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Background Chronic granulomatous disease (CGD) is a rare disease in China, and very little large‐scale studies have been conducted to date. We aimed to investigate the clinical and genetic features of CGD in Chinese pediatric patients. Methods Pediatric patients with CGD from Beijing Children's Hospital, Capital Medical University, China, were enrolled from January 2006 to December 2016. Results A total of 159 pediatric patients with CGD were enrolled. The median age of clinical onset was 1.4 months, and 73% (116/159) had clinical onset symptoms before the 1 year of age. The most common site of invasion was the lungs. The lymph nodes, liver, and skin were more frequently invaded in X‐linked (XL) CGD patients than in autosomal recessive (AR) CGD patients (P

A trifluoroacetic acid (TFA)-mediated cascade oxidation/1,3-dipolar cycloaddition reaction of stabilized pyridinium salts with dimethyl sulfoxide (DMSO) has been developed in the presence of K2S2O8 and trimethylethylenediamine (TMEDA). In this transition-metal-free reaction, DMSO acts as a one-carbon source, thus providing a convenient method for the efficient and direct synthesis of various indolizine derivatives.

Superimposition of dual frequencies (DFs) is one of the methods used for controlling plasma distribution in an inductively coupled plasma (ICP) source. The effects of a superimposed DF on the argon plasma characteristics have been investigated using a two-dimensional self-consistent fluid model. When both currents are fixed at 6 A, the plasma density drops with decrease in one of the source frequencies due to less efficient heating and the plasma uniformity improves significantly. Moreover, for ICP operated with superimposed DFs (i.e., 4.52 MHz/13.56 MHz and 2.26 MHz/13.56 MHz), the current source exhibits the same period as the low frequency (LF) component, and the plasma density is higher than that obtained at a single frequency (i.e., 4.52 and 2.26 MHz) with the same total current of 12 A. However, at superimposed current frequencies of 6.78 MHz/13.56 MHz, the plasma density is lower than that obtained at a single frequency of 6.78 MHz due to the weaker negative azimuthal electric field between two positive maxima during one period of 6.78 MHz. When the superimposed DF ICP operates at 2.26 and 13.56 MHz, the rapid oscillations of the induced electric field become weaker during one period of 2.26 MHz as the current ratio of 2.26 MHz/13.56 MHz rises from 24 A/7 A to 30 A/1 A, and the plasma density drops with the current ratio due to weakened electron heating. The uniformity of plasma increases due to sufficient diffusion under the low-density condition.

Objective: To summarize and analyze the manifestations of stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI).Methods: A systematic literature review was performed including cases from January 1, 2014, to February 1, 2020, using PubMed, OVID, CNKI, and WanFang. This included all the literature containing comparatively complete clinical data. Statistical analysis was performed using SPSS 20.0 to analyze the difference in age of onset, severity of skin lesions, and respiratory symptoms between SAVI patients with p.N154S and p.V155M mutations.Results: A total of 25 papers were included reporting on 51 individuals, of whom 17 had familiar inheritance of their mutation. Patients included 27 males and 24 females, and 8 fatal cases were observed. A total of 10 mutation sites have been reported in the STING gene, with p.V155M being the most prevalent. We identified SAVI as an early-onset disease with a median age of onset of 3 months after birth. Skin lesions were the most common symptoms of SAVI, found in 94.1% (48/51) of patients, while 76% (19/25) who had undergone a skin biopsy showed vasculopathy. Involvement of the lungs was identified in 68.6% (35/51) of patients, while only 22.2% (4/18) who had undergone a lung biopsy showed vasculopathy. Of 20 patients, 19 had increased immunoglobulin, mainly IgG. Furthermore, 45.1% (23/51) of patients had a positive low titer or were transiently positive for antinuclear antibodies. Of the 18 patients treated with JAK inhibitors, 6 relapsed and 2 died of acute respiratory failure caused by viral infection. Patients with p.N154S mutation had an earlier disease onset (p = 0.002) and more severe skin lesions (p < 0.001) than those patients with p.V155M mutation.Conclusion: SAVI is an early-onset disease accompanied by skin and lung lesions whose clinical presentation varies among patients with different genotypes. Therapeutic effects of JAK inhibitors are unsatisfactory.

Superficial angiomyxoma (SAM) is an extremely rare soft tissue tumor. It is especially rare in the vulva, with only a few such cases reported in the medical literature. Here, we report a case of SAM of the vulva that was initially suspected to be a Bartholin gland cyst. The patient underwent local excision of the vulvar cyst under lumbar anesthesia. Clinical manifestations and B-scan ultrasonographic features are similar between SAM and cysts. Echoes in the mass are uneven and exhibit low echoes and punctate hyperechoic floating. Thus, increasing sonographers' awareness of the high-frequency ultrasonography findings associated with this rare tumor could broaden their knowledge base.

