Objective To study the clinical features of a heterogeneous immunodeficiency disease,common vailable immunodeficiency(CVID),and to enhance the understanding of it.Methods 12 cases of CVID treated in Peking Union Medical College Hospital from January 1990 to March 2007 were analyzed retrospectively,including the clinical characteristics,laboratory results,treatment and prognosis.Results Among the 12 patients,the ratio of male to female Was 2:1,the average onset age(26±9)years old and the median time from onset to diagnosis 18 months.The main symptoms were fever(with a percentage of 67.0%),recurrent Cough and expectoration(58.3%)and diarrhea(41.6%).Anemia and leukocytopenia were the common laboratory changes.All the cases were diagnosed due to the presence of hypo- immunoglobulinemia.The tests available for subtypes of lymphocytes in 9 patients showed that B cells and CD4+ T cells decreased obviously,with an inverse ratio of CD4/CD8,indicating T cell dysfunction.Clinical improvement was demonstrated after treatment with intravenously administered immunoglobulin(IVIG)in 10 cases.Conclusions CVID is a heterogeneous group of immunologic disorders of unknown etiology,characterized by impaired antibody responses and recurrent airway and/or gastrointestinal infection and accompanied with autoimmune diseases or cancer.Hypo-immunoglobulinemia is the main evidence of its diagnosis.IVIG as a replacement therapy is an effective way of management.

The air flow ratio and the pulverized-coal mass flux ratio between the rich and lean sides are the key parameters of horizontal bias burner. In order to realize high combustion efficiency, excellent stability of ignition, low NOx emission and safe operation, six principal demands are presented on the selection of key parameters. An analytical model is established on the basis of the demands, the fundamentals of combustion and the operation results. An improved horizontal bias burner is also presented and applied. The experiment and numerical simulation results show the improved horizontal bias burner can realize proper key parameters, lower NOx emission, high combustion efficiency and excellent performance of part load operation without oil support. It also can reduce the circumfluence and low velocity zone existing at the downstream sections of vanes, and avoid the burnout of the lean primary-air nozzle and the jam in the lean primary-air channel. The operation and test results verify the reasonableness and feasibility of the analytical model.

Objective To study the quality of life(QOL) of the type 2 diabetes mellitus and its influencing factors.Methods The three hundred and seventy-six cases of the type 2 diabetes mellitus were investigated with the short form 36(SF-36) measuring scale in Changsha.Results The results demonstrated that medical care,alcohol intake,physical activity,dwelling coneition,occupation and marriage were the main effective factors.Conclusion It was very important to pay more attention to enhancing psychological and behavioral health education,and improving the QOL in patients of diabetes mellitus.

ObjectiveTo analyze the clinical characteristics of Behcet's disease with intracardiac thrombus.MethodsThe data of 8 patients diagnosed as Behcet's disease with intracardiac thrombus in Peking Union Medical College Hospital from January,1990 to January,2011 were studied retrospectively.ResultsBehcet's disease with intracardiac thrombus was found in 8 patients (5 men and 3 women) with a median age of 28.5 years.Most of them were young men.Thrombus was mostly found in the right side of the heart.Most of the patients had pulmonary thromboembolism with negative anticardiolipid antibody and basically normal C-reactive protein and erythrocyte sedimentation rate.ConclusionIntracardiac thrombus associated with Behcet's disease most commonly occurs in young men and usually involves the right side of the heart.

Objective To improve the diagnosis and treatment of infective endocarditis(IE)by exploring its causes,pathogenic microorganism and clinicsI characteristics.Methods The clinical data of 120 IE patients treated in Peking Union Medical College Hospital from October 1997 to September 2007 were analyzed retrospectively.Results Of the 120 consecutive cascs diagnosed as IE according to the Duke's new criteria,79 were male and 41 female with a average age of(43.2±16.7)years old.Twelve cases were prosthetic valve endocarditis(PVE)and 108 cases native valve endocarditis(NVE)and there were no previously known heart diseases in 29 of the cases.Seventy-nine of the 108(73.1%)NVE patients had basic cardiac abnormalifies before IE diagnosis,such as congenital cardiovascular disease(30 cases),idiopathic mitral valve prolapse(23 cases)and rheumatic heart disease(11 cases).Fever(100.0%),anemia(54.2%)and embolism(48.3%)were the most common clinical manifestations in the IE development.Of the 83 patients who had a positive blood culture result,Streptococcus vividaus(51.8%)was the most common isolated microorganism.Conclusions Congenital cardiovascular diseases and idiopathic mitral valve prolapse are the two most commonly heart diseases in IE.Blood culture and echocardiogram should always be done to exclude IE,especially presenting with fever of unknown reasons.

Objective To investigate the effects of somatostatin (SST) on levels of IL-6,IL-8 and TNF-αduring CPB in pigs. Methods Twenty four healthy pigs were randomly divided into control group (SS00) and 3 experimental groups, SS05, SS10 and SS20. All pigs were performed by CPB for cardiac arrest in 45 min. the levels of IL-6, IL-10 and TNF-αwere tested and compared. Results The TNF-α, IL-6 and IL-10 levels were higher than T0 among all groups after CPB(P<0.05);The levels of TNF-αand IL-6 after CPB in SS10, SS20 groups were lower than SS00 group(P<0.05), and the IL-10 level was higher(P<0.05). Conclusion Infusing with a certain dose of SST before CPB could down-regulate acute inflammatory response;SST has no impact on the operative safety.

