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 investigate the timing of pericardial drainage catheter removal and restart of the anticoagulation in patients with atrial fibrillation (AF) suffered from perioperative pericardial tamponade during atrial fibrillation catheter ablation and uninterrupted dabigatran. Methods: A total of 20 patients with pericardial tamponade, who underwent AF catheter ablation with uninterrupted dabigatran in Beijing Anzhen Hospital from January 2019 to August 2021, were included in this retrospective analysis. The clinical characteristics of enrolled patients, information of catheter ablation procedures, pericardial tamponade management, perioperative complications, the timing of pericardial drainage catheter removal and restart of anticoagulation were analyzed. Results: All patients underwent pericardiocentesis and pericardial effusion drainage was successful in all patients. The average drainage volume was (427.8±527.4) ml. Seven cases were treated with idarucizumab, of which 1 patient received surgical repair. The average timing of pericardial drainage catheter removal and restart of anticoagulation in 19 patients without surgical repair was (1.4±0.7) and (0.8±0.4) days, respectively. No new bleeding, embolism and death were reported during hospitalization and within 30 days following hospital discharge. Time of removal of pericardial drainage catheter, restart of anticoagulation and hospital stay were similar between patients treated with idarucizumab or not. Conclusion: It is safe and reasonable to remove pericardial drainage catheter and restart anticoagulation as soon as possible during catheter ablation of atrial fibrillation with uninterrupted dabigatran independent of the idarucizumab use or not in case of confirmed hemostasis.
Humans ; Atrial Fibrillation/drug therapy* ; Dabigatran/therapeutic use* ; Cardiac Tamponade/complications* ; Anticoagulants/therapeutic use* ; Retrospective Studies ; Treatment Outcome ; Drainage/adverse effects* ; Catheter Ablation ; Catheters/adverse effects*

We established an indirect ELISA method using Trichinella spiralis trehalase(TsTRE)protein expressed in prokaryotic cells.The TsTRE gene was amplified by RT-PCR and ligated into the pCold I plasmid,which was expressed in E.coli BL21 competent cells.The rTsTRE protein was purified through affinity column chromatography.The TsTRE protein was localized with immunofluorescence techniques,and the immunogenicity of rTsTRE was detected by westernblotting.Subse-quently,rTsTRE protein was used as a coating antigen to establish an indirect ELISA.We optimized the antigen-coating con-centration,serum dilution concentration,antigen-coating incubation time,type of blocking solution,blocking incubation time,HRP-labeled goat anti-rabbit IgG serum dilution concentration,HRP-labeled goat anti-rabbit IgG serum incubation time and response time of TMB.Subsequently,the critical value,repeatability,sensitivity,specificity and clinical detection rate of the ELISA were evaluated.Immunofluorescence indicated that trehalase was abundant in the rod-shaped body,tail and epidermis of Trichinella spiralis muscle larvae.Western-blot indicated that rTsTRE protein combined with the positive serum of mice infected with T.spiralis for 42 d;the band was approximately 60 kDa.The established indirect ELISA had a positive threshold of 0.384;the intra-run and inter-run coefficients of variation were 5.504％-7.630％ and 4.664％-9.929％,and did not exceed 10％.The lowest detectable titer was 1:1 280.No cross reaction was observed with antibodies to Clonorchissinensis,Schistosoma ja-ponicum,Ascaris suum,Toxocara gondii and Toxocara canis,and the clinical negative detection rate was 0％.Thus,we suc-cessfully expressed the rTsTRE protein.Moreover,the established indirect ELISA method using the TsTRE protein as the coating antigen had good repeatability,sensitivity,specificity and clinical detectability,and can be applied to the detection of clinical samples.