Paediatric Mycoplasma pneumoniae pneumonia (MPP) is a major cause of community-acquired pneumonia in China. Data on epidemiology of paediatric MPP from China are little known. This study retrospectively collected data from June 2006 to June 2016 in Beijing Children's Hospital, Capital Medical University of North China and aims to explore the epidemiological features of paediatric MPP and severe MPP (SMPP) in North China during the past 10 years. A total of 27 498 paediatric patients with pneumonia were enrolled. Among them, 37.5% of paediatric patients had MPP. In this area, an epidemic took place every 2–3 years at the peak, and the positive rate of MPP increased during these peak years over time. The peak age of MPP was between the ages of 6 and 10 years, accounting for 75.2%, significantly more compared with other age groups (χ2 = 1384.1, P < 0.0001). The epidemics peaked in September, October and November (χ2 = 904.9, P < 0.0001). Additionally, 13.0% of MPP paediatric patients were SMPP, but over time, the rate of SMPP increased, reaching 42.6% in 2016. The mean age of paediatric patients with SMPP (6.7 ± 3.0 years old) was younger than that of patients with non-SMPP (7.4 ± 3.2 years old) (t = 3.60, P = 0.0001). The prevalence of MPP and SMPP is common in China, especially in children from 6 to 10 years old. Paediatric patients with SMPP tend to be younger than those with non-SMPP. MPP outbreaks occur every 2–3 years in North China. September, October and November are the peak months, unlike in South China. Understanding the epidemiological characteristics of paediatric MPP can contribute to timely treatment and diagnosis, and may improve the prognosis of children with SMPP.

A novel DMSO-involved cascade reaction of stabilized sulfonium salts has been established for direct construction of polyfunctional furans. This one-pot sequential reaction involving in situ generated α-methylene sulfonium salts was followed by [4 + 1] annulation with sulfur ylides. Notably, DMSO plays a very important role in this transformation, not only as a solvent but also as one carbon source.

Severe combined immunodeficiency (SCID) is the most serious disorder among primary immunodeficiency diseases threatening children's life. Atypical SCID variant, presenting with mild reduced T cells subsets, is often associated with infection susceptibility but poor clinical diagnosis. The atypical X-SCID patient in the present study showed a mild clinical presentation with a TlowNK+B+ immunophenotype. The patient has reduced T- cell subpopulations with a subdued thymic output measured by sjTRECs. Further analysis showed that T cells maintained a normal proliferation and a broad Vβ repertoire. NK cells, however, exhibited a skewed development toward immature CD3-CD16+CD56- cells. Genetic analysis revealed a novel deletion at nucleotide 52 in exon 1 of IL2RG gene. Sequence alignment predicted a truncated IL2RG protein missing signal peptide derived from a possible alternative reading frame. The novel mutation in IL2RG gene identified in our study may help the early diagnosis of atypical X-SCID.

A fluid model is employed to investigate the effect of radio frequency bias on the behavior of an argon inductively coupled plasma (ICP). In particular, the effects of ICP source power, single-frequency bias power, and dual-frequency bias power on the characteristics of ICP are simulated at a fixed pressure of 30 mTorr (1 Torr = 1.33322 × 102 Pa). When the bias frequency is fixed at 27.12 MHz, the two-dimensional (2D) plasma density profile is significantly affected by the bias power at low ICP source power (e.g., 50 W), whereas it is weakly affected by the bias power at higher ICP source power (e.g., 100 W). When dual-frequency (27.12 MHz/2.26 MHz) bias is applied and the sum of bias powers is fixed at 500 W, a pronounced increase in the maximum argon ion density is observed with the increase of the bias power ratio in the absence of ICP source power. As the ratio of 27.12-MHz/2.26-MHz bias power decreases from 500 W/0 W to 0 W/500 W with the ICP source power fixed at 50 W, the plasma density profiles smoothly shifts from edge-high to center-high, and the effect of bias power on the plasma distribution becomes weaker with the bias power ratio decreasing. Besides, the axial ion flux at the substrate surface is characterized by a maximum at the edge of the substrate. When the ICP source power is higher, the 2D plasma density profiles, as well as the spatiotemporal and radial distributions of ion flux at the substrate surface are characterized by a peak in the reactor center, and the distributions of plasma parameters are negligibly affected by the dual-frequency bias power ratio.

An interesting σ-bond insertion/benzannulation reaction for the synthesis of polysubstituted naphthalene derivatives has been developed from readily accessible ketones, arynes, and alkynoates. This practical and transition-metal-free method provides a novel route to diverse naphthalenes through a substrate-controlled rearrangement reaction with the cleavage of C–C bonds.

As the wafer size increases, pulsed dual frequency inductively coupled plasma sources have been proposed as an effective method to achieve large-area uniform plasmas. A two-dimensional (2D) self-consistent fluid model, combined with an electromagnetic module, has been employed to investigate the influence of the pulse duty cycle and the pulse phase shift on the plasma radial uniformity in an argon discharge. When both antennas are pulsed, the best radial uniformity is obtained at 30%, due to the balance between the positive feedback and diffusion loss. When the duty cycle decreases, the bulk plasma density becomes lower since the power absorption is limited during the shorter active-glow period. As the duty cycle decreases to 10%, the plasma density is characterized by an edge-high profile because of the strong diffusion. When the pulse duty cycle of the outer two-turn coil is fixed at 70%, the plasma density profiles shift from center-high over uniform to edge-high as the pulse duty cycle of the inner coil decreases from 50% to 10%, and the best plasma uniformity appears at 30%. In addition, by adjusting pulse phase shifting of two antennas, the plasma uniformity could also be improved, and the nonuniformity degree decreases from 0.138 for the synchronous pulse to about 0.101 for the asynchronous pulse.

