Objective: To report the clinical manifestations and laboratory features of five patients with congenital thrombotic thrombocytopenic purpura (cTTP) and explore its standardized clinical diagnosis and treatment along with a review of literature. Methods: Clinical data of patients, such as age of onset, disease manifestation, personal history, family history, and misdiagnosed disease, were collected. Treatment outcomes, therapeutic effects of plasma infusion, and organ function evaluation were observed. The relationship among the clinical manifestations, treatment outcomes, and ADAMTS13 gene mutation of patients with cTTP was analyzed. Additionally, detection of ADAMTS13 activity and analysis of ADAMTS13 gene mutation were explored. Results: The age of onset of cTTP was either in childhood or adulthood except in one case, which was at the age of 1. The primary manifestations were obvious thrombocytopenia, anemia, and different degrees of nervous system involvement. Most of the patients were initially suspected of having immune thrombocytopenia. Acute cTTP was induced by pregnancy and infection in two and one case, respectively. ADAMTS13 gene mutation was detected in all cases, and there was an inherent relationship between the mutation site, clinical manifestations, and degree of organ injury. Therapeutic or prophylactic plasma transfusion was effective for treating cTTP. Conclusions: The clinical manifestations of cTTP vary among individuals, resulting in frequent misdiagnosis that delays treatment. ADAMTS13 activity detection in plasma and ADAMTS13 gene mutation analysis are important bases to diagnose cTTP. Prophylactic plasma transfusion is vital to prevent the onset of the disease.
Female ; Pregnancy ; Humans ; Adult ; Blood Component Transfusion ; Plasma ; Purpura, Thrombotic Thrombocytopenic/therapy* ; Mutation ; Purpura, Thrombocytopenic, Idiopathic ; ADAMTS13 Protein/therapeutic use*

Objective:To study the soil physical and chemical properties， microorganisms， and metabolites in different culture environments of Gastrodia elata， so as to provide scientific basis for subsequent cultivation of G. elata in multiple environments. Method:The tubersphere soil of G. elata cultured in different environments was collected for analyzing the soil nutrients， microbial numbers， and metabolite differences using the agrochemical method， plate-count method， and gas chromatography-time-of-flight mass spectrometry （GC-TOF-MS）-based non-targeted metabonomic approach. Result:The analysis of soil physical and chemical properties revealed the highest soil moisture， pH， available potassium， and available phosphorus in the spinney and the highest electrical conductivity， total nitrogen， alkali-hydrolyzable nitrogen， and organic matter in the pinewood. As demonstrated by the quantitative analysis of soil microorganisms， the cultivable microorganisms were bacteria， actinomycetes， and fungi， with the bacterial population and total microbial biomass in the spinney and the number of fungi and actinomycetes in the barren slope detected to be the largest. The ratio of bacteria to fungi （B/F value） in the pinewood was the highest， while that in the barren slope was the lowest. The results of metabonomic research demonstrated that the compositions and quantities of soil metabolites in the spinney （Z group）， pinewood （S group）， and barren slope （HD group） varied. Through comparisons between S and Z groups， between HD and Z groups， as well as between HD and S groups by orthogonal partial least squares discriminant analysis （OPLS-DA）， 18， 35， and 24 differential metabolites were separately screened out， and the Kyoto Encyclopedia of Genes and Genomes （KEGG） pathway enrichment analysis yielded 5， 9， and 13 metabolic pathways. There existed a significant causal relationship of the soil physical and chemical properties and microbial numbers with the metabolites. Conclusion:The soil physical and chemical properties， microbial numbers， and metabolite changes differed significantly in different culture environments of G. elata， which were sorted by the suitability in a descending order as follows： spinney > pinewood >barren slope. The soil physical and chemical properties and microbial numbers are the crucial factors driving changes in soil metabolites， suggesting that regulating the soil physical and chemical characteristics and microbial characteristics in the culture environment is an important mechanism for maintaining the G. elata-soil-microbial symbiotic system.

