Primary immunodeficiency diseases (PIDs) represent a typical group of rare diseases with high mortality and disability with regard to long-term outcome.Saving life and improving quality of life of the patients with PID remain to be the responsibility of the stakeholders from all aspects of the society.As the central player in the long run of PID campaign,pediatric immunologists should play a critical role in providing key epidemiological data in favor of political decision-making and social resource distribution.

BACKGROUND: The role of mechanical stress in the functional regulation of osteoblasts becomes an emphasis in osseous biomechanical researches recently. There are few reports on whether fluid shear stress can induce the expression of core-binding factor α1 (Cbfα1) or not and what is its rule.OBJECTIVE: To investigate the effects of fluid shear stress (FSS) on the gene expression of Cbfα1 in human osteosarcoma cells.DESIGN: A controlled observation experiment.SETTING: Department of Aerospace Biodynamics, Faculty of Aerospace Medicine, Fourth Military Medical University of Chinese PLA.MATERIALS: Subjects were human osteosarcoma cells (MG-63) METHODS: This study was carried out at the Department of Aerospace Biodynamics, Faculty of Aerospace Medicine, Fourth Military Medical University of Chinese PLA from November 2004 to April 2005. ①After cultured for 60 hours, MG-63 were treated with 0.5 Pa (0.5 Pa stress treated group ) or 1.5 Pa (1.5 Pa stress treated group ) FSS in a flow chamber for 15, 30, 60minutes, respectively. Cover glass was put in the Petri dish containing culture medium at the same time, serving as the control group of FSS treated group. ②The total RNA in cells was isolated. Reverse transcription polymerase chain reaction analysis was made to examine the gene expression of Cbfα1 mRNA. The ratio of Cbfα1 mRNA and GAPDH mRNA was calculated.MAIN OUTCOME MEASURES: The mRNA expressions of Cbfα1 at different FSS and different time.RESULTS: ① Compared with control group, Cbfα1 mRNA expression increased significantly at 30 and 60 minutes with the treatment of FSS.Within certain time range (15 to 60 minutes), Cbfα1 mRNA expression was strengthened with the elongation of time and the increase of stress level. ②The Cbfα1 mRNA expression at 30 and 60 minutes were significantly increased in 1.5 Pa stress treated group in comparison with 0.5 Pa stress treated group (P＜0.01).CONCLUSION: FSS can significantly increase the gene expression of Cbfα1 in human osteosarcoma cells.

Objective To observe pre-syncopal limited tolerance and cardiovascular responses to head-up tilt combined with lower body negative pressure (HUT/LBNP) following exposure to head-down tilt (HDT, -1 Gz). Method Exposures to HUT/LBNP (-60 mmHg) in control session (without preceding 30 s -1 Gz treatment) and in simulated push-pull effect (PPE) session (with preceding 30 s -1 Gz treatment) were performed in 8 healthy adults. The changes of hemodynamic parameters were monitored by electrical impedance instrument during the experiments. Result The mean endurance time in presyncopal symptom limited HUT/LBNP in control session and in simulated PPE session were 8.4±2.1 min and 4.5±2.4 min, respectively, the two means were significantly different (P< 0.01). In simulated PPE session, as compared with baseline, heart rate (HR) during HDT was significantly lowered (P<0.01), while stroke volume (SV) and cardiac output (CO) were increased significantly (P<0.01). During HUT/LBNP, the increased percentage (relative to baseline) of HR in PPE session was lower than these in control session (P<0.05); the decreased percentages of SV and CO during HUT/LBNP in PPE session were both higher than those in control session (P<0.05). During HUT/LBNP, arterial pulse pressure (PP) of control session was significantly decreased than the value of baseline value (P<0.05); Total peripheral resistance (TPR) of PPE session was significantly increased than baseline value (P<0.05). Conclusion Tolerance time before the appearance of presyncopal symptoms during HUT/LBNP decreases and cardiovascular responses to HUT/LBNP are impaired, preceding exposure to HDT.

Objective To evaluate the clinical efficacy of expulsive therapy using alpha 1-selective adrenoblocker and prostaglandin synthetase inhibitor for distal ureteral stones.Methods 94 patients with distal ureteral stone were randomly divided into study group and control group.In study group,47 cases received tamsulosin 0.2mg daily and dielofenac rectal suppositories 50mg 2 times daily,while watchful waiting without tamsulosin and diclofenac suppository in 47 cases of control group.Observation lasted 2 weeks and also stone expelled as end point.Results No severe adverse reactions related to the drugs were noted and no patients withdrew from the study.The stone-free rate was 91.5%(43/47) of the study group and 25.5% (12/47) of the control group (P＜0.01).A mean stone expelling date of the control group was(8.9±4.3)d and that of the study group was(6.4±3.7)d(P＜0.01).Rates of renoureteral colic recurred in the study group and the control group were 4.3%(2/47) and 48.9%(23/47),respectively (P＜0.01).Conclusion Treatment of distal ureteral stones wlth tamsulosin and dielofenac suppository can increase the stone-free rate,shorten mean stone expelling date,decrease rate of renottreteral colic recurred.