New enterprises for products in prepackages with fixed content always use sampling inspection to make sure the accuracy of the net quantity of their products according to JJF 1070-2005 “Rules of Metrological Testing for Net Quantity of Products in Prepackages with Fixed Content”, but this sampling inspection plan is expensive. The theoretical basis of the variable sampling plan in JJF 1070 was analyzed first. Then based on ISO 3951-1, the variable acceptance standard, a new sampling plan for products in prepackages with fixed content was developed, and it’s operation characteristic curve was drawn. Taking one company in Jinhua city of Zhejiang province of China as an example, a comparative analysis for the practical application of these two sampling plans was carried out. Under the premise that the sampling plan’s discrimination power was unchanged, the results showed that the acceptance effects of two sampling plans waere almost the same, while the reduction of sample size for the new one was 44%, the inspection time and cost were all reduced.

A novel and simple technique to control the search direction of the differential mutation was proposed. In order to verify the performance of this method, ten widely used benchmark functions were chosen and the results were compared with the original differential evolution (DE) algorithm. Experimental results indicate that the search direction controlled DE algorithm obtains better results than the original DE algorithm in term of the solution quality and convergence rate.

Differences in secondary structure among Bombyx mori (B. mori) silk and two wild silks of Antheraea yamamai (A. yamamai) and Antheraea pernyi (A. pernyi) were investigated by CP/MAS 13C NMR Spectroscopy. The β-sheet structure was primary in three silk, and B. mori silk had the highest β-sheet structure. Although amino acid compositions are very similar for two wild silk, their secondary structures had significant difference. A. yamamai silk contained more α-helix structure, whereas more β-turn and random coil structures formed in A. pernyi silk. B. mori silk was mainly composed of anti-parallel β-sheet structure, however, the parallel β-sheet structure was advantage in the two wild silks, and A. yamamai silk contained more anti-parallel β-sheet conformation than A. pernyi silk.

Silk fibroin/cellulose acetate blend Nanofibrous membranes were prepared by electro- spinning and their performances were evaluated as a heavy metal ion adsorbent. The electrospun nanofibrous membranes were comprised of randomly oriented ultra-fine fibers of 100-600nm diameters. As a result of field emission electron microscope (FEEM), compared with pure nanofibrous membranes, the anti-felting shrinkage of SF/CA blend nanofibrous membranes with 20% CA content was markedly improved after treatment with 100% ethanol. Metal ion adsorption test was performed with Cu2+ as a model heavy metal ion in a stock solution. The pure SF nanofibrous membranes exhibited high metal ion capacities compared with that pure CA nanofibrous membrane. Especially, the SF/CA blend nanofibrous membranes had an exceptional performance for the adsorption of metal ions, and the maximum milligrams per gram of metal ions adsorbed reached 22.8mg/g for Cu2+. This indicated that SF and CA had synergetic effect.

Four kinds of solutions were prepared by dissolving cellulose acetate in dichloromethane, formic acid, acetic acid and trifluoroacetic acid respectively, and then were electrospun into fibers. The solutions were characterized for their viscosity and conductivity using rotary viscosimeter and conductivity meter, respectively. The morphology and performance of the electrospun CA fibers were investigated by scanning electron microscopy (SEM), digital microscope and X-ray diffraction (XRD). Results showed that the important factors determining the electro-spinnability of the CA solutions were boiling point, viscosity and conductivity of the resulting solutions. 8% CA spinning solution with trifluoroacetic acid as solvent had good spinnability, and the average diameter reached 236nm; the obtained fibers were mainly amorphous, but characterized for good mechanical properties.

UHMWPE filaments have a low surface free energy and therefore often require a modification of their surface properties before any use. Atmospheric pressure plasmas treatment is a convenient and environmentally friendly way to obtain these modifications by introducing new chemical groups at the surface without affecting the bulk properties. This paper studies the influence of nano-SiO2 sol-gel coating pretreatment on atmospheric pressure plasma jet (APPJ) treatment of ultrahigh molecular weight polyethylene (UHMWPE) fibers when a mixture of 100% helium and 1% oxygen used as the treatment gas. The surface properties of the plasma-treated UHMWPE filaments are characterized using contact angle measurements and ATR-FTIR spectroscopy. The UHMWPE filaments show a remarkable increase in surface free energy after plasma treatment. ATR-FTIR spectroscopy of the plasma-treated UHMWPE filaments reveals that plasma treatment introduces oxygen-containing functionalities on the UHMWPE filaments surface leading to the increased surface free energy.

Ultrahigh molecular weight polyethylene (UHMWPE) Filaments has a low surface free energy and therefore modification of their surface properties before any use is often needed. Atmospheric pressure plasmas treatment is a convenient and environment friendly way to obtain these modifications by introducing new chemical groups at the surface without affecting the bulk properties. This paper studies the influence of nano-SiO2 Sol-gel coated pretreatment on atmospheric pressure jet (APPJ) treatment of UHMWPE fibers with helium used as the treatment gas. The surface properties of the plasma-treated UHMWPE Filaments are characterized using contact angle measurements and ATR-FTIR spectroscopy. The UHMWPE Filaments show a remarkable increase in surface free energy after plasma treatment. ATR-FTIR spectroscopy of the plasma-treated UHMWPE Filaments reveals that plasma treatment introduces oxygen-containing functionalities, such as ketones, aldehydes, alcohols and carboxylic acids on the UHMWPE Filaments surface leading to the increase of surface free energy.

