OBJECTIVETo explore the short-term impact of ambient PM(10) on daily non-accidental death, cardiovascular and respiratory death of residents in Beijing.

METHODSMortality data of residents in Beijing during 2006 to 2009 were obtained from public health surveillance and information service center of Chinese Center for Disease Control and Prevention, contemporaneous data of average daily air concentration of PM(10), SO(2), NO(2) were obtained from Beijing Environment Protection Bureau (year 2005 - 2006) and public website of Beijing environmental protection (year 2007 - 2009), respectively, contemporaneous meteorological data were obtained from china meteorological data sharing service system. Generalized addictive model (GAM) of time serial analysis was applied. In additional to the control of confounding factors such as long-term trend, day of the week effect, meteorological factors, lag effect and the effects of other atmospheric pollutants were also analyzed.

RESULTSDuring year 2006 to 2009, the number of average daily non-accidental death, respiratory disease caused death, cardiovascular and cerebrovascular diseases caused death among Beijing residents were 140.1, 15.0, 65.8, respectively;contemporaneous medians of average daily air concentration of PM(10), SO(2), NO(2) were 123.0, 26.0, 58.0 µg/m(3), respectively;contemporaneous average atmosphere pressure, temperature and relative humidity were 10.1 kPa, 13.5°C and 51.9%, respectively. An exposure-response relationship between exposure to ambient PM(10) and increased daily death number was found as every 10 µg/m(3) increase in daily average concentration of PM(10), there was a 0.1267% (95%CI: 0.0824% - 0.1710%) increase in daily non-accidental death of residents, 0.1365% (95%CI: 0.0010% - 0.2720%) increase in respiratory death and 0.1239% (95%CI: 0.0589% - 0.1889%) increase in cardiovascular death. Ambient PM(10) had greatest influence on daily non-accidental and cardiovascular death of the same day, while its greatest influence on respiratory death occurred 5 days later.

CONCLUSIONThe ambient PM(10) pollution increased daily non-accidental, respiratory disease caused, cardiovascular and cerebrovascular diseases caused deaths among residents in Beijing, and lag effect existed as for the effect of ambient PM(10) pollution on respiratory disease caused death.

Air Pollutants ; analysis ; Air Pollution ; analysis ; Cardiovascular Diseases ; mortality ; China ; epidemiology ; Environmental Exposure ; analysis ; Humans ; Mortality ; Particle Size ; Particulate Matter ; analysis ; Respiratory Tract Diseases ; mortality ; Time Factors

Objective To report a large family with an autosomal dominant dementia associated with mutation in the prion protein gene(PRNP)and the detailed clinical,neuroimaging and pathological manifestations.Methods Two patients from a large family of dementia were admitted to our ward and the data of their medical history,physical examination,video electroenceplialogram,neuroimaging were colleted.A sterotactic biopsy of the right frontal lobe of the proband was done.After the informed consent from the family members obtained,the genomic DNA was extracted from peripheral blood leucocytes of 5 persons followed by in,vitro amplification using polymerase chain reaction(PCR).The PCR products were directly sequenced by Sanger method.PRNP gene sequence was also examined in 150 normal Chinese to exclude single nueleotide polymorphism.Results A missense mutation of PRNP gene in 5 farnily members was detected,resulting in Gll4V mutation in the prion protein,with M/M genotype of eodon 129.This mutation was not detected in 150 normal Chinese.The proband was diagnosed as inherited prion disease by her clinical features,including neuropsychiatrie disturbances and progressive dementia,and manifestations of neuroimaging,EEG,neuropathology and PRNP gene mutation.Conclusion The first autosomal dominant pedigree of family prion disease is found in China with G114V mutation in PRNP gene which may lead to the prion disease directly.

The high-quality development requirements for public hospitals,national examination orientations,and DIP medical insurance payment reforms present significant challenges to the refined management of public hospitals.Therefore,it is essential to enhance the operational management of these institutions.This paper aims to develop an operation evaluation index system for clinical departments using the Delphi method for assessing the efficiency of resource input and output across various clinical departments.It provides a scientific basis for decision-making regarding resource allocation,transformation towards re-fined management,and the enhancement of operational guidance for departments.

