In this feature article,we focused on rare hereditary bleeding disorders with the exception of hemophilia,hereditary factor Ⅺ deficiency and von Willebrand's disease.In order to raise the awareness and promote the diagnosis rate of the rare hereditary bleeding disorders,we discussed the classification according the bleeding mechanism,clinical features and therapeutic advance.

The mechanisms of childhood primary immune thrombocytopenia(ITP) are the destruction of platelet by platelet antibody and cytotoxic T cells,which also influence the function of megakaryocyte and cause the reduction of platelet.Novel agents targeted to the immune abnormalities of ITP includes 4 types:(1) CD40-CD154 pathway blocker,such as humanized CD154 antibody Toralizumab,which is monoclonal antibody and modify Fc,hopefully to be used in treatment of ITP.(2) The drugs which block the binding between Fc receptor and anti-platelet antibody such as Rozrolimupab,which is the combination of 25 kinds of anti-RHD and presents 62％ efficacy but has severe hemolysis as adverse effects which restricts the application on ITP.Soluble FcγRⅡb(SM101) is also effective on ITP and it conquer the shortcome of activating of B cells to induce autoimmune diseases.(3) The drugs which block the key molecular Syk of activation the platelet phagocytosis by microphage such as R788,with 44％ effectiveness.(4)The drugs which stimulate thrombopoiesis such as Avatrombopag et al.

Microcystins (MCs) is a potent cyclic heptapeptide hepatotoxin produced by the blue-green algae,Microcystis aeruginosa. The pollution caused by microcystins has become a global concern. Facing to MCs-induced health hazards and potential economic losses,it has an important practical significance to study the effective chemoprotectants against MCs. In this paper,both domestic and abroad researches on the chemoprotectants (silymarin,curcumin,green tea polyphenols,cyclosporin-A,rifampin,trace element supplementation) against MCs were reviewed.

Objective To investigate the performances in line bisection(LB)and line cancellation(LC)tasks of patients with unilateral neglect.Methods The unilateral neglect patients,performances in LB and LC tasks before and after rehabilitation treatment were retrospectively analyzed.Results Before treatment,statistical analyses revealed the patients,performances in LC and LB tasks did not differ from each other(P =0.902).After treatment,statistical analyses revealed a significant difference between the two tasks(P =0.007).With LB only,29/30(97%)patients with neglect were detected;with LC only,22/30(73%)patients with neglect were detected.Conclusions As the performances of unilateral neglect patients improved,patients revealed difference between the performances in LB and in LC tasks.One test alone would miss some subjects with neglect.Combinative use of the two tests was more sensitive than either of the 2 tests alone.

Objective:To assess the prevalence of depressive symptoms in type 2 diabetic patients with hypertension.Methods:A systematic electronic search was conducted in CNKI,Wanfang database,SinoMed,Pubmed and Embase.Literatures that reported the depressive symptoms status of type 2 diabetic patients with hypertension were included.All patients were divided into case group and control group,according to with or without hypertension.Meta-analysis was performed with Statal2.0 software after data extraction and the quality of studies assessment.Results:A total of 11 articles involving 8031 type 2 diabetic patients were included in the meta-analysis,including 4711 patients in case group and 3320 patients in control group.The pooled prevalence of depressive symptoms in case group was 43.5％ (95％ CI:26.1％-60.8％).The pooled prevalence of depressive symptoms in control group was 34.0％ (95％ CI:19.6％-48.5％).The pooled odds ratio was 1.32 (95％ CI:1.05-1.67,P ＜0.05).Conclusion:Hypertension increases the risk of developing depressive symptoms in type 2 diabetic patients.Therefore,medical staff should pay more attention to the mental health of this population.