Simple nephrotic syndrome (SNS) of 55 cases of hospitalized patients from 1984 to 1988, boy 46 cases, girl 9 cases. Age 7 months to 13 years were studied. In measurementsof serum immunoglobulin (Ig) 55 cases, IgG decreased 70.9%, but IgA and IgM were mostly normal. Serum IgG were decreased of SNS in 8 cases: IgG1 were significant ly lower than normal (P0.05); IgG3 were significantly higher of than normal (P

The article described the characteristics of research teaching and try to adopt a different research teaching methods based on the characteristics of the contents of each chapter of aerospace biodynamics to enhance the effectiveness of military medicine lesson and cultivate innovative thinking ability of students in military schools.

Objective To explore factors influencing the prognosis of hepatocellular carcinoma (HCC) patients after hepatectomy. Methods From May 1994 to January 1998, 189 patients who underwent hepatectomy for HCC were enrolled for reviewing their clinical characteristics, treatment and prognosis. Twenty-two parameters contributing to long-term survival rate (SR) and disease-free SR were analysed. Results The 3,5-year cumulative SR of the whole group was 63%,45%, respectively. The 3,5-year SR and disease-free SR in the curative resection (CR) group ( n =162) were 67%,47% and 45%,26% respectively. Results showed that the way by which a tumor was found, tumor size, portal tumor thrombi, satellite nodule, TNM stage, cirrhosis type, recurrent and treatment, blood transfusion, differentiation grade,and CR were risk factors by individual variable analysis( P =0.0000～0.0034); A multivariable analysis showed that CR, tumor size, tumor finding mode and reoperation were significant factors associating with prognosis( P =0.0000～0.0024). Blood transfusion and type of cirrhosis were closely correlated with tumor-free survival ( P =0.0001). Conclusions Curative resection, tumor size, reoperation for recurrence were important factors for recurrence by multivariate analysis. The severity of concomittant liver cirrhosis and perioperative blood transfusion were closely correlated with postoperative tumor free survival.

Objective To optimize the preparation of nanoparticles encapsulating antisense oligodeoxynucleotides in a-butyleyanoacrylate carrier (ASODN in NP) and investigate their stability. Methods ASODN in NP were prepared by interfacial polymerization of butyleyanoacrylate (BCA). The formulation and technology of the prepared NP was optimized by using orthogonal design based on the single-factor experiment. The morphology of NP was examined by transmission electron microscope; The size and size distribution of NP were determined by Malvern laser granularity equipment;The encapsulation efficiency and drug loading were determined by HPLC; The ability of protecting oligodeoxynucleotides from serum was investigated on a 20% polyacrylamide-7 Murea sequencing gel (PAGE). Results The nanoparticles in the optimal conditions were of regular spherical surface and discrete. The average size was 97.1 nm,the average encapsulation efficiency and drug loading of ASODN in NP were 96.7% and 10.1% respectively; The oligonucleotides were more efficiently protected from degradation by nucleases than by oligonucleotides adsorbed into nanospheres.Conclusion ASODN in NP has good stability,encapsulation efficiency,drug loading and great potential for ASODN delivery.

OBJECTIVETo study on characteristics and application of key acupoints in Elongated Needle Therapy.

METHODSProbe into method of determining key acupoints and their clinical application from characteristics of channels, characteristics of acupoints, position of diseases, pathogenesis, syndromes and other aspects.

CONCLUSIONEstablishment of the theory of key acupoints of enlongated needle therapy reflects the principle of "fewer but better" in acupoint selection of enlongated needle therapy, which can reduce pain of the patient and increase the therapeutic

Objective To investigate the feasibility, rationality and efficacy of the autologous dermal-fat composite tissue used as filling materials in the repair of nasal dorsum collapse.Methods The dermal fat composite tissue block (7.0 cm× 1.0 cm × 1.0 cm) was removed from the buttocks or abdoman as filling materials. 13 cases of nasal dorsum and nasal-shaped misfits were treated by using an umbrella graft of the auricular cartilage plus dermal-fat composite tissue graft to reconstruct natual shape of nasal dorsum and tip. Results The nasal dorsum and tip were repaired in 13 patients with collapsed nasal dorsum. The dermal-fat composite tissue survived well, and the incisions were healed in stage Ⅰ and the incision trace was not obvious. After follow-up for one year, the shape of nose was much satisfactory. Conclusion It is a well-accepted and easy-going procedure to repair collapsed nasal dorsum with autologous dermal-fat or a combination of composite ear cartilage tissue graft, with satisfactory effect and no rejection reactions.