OBJECTIVETo evaluate the efficacy and safety of telbivudine use during the second and third trimester of pregnancy for reducing hepatitis B virus (HBV) transmission from highly viremic hepatitis B e antigen-positive (HBeAg+) mothers to their fetuses.

METHODSPregnant women, between weeks 20 to 32 of gestation, who were HBeAg+ and had HBV DNA more than 1.0*10(7) copies/mL were enrolled in our study. The women were offered inclusion into one of two treatment arms, based upon their personal preference: telbivudine or no telbivudine. The patients in the telbivudine treatment arm were administered 600 mg/d telbivudine at least until postpartum week 4. All delivered infants in both treatment arms were administered hepatitis B immune globulin (HBIG; 200 IU) within 12 hours of delivery and recombinant HBV vaccine (20 mug) at 0, 1 and 6 months. The HBV perinatal transmission rate was determined by measuring HBsAg and HBV DNA in infants at postpartum week 28.

RESULTSA total of 220 pregnant women were enrolled in our study, 120 chose the telbivudine arm and 100 chose the control arm. All telbivudine treated subjects were registered in the Antiretroviral Pregnancy Registry. Telbivudine treatment was associated with a marked reduction in the mothers' serum HBV DNA, HBeAg and ALT levels before delivery. A striking decline of HBV DNA levels in treated mothers was observed at week 2 of treatment, which was followed by a gradual and steady decrease that continued until delivery. Thirty-seven (31%) of the telbivudine-treated mothers and none (0%) of the untreated controls had polymerase chain reaction-undetectable viremia at delivery. At week 28, 0% of the infants delivered from telbivudine-treated mothers were HBsAg+ or HBV DNA+, as compared to 8% HBsAg+ or HBV DNA+ in the untreated control arm (P = 0.002). No telbivudine discontinuations occurred from adverse events, and no congenital deformities were observed in the infants delivered to telbivudine-treated mothers. Eighty mothers discontinued telbivudine at week 4 postpartum, and there were no cases of severe hepatitis. There were no significant differences between the two treatment arms for postpartum hemorrhage, adverse events during pregnancy, cesarean section, gestational age, or infants' height/weight or Apgar scores.

CONCLUSIONSTelbivudine use during the second and third trimester of pregnancy in HBeAg+ highly viremic mothers can safely reduce perinatal HBV transmission rates. Telbivudine was well-tolerated by our patient group. Furthermore, no safety concerns were observed in either the telbivudine-treated mothers or their delivered infants in short term follow-up.

Adult ; DNA, Viral ; Female ; Hepatitis B ; transmission ; virology ; Hepatitis B virus ; Humans ; Infectious Disease Transmission, Vertical ; prevention & control ; Nucleosides ; adverse effects ; therapeutic use ; Pregnancy ; Pregnancy Complications, Infectious ; prevention & control ; virology ; Pregnancy Trimester, Second ; Pregnancy Trimester, Third ; Pyrimidinones ; adverse effects ; therapeutic use ; Thymidine ; analogs & derivatives ; Viral Load ; Young Adult

OBJECTIVETo investigate the role of 99mTc-TRODAT-1 SPECT in diagnosis and assessing severity of idiopathic Parkinson's disease (PD).

METHODSThirty-eight patients with primary, tentative diagnosis of PD and eighteen age-matched normal controls were studied with 99mTc-TRODAT-1 SPECT imaging. The regions of interests (ROIs) were drawn manually on cerebellum (CB), occipital cortex (OC) and three transverse plane slice-views of striatums, the semiquantitative BG (background)/[(OC+CB)/2] were then calculated.