Objective To construct the pcDNA3. 1-Brugia malayi (Bm) glyceraldehyde-3-phosphate dehydrogenase (GAPDH) eukaryotic recombinant plasmid and to study its effect on mouse cellular immunity response. Methods Total RNA was prepared from periodic Bm. The target gene fragments were amplified by reverse transcription-polymerase chain reaction (RT-PCR) technique and then were inserted into the cloning vector. pGEM-T Easy, and sub-cloned into pcDNA3. 1. Purified pcDNA 3. 1-BmGAPDH recombinant plasmid and CpG were injected into the anterior tibial muscle of BALB/c mice in order to induce host immunity response. Mice injected with PBS and mice injected with blank plasmid were prepared as controls. The mouse models were immunized for 3 times with an interval of 2 weeks. RT-PCR was utilized to detect target gene expression in the muscle tissue. MTT method was used to measure the immunized mice T lymphocytes stimulation index, while enzyme-linked immunoassay (ELISA) was used to determine the serum interleukin (IL)-4 and interferon (IFN)-γ level. Means were compared using t test with SPSS software. Results The recombinant plasmid pcDNA3. 1-BmGAPDH was constructed sucessfully. The target gene was 1020 bp long and its homology with known gene sequence in database was 99%. BmGAPDH gene in the injected muscle of the immunized mice was detected by PCR. The proliferation of spleen T lymphocytes was higher in pcDNA3-BmGAPDH group than in the 2 control groups which were 1. 398, 1. 006 and 1. 017,respectively (P< 0. 05). The levels of IFN-γ and IL-4 in serums from the immunized mice were significantly higher than those of the PBS control group and blank plasmid control group which were 163.905, 58.589, 51. 317 and 107. 906, 27.111, 34.627, respectively (P<0. 05). Immune adjuvant CpG could accelerate and boost antigen-specific immune responses induced by vaccine, which presented as significant increase of IFN-γ and lymphocyte proliferation at 4 weeks after immunization.Conclusion The recombinant eukaryotic plasmid pcDNA3. 1-BmGAPDH is constructed and could elicit cellular immune responses in immunized mice.

Objective To investigate the value of the application of fibrobronchoscopy in extubation for patients suffering from acute exacerbation of chronic obstructive pulmonary disease(AECOPD)with low cough peak expiratory flow(CPEF). Methods A single-center prospective controlled study was conducted. The ventilated AECOPD patients who were cooperative at the time of extubation in Department of Critical Care Medicine of Guangzhou Institute of Respiratory Disease of Guangzhou Medical University from June 2009 to May 2014 were enrolled. All patients successfully passed the spontaneous breathing trial(SBT). Extubation was performed after determination of CPEF following energetic coughing. According to the CPEF,the patients were divided into CPEF≥60 L/min group (high CPEF group)and CPEF<60 L/min group(low CPEF group). After extubation,fibrobronchoscopic drainage was given to the patients in high CPEF group when necessary. Fibrobronchoscopic drainage was given to the patients in low CPEF group at least once a day,and the frequency of such treatment could be increased according to the patient's condition. If the patients did not require re-intubation within 48 hours,extubation was recorded as successful. The gender,age,acute physiology and chronic health evaluationⅡ(APACHEⅡ)score before extubation,ventilation time,the time of intensive care unit(ICU)stay,the mortality in ICU,the rate of re-intubation,the ability to cough and the frequency of application of fibrobronchoscopy after extubation were recorded. Results A total of 102 patients with AECOPD were enrolled,58 patients in high CPEF group and 44 in low CPEF group. Compared with high CPEF group,the mean age in low CPEF group was older(years:74.3±15.2 vs. 69.5±11.4,t=2.164,P=0.041),the time of ICU stay was significantly longer(days:20.1±11.2 vs. 17.4±7.3,t=2.274,P=0.030), but there was no significant difference in gender〔male/female(cases):35/9 vs. 45/13,χ2=0.057,P=0.812〕, APACHEⅡscore(11.9±1.9 vs. 10.3±4.2,t=1.290,P=0.200),mechanical ventilation time(days:14.8±10.8 vs. 13.3±9.6,t=0.677,P=0.501)and the rate of re-intubation〔18.18%(8/44)vs. 12.07%(7/58),χ2=1.412, P=0.235〕between low CPEF group and high CPEF group. The cough strength of patients in high CPEF group was almost alwaysstrong(52 cases),and in the low CPEF group,most of them wasmoderate(14 cases)orweak(26 cases). The frequency of application of fibrobronchoscopy in low CPEF group was higher than that in high CPEF group(times:4.1±1.8 vs. 1.3±0.9,t=2.626,P=0.011). All patients underwent weaning successfully,and no death occurred. Conclusion The application of fibrobronchoscopy in the extubated AECOPD patients with low CPEF can reduce the rate of re-intubation,avoid the prolonged ventilation,but cannot reduce the time of ICU stay.

