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.

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 clinical features of amebic liver abscess, the causes of misdignosis and the effect of medical and surgical therapy on patient′s prognosis. [Methods] The clinical features of 36 patients with amebic liver abscess admitted from 1982 to 1997 in our hospital were retrospectively reviewed. [Results] The major clinical manifestations were: abdominal pain (86 1%), fever (86 1%),hepatomegaly with tenderness (83 3%) and right intercostal tenderness(58 3%). Leukocytosis was observed in 61 1%, and increased of ESR in 88 5% (23/28). Serologies against Entamoeba histolytica were noted in 92 6%. Ultrasonography showed single lesions in 75% and right\|lobe involvement in 75%. All patients were treated with metronidazole and 27 patients received treatment with needle aspiration or draining at the same time. After treatment, 10 patients were cured, 25 patients were improved significantly and effective rate was 97 2%. One patient died of hepatic failure. [Conclusion] Medical therapy alone was excellent for small abscesses, while percutaneous needle aspiration or draining was a successful approach in patients with large abscesses.

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 evaluate clinical features,predisposing factors,therapeutic regimen and prognosis of non-traumatic rhabdomyolysis.Methods Clinical picture,therapeutic regimen and prognosis were investigated in 39 cases with non-traumatic rhabdomyolysis by retrospective analysis.Results Non-traumatic rhabdomyolysis mostly presented fever,asthenia,myalgia and/or muscular tenderness,swelling of involved muscles,red urine and oliguria or anuria.The complications and comorbidity of rhabdomyolysis included acute renal failure(ARF),disorders of metabolites and electrolytes,compartmental syndrome,infection,and multiple organ dysfunction.Infection(33.3%)was the most common etiology of non-traumatic rhabdomyolysis,followed by drugs(25.6%),metabolite or electrolyte derangements(10.3%)and alcohol intoxication(7.7%)etc.Therapeutic regimen covered treatment of the underlying diseases,volume repletion,alkalization and dealing with the complications.For the patients with established renal failure,renal replacement therapy was essential.Overall mortality was 15.4%,while the mortality in the patients with ARF was 20.7%.If surviving ARF,the patients' renal function promised to be normalized consequently.Conclusion Non-traumatic rhabdomyolysis is a syndrome with a variety of causes,different clinical presentations and versatile combination of complications,which confounds the diagnosis.However,if treated properly and in time,the survivors in all probability will recover from ARF.

Objective:To analyze the epidemiological characteristics of human immunodeficiency virus (HIV) and hepatitis B virus (HBV) co-infected patients in Beijing and investigate the associated factors.Methods:The clinical data of patients with HIV infection who were treated in HIV/AIDS designated hospitals (Peking Union Medical College Hospital, Beijing Ditan Hospital and Beijing Youan Hospital) were retrospectively analyzed.Results:A total of 11 572 patients were finally included in the study, among whom 532 patients (4.6%) were co-infected with HIV and HBV. Most of the co-infected patients were young male adults (28~48 years old), accounting for 85.9%. The main transmission route was homosexual behavior (74.8%). There were 87.4% co-infected patients treated with two anti-HBV drugs, including lamivudine (3TC) and tenofovir (TDF). From 2013 to 2018, the annual prevalence of HIV and HBV co-infection decreased gradually, with the rate of 6.37%, 4.55%, 3.92%, 4.68%, 4.24% and 2.74%, respectively. In our study, The main influencing factors of HIV and HBV co-infection were age older than 28 years old versus<28 years old ( OR=2.807, 95% CI 1.241-6.345) and marriage status (married versus unmarried, OR=1.259, 95% CI 1.004-1.579). Conclusions:The proportion of HBV infection in HIV-infected patients is 4.60% (532) in our cohort. From 2013 to 2018, the prevalence of HIV and HBV co-infection in Beijing shows a decreasing trend. The risk of co-infection is higher in married young adults (28~48 years old).