BACKGROUND A relationship between coronary artery spasm (CAS) and myocardial bridge (MB) has been noticed. This study was designed to investigate the differences of stress tests and symptoms between CAS patients with or without MB. METHODS Two hundred and sixteen patients with atypical chest pain who underwent coronary angiography and acetylcholine provocation test were divided into MB group (n=68) and nonmyocardial bridge group (NMB, n=148). The results of acetylcholine test, treadmill exercise electrocardiography, myocardial scintigraphy, and levels of plasma endothelin-1 and nitric oxide were compared between the two groups. RESULTS Among these atypical chest pain patients, CAS was induced by acetylcholine in 85% MB patients and 53% NMB patients (P

Matrine, a main active component extracted from dry roots of Sophora flavecens , has been reported to exert antitumor effects on A549 human non-small lung cancer cells, but its mechanisms of action remain unclear. To determine effects of matrine on proliferation of A549 cells and assess possible mechanisms, MTT assays were employed to detect cytotoxicity, along with o flow cytometric analysis of DNA content of nuclei of cells following staining with propidium iodide to analyze cell cycle distribution. Western blotting was performed to determined expression levels of Bax, Bcl-2, VEGF and HDAC1, while a microarray was used to assessed changes of miRNA profiles. In the MTT assay, matrine suppressed growth of human lung cancer cell A549 in a dose- and time- dependent manner at doses of 0.25-2.5 mg/ml for 24h, 48h or 72h. Matrine induced cell cycle arrest in G0/G1 phase and decreased the G2/M phase, while down-regulating the expression of Bcl2 protein, leading to a reduction in the Bcl-2/Bax ratio. In addition, matrine down regulated the expression level of VEGF and HDAC1 of A549 cells. Microarray analysis demonstrated that matrine altered the expression level of miRNAs compared with untreated control A549 cells. In conclusion, matrine could inhibit proliferation of A549 cells, providing useful information for understanding anticancer mechanisms.

In this study, we assessed the toxic effects of formaldehyde (FA) on mouse bone marrow mesenchymal stem cells (BM-MSCs). Cytotoxicity was measured by using MTT assay. DNA strand breakage was detected by standard alkaline comet assay and comet assay modified with proteinase K (PK). DNA-protein crosslinks (DPCs) were detected by KCl-SDS precipitation assay. We found that FA at a concentration from 75 to 200 μM inhibited cell survival and induced DPCs over 125 μM. The PK-modified comet assay showed that FA-induced DNA strand breakage was increased in a dose-dependent manner from 75 to 200 μM. On the other hand, standard alkaline comet assay showed that DNA strand breakage was decreased with FA concentration over 125 μM. We confirmed by using Pearson correlation that there was a negative linear correlation between DPCs and survival rate (r = -0.987, P < 0.01) and positive linear relationships between DPCs and (i) sister chromatid exchange and (ii) micronucleus (r = 0.995, P < 0.01; r = 0.968, P < 0.01). DNA damage RT(2) profiler polymerase chain reaction array was used to investigate the changes in the expression of damage response genes. Xpa and Xpc of the nucleotide excision repair pathway and Brca2, Rad51, and Xrcc2 of the homologous recombination pathway were all up-regulated in both 75 and 125 μM FA. However, the same genes were down-regulated with 175 μM FA. The expressions of Chek1 and Hus1, which are involved in cell cycle regulation, were altered in the same manner with 75, 125, and 175 μM FA. These results indicated that Xpa, Xpc, Brca2, Rad51, Xrcc2, Chek1, and Hus1 were essential for the BM-MSCs to counteract the effects of FA.

Cystic fibrosis (CF) is rare in Chinese mainland. We present two cases of CF patients diagnosed by gene analysis. Their clinical manifestations and genetic mutation features are analyzed in this article. It will be of special interest to pediatricians in recognition of CF.The clinical material of two CF patients who were diagnosed by gene analysis was retrospectively analyzed.The first patient is a 13-year-old girl. She had a complaint of recurrent fever and cough for 6 months, expectoration for 2 months and hemoptysis for 20 days. After 3 months of her birth, she was operated on for bullae of lung. She was susceptible to upper respiratory tract infection. There was no family history of recurrent wheeze and other special diseases. Aspergillus fumigatus specific IgE was at grade 3 and aspergillus fumigatus IgG was high. Pseudomonas aeruginosa was positive in sputum culture. Sweat testing was performed and Na+ was higher. Pulmonary CT indicated bronchiectasis. Nasal sinus CT showed optical density of soft tissue within maxillary sinus and chronic bilateral sinusitis. The electron microscopy of cilia suggested immobile cilia syndrome. A heterozygotic mutation (263TG, 2909GA) was found after CFTR genetic mutation analysis. Both her parents were carriers. She was treated with inhalation of nebulized hypertonic saline and postural drainage for a long time. And she got better during a follow up period of 1 year. The second patient was a 10-year-old girl who complained of recurrent expectoration for 3 years and shortness of breath for half a year. She had a history of sinusitis and steatorrhea. The family history was normal. Both the lipase and insulin level in blood serum was lower.Pseudomonas aerugino and Aspergillus fumigatus were both positive in sputum culture. Aspergillus fumigatus IgE was normal. Pulmonary CT indicated bronchiolitis and bronchiectasis. Nasal sinus CT showed bilateral maxillary sinusitis. CFTR genetic mutation analysis revealed a homozygous mutation (3196CT). Her parents and relatives did not participate in this study. Unfortunately, this child died of respiratory failure 3 months after discharge.CFTR gene mutation was a main cause of CF. Common symptoms are those of bronchiectasis, pancreatitis and sinusitis. The two Chinese patients were diagnosed by gene analysis. One had a heterozygous mutation (263TG, 2909GA) and the other had a homozygous mutation (3196CT), not ΔF508 which is common in western countries.