Objective To investigate the influence of remote care management on the diet compliance and disease recurrence of patients with pancreatitis. Methods Seventy-four simple pancreatitis patients in our hospital from January 2013 to March 2014 were involved and numbered according to the administration order and divided into the observation group and the control group according to the random number table with 37 cases in each group. The two groups accepted conventional health education during hospitalization time. The control group was given intensive classes after discharge, while the observation group received remote care management. Twelve months later, the rate of disease recurrence and diet compliance of the two groups were observed and compared. Results After the management, the observation group was superior to the control group in diet compliance. The rate of disease recurrence was significantly lower in the observation group than that of the control group (all P<0.05). Conclusion The remote care management can effectively improve the diet compliance of pancreatic patients, lower the recurrence rate, and have positive effect on prognosis.

To evaluate the status of diabetes-related knowledge and training requirement among general physicians,a questionnaire survey was conducted in 676 general physicians from 32 primary medical institutions and 5 secondary hospitals of Shanghai Pudong New Area.48.2% (261/541) of the surveyees had bachelor degree and most of them worked in secondary hospitals or primary medical institutions in affluent areas.The awareness rate of diabetic knowledge among the surveyeos was 51.6%,and there were significant differences in the awareness rate of diabetes-related knowledge between different levels of hospitals and physicians with different academic background.Only 26.6% of physicians received diabetes-related continuing education or training,there was a popular request for professional training from the surveyed physicians.

Objective To investigate the significance of B-type natriuretic peptide (BNP) in acute coronary syndrome(ACS).Methods 151 in-patients of ACS during May 2006 to June 2008 were selected as the treatment group.Other 212 impatients with essential hypertension were selected as control group.Blood lipid,blood glucose,blood routine,renal function,high sensitive C-reaction protein (hs-CRP) and BNP were examined in all patients.Their blood pressure,heart rate,and history of smoking,hypertension,diabetes mellitus and hyperlipidemia 62.0±14.2,t = 5.043,P＜0.001 ),blood creatinine [ (95.0±67.4 ) vs.(72.8±29.0) μmol/L,t = 4.164,P ＜0.001 ),blood uric acid[ (360.2±104.4 ) vs.(300.8±92.7 ) μmol/L,t = 5.521,P ＜ 0.001 ],BNP (80.81 vs.352.38 ng/L,Z = - 7.295,P ＜ 0.001 ),hs-CRP (7.99 vs.17.08,Z = - 3.983,P ＜ 0.001 ),white blood cells (WBC) [ (7.843 +3.025)×109/L vs.(6.333±3.569)×109/L,t =4.209,P ＜0.001 ],monocytes [ (0.522±0.201)×109/L vs.(0.417±0.157)×109/L,t = 5.084,P ＜0.001 ] and blood glucose [ (6.518±2.303 ) vs.(5.691±2.085 ) mmol/L,t = 3.461,P = 0.001 ] had significant difference.Systolic blood pressure had difference between two groups [ (134.7±27.8 ) vs.(142.0±26.3 ) mm Hg,t = 2.536,P=0.012 ].However,the percentage of smoking[ 43.7% (66/151)vs.23.1% (49/212),X2 = 17.283,P ＜0.001 ] and male gender[ 74.8% ( 113/151 )vs.48.6% ( 103/212),X2 = 25.217,P ＜ 0.001 ) ] had significant difference between two groups ( P ＜0.001 ).Multivariable regression analysis showed that age (X2 = 10.369,P =0.001 ),gender (X2 = 12.836,P ＜ O.001 ),BNP (X2 =4.807,P =0.028) and WBC(X2= 10.788,P =0.001 ) were the main factors of difference between the two groups.Moreover,BNP was linearly correlated with left ventricular ejection fraction(EF) in ACS patients (t =5.789,P ＜ 0.001 ).Conclusion Old age,male,WBC and BNP are closely related with ACS,of which BNP has certain predictive value for ACS.