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Background and aims Chronic granulomatous disease (CGD) is a rare primary immunodeficiency caused by mutations in the NADPH oxidase in phagocytic leucocytes, leading to severe life-threatening bacterial and fungal infections. The most common treatments are prophylactic antibiotics sulfamethoxazole (SMZ) and occasionally antifungal or anti-tuberculosis therapy. Haemopoietic stem-cell transplantation (HSCT) is currently the only curative option for these patients. We summarized the treatments and prognosis of 50 cases of CGD patients in a single center in Chongqing, China. Methods 50 CGD patients were recruited between 2005 and 2017 based on clinical diagnosis, survival analysis was performed. The primary endpoints were overall survival and event-free survival (EFS), frequency of infections, incidence of acute and chronic graft-versus-host disease (GVHD) after at least 18 months of follow-up. Results SMZ was begun for all patients since diagnosis. The overall survival was 80%. Among the 50 patients enrolled, 20 X-linked CGD patients received HSCT with a success rate of 95%. 10 HLA-matched related-donor and 10 HLA-matched unrelated-donor transplants were done. The cumulative incidence of acute GVHD of grade I-II and grade III-IV were 60% and 5%, respectively. The myeloid donor chimerisms were all at least 94% except one 86.1%. Conservative treatment and HSCT decreased separately the frequency of infections to 5.1% and nearly 0 per 100 patient-months in patients. Conclusions Severe infections were the main cause of death and the overall mortality was still high in China. HSTC is a potentially curative therapy for CGD leading resolution of infections and complications. CGD children who had undergone HSCT have better quality of life and fewer infections compared with those treated conservatively.

Background and aims STAT3 gene mutation causes autosomal dominant hyper- IgE syndrome (AD-HIES)is a rare primary immunodeficiency disease. We described the clinical and molecular features of 20 AD-HIES patients in Mainland China. Methods We diagnosed patients with AD-HIES on the basis of NIH score of ≥40 points, described the clinical features, sequenced the STAT3 gene of 20 patients, and quantified Th17 cells in peripheral blood of 19 patients by flow cytometry. Results Among the 20 patients (14 males and 6 females), the onset age ranged from 1 days to 6 months, and the diagnostic age ranged from 1 to 12 years. All patients (100%) had characteristic manifestation of HIES with the range of NIH scores 41–77 points, including recurrent eczema and pneumonia, particularly high serum IgE levels and eosinophilia. Characteristic facial features (100%), bone abnormalities, recurrent skin abscesses (85%), lung abscesses (60%), recurrent oral thrush (55%) were also present in the studied patients. STAT3 hot mutations V637M or R382W/Q were identified in 12 patients (60%), two novel heterozygous missense mutations (T620S and R609G) were identifiedin 2 patients respectively, and other mutations (C712G, S636F, Q469H, Y705H, V432M, c.1139+5G>T) were identified in other 6 patients. The percentage of Th17 cells in CD4+ T cells (CD3+CD8IL-17A+/CD3+CD8 cells) of 19 patients with STAT3 mutation was significantly decreased or deficient as compared with healthy controls. Conclusions Children with repeated Staphylococcus aureus pneumonia, cold skin abscess, fungal infection, eczema, increased serum IgE level and eosinophil, should be alert to AD-HIES. Detection of Th17 cell by flow cytometry assist clinical diagnosis when the NIH score ≥40 points. Further STAT3 gene analysis can confirmed the diagnosis.Early diagnosis and active prevention of infection can significantly improve the quality of life of patients.

Tool inventory has a large difference with the traditional static inventory since the tool can be reused by grinding. In this paper, according to the life characteristics of tool, the tool inventory cost model has been built by studying the using process of tool. The model takes all costs which includes the shortage cost in the product life cycle into account under the circumstances of demand uncertainty. The compact genetic algorithm is successfully used in the cost function optimization process. The reasonableness of the tool inventory cost model and the effectiveness of the compact genetic algorithm have been demonstrated by simulation results. c 2014 IEEE.

A serial of new issues need to be tackled before Ad Hoc network is used in military. There are many challenges: the lack of simulation platform of military Ad Hoc network, the difficulty of concept demonstration on maneuver combat, the hardness of quantitative analysis, etc. So, simulation system on maneuver combat is designed; frame and structure of the system are given. Many new issues on maneuver combat are researched: responsibility area programming, maneuver area programming, maneuver route programming, combat capability assessment, etc. This simulation system has a good application foreground. It can raise the cooperation capability of many single weapons. It can strengthen the capability of associated systems.

A clear understanding of the nucleation, growth, coarsening, and aggregation processes of nanomaterials is necessary to enable the preparation of highly controlled nanostructures. Among wet chemical synthetic methods, ultrasound-assisted preparation has become an important tool in material science. The formation and crystal growth mechanism under ultrasound is special compared with other wet chemical synthetic routes. In this review, we discussed the chemical and physical effect of ultrasound and summarized the ultrasonic effect on crystallization. The sonolysis of water and the cavitation-induced microjet impact and shockwave are the two key factors in the sonochemical formation of inorganic nanomaterials. The ultrasonic-assisted Ostwald ripening and oriented attachment processes have been reviewed for the possible crystal growth mechanisms in the fabrication of inorganic nanostructures.