To summarize clinical and molecular features of two children with autosomal recessive chronic granulomatous disease caused by CYBA mutations.The clinical records and CYBA mutations were reviewed for analysis of infections and inflammatory complications.The first case was a girl diagnosed with "liver and spleen abscess" in our hospital when she was 2.9 years old, with past history of neonatal impetigo and recurrent purulent lymphadenitis and positive family history. The results of DHR123 flow-cytometry showed that positive phagocytes after phorbol ester (PMA) stimulation was 84.63%. CYBA mutation analysis showed that she had heterozygous 35CT, Q3X and IVS-2AG. The second case was a boy diagnosed with "sepsis (salmonella D)" when he was 4 years old with a past history of impetigo, sepsis, perianal abscess, skin infection and positive family history. The results of flow cytometry showed that positive phagocytes after PMA stimulation was 96.13%. CYBA mutation analysis showed that he had homozygous 35CT, Q3X and his parents were all carriers. All of them had BCG related axillary lymphnode calcification.A22CGD cases had recurrent purulent infections (skin, lymphnode, liver and spleen, lung, blood), DHR123 flow cytometric analysis helped the diagnosis of CGD, CYBA mutation analysis ascertained the diagnosis of A22CGD.

In this paper, Calculates, simulates and analyzes the ground clutter power spectrum of airborne weather radar; describes the simulation process and gives a calculation formula. Analyzes the power spectrum of the main lobe ground clutter; Based on the analysis, designs the complex coefficient filter to filter out the main lobe clutter, and gives the simulation results.

To screen enhancer-like sequences from Escherichia coli strain C600 genome, to construct an expression vector harboring prokaryotic enhancer-like sequence and study the effect of interferon gene expression.Enhancer-like element from Escherichia coli strain C600 genome was obtained by using the chloramphenicol acetyl-transferase (CAT) gene as reporter gene. An expression vector harboring prokaryotic enhancer-like sequence from Escherichia coli strain C600 was constructed. Interferon was expressed and assayed.An enhancer-like sequences with distance and orientation independence property were screened and named 3A. Quantification test showed that the direct and reverse orientation of 3A could increase the activity of beta-galactosidase with 7.11 and 2.93 times. The enhancing activity of the element was on transcription level. An expression vector harboring the prokaryotic enhancer-like sequence 3P3 which was enhancing function region of sequence 3A was constructed. Using this vector the antiviral activity of interferon alpha-2b was increased by 3.7 times in comparison with the original expression plasmid.3A enhancer-like sequence was screened from Escherichia coli strain C600 genome. Interferon gene was highly expressed by using an expression vector harboring enhancer-like sequences.

White noise pollution mainly reduce the precision of spectral moments estimation for weather echoes, this paper presents a novel spectral estimation method based on fitting to decrease the impact of white noise on spectral parameters estimation. Fitting polynomial is firstly formed by analyzing the spectral characteristics of weather echoes, and then calibrates the received fitted spectrum by triangle. The simulation results show that the method can successfully reduce the impact of white noise on the spectral moments estimation, and can get higher performance than FFT method with a measurement mean error less than 0.4%. So the proposed method can be applied in weather radar

To explore the clinical manifestation, immune abnormality and outcome of disseminated Bacille Calmette-Guérin (BCG) infection in children.The clinical data of 18 children with disseminated BCG infection seen from January 2000 to December 2007 were analyzed retrospectively.Thirteen of the children were male among 18 patients. Disseminated infection first appeared in armpit lymph nodes ipsilateral to the vaccination site, then spread to lungs in 15, lymphnodes of mediastinum or abdominal cavity in 18, skin and soft tissues in 5, skeletons in 4, liver in 4, spleen in 8, kidney, adrenal gland or meninges in 3. Twelve children were diagnosed to have primary immunodeficiency; 3 had severe combined immunodeficiency (SCID); 7 had chronic granulomatous disease (CGD), 2 had IL-12/IFN-gamma passageway deficiency. Eleven of the 18 patients died, and the remaining 7 patients were followed up from 1 to 9 years and are alive at present, but presented recurrent skin and bone tuberculosis in 4 and recurrent other infection in 3.Most Children with disseminated BCG infection had primary immunodeficiency. CGD and IL-12/IFN-gamma passageway deficiency accounted for considerable proportion, so special immune function should be detected in these patients. The prognosis was poor. The type of the immunodeficiency diseases should be identified in early stage and the specific immune treatment should be given to the patients.