Objective: To evaluate the prognostic value of kinetic changes in minimal residual disease (MRD) status, as well as its relationship with risk stratification, therapeutic response and treatment in patients with newly-diagnosed multiple myeloma (MM) . Methods: A total of 135 patients with newly-diagnosed MM were screened, and 105 patients who achieved VGPR or more as the best responses were included into this study. The MRD status was determined by multiparameter flow cytometry (MFC) at multiple intervals after two cycles of treatment until clinical relapse, death, or last follow-up. The statistical methods included Kaplan-Meier analysis, Cox regression, etc. Results: ①In all 135 patients, 57.8% (78/135) patients achieved MRD negativity (MRD(-)) after treatment. In 105 patients who achieved VGPR and thus included in this study, the MRD(-) rate was 72.4% (76/105) , with a median interval of 3 months from starting treatment to achievement of MRD(-) status. ②The 2-year PFS rate of patients with MRD(-) status was significantly higher than that of MRD(+) status (62.2% vs 41.3%, P=0.001) , while MRD persistence (MRD(+)) was an independent factor for poor prognosis (multivariate analysis for PFS: P=0.044, HR=3.039, 95%CI 1.029-8.974) . ③Loss of MRD(-) status (i.e., MRD reappearance) showed inferior outcomes compared with MRD sustained negative ones, the PFS was 18 months versus not reach (P<0.001) and the OS was not reach for both (P=0.002) . ④The 2-year PFS and OS rates of patients with duration of MRD(-)status≥12 months were significantly higher than those of the control group (PFS: 77.7% vs 36.7%, P<0.001; OS: 96.4% vs 57.9%, P<0.001 respectively) . Duration of MRD(-) status was associated with a marked reduction in risk of relapse or death (univariate analysis for PFS: P<0.001, HR=0.865, 95%CI 0.815-0.918; for OS: P=0.001, HR=0.850, 95%CI 0.741-0.915 respectively) . ⑤Moreover, even in patients carrying high-risk cytogenetic abnormalities (CA) or ineligible for ASCT, MRD negativity remained its prognostic value to predict PFS (high-risk CA medianPFS: not reach vs 19 months, P=0.006; ineligible for ASCT medianPFS: not reach vs 25 months, P=0.052 respectively) . ⑥Last, treatment with the bortezomib-based regimens contributed to prolonged MRD(-) duration (median MRD(-) duratio: 25 months vs 10 months, P=0.034) . Conclusion: Our findings supported MRD(+) status as an independent poor prognostic factor in MM patients, which implicated that duration of MRD(-) status also played a significant role in evaluation of prognosis, while loss of MRD(-)status might serve as an early biomarker for relapse. Therefore, monitoring of MRD kinetics might more precisely predict prognosis, as well as guide treatment decision, especially for when to start retreatment in relapsed patients.
Bortezomib/therapeutic use* ; Humans ; Multiple Myeloma/therapy* ; Neoplasm Recurrence, Local ; Neoplasm, Residual/diagnosis* ; Prognosis ; Risk Assessment ; Treatment Outcome