PURPOSE: The purpose of this study was to assess and compare elbow range of motion, triceps extension strength and functional results of type C (AO/OTA) distal humerus fractures treated with bilateral triceps tendon (BTT) approach and olecranon osteotomy (OO). At the same time, we are also trying to know whether BTT approach can provide sufficient vision for comminuted intra-articular fractures of the distal humerus, and whether it is convenient to convert to the treatment to total elbow arthroplasty (TEA) or OO. METHODS: Patients treated with OO and BTT approaches for type C distal humerus fractures between July 2014 and December 2017 were retrospectively reviewed. Inclusion criteria include: (1) patients' age were more than 18 years old, (2) follow-up was no less than 6 months, and (3) patients were diagnosed with type C fractures (based on the AO/OTA classification). Exclusion criteria include: (1) open fractures (Gustillo type 2 or type 3), (2) treated by other approaches, and (3) presented with combined injuries of ipsilateral upper extremities, such as ulnar nerve. Elbow range of motion and triceps extension strength testing were completely valuated, when the fractures had healed. Assessment of functional results using the Mayo elbow performance score and complications were conducted in final follow-up. The data were compared using the two tailed Student's t-test. All data were presented as mean ± standard deviation. RESULTS: Eighty-six patients of type C distal humerus fractures, treated by OO and BTT approach were retrospectively reviewed between July 2014 and December 2017. Fifty-five distal humerus fractures (23 males and 32 females, mean age 52.7 years) treated by BTT approach or OO were included in this study. There were 10 fractures of type C1, 16 type C2 and 29 type C3 according to the AO/OTA classification. Patients were divided into two surgical approach groups chosen by the operators: BTT group (28 patients) and OO group (27 patients). And the mean follow-up time of all patients was 15.6 months (range, 6-36 months). Three cases in BTT group were converted to TEA, and one converted to OO. Only one case in BTT group presented poor articular reduction with a step more than 2 mm. There were not significantly different in functional outcomes according to the Mayo elbow performance score, operation time and extension flexion motion are values between BTT group and OO group (p > 0.05). Complications and reoperation rate were also similar in the two groups. Triceps manual muscle testing were no significant difference in the two groups, even subdivided in elder patients (aged >60 years old). CONCLUSION BTT is a safe approach to achieve similar functional result comparing with OO. BTT were not suitable for every case with severe comminuted pattern, but it avoids the potential complications related to OO, and has no complications concerning with triceps tendon. It is convenient for open reduction internal fixation and flexible to be converted to OO, as well as available to be converted to TEA in elder patients.
Adolescent ; Aged ; Elbow Joint/injuries* ; Female ; Fracture Fixation, Internal/methods* ; Fractures, Comminuted ; Humans ; Humeral Fractures/surgery* ; Humerus ; Infant ; Male ; Middle Aged ; Range of Motion, Articular ; Retrospective Studies ; Tendons ; Treatment Outcome

OBJECTIVE: To explore clinical effects of ultrasound-guided minimal traverse-cross technique repair for acute closed Achilles tendon ruptures. METHODS: From January 2015 to March 2017, 20 patients with acute closed Achilles tendon rupture were treated by minimal traverse-cross technique repair with ultrasound guided. Among them, including 13 males and 7 females, aged from 28 to 49 years old with an average of(31.3 ±4.5) years old. All patients were single side injury. Fifteen patients on the left side and 5 patients were on the right side. The time from injury to operation ranged from 1 to 5 days with an average of (2.5±0.7) days. Operative time, postoperative complications were observed, and AOFAS score before and after operation at 12 months were compared. RESULTS: All patients were followed up for 12 to 27 months with an average of(15.2±4.9) months. Operative time ranged from 33 to 65 min with an average of(43.7±5.6) min. Incision of one patient were continued oozing and improved after changing dressings, other patients were healed at stage I. No sural nerve irritation symptoms and palindromic rapture of heel tendon occurred. AOFAS score was improved from 65.2±7.4 before operation to 97.7±4.7 after operation at 12 months (t=22.5, <0.01); 18 patients got excellent results and 2 good. CONCLUSIONS Ultrasound-guided minimal traverse-cross technique repair for acute closed Achilles tendon ruptures, which promise minimal incision, protect sural nerve, ensure quality of tendon anastomosis and fixation, and is a ideal method for repairing acute closed Achilles tendon ruptures.
Achilles Tendon ; Adult ; Female ; Humans ; Male ; Middle Aged ; Rupture ; Sural Nerve ; Sutures ; Tendon Injuries ; Treatment Outcome