RESULTSA lower uptake of 99mTc-TRODAT-1 in striatums were displayed in thirty-six out of thirty-eight PD patients by visual inspection, compared to controls. In twenty-four PD cases with (Hoehn and Yahr scale) HYS stage I, a greater loss of DAT uptake was found in striatum and its subregions contralateral striatum to the affected limbs than in the same regions of the controls, although the striatal uptake was bilaterally reduced. Using Spearman correlation analysis showed that the reduction of the uptake ratios significantly correlated with the UPDRS in striatum and all its subregions in the PD group (P<0.05), a similar change was also found in the putamen by using the rating scale of Hoehn and Yahr (P<0.05). However, analysis of variance (ANOVA) did not show any relationship between the decreasing uptake of 99mTc-TRODAT-1 and increasing severity of PD patients, although the specific uptake of 99mTc-TRODAT-1 was continuously decreased in the striatum by visual inspection with the progress of PD from HYS stage I to III.

CONCLUSION99mTc-TRODAT-1 SPECT imaging may serve as a useful method for improving the correct diagnosis of PD. In assessing the role of 99mTc-TRODAT-1 SPECT in disease severity of PD, UPDRS can offer a comprehensive index, although the Hoehn and Yahr assessment may be available in part.

Adult ; Aged ; Corpus Striatum ; diagnostic imaging ; metabolism ; Dopamine Plasma Membrane Transport Proteins ; Feasibility Studies ; Female ; Humans ; Male ; Membrane Glycoproteins ; metabolism ; Membrane Transport Proteins ; metabolism ; Middle Aged ; Nerve Tissue Proteins ; metabolism ; Organotechnetium Compounds ; pharmacokinetics ; Parkinson Disease ; diagnostic imaging ; metabolism ; Radiopharmaceuticals ; pharmacokinetics ; Reproducibility of Results ; Sensitivity and Specificity ; Severity of Illness Index ; Tomography, Emission-Computed, Single-Photon ; methods ; Tropanes ; pharmacokinetics

This study was aimed to investigate the pro coagulation effects of hemocoagulase atrix and its effective components (batroxobin and factor X activator) on plasma of normal subjects and patients with bleeding disorders and their mechanisms. Activated partial thromboplastin time (APTT) and prothrombin time (PT) were measured. The factor (F)X activation and thrombin generation were analyzed by using chromogenic substrate method. The results showed that the plasma APTT of normal subjects was shortened by hemocoagulase atrix, batroxobin and FX activator, and the effect of FX activator was found to be concentration-dependent (r = 0.889, P < 0.05). The prolonged APTT of plasma from patients with bleeding disorders could be corrected by hemocoagulase atrix, batroxobin and FX activator, but PT showed no great changes resulted from the treatments. FX activator could promote FX activation and thrombin generation, while neither hemocoagulase atrix nor batroxobin showed such abilities. It is concluded that hemocoagulase atrix promotes coagulation process, and corrects coagulation abnormalities in patients with bleeding disorders, its main component batroxobin directly acts on fibrinogen, and FX activator promotes thrombin generation through activating FX.
Adult ; Batroxobin ; pharmacology ; Blood Coagulation ; drug effects ; Blood Coagulation Disorders ; blood ; Case-Control Studies ; Cysteine Endopeptidases ; pharmacology ; Factor X ; metabolism ; Female ; Humans ; Male ; Middle Aged ; Neoplasm Proteins ; pharmacology ; Partial Thromboplastin Time ; Thrombin ; metabolism ; Young Adult

OBJECTIVETo study the clinical features and ABCG5/ABCG8 gene mutations of three pedigrees of phytosterolemia presented with macrothrombocytopenia and hemolysis.

METHODSErythrocyte and platelet morphology were examined under light microscope. Plasma sterol levels were measured by high pressure/performance liquid chromatography method. All of ABCG5 and ABCG8 exons and intron-exon boundaries were directly sequenced to identify mutations, the corresponding gene mutation sites of three families members and healthy individuals were detected.

