Introduction Haemophilia A (HA) is a rare X chromosome‐linked bleeding disorder resulting in missing or defective clotting factor VIII (FVIII) and causes large disease burden. Aim As a member of World Federation of Hemophilia, China seeks to understand the current epidemiology, disease profile and treatment landscape of patients with HA through the Hemophilia Treatment Center Collaboration Network of China (HTCCNC). Methods The HTCCNC enabled data collection on patients with HA from 166 member hospitals (2007–2019) across China. The distribution of patients across 31 divisions was summarized using a heat map. Patient demographics, disease severity and clinical and treatment information were summarized using descriptive statistics. Results HTCCNC identified 17,779 patients with HA during 2007–2019. Patients were predominantly male (99.99%), and 28.3% had a known family history of haemophilia. Among patients with lab‐measured disease severity (N = 13,116), 6,519 had severe HA (49.7%), 4,788 had moderate HA (36.5%), and 1,809 had mild HA (13.8%). Among patients with information on the delays, delays in diagnosis and in treatment initiation were observed in 1,437 (28.8%) and 1,750 (39.2%) patients, respectively. On average, those patients had an 8.4 years gap between the first bleed and HA diagnosis and a delay of 8.6 years from the first bleed to treatment initiation. Additionally, 44.33% of patients relied solely on episodic treatments, and 16.2% received any prophylaxis treatments. Conclusions Using data from the largest haemophilia registry in China, this study indicated that delayed diagnosis and treatment, together with low utilization of prophylaxis, are key challenges for patients with HA.
ObjectiveTo identify the right and left difference of the facial soft tissue landmarks three-dimensionally from the subjects of normal occlusion individuals.Materials and MethodsCone-beam computed tomography (CT) scans were obtained in 48 normal occlusion adults (24 men, 24 women), and reconstructed into 3-dimensional (3D) models by using a 3D image soft ware. 3D position of 27 soft tissue landmarks, 9 midline and 9 pairs of bilateral landmarks, were identified in 3D coordination system, and their right and left differences were calculated and analyzed.ResultsThe right and left difference values derived from the study ranged from 0.6 to 4.6 mm indicating a high variability according to the landmarks. In general, the values showed a tendency to increase according to the lower and lateral positioning of the landmarks in the face. Overall differences were determined not only by transverse differences but also by sagittal and vertical differences, indicating that 3D evaluation would be essential in the facial soft tissue analysis.ConclusionsMeans and standard deviations of the right and left difference of facial soft tissue landmarks derived from this study can be used as the diagnostic standard values for the evaluation of facial asymmetry.
In order to improve anti-interfere ability and increasing stability and reliability of automatically-controlled door Control System used in high speed Train. In this paper, we analysis the reason of electromagnetic interference and electromagnetic radiation on the system. the restrained technology of t he hardware interference was studied from the ranges of circuit board design , GND design , insulated technology and power interference rest rained aspects , and the software anti-interference mechanism was al so studied from redundancy. Based on this , the design of electromagnetic compatibility in automatically-controlled door Control System used in high speed Train was accomplished.Through actual vehicle experiments , the system design was verified , and the result showed that the system design is accuracy , reliable and creditable.
The electric cable is one of the most important materials in the high speed train, accurate service life directly affects the safety of the operation of the motor car group and the operating cost of the vehicle. Due to the importance of cable life assessment, based on Arrhenius model, this paper evaluates the service life of the cable. First, the mathematical model of Arrehenius is deduced, at the same time in order to solve the problem to facilitate the corresponding simplification, the parameters of the model were obtained by the least square method. Finally, the feasibility of the method is verified by the accelerated thermal aging test of the cable.
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