Background:Clinical outcomes of undifferentiated arthritis (UA) are diverse,and only 40 ％ of patients with UA develop rheumatoid arthritis (RA) after 3 years.Discovering predictive markers at disease onset for further intervention is critical.Therefore,our objective was to analyze the clinical outcomes of UA and ascertain the predictors for RA development.Methods:We performed a prospective,multi-center study from January 2013 to October 2016 among Chinese patients diagnosed with UA in 22 tertiary-care hospitals.Clinical and serological parameters were obtained at recruitment.Follow-up was undertaken in all patients every 12 weeks for 2 years.Predictive factors of disease progression were identified using multivariate Cox proportional hazards regression.Results:A total of 234 patients were recruited in this study,and 17 (7.3％) patients failed to follow up during the study.Among the 217 patients who completed the study,83 (38.2％) patients went into remission.UA patients who developed RA had a higher rheumatoid factor (RF)-positivity (42.9％ vs.16.8％,x2=8.228,P=0.008),anti-cyclic citrullinated peptide (CCP) antibodypositivity (66.7％ vs.10.7％,x2 =43.897,P ＜ 0.001),and double-positivity rate of RF and anti-CCP antibody (38.1％ vs.4.1％,x2 =32.131,P ＜ 0.001) than those who did not.Anti-CCP antibody but not RF was an independent predictor for RA development (hazard ratio 18.017,95％ confidence interval:5.803-55.938;P ＜ 0.001).Conclusion:As an independent predictor of RA,anti-CCP antibody should be tested at disease onset in all patients with UA.

Objective To study on the effects of Cinnamic aldehyde on leukemia cell line K562 by Caco -2 cells in vitro absorption model.Methods The effective components of cinnamon(0,50,100,200,400, 600,800,1000 μg· mL-1 ) were determined by Caco-2 cell model of Transwell, and the concentration was determined by HPLC.No cytotoxic concentration range of Cinnamic aldehyde acting on K562 cells for 72 h is detected by MTT assay.After 72 h incubation of Cinnamic aldehyde standard(50,75 μg· mL-1 ) and leukemia K562 cells, the cells surface antigens including CD235a, CD36, CD41, CD61, CD13, CD33 and CD14 were determined by Flow cytometry.Results The active ingredi-ent of cinnamon is extracted by transwell transport pool of Caco-2 cell model and no cytotoxic concentration is 200 μg · mL-1.The cinnamicaldehyde is the component which goes through the model by HPLC.The 24 h inhibition rates ( IRs ) of Cinnamic aldehyde on K562 cells are (25.29 ±0.97)%and (36.60 ±0.18)%at the concentrations of 50 and 75 μg· mL-1 , respectively;IRs for 48 h are ( 48.23 ±0.63 )% and ( 57.15 ±0.58 )%; IRs for 72 h are ( 58.23 ±0.63 )% and (57.15 ±0.58)%.Compared with the control group, the inhibitory activity is obvious(P<0.05).After incubation 72 h, the expressions of myeloid differentiation phenotypes including CD13, CD33, CD36 on K562 cells are (0.33 ±0.21)%, ( 32.89 ±0.19 )%, ( 7.73 ±0.57 )% and ( 0.72 ±0.43 )%, ( 38.80 ±0.03 )%, (10.90 ±0.82)%at the concentrations of 50 and 75 μg· mL-1 , respectively.Compared with the control group, the inhibition increased ( P <0.05 ).The phenotypic expressions of erythroid differentiation are ( 52.38 ±0.65 )%, (57.48 ±0.70)%.Compared with the control group, the inhibition increased( P<0.05).Megakaryocyte differentia-ted phenotype CD41, CD61 expression has no significant change ( P >0.05 ).Conclusion The Cinnamic aldehyde can go through the Caco-2 in vitro absorption model and enables the K562 cells to differentiate into myeloid and erythroid.

OBJECTIVETo investigate the early diagnosis and treatment for trauma around the knee with popliteal vascular injury.

METHODSA retrospective analysis was employed to analyze the clinical data from 15 patients (9 males and 6 females were with a mean age of 39.2 years old,ranging from 26 to 62 years old) with fracture or dislocation around the knee with popliteal vascular injury from January 2007 to January 2013. Combined with clinical symptoms and signs, oxygen saturation monitors, color ultrasound, DSA angiography and interventional surgery were used to determine the vascular injury. The knee fracture and dislocation were fixed with hybrid external fixation and plate-screw fixation, respectively. Then, the blood circulation was reconstructed by thrombectomy, repair and autologous vein graft for individual injured vascular. The average total operation time, average hospitalization days, predictive salvage index (PSI), average blood transfusion amount, average medical expenses and infection cases were recorded to determine the effect of early diagnosis and treatment.