RESULTSAll the patients presented macrothrombocytopenia, hemolysis, splenomegaly and xanthomas. The blood smears showed large platelets, some as large as erythrocytes, and abnormal erythrocyte shapes, such as stomatocytes. Plasma concentrations of phytosterols, especially sitosterol were markedly elevated (30 fold) in the affected patients. Four mutations were identified in these three pedigrees, ABCG5 C20896T (R446X) and A20883G, ABCG8 del43683-43724 and del1938C-1939G/ins1938T. The latter three were novel mutations reported for the first time.

CONCLUSIONSPhytosterolemia associated with macrothrombocytopenia and hemolysis is a new subtype of this disease. Plasma phytosterols and related gene analysis should be performed when ever an unexplained macrothrombocytopenia, especially combined with haemolysis or/and stomatocytosis.

ATP Binding Cassette Transporter, Sub-Family G, Member 5 ; ATP Binding Cassette Transporter, Sub-Family G, Member 8 ; ATP-Binding Cassette Transporters ; genetics ; Adult ; Blood Platelets ; cytology ; DNA Mutational Analysis ; Erythrocytes, Abnormal ; Female ; Hemolysis ; genetics ; Humans ; Hypercholesterolemia ; genetics ; pathology ; Intestinal Diseases ; genetics ; pathology ; Lipid Metabolism, Inborn Errors ; genetics ; pathology ; Lipoproteins ; genetics ; Male ; Middle Aged ; Mutation ; Pedigree ; Phytosterols ; adverse effects ; blood ; genetics ; Platelet Count ; Thrombocytopenia ; genetics ; pathology

OBJECTIVETo investigate the impact of penile surgery on the erectile function of the patient and to evaluate the role of small-dose vardenafil in restoring the impaired penile erection.

METHODSSixty cases of penile cavernosum surgery were equally and randomly divided into a vardenafil and a control group, the former treated 5 - 7 days after surgery with 10 mg vardenafil every other day, while the latter given vitamin E at 100 mg once a day, both for 12 weeks. The penile erectile function of the patients was evaluated with the IIEF-5 questionnaire before surgery and at 3 and 6 months after vardenafil medication.

RESULTSThe mean IIEF-5 scores of the vardenafil group were 18.83 +/- 2.98 and 20.13 +/- 2.98 at 3 and 6 months after vardenafil medication, significantly higher than 14.21 +/- 3.62 before surgery (P > 0.05), while that of the control group was significantly decreased at 3 months as compared with the preoperative score (13.38 +/- 2.82 versus 15.80 +/- 3.02, P < 0.05). The vardenafil group showed the highest IIEF-5 score after surgery (P < 0.05).

CONCLUSIONLong-term administration of small-dose vardenafil after penile surgery helps to restore and maintain penile erectile function.

Adult ; Erectile Dysfunction ; drug therapy ; Humans ; Imidazoles ; administration & dosage ; therapeutic use ; Male ; Middle Aged ; Penile Erection ; Penis ; surgery ; Piperazines ; administration & dosage ; therapeutic use ; Postoperative Period ; Recovery of Function ; Sulfones ; administration & dosage ; therapeutic use ; Treatment Outcome ; Triazines ; administration & dosage ; therapeutic use ; Vardenafil Dihydrochloride ; Vasodilator Agents ; administration & dosage ; therapeutic use

OBJECTIVETo explore the effects of adenovirus vector-mediated gene transfer of ICOSIg fusion protein on experimental autoimmune myocarditis (EAM) in Lewis rats.

METHODSExpression vector containing ICOSIg (p-Adeno-ICOSIg) was constructed by fusion of human ICOS and IgGFc segment. Adenovirus vector was digested by PacI enzyme and transfected into HEK 293 cells. Adenovirus expressing ICOSIg was produced. EGFP was constructed into adenovirus vector and used as control. EAM was induced in Lewis rats by injection of porcine cardiac myosin. All immunized Lewis rats were divided into 4 groups. Group A (n = 15) and B (n = 15) received adenovirus containing ICOSIg on day 0 and day 14 respectively to study the effects of costimulatory molecules gene therapy on T cell activation and inflammation; group C (n = 10) and group D (n = 10) received adenovirus containing EGFP on day 0 and day 14 respectively as controls. Group E (n = 10) was normal controls that did not receive immunization. On day 28, all rats were killed after echocardiography examination. Histopathological examination was performed to observe myocardial inflammation. Protein levels of ICOS, ICOSL, B7-1 and B7-2 were detected by Western blot. INF-gamma, IL-2 and IL-4 mRNA were determined by realtime RT-PCR.