Objective To evaluate the pharmacokinetic profiles of lopinavir(LPV) in Chinese HIV-infected patients.Methods A total of 16 patients were enrolled in the LPV pharmacokinetic study.Blood samples were collected before LPV intake and 0.5,1.0,1.5,2.0,2.5,3.0,4.0,6.0,8.0,10.0,12.0 h after administration.Serum level of LPV was determined by the developed high performance liquid chromatography (HPLC) method.The pharmacokinetic profiles were assessed by WinNonlin software.Results The non-compartment model pharmacokinetic (PK) parameters were as follows:the peak time of LPV (Tmax) (3.88 ± 0.23) h,maximum plasma concentration (Cmax) (10.36 ± 3.42) mg/L,minimum plasma concentration (Cmin) (2.18 ± 0.34) mg/L,the 24 h area under plasma-concentration-time curve (AUC0-24) (116.22 ± 15.68) mg · h · L-1,half life(T1/2) (4.5 ± 0.13) h,and clearance rate (CL/F) (3.44 ± 1.34) L/h respectively.Conclusions The pharmacokinetic profiles of LPV in Chinese HIV-1 infected patients demonstrate lower Cmin than those of reported studies,while other parameters are similar.Patients should be educated for compliance based on the narrow gap between Cmin and minimum effect concentration.

Objective To improve the diagnosis and treatment of Q fever endocarditis.Methods From 2008 to 2013,four cases of Q fever endocarditis were diagnosed in Peking Union Medical College Hospital.Clinical features,laboratory test,management and prognosis were analyzed with literature review.Result All four cases had long period of fever and heart murmur.Two patients represented with respiratory symptom and one with non-specific rash.General laboratory tests including complete blood cell count,ESR,C-reactive protein(CRP),liver function and radiology of lung did not show specific abnormalities.Signs of endocarditis were shown by ultrasound and important for diagnosis.Repeated blood culture was negative.All of the diagnoses were confirmed by serum antibody detection and the patients recovered well with treatment based on doxycycline or minocycline.Conclusions Endocarditis is the most common form of chronic Q fever,which is easily misdiagnosed because its blood culture is negative and may accompanied with varied manifestation such as pneumonia and liver injury.For the patients with chronic fever and blood culture negative endocarditis,chronic Q fever should be considered as differential diagnosis.The confirmatory method for diagnosis is serum antibody detection.Early and sufficient treatment may improve the prognosis.

OBJECTIVE: To study the pharmacological activities and toxicity of the crude alkaloids of Corydalis adunca maxim and to provide pharmacological data for further development of this herbal medicine. METHODS: The anti- inflammatory effects of the crude alkaloids of Corydalis adunca maxim were observed by using xylol and agra to induce the turgidness in the rats. The analgesic effects were observed by body distortion methods. The LD50 and 95% creditability were calculated with developed Karber Method. RESULTS: The administration of alkaloids of Corydalis adunca maxim had the function of inhibiting the auricle swelling caused by xylol and joint swelling caused by agar, and of decreasing the body- distortion of the rats. The LD50 of the crude alkaloids of Corydalis adunca maxim was 1. 833g. kg- 1 and the 95% creditability was 1. 18～ 2. 06g? kg- 1. CONCLUSIONS: The crude alkaloids of Corydalis adunca maxim has anti- inflammatory and analgesic effects in rats.

ObjectiveTo evaluatethe microbial spectrum and clinical characteristics of microbiological diagnosed bloodstream infections ( BSI ) with identified infective sources. Methods The hospitalized patients microbiologically diagnosed as BSI with identified infective sources were included in this study from January 2008 to December 2009.Data were collected retrospectively and analyzed by software SPSS 17.0.ResultsIn this 2-year study,301 strains of microbes were isolated from 249 patients.There were 205 ( 82.33％ ) patients with monomicrobial BSI,while the other 44 ( 17.67％ ) patients with polymicrobial BSI.The most common identified source of bloodstream infections was lower respiratory tract infection (125,41.5％ ),followed by intraabdominal infection (55,18.3％ ) and intravascular devices related infection (54,17.9％ ).The four most common isolated pathogens were Acinetobacter species (60,19.9％ ),Escherichia coli (50,16.6％ ),Pseudomonas species (35,11.6％ ) and Staphylococcus Aureus (34,11.3％ ).Eighty-eight (35.3％) patients died during hospitalization due to all causes,out of which 62(24.9％ ) patients died owing to BSI.The patients with BSI originated from lower respiratory tract had a higher crude in-hospital case-fatality ratio than those with BSI originated from other resources ( OR =2.186 ; 95％ CI 1.260-3.792; x2 =7.879,P =0.005). In the multivariate regression,age ≥ 65,invasive mechanical ventilation, reservation of central line and polymicrobial BSI during hospitalization were independent risk factors of death due to all causes. Conclusions Lower respiratory tract is the most common originated source of BSI with microbiological identified sources. Gram-negative bacillus taking advantage,the microbial spectrum of BSI with identified sources in our study is different from those reported before both in primary and secondary BSI.The patients with BSI originated from respiratory tract have a higher crude in-hospital case-fatality ratio.