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.

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.

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

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.

BACKGROUND: Left ventricular hypertrophy induced by primary hyper tension is a risk factor which has a dependent effect on various cardiovas cular complications. Reversal left ventricular hypertrophy has been a keyobject for treating hypertension currently. Plasma neuropeptide Y has en dothelium-derived contracting blood vessel, and has adjusting effect on va somotor function through endothelial cell to participate in onset and main tenance of hypertension. OBJECTIVE: To investigate effect of yixin jiangya capsule on left ven tricular mass index (LVMI) and content of neuropeptide Y of primary grade2 hypertensive patiepts with left ventricular hypertrophy of hyperactivity ofyang due to yin deficiency blood stasis. DESIGN: Case analysis on the basis of patients. SETTING: Department of Traditional Chinese Medicine, General Hospitalof Jinan Military Area Command of Chinese PLA; Department of InternalMedicine, Affiliated Hospital of Shandong University of Traditional Chi nese Medicine. PARTICIPANTS: Totally 93 primary grade 2 hypertensive patients withleft ventricular hypertrophy of hyperactivity of yang due to yin deficiency blood stasis were selected from General Hospital of Jinan Military AreaCommand of Chinese PLA and Affiliated Hospital of Shandong University of Traditional Chinese Medicine from March 2003 to August 2005. All cases were divided into two groups according to random digital table. There were 62 cases in observational group including 40 males and 22 females aged from 46 to 70 years with mean age of 58.94 years and coursed from 2 to 19 years with mean course of 8.93 years. There were 31 cases in control group including 19 males and 12 females aged from 44 to 69 years with mean age of 59.27 years and coursed from 2.5 to 18 years with mean course of 9.11 years. Another 30 people were regarded as healthy group.METHODS: Patients in both groups were treated with enalaprol orally with 10 mg each time of once a day. Patients in observational group were also treated with yixin jiangya capsule (component: shengrlihuang, shengshouwu, sangjisheng, chuanniuxi, gouteng, shijueming, xiakucao, tianma,mudanpi, chishaoyao, dilong, chuanshanjia, etc. Mixed according to ratio,extracted, concentrated, made of capsule, 0.5 g/pill, 4.65 g/raw drug, provided by Shandong Jinan Hongjitang Pharmacy Limited-liability Company,batch number:. 021214), 5 pills once for 3 times every day. Courses in both groups were 24 weeks respectively. LVMI and content of neuropeptide Y were measured with ultrasonic cardiograph and radio-immunity analysis method before and after treatment. Meanwhile, indexes mentioned above were also measured in healthy group so as to regard as the controls.MAIN OUTCOME MEASURFS: LVMI, content of neuropeptide Y,changes of blood pressure, adverse events and side effects.RESULTS: Totally 93 patients entered the final analysis. ① LVMI and content of neuropeptide Y were increased both observational group and control group before treatment [(147.2±16.9), (146.7±17.2), (101.8±.22.4) g/m2;(135.4±33.2), (133.8±31.1), (78.6±24.5) ng/L.; P ＜ 0.01]. ② LVMI and content of neuropeptide Y were decreased both observational group and control group after 24-week treatment, and those in observational group were superior to those in control group [(103.9±24.6), (122.6±19.9) g/m2;(82.9±25.2), (113.7±26.2) ng/L; P ＜ 0.05-0.01]. ③ LVMI and content of neuropeptide Y in observational group were not significantly different from those in healthy group increased both in observational group and control group after treatment (P.＞ 0.05), and those in control group were higher than those in healthy group (P ＜ 0.01). ④ Descent levels of systolic pressure and diastolic pressure were not significantly different between observational group and control group [(137.4±12.8), (134.2:t:11.9) mm Hg;(80.1±8.3), (81.7±7.4) mm Hg, P ＞ 0.05]. Blood routine and liver-kidney function were not changed obviously both groups before and after treatment, and side effect was not observed during the process.CONCLUSION: LVMI and content of neuropeptide Y of primary grade 2 hypertensive patients with left ventricular hypertrophy of hyperactivity of yang due to yin deficiency blood stasis are higher than those of healthy people. Yixin jiangya capsule can deteriorate left ventricular hypertrophy and decrease the content of neuropeptide Y on the basis of application of enalaprol.

Objective To study the impact of preprocedure intravenous urography(IVU)on the outcome of shock wave Iithotripsy(SWL)for the middle and lower ureteral stones.Methods 112 patients with solitary radiopaque the middle and lower ureterat stones on plain radiographs and no severe hydronephrosis on ultrasonographic examination were divided randomly to two treatment groups.IVU group(n=56)had IVU before the start of SwL,whereas patients in the control group(n=56)underwent SWL without IVU.Postoperative success,stone-free rates and complications were evaluated in both groups.Results Eleven patients in the IVU group were excluded from the study.The SUCCESS rate[91.1%(41/45)in IVP group VS 94.6%(53/56)in control group],stone-free rate[88.9%(40/45)vs 89.3%(50/56)],and complication rate[22.2%(10/45)vs 21.4%(12/56)]were similar in two groups(P＞0.05).ConclusionIt is not necessary to obtain an IVU for patients who have solitary radiopaque the middle and lower calculi on plain radiographs with no severe hydronephrosis on ultrasonographic examination before scheduling them for SWL,thus minimizing the cost,avoiding exposure to contrast medium,and reducing radiation exposure.

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.