Objective To investigate the effect of Glycyrrhizin combined with puerarin on serum Leptin and insulin resistance in non-alcoholic fatty liver disease(NAFLD) patients.Methods One hundred and twenty patients with NAFLD were randomized into 4 groups,which were control group,compound Glycyrrhizin group,puerarin group,combined group,and each group was 30 cases.Patients in control group were received the regular liver protecting therapy including vitamins,amino acids,glucurolactone,in compound Glycyrrhizin group were given 60 ml compound glycyrrhizin solution (iv),in puerarin group were given puerarin at dose of 400 mg by intravenous infusion,and in combined group were given both compound glycyrrhizin and puerarin combination.All treatment period was 4 weeks.The levels of serum serum alanine aminotransferase (ALT),aspartate aminotransferase (AST),total cholesterol (TC),triglyceride (TG),leptin (LP),fasting blood glucose (FBG) and insulin(INS) were measured,and the insulin resistance index(IRI) was calculated.The liver CT image of patients were performed by Germany Siemens dual source CT instrument.Results The levels of serum ALT,AST,TC,TG,LP and IRI in control group at before and after treatment were ((83.08 ± 115.68) U/L vs.(43.32 ±11.72) U/L,(52.12±15.62) U/Lvs.(36.08 ±7.28) U/L,(6.20±1.30) mmol/Lvs.(5.60 ±0.70) mmol/L,(2.70 ±0.50) mmol/L vs.(2.10 ±0.40) mmol/L,(14.63 ±3.26) μg/L vs.(7.61 ± 2.46) μg/L,(7.9 ± 1.8) vs.(7.0 ± 1.2)),and the difference were statistically significant (t =12.828,4.244,16.648,21.442,3.341,16.152 respectively,P ＜ 0.01).The levels of serum ALT,AST,TC,TG,LP and IRI in compound glycyrrhizin group after treatment were ((43.28 ± 11.06) U/L,(37.28 ± 7.22) U/L,(5.70± 0.80) mmol/L,(2.20 ± 0.50) mmol/L,(7.89 ± 2.26) μg/L,(7.1 ± 1.6) respectively,significant different from before treatment ((83.06 ± 14.38) U/L,(51.68 ± 15.48) U/L,(6.30 ± 1.50) mmol/L,(2.60 ± 0.40) mmol/L,(15.13 ± 3.87) μg/L,(7.8 ± 2.2) respectively,t =8.893,4.225,16.520,24.708,6.353,21.137 respectively,P ＜ 0.01).The levels of serum ALT,AST,TC,TG,LP and ISI in puerarin group after treatment were (44.26 ± 9.68) U/L,(36.86 ± 6.88) U/L,(5.60 ± 0.70) mmol/L,(2.26 ± 0.48) mmol/L,(6.89 ± 2.18) μg/L,(7.0 ± 1.8) respectively,significant different from that before treatment ((82.68±14.36) U/L,(50.06±15.23) U/L,(6.20±1.60) mmol/L,(2.70±0.52) mmol/L,(15.68 ±3.26)μg/L,(7.7 ±2.8) respectively,t =7.087,8.138,18.159,7.244,7.470,32.283 respectively,P ＜ 0.01).The levels of serum ALT,AST,TC,TG,LP and ISI in combined treatment group after treatment were (22.28 ± 9.38)U/L,(28.48 ± 9.06) U/L,(5.00 ± 0.60) mmol/L,(1.70 ± 0.40) mmol/L,(4.63 ± 2.36) μg/L,(6.20± 1.6) respectively,significantly different from that before treatment ((84.62 ± 14.88) U/L,(49.12 ± 16.56)U/L,(5.70 ± 1.60) mmol/L,(2.78 ± 0.50) mmol/L,(14.78 ± 3.68) μg/L,(7.6 ± 2.1),t =14.255,11.272,8.371,9.941,8.102,37.626,P ＜ 0.01).The levels of serum ALT,AST,TC,TG,LP and ISI of patient were no significant difference before treatment,but after treatment,these indexes in combined therapy group were the lowest among 4 groups (P ＜ 0.05).And there were no significant difference among control group,compound glycyrrhizin group,puerarin group (P ＞ 0.05).Conclusion Compound glycyrrhizin combined with puerarin is proved to be an effect treatment method for NAFLD through decreasing serum leptin,improving insulin resistance.

Objective To evaluate the etiological and clinical characteristics of fungemia in Peking Union Medical College Hospital.Methods Microbial and clinical information of patients with fungemia consulted in Peking Union Medical College Hospital during 2008 to 2010 were retrospectively analyzed.Results A total of 70 patients were diagnosed with fungemia,and 100％ of them had underlying diseases or potential risk factors.Of them,40 (57.1％) patients were monomicrobial fungemia,and the other 30 (42.9％) patients with positive blood cultures were caused by at least two different microbes during hospitalization.Among 122 strains of microbes isolated from blood cultures,72 were fungi and 50 were bacteria.Among the isolated fungi,61 (84.7％) were Candida species,31 (50.8％) were Candida albicans and 30 (49.2％) were non-albicans.According to the colonization or infected sites other than blood of the isolated fungi,35 cases (50.0％) were primary fungemia; 18(25.7％) were colonizing at lower respiratory tract simultaneously; 10 (14.3 ％) caused central-line related fungemia; 3 (4.3％) were secondary to intraabdominal fungal infection; and another 4 (5.7％) isolates had multiple colonization sites.During hospitalization,37 cases died with a crude mortality rate of 52.9％,and 22 (32.9％) died of fungemia itself.In single factor analysis,ICU hospitalization(x2 =15.136,P ＜ 0.001),operation history within 30 days (x2 =3.540,P =0.060) and invasive mechanical ventilation (x2 =4.450,P =0.035) were related to crude mortality.Bacteremia during hospitalization (x2 =5.657,P =0.017),circulatory underlying diseases (x2 =3.399,P =0.065) and ICU treatment (x2 =4.955,P =0.026) increased attributable mortality.In the multivariate analysis,ICU history increased mortality during hospitalization,however,the operation history within 30 days was independently irrelevant to crude mortality during hospitalization.ICU history and bacteremia during hospitalization were independently correlated to attribution mortality of the patients with fungemia.Conclusions Fungemia,usually accompanied with bacteremia,occurs often in the patients with underlying diseases.Patients with fungemia have poor prognosis and more than 50％ patients die.ICU history increases the risk both to crude and attributable mortality.The patients with fungemia who had polymicrobial bloodstream infection have a higher attribution mortality.Operation history within 30 days is independently negatively correlated to attributable mortality.

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.