RESULTSThere was one patient with death, 8 patients with amputation, and 6 patients with successful repair surgery for popliteal artery, anterior tibial and posterior tibial arteries. These six patients with surviving limbs were followed up for an average of 28.3 months (ranged, 12 to 60 months). Among the 6 successful patients, the joint function of 4 patients was good and excellent.

CONCLUSIONThe trauma around the knee with popliteal vascular injury is characterized by complex and serious injury, easy misdiagnosis and loss diagnosis, poor prognosis and high risk of amputation. The early diagnosis of trauma around the knee with popliteal vascular injury should depend on the mechanism of trauma, local anatomical characteristics of injury site, clinical presentations and appropriate auxiliary examinations. The appropriate indications for limb salvage and amputation should be used to achieve more effective clinical results.

Adult ; Early Diagnosis ; Female ; Humans ; Knee Injuries ; diagnosis ; surgery ; Male ; Middle Aged ; Popliteal Artery ; injuries ; surgery ; Retrospective Studies

Objective To learn the prevalence of overweight and obesity among city residents in Wenzhou city.Methods Byusing multistage stratified cluster sampling method,a total of 10 449 residents aged 18 years and above were selected forquestionnaires interview,physical examination and laboratory testing ,analyzed the prevalence and risk factors of overweightand obesity among city residents.Results The crude prevalence rates of overweight and obesity were 32.60% and7.70%,and the age -adjusted rate were 27.50% and 6.51%.The multivariate logistic regression analysis showed that age(OR =1.01,95%CI:1.01 -1.02)and drinking (OR =1.14,95%CI:1.02 -1.27)were risk factors of overweight andobesity,while high degree of education(OR =0.87,95%CI:0.84 -0.91)and active physical exercise (OR =0.75,95%CI:0.57 -0.98)were protective factors of overweight and obesity.The prevalence rates of hypertension,diabetes,dyslipidemia and hyperuricemia were much higher among adults with overweight and obesity,compared with that of normalweight adults (P <0.01).Conclusion Prevalence of overweight and obesity is high in Wenzhou City.Intervention shouldbe actively carried out for the prevention and control of overweight and obesity.

OBJECTIVETo explore effects of Shenqi preparation,Traditional Chinese Medicine, on anti-fatigue and anti-oxidant functions.

METHODSOne hundred and twenty mice were randomly divided into control group and 3 experimental groups. The high, medium and low-dose of Shenqi preparation were given to the 3 experimental groups respectively, while distilled water to the control group for 15 d. The loaded swimming time, the level of lactate, serum urea nitrogen (SUN), muscle and liver glycogen, liver super-oxide dismutase (SOD), the content of malondialdehyde (MDA), glutathione peroxidase(GSH-Px) were assayed.

RESULTSThe loaded swimming test showed that the exhausted swimming time of 3 experimental groups [(296.0 +/- 25.3)s, (437.0 ĝ 38.9)s, (595.0 +/- 53.9)s respectively] was longer than that of control group [(231.0 +/- 22.5)s, P < 0.05, P < 0.01]. The liver glycogen content of the high and medium-dose experimental groups were higher than that of control group respectively (P < 0.01). The SUN content of each experimental group was less than that of the control group (P < 0.01, P < 0.05). Moreover,in the medium and high dose experimental groups, less accumulation of lactate was found (P < 0.01, P < 0.05), and the content of liver SOD and GSH-Px was higher (P < 0.01, P < 0.05). The content of liver MDA in high-dose experimental group was less than that of the control group (P < 0.05).

CONCLUSIONShenqi preparation, especially the high and medium-dose experimental groups, is able to improve exercise tolerance and has anti-fatigue and anti-oxidant effects in mice.