To explore an effective method of emergency remedial cardiac pacing and evaluate its clinical application.The transthoracic cardiac pacing was used by a steel wire loop electrode to 18 patients with cardiac arrest or serious bradycardia, after a routine cardiac pacing was failed by way of jugular or subclavian vein.It is a simple procedure used by steel wire loop. All the pacing had been successfully captured the hearts except 5 cases without capturing. There were 3 cases of cardiac arrest, 2 cases of cardiac arrest after electric defibrillation, 1 case of cardiac arrest after electric shock because of ventricular running and all the 5 cases of severe cardiac bradycardia due to serious atrioventricular block caused by high blood potassium were survived. Eight cases of cardiac arrest were successful capture, that was due to the short period of time from cardiac arrest to ventricle puncture than that of 5 cases of ventricular pacing which was not successful [(17.6+/-4.6) minutes vs. (26.4+/-5.4) minutes, P0.05].The steel wire loop electrode is a safe and reliable emergency cardiac pacing method, which contribute to the rapid establishment of an effective emergency cardiac pacing. When the conventional intravenous cardiac pacing was not successful, the method of emergency transthoracic cardiac pacing used by steel wire loop electrode should be bold and use to improve the successful rate of resuscitation.

in this paper, low rang time side lobes for pulse compression in weather radar can be achieved by an integrated scheme using Non-linear FM, tapering transmission waveform, mismatched windowed filter. The simulation process to search optimum realization scheme is presented to make sure least enlargement of main lobe pulse-width while mitigating energy of side lobes. The result of simulation indicates that side lobes can be less than 60dB while main lobe width will not surpass 0.66 μs. In the end, the implement of the scheme is designed for realization. Keywords-Side lobes Suppression; Mismatched filter; weather radar

To study the expression of inducible co-stimulator ligand(ICOSL) on human coronary artery endothelial cells(HCAEC) and its being interferentialed by oxidized low density lipoprotein(ox-LDL).ICOSL expression levels were determined by the fluorescence, reverse transcription PCR (RT-PCR) and Western blot, respectively.The ICOSL mRNA OD values of control and 100 mg/L ox-LDL group was 0.071+/-0.035 and 0.186+/-0.044, respectively. The Western blot A values of control and 100 mg/L ox-LDL group was 10.88+/-1.53 and 16.03+/-4.08, respectively. ox-LDL increased expression of ICOSL mRNA and protein(P0.05).ICOSL can express on the HCAEC. ox-LDL can up-regulate the expression of ICOSL.

To study the effect of oxidized low density lipoprotein (ox-LDL) on the expression of B7-related protein-1 (B7RP-1) on human coronary artery endothelial cells (HCAECs).HCAECs were incubated in the presence of 100 mg/L ox-LDL for 24 h, and B7RP-1 expression levels were determined using fluorescence reverse transcription PCR (RT-PCR) and Western blotting.B7RP-1 expression was detected HCAECs, with spots of fluorescence signals distributing on the cell membrane as observed under fluorescence microscope. RT-PCR with B7RP-1 specific primers yielded products of expected size (496 bp). Western blotting identified B7RP-1 expression in the HCAECs as a cell-associated protein with an apparent molecular mass of 70,000. Treatment of the cells with ox-LDL significantly increased B7RP-1 expression at both the mRNA and protein levels (P0.05).B7RP-1 is expressed on the membrane of HCAECs. ox-LDL can promote up-regulate the expression of B7RP-1, which might be one of the immunopathogenesis of atherosclerosis.

To study the effects of oxygen and calcium on the expression of eukaryotic vectors harboring wild-type or mutated hypoxia-inducible factor-1alpha (HIF-11alpha) in HEK293 cells.HEK293 cells were transiently transfected with pcDNA3.1+/HIF-11alpha, pcDNA3.1+/HIF-11alpha-564Ala and pcDNA3.1+/HIF-11alpha-564Ala-803Ala via lipofectin. Western blotting were used to detect HIF-11alpha protein after normoxic or hypoxic exposure of the transfected HEK293 cells in the presence or absence of Ca(2+). The levels of vascular endothelial growth factor (VEGF) mRNA in the transfected cells in normoxic condition was detected using RT-PCR.The levels of HIF-11alpha protein and VEGF mRNA increased in HEK293 cells transfected with the vectors harboring mutated HIF-11alpha, but not in the cells transfected with wild-type HIF-11alpha vectors in normoxia. Hypoxia increased the levels of HIF-11alpha protein in the cells transfected with wild-type HIF-11alpha vectors, which was inhibited by the application of Ca(2+). Ca(2+) showed no inhibitory effect on HIF-11alpha levels in HEK293 cells transfected with the vectors containing mutated HIF-11alpha.The protein products of pcDNA3.1+/HIF-11alpha-564Ala and pcDNA3.1+/HIF-11alpha- 564Ala-803Ala in HEK293 cells enhance the cell tolerance to oxygen and protease.

