Wt or Mcl-1 3' UTR-Wt plasmid significantly reduced the luciferase activity compared with the control group.Conclusions The miR-29a may regulate apoptosis by targeting the genes Bcl-2 and Mcl-1,and miR-29a may play a pivotal role in the process of apoptosis in immune cells.

OBJECTIVETo investigate whether propranolol application as collyrium or intraperitoneal (IP) injection can promote the recovery of oxygen-induced retinopathy (OIR).

METHODThirty-six 7-day-old mice were divided into the following 6 groups: normal control, propranolol eye drops, propranolol IP injection, eye drops negative control, IP injection negative control, and pathological model with 6 mice in each. In a typical model of OIR, litters of mice pups with their nursing mothers were exposed to an infant incubator to high oxygen concentration (75 ± 5)% between postnatal day (PD) 7 and PD12, prior to returning to room air. Two routes of propranolol treatment were assessed from PD12 to PD17: IP injection and eye drop, with doses 2 mg/(kg·time), three times a day. Another three groups were given citric acid buffer eye drops, IP injection of citric acid buffer, and negative control were not treated with any drug. Neonatal mice fed in normal conditions served as normal control. Mice were sacrificed at PD17 to evaluate the morphological changes of retinal vessels by fluorescein isothiocyanate-dextran perfusion and retinal whole mount. The retinal neovascularization was evaluated by counting the number of nuclei of the endothelial cell breaking through the internal limiting membrane (ILM).

RESULTCompared with the oxygen-exposed group, the branches of retinal vessels went normal with a less un-perfused area in the propranolol eye drops and propranolol IP injection groups [(38.9 ± 9.9)% and (5.6 ± 2.3)% vs. (16.2 ± 10.0)% and (2.2 ± 0.8)%, (25.9 ± 5.0)% and (2.1 ± 2.7)%, F=36.12 and 14.55, P both<0.001]. The number of nuclei of endothelial cells breaking through the ILM on the retinal cross-section in the propranolol eye drops group decreased (14.2 ± 5.1) per slide, which was less than that in the oxygen-exposed group (49.1 ± 8.9) per slide and the propranolol IP injection group (18.0 ± 5.9) per slide; it was also less than that in the eye drops negative control group (47.4 ± 8.1) per slide (F=187.60, P<0.05). Moreover, the number of nuclei of endothelial cells breaking through the ILM on the retinal cross-section in the propranolol IP injection group was less than that in the IP injection negative control group (49.9 ± 7.1) per slide (P<0.05).

CONCLUSIONPropranolol could effectively inhibit the formation of retinal neovascularization in mice; the eye drops was more effective than the IP injection.

Animals ; Dextrans ; Disease Models, Animal ; Endothelial Cells ; Fluorescein-5-isothiocyanate ; analogs & derivatives ; Injections, Intraperitoneal ; Mice ; Ophthalmic Solutions ; Oxygen ; adverse effects ; Propranolol ; therapeutic use ; Retina ; drug effects ; Retinal Neovascularization ; chemically induced ; drug therapy ; prevention & control ; Retinal Vessels ; drug effects

Objective: To analyze the epidemiological characteristics, clinical manifestations and imaging charac-teristics of 76 cases with neonatal cerebral infarction, in order to provide evidence for the diagnosis, treatment and prognosis for neonal cerebral infarction. Method: Newborns with cerebral infarction admitted into the Neonatal Center, Beijing Children′s Hospital, Capital Medical University from January 2007 to December 2017 were enrolled.According to neurological sequelae, the participants were divided into good prognosis group and poor prognosis group for retrospective analysis, and their cranial magnetic resonance imaging(MRI) changes and mental development were followed up. Results: The incidence of convulsion was the highest in 76 cases (73.7%, 56/76 cases), among which unilateral limb twitching was characteristic in 39.2% (22/56 cases). Clinical physical examination showed no significant positive signs in 13.2%(10/76 cases) of the patients, but middle cerebral artery involvement accounted for 80.3% (61/76 cases), and there was no abnormal discharge of electroencephalogram in 29.2% (19/65 cases) of the patients.Among 41 children (29.3%) received follow-up, 12 cases had neurological sequelae, and 15 cases (36.6%) received anticoa-gulant therapy.The median area of original damage of T2 sequence cerebral infarction was 10.3%, the median area of diffusion weighted image damage was 2.0%, and the median area of damage of T2 sequence was 1.0%.The median ratio of craniocerebral lesion damage was 8.4%.The ratios of poor prognosis were 14.9%, 4.8%, 0.5% and 14.4%, respectively. Conclusions Neonatal convulsion is the most common manifestation of neonatal cerebral infarction.Unilateral limb clonus is characteristic.The larger the infarct involvement area and the larger the intracranial lesion change area through reexamination, the poorer prognosis of the children.Early anticoagulant therapy could not improve the prognosis of neonatal cerebral infarction.