Animals ; Antioxidants ; metabolism ; Blood Urea Nitrogen ; Disease Models, Animal ; Drugs, Chinese Herbal ; pharmacology ; Fatigue ; drug therapy ; metabolism ; Glutathione Peroxidase ; metabolism ; Glycogen ; metabolism ; Lactic Acid ; blood ; Liver ; drug effects ; metabolism ; Male ; Malondialdehyde ; metabolism ; Mice ; Physical Conditioning, Animal ; Superoxide Dismutase ; metabolism

OBJECTIVETo explore optimal choice of surgical treatment and operative approach for closed complex tibial plateau fractures and its influencing factors.

METHODSFrom January 2003 to January 2011, 95 patients with closed complex tibial plateau fractures were estimated Schatzker V and Vl, and treated with three different surgical methods. The methods included single plate through anterolateral incision (Group A, 22 cases), double plates through inside and outside incisions (Group B, 36 cases), and double plates through antero-midline incisions (Group C, 37 cases). There were 56 males and 39 females, ranged the age from 19 to 57 years (averaged, 36.3 years), 50 cases in left, 45 cases in right. According to Schatzker classification, 51 cases were type V, 44 cases were VI. The data of operation time, intraoperative blood loss, complications (infectious of wound, necrosis, bad incision, collapse fracture, loosen of internal fixation, fracture failure)and recovery of function of lower limb joint were collected.

RESULTSThere were no significant difference among three groups in operation time (P > 0.05); blood loss in group A was obvious better than other groups (P < 0.05); collapse of joint surface and failure rate of internal fixation in group A was higher than other groups (P > 0.05); Merchant score after 1 year were higher in group B, C than group A. For lower limb function, 10 cases got excellent results, 5 good, 4 fair and 3 poor in group A; 21 cases got excellent results, 11 good, 3 fair and 1 poor in group B; 23 cases got excellent results, 11 good,2 fair and 1 poor in group C.

CONCLUSIONThe blood loss in group A was least, but fracture exposure and joint surface was not satisfactory, and stable fixation could not be achieved, the long-term result was not good. For fractures with double condyles and dislocated involved, double plates through inside and outside incisions or double plates through antero-midline incisions was suggested,which benefit good reduction of joint surface, stable fixation, and erlier exercise.

Adult ; Bone Plates ; Case-Control Studies ; Female ; Fracture Fixation ; adverse effects ; methods ; Fractures, Closed ; surgery ; Humans ; Male ; Middle Aged ; Tibial Fractures ; surgery

OBJECTIVETo observe the therapeutic effects of Qinggan Huatan Huoxue Recipe (QHHR) on patients with non-alcoholic steatohepatitis (NASH).

METHODSOne hundred and fifty NASH patients were randomly assigned to the treatment group (78 cases) and the control group (72 cases). QHHR was given to patients in the treatment groups, while Danning Tablet was given to those in the control group. The therapeutic course for all was three months. Before and after treatment changes of clinical symptoms and physical signs, liver imageology, liver functions, blood lipids, and insulin resistance index (IRI) were observed.

RESULTSCompared with before treatment, obvious improvement of clinical symptoms, weight, body mass index (BMI), liver functions, blood lipids, and integral of liver ultrasound B was obtained in the two groups (P < 0.05). The IRI of the treatment group was significantly reduced after treatment (P < 0.05). Better effects were obtained in lowering the body weight, BMI, alanine aminotransferase (ALT), gamma glutamyltransferase (gamma-GT), triglyceride (TG), total cholesterol (TC), integral of liver ultrasound B, and the total effective rate (P < 0.05).

CONCLUSIONSQHHR had definite effects on NASH. Its therapeutic effects were better than Danning Tablet.

Adolescent ; Adult ; Aged ; Drugs, Chinese Herbal ; therapeutic use ; Fatty Liver ; drug therapy ; Female ; Humans ; Male ; Middle Aged ; Non-alcoholic Fatty Liver Disease ; Phytotherapy ; Young Adult