OBJECTIVE To study the efficacy and possible mechanism of Yiqi Huayu Recipe (YQHYR), a compound traditional Chinese herbal medicine, in preventing and treating degeneration of the articular cartilage in rats with osteoarthritis (OA). METHODS A total of 90 one-month-old SD rats were randomly divided into normal control group, untreated group and YQHYR group, with 30 rats in each group. The osteoarthritis was induced by shoulder disarticulation plus upright posture in rats. The rats in YQHYR group were treated with YQHYR at 5-, 7- and 9-month old (4, 6 and 8 months after the surgery) for one month. Ten rats in each subgroup were sacrificed at 6-, 8- and 10-month old (5, 7 and 9 months after the surgery) respectively and the knee joint samples were harvested for detection. Safranin-O/fast green staining was performed to examine the morphology of articular cartilage. The expressions of type II collagen (Col2A1), aggrecan-1 (Agc1), matrix metalloproteinase-13 (MMP-13) and tissue inhibitor of metalloproteinase-1 (TIMP-1) mRNAs were evaluated by real-time fluorescent quantitation polymerase chain reaction. RESULTS Histological analysis showed that the structure of articular cartilage was seriously destroyed in rats in the untreated group. On the contrary, the degenerative changes of the articular cartilage in the YQHYR group were dropped off. Real-time fluorescent quantitation polymerase chain reaction showed that expressions of Agc1, TIMP-1 and Col2A1 mRNAs were up-regulated in 6- and 10-month-old rats in the YQHYR group as compared with those in the untreated group (P

To investigate the clinical features of coronary artery spasm patients with or without myocardial bridge and explore the roles of endothelial dysfunction in these patients.One hundred eighteen patients undergone acetylcholine provoking test were divided into myocardial bridge (MB) group (n = 26) and non-myocardial bridge (NMB) group (n = 92). The results of acetylcholine test, treadmill exercise electrocardiography, stress myocardial perfusion scintigraphy, plasma level of endothelin-1 and nitric oxide were compared between MB group and NMB group.Coronary artery spasm was induced in 21 patients in MB group (81%) and 52 patients in NMB group (57%, P0.05). Positive treadmill electrocardiography was obtained in 19 patients in MB group (73%) and 7 patients in NMB group (8%, P0.001). Ischemic perfusion defect in 20 (77%) and 9 patients (10%, P0.001) and reverse redistribution in 23 (88%) and 68 patients (74%, P0.05). Patients showed different clinical features in MB group and NMB group (more short-duration exertional angina and could not be readily released by nitroglycerine in MB group while more patients experienced long-lasting variant angina and symptoms could be readily released by nitroglycerine). Plasma endothelin-1 level was significantly higher [(132.1 +/- 6.5) ng/L vs. (108.5 +/- 8.2) ng/L, P0.01] while nitric oxide was significant lower [(84.7 +/- 17.5) ng/L vs. (99.8 +/- 18.2) ng/L, P0.05] in MB group compared to NMB group.MB patients were prone to coronary artery spasm partly due to endothelial dysfunction. Patients with MB and coronary artery spasm also showed classic clinical symptoms and positive stress tests for ischemia.

To observe effect of porcine relaxin(pRLX) on NO production of human microvascular endothelial cells(HMVECs) and discuss its possible mechanism.iNOS and cNOS expression of HMVECs with or without pRLX were detected using western blotting. NO production of HMVECs with pRLX at different concentration or different time were determined by method of Griess. NO production of pRLX of HMVECs plus Non-selective NOS inhibitor NG-monomethyl-L-arginine(L-NMMA), selective iNOS inhibitor aminoguanidine(AG) or nuclear factors-kappaB (NF-kappaB) inhibitor pyrrolidine dithiocarbamate(PDTC) were also analysed.pRLX promoted iNOS protein expression of HMVECs, but not cNOS protein expression. NO production of HMVECs was promoted by pRLX on concentration-dependent pattern instead of time-dependent one. AG, L-NMMA and PDTC were showed to block the effect of pRLX on NO production of HMVECs.pRLX promote iNOS expression and NO production of HMVECs.

To investigate clinical features of X-linked agammaglobulinemia (XLA) in children.The medical records of 17 children with XLA between January 2001 and April 2007 were reviewed.The age at first diagnosis in 88.2% of patients was more than 6 years, with a mean of 7.7 years. Twelve patients (70.6%) presented first symptoms over 2 years old, with a mean of 4.2 years. Respiratory infections as first symptoms and complaints occurred in 64.7% of the patients and 35.3% of the patients presented with polyarthritis. Skin and soft tissue infections were rarely seen in less than 1 year old group children. Abrupt sepsis and abscess in deep tissues were seen in the older children. CD4+ T cells decreased and CD8+ T cells increased in 9 patients and an inversed ratio of CD4+/ CD8+ was observed in 11 patients.Both the age presenting first symptoms and the age at first diagnosis in children with XLA in this study were later than the reported data. Respiratory infection was the most common manifestation. High prevalence of polyarthritis was observed. Abnormal T cell phenotypes occurred in more than one half of patients.