OBJECTIVE: To establish 293T cell lines stably expressing Calpain-cleavage related α3 cytoplasmic tail mutants, and to explore the effect of amino acid motifs in integrin β3 cytoplasmic tail on αⅡbβ3-mediated cell function. METHODS: 293T cell lines stably co-expressing human wild type integrin αⅡb and full length β3 or mutant β3, including β3-ΔNITY (β3 cytoplasmic tail NITY motif deleted), β3-Δ754 (β3 cytoplasmic tail TNITYRGT motif deleted) and β3-Δ759 (β3 cytoplasmic tail RGT motif deleted) were established. Spreading and adhesion of these stable cell lines on immobilized fibrinogen were tested. RESULTS: 293T-αⅡbβ3ΔNITY, 293T-αⅡbβ3Δ754, 293T-αⅡbβ3Δ759 and 293T-αⅡbβ3 cell lines were successfully established. Compared with the 293T cells, 293T-αⅡbβ3 cells which expressed full β3, possessed well adhesion and spread ability on immobilized fibrinogen, suggesting it can be as a surrogate for platelet. Compared with 293T-αⅡbβ3 cells, the 293T-αⅡbβ3ΔNITY cells showed a partial impairment of adhesion and spreadability on immobilized fibrinogen. while the 293T-αⅡbβ3Δ754 cells and 293T-αⅡbβ3Δ759 cells failed to adhere or spread on immobilized fibrinogen. CONCLUSION To the cell spreading function mediated by integrin β3, RGT motif is vital, while NITY can be dispensable. These established 293T cell lines stably expressing different β3 mutants provide a solid basis for a further analysis of mass spectrometry.
Amino Acid Motifs ; Animals ; CHO Cells ; Cell Adhesion ; Cricetinae ; Cricetulus ; HEK293 Cells ; Humans ; Integrin beta3 ; genetics ; metabolism ; Platelet Glycoprotein GPIIb-IIIa Complex ; genetics ; metabolism ; Signal Transduction

Catheter ablation has been recommended as a treatment option for paroxysmal atrial fibrillation (PAF) patients complicated with type 2 diabetes mellitus (T2DM). PAF patients with T2DM have a higher recurrence rate after catheter ablation. Prolongation of corrected QT (QTc) interval has been linked to poor outcomes in T2DM patients. Whether the abnormal QTc interval is associated with the ablation outcome in the PAF patients with T2DM remains unknown. In this study, 134 PAF patients with T2DM undergoing primary catheter ablation were retrospectively enrolled. Pre-procedural QTc interval was corrected by using the Bazett's formula. Cox proportional hazards models were constructed to assess the relationship between QTc interval and the recurrence of AF. After a 29.1-month follow-up period, 61 patients experienced atrial tachyarrhythmia recurrence. Recurrent patients had a longer QTc interval than non-recurrent patients (425.2±21.5 ms vs. 414.1±13.4 ms, P=0.002). Multivariate Cox regression analysis revealed that QTc interval [hazard ratio (HR)=1.026, 95% confidence interval (CI) 1.012-1.040, P=0.005] and left atrial diameter (LAD) (HR=1.125, 95% CI 1.062-1.192, P=0.003) were independent predictors of recurrent atrial tachyarrhythmia. Receiver operating characteristic analysis demonstrated that the cut-off value of QTc (418 ms) predicted arrhythmia recurrence with a sensitivity of 55.7% and a specificity of 69.9%. A combination of LAD and QTc was more effective than LAD alone (P<0.001) in predicting arrhythmia recurrence after the procedure. QTc interval could be used as an independent predictor of arrhythmia recurrence in T2DM patients undergoing AF ablation, thus providing a simple method to identify those patients who likely have a better outcome following the procedure.
Aged ; Atrial Fibrillation ; complications ; physiopathology ; surgery ; Catheter Ablation ; adverse effects ; Diabetes Mellitus, Type 2 ; complications ; physiopathology ; surgery ; Electrocardiography ; Female ; Heart Atria ; physiopathology ; Humans ; Male ; Middle Aged ; Proportional Hazards Models ; Risk Factors ; Tachycardia ; physiopathology ; Treatment Outcome

OBJECTIVETo evaluate the clinical efficacy of mini-open repair for the treatment of acute closed achilles tendon ruptures.

METHODSFrom April 2012 and October 2013,14 patients (14 feet) with acute closed achilles tendon ruptures were treated in our department. They were 9 males and 5 females, with an average age of 30.5 years old (ranged, 25 to 49 years old). The interval between injury and operation ranged from 1 to 13 days (8 days on average). A longitudinal incision approximately 1.5 to 2.0 cm in length was made around the ruptured achilles tendon for mini-open repair after insertion of oval clamp. Postoperative rehabilitation was carried out.

RESULTSThe wounds healed at the first stage except 2 cases with slow recovery. All the patients were followed up for 6 to 24 months, with an average of 11 months. According to the ankle-hindfoot scoring system of American Orthopaedic Foot & Ankle Society (AOFAS),the score was 92.71 ± 6.58 (82 to 100).