RESULTSOn day 28, cardiac function was significantly improved and myocardial inflammation significantly attenuated in group B compared to group A, C and D (all P < 0.05). B7-1 expression at protein level was significantly lower in group B than that of group C (P < 0.05). ICOS and ICOSL expressions at protein level were significantly decreased in both group A and B compared with group C and D (P < 0.05). IFN-gamma mRNA level significantly decreased and IL-4 mRNA significantly increased in group A and B compared to group C and D (P < 0.05).

CONCLUSIONSBlockade of costimulatory pathway with gene therapy of ICOSIg alleviated autoimmune inflammatory damage and improved cardiac function in Lewis rats with EAM. Down-regulated costimulatory molecules in the myocardium and reduced inflammatory cytokine secretion might be responsible for the beneficial effects of ICOSIg in this model.

Adenoviridae ; genetics ; Animals ; Antigens, Differentiation, T-Lymphocyte ; genetics ; Autoimmune Diseases ; immunology ; pathology ; therapy ; Disease Models, Animal ; Gene Transfer Techniques ; Genetic Therapy ; Genetic Vectors ; Immunoglobulin Fc Fragments ; genetics ; Inducible T-Cell Co-Stimulator Protein ; Male ; Myocarditis ; immunology ; pathology ; therapy ; Rats ; Rats, Inbred Lew ; Recombinant Fusion Proteins ; genetics

OBJECTIVETo investigate clinical features, laboratory alterations and gene mutations of 6 patients with Wiskott-Aldrich syndrome (WAS).

METHODST lymphocyte subtypes were measured by flow cytometer. The routine blood tests including platelet count and mean platelet volume were performed by complete blood analyzer Sysmex XE2100. Serum immunoglobulin was measured by immunoturbidimetry. Mutations in WAS protein (WASP) gene (including all the exons and exon-intron boundaries and 3', 5' untranslation region) of 6 patients and their family members were identified by PCR and sequencing.

RESULTSThe patients presented with petechiae, easy bruise, eczema, bloody diarrhea, recurrent infection and fever, and the clinical scores were 3 or 4. They were thrombocytopenia with smaller mean platelet volume, anemia and leukocytosis. Megakaryocyte number was normal or slightly increased in bone marrow. In the probands, the percentage of CD3+ T cells was decreased, the CD4+/CD8+ ratio was abnormal, while the fractions of CD19+ and CD16+ CD56+ cells were in normal range. In most of the patients, the serum levels of IgG and IgA were increased. Six mutations were identified in the patients, including 10250 C-->T, and five novel mutations: 6783 C-->G,10216-10221 Ins G, 9964 Del T,10192-10203 Del GCCTGCCGGGG and 10052-10059 del GCTACTG. The 6783 C-->G in exon 3 resulted in premature stop at Tyr102, and the remaining four mutations in exon 10 resulted in frame shift and premature stop.

CONCLUSIONThe main characteristics of these WAS patients were thrombocytopenia with smaller mean platelet volume and immunological disturbance. Their gene mutations were deletion, insertion or nonsense mutations. All the patients had been misdiagnosed as ITP, indicating the importance of differential diagnosis.

Child, Preschool ; DNA Mutational Analysis ; Humans ; Infant ; Male ; Platelet Count ; Sequence Deletion ; Wiskott-Aldrich Syndrome ; diagnosis ; genetics ; pathology ; Wiskott-Aldrich Syndrome Protein ; genetics