Background: Coronary artery spasm usually attacks while at rest and the diagnosis depends on provoking tests which mostly are invasive, expensive and not practical. Previous investigations demonstrated that in most patients with coronary artery spasm, treadmill exercise electrocardiography (TEEC) did not induce any ischemic changes, and myocardial perfusion scintigraphy (MPS) showed reverse redistribution (RR). Hypothesis: Combination of resting chest pain, negative TEEC and RR might be a rational noninvasive set to predict coronary artery spasm. Methods: Patients with chest pain at rest, no significant coronary artery stenosis, and willing to undergo acetylcholine test were included. TEEC, dipyridamole and rest thallium-201 MPS were performed before or after coronary angiography. The patients were divided into spasm and nonspasm groups by acetylcholine test. The clinical features and results of TEEC and MPS were compared between the spasm and nonspasm groups. Results: Acetylcholine test was performed in 92 patients and coronary artery spasm was successfully provoked in 68 patients. Positive TEEC was induced in only 4 patients in the spasm group. RR was present in totally 77 patients and 68 of them were from the spasm group. By a combination of resting chest pain, negative TEEC and RR to diagnose coronary artery spasm, the sensitivity and specificity were 94% and 96% respectively. Conclusion: Combination of resting chest pain, negative TEE and RR appears to be a rational noninvasive set to predict coronary artery spasm. Copyright © 2007 Wiley Periodicals, Inc.

To explore the relationship between plasma low-density lipoprotein (LDL) and oxidized low-density lipoprotein (ox-LDL) levels and the severity of coronary atherosclerosis.Fasting plasma ox-LDL was measured by enzyme-linked immunosorbent assay and plasma LDL was measured by biochemical autoanalyser in 31 patients with coronary artery spasm (CAS group, chest pain with positive acetylcholine provocation test but without significant coronary artery stenosis), 35 patients with stable angina pectoris (SAP group) and 24 healthy persons (control group).Plasma LDL levels were similar between CAS and SAP groups but significantly higher than that in control group. Plasma ox-LDL levels significantly increased in proportion to coronary lesion severities [SAP (575 +/- 219 microg/L)CAS (299 +/- 117 microg/L)control (218 +/- 35 microg/L)]. In SAP group, plasma ox-LDL level was also significantly higher in multi-vessel disease group than that in single-vessel disease group (672 +/- 92 vs. 462 +/- 72 microg/L, P0.05).Plasma ox-LDL but not LDL level is significantly correlated to the severity of coronary atherosclerosis and should therefore be the focused therapy target in patients with coronary artery disease.

To investigate the value of stress electrocardiography (S-ECG) and stress myocardial perfusion scintigraphy (S-MPS) in the differential diagnosis of patients with atypical chest pain.Patients with atypical chest pain were undergone S-ECG, S-MPS, coronary angiography and coronary artery spastic provocation with intracoronary acetylcholine test. The final diagnoses of those patients were coronary heart disease, coronary spasm, coronary artery muscular bridge, microvascular angina pectoris and chest pain with non-coronary heart disease. Those patients were grouped by final diagnoses to retrospectively compare the results of S-ECG and S-MPS between groups.Totally 186 patients with integrated data were included. The final diagnoses were coronary artery stenosis (above 50% stenosis in diameter) in 20%, coronary artery spasm in 27%, coronary artery muscular bridge in 14%, microvascular angina pectoris in 5%, and chest pain with non-coronary artery disease in 34%. The sensitivity and specificity to diagnose ischemic coronary artery disease (including coronary stenosis, coronary artery muscular bridge and syndrome X but not coronary artery spasm) were 92% and 65% in S-ECG, 62% and 79% in S-MPS, respectively. Combination of atypical chest pain, negative S-ECG and reversal redistribution of S-MPS was an accurate non-invasive method to diagnose coronary artery spasm with sensitivity of 94% and specificity of 96%.Most of patients with atypical chest pain have organic or functional ischemic coronary artery disease. Combination of heart stress tests are helpful to differentiate the etiology of atypical chest pain.

This study is aimed to compare the clinical characteristics of patients with typical and atypical coronary artery spasm.Out of 64 patients with chest pain at rest and without significant coronary artery stenosis, coronary artery spasm was provoked by intracoronary injection of acetylcholine in 46 patients, including 12 with ST segment elevation (typical coronary artery spasm group) and 34 without ST segment elevation (atypical coronary artery spasm group). The demographic data, coronary angiographic findings, treadmill electrocardiogram, dipyridamole and rest thallium-201 myocardial perfusion computed tomography, and the follow-up clinical data of the two groups were compared.The patients with typical coronary artery spasm were younger (47 +/- 6 vs. 58 +/- 12, P0.05) than patients with atypical coronary artery spasm group. Hyperlipidemia were more common in atypical coronary artery spasm group (74% vs. 33%, P0.05) and myocardial bridging was more common in patients with typical coronary artery spasm group (67% vs. 32%, P0.01). Focal coronary spasm during acetylcholine provocation was seen in 92% patients with typical coronary spasm and in 32% patients with a atypical coronary artery spasm (P0.01) while diffuse coronary spasm was seen in 8% patients with typical coronary spasm and in 68% patients with a atypical coronary artery spasm (P0.01). All patients with coronary artery spasm were treated with aspirin, calcium channel blockers, long-acting nitroglycerine, with or without lipid-lowering drugs, 2 patients with typical coronary spasm and 4 patients with atypical coronary spasm were rehospitalized due to chest pain and rest of the patients remained free of chest pain during the median follow-up period of 18 +/- 14 months.Atypical coronary artery spasm is common in patients with rest angina and diffuse coronary microvascular spasm might be the cause of chest pain in these patients.