CONCLUSIONThe surgical treatment of acute achilles tendon rupture with mini-open repair has advantages of little invasion, a low rate of incision problems, quick function recovery, and simple operation, and it is suitable for primary hospital.

Achilles Tendon ; injuries ; physiopathology ; surgery ; Adult ; Female ; Humans ; Male ; Middle Aged ; Minimally Invasive Surgical Procedures ; methods ; Tendon Injuries ; physiopathology ; surgery ; Treatment Outcome ; Wound Healing

OBJECTIVETo investigate the association between physical activity (PA) and the incidence of metabolic syndrome (MS) in Chinese adults.

METHODSData on PA and other variables were obtained at the baseline examination of China Multi-center study of Cardiovascular Epidemiology in 1998 and of International Collaborative study of Cardiovascular Disease in Asia(InterASIA) during 2000 - 2001. Follow-up study was conducted in 2007 - 2008. A total of 11 512 Chinese adults aged 35 - 74 years (5563 men and 5949 women) were included in the final data analysis. Information on demographics, PA, smoking and alcohol consumption were obtained and components of MS were examined. Participants were divided into four groups according to quartile of total metabolic equivalent (MET) values per day. In addition, subjects were grouped into the following categories according to occupational PA: inactive, light, moderate and vigorous. Binary logistic model was used to examine the association between PA and the incidence of MS.

RESULTSA total of 2527 cases with MS were documented during an average following up of 8.1 years. The annual incidence rate of MS was 2.71% (2527/93 178.68). After multivariate logistic regression analysis, compared with participants with total PA volume < 32.0 MET×h×d(-1) (annual incidence rate was 3.19% (697/21 830.74)), the RR (95%CI) value of participants with total PA volume during 32.00 - 37.85, 37.86 - 52.29, and ≥ 52.30 MET×h×d(-1) was 1.05(0.92 - 1.19), 0.98(0.86 - 1.12), and 0.68(0.59 - 0.80), respectively (χ(2)trend = 34.23, P < 0.05), with corresponding annual incidence rates of 2.82% (690/24 504.25), 2.73% (661/24 179.36) and 2.11% (479/22 664.33). In addition, compared to inactive occupational PA (annual incidence rate was 2.76% (402/14 588.33)), the corresponding RR (95%CI) value was 0.80 (0.69 - 0.92), 0.70 (0.59 - 0.82), and 0.54 (0.45 - 0.65) (χ(2)trend = 42.34, P < 0.05), and the annual incidence rates were 2.86% (648/22 663.41), 2.40% (455/18 956.14) and 1.89% (344/18 173.86) in participants with light, moderate and vigorous occupational PA, respectively.

CONCLUSIONBoth increased total PA volume and occupational PA intensity are significantly associated with decreased risk of incidence of MS.

Adult ; Aged ; China ; epidemiology ; Exercise ; Female ; Humans ; Incidence ; Male ; Metabolic Syndrome ; epidemiology ; Middle Aged ; Prospective Studies ; Risk Factors

Objective To determine whether the novel surface-anchored protein SasX promotes aggregation of S.aureus and adherence of S.aureus to human nasal epithelial cells.Methods MRSA ST-239 HS770 sasX gene mutant ( HS770 △sasX) and complement [ HS770 △sasX (pRBsasX) ] were gotten by gene knock-out and complement methods.The aggregation ability of S.aureus was observed through microscope.By adherence assay which was used for detection of the adherence ability of wild type and mutant to human nasal epithelial cells and blocking experiments which detected the ability of the purified recombinant SasX protein in blocking the adherence of S.aureus to human nasal epithelial cells,we investigated the influence SasX on colonization of S.aureus.Results Compared to wild type,HS770△sasX showed a reduction in cell aggregation,while the complement had no difference with wild type in aggregation. HS770 △sasX showed a significant reduction of adherence to human nasal epithelial cells compared to wild type ( P＜0.01 ),and the complement showed a very clear increasement of adherence to human nasal epithelial cells compared to wild type(P＜0.01 ).Preincubation of nasal epithelial cells with the purified recombinant SasX protein inhibited S.aureus binding significantly.Conclusion SasX had an influence in aggregation of S.aureus and its adherence to human nasal epithelial cells.By acquiring sasX,S.aureus colonized more easily to the susceptible sites of the host,and thus caused infection.