Objective To establish the national standard materials for haemiglobincyanide (HiCN) for the traceability assays of hemoglobin. Methods HiCN national standard materials were established according to the document of International Committee for Standardization in Haematology (ICSH). The standard materials were certificated according to ISO Guide 35, including homogeneity and stability. Then they were characterized by the calibrated spectrophotometer which can be traceable to National Institute of Standards and Technology (NIST). The international reference materials of HiCN were compared with the result of the WHO reference laboratory to confirm the reliability. Results The uncertainty of the HiCN standard materials was 0.000 4 g/L and the variation coefficient (CV) was 0.09%. The uncertainty of long-term stability was 0.000 6 g/L; the certificated value of the standard materials was 0.615 9 g/L with uncertainty of 0.000 4 g/L. The combined uncertainty was 0.000 9 g/L and the expanded uncertainty was 0.001 8 g/L when the cover factor was 2. The relative error was 0.08% between the result of the standard materials and the international certificated value. Conclusion The homogeneity and stability of the standard material is acceptable and the method of characterization is accurate and reliable.

Objective To evaluate the accuracy and comparability of secondary hematology analyzer for platelet enumeration in order to determine the accuracy and reliability of assigned value of fresh blood.Methods The results between secondary standard hematology analyzer and the reference method of platelet enumeration of 40 specimens were compared according to the document from CLSI EP9-A2.The correlation and bias were calculated.At the same time,the results of secondary standard hematology analyzer between our laboratory and Japan reference laboratory were compared.The fresh blood from normal people was prepared to be used as calibrator after assigned value by secondary standard hematology analyzer.And 36 hematology analyzers were performed correctness validation and calibrated by 36 fresh bloods.Results The results of 40 specimens by secondary standard hematology analyzer and the reference method were ( 108 -326) × 109/L and( 110 -327 ) × 109/L respectively.Correlation coefficient between the secondary standard hematology analyzer and the reference method was 0.993.The bias between two methods was from -3.8％to 3.4％.The results of NCCL and Japan reference laboratory from 2009 to 2010 were( 185 -203) × 109/L and (185 - 198) × 109/L The bias range between our laboratory and reference laboratory in Japan was from - 1.4％ to 3.7％.The ranges of coefficient variations of two laboratories were from 2.0％ to 3.0％ and from 2.6％ to 3.4％,respectively.The biases of 20 hematology analyzers were from - 2.6％ to 2.1％ and they passed the correctness validation.The biases of 16 hematology analyzers were decreased from 3.4％ - 12.6％of pre-calibration to 0％ - 2.8％ of post-calibration.Conclusions The results of secondary standard hematology analyzer are assured to be accurate and comparable by the comparison of reference laboratories.It is feasible that fresh blood assigned value by secondary standard hematology analyzer can be used as calibrator for the hematology analyzer.

Objective To prepare and evaluate the reference materials for plasma von Willebrand Factor antigen testing with fresh frozen plasma.Methods The candidates were prepared by low temperature centrifugation in 5 different concentration levels.The homogeneity and stability of the preparation was evaluated according to the ISO Guide35 and CNAS-GL03.The comparability between STAGO and IL system was evaluated according to the WS/T 356-2011.Then the preparations were characterized by six laboratories with the Secondary Coagulation Standard established by NIBSC(SSCLOT4).Results Homogeneity evaluation of the preparation showed that there was no statistically significant difference between the groups (P >0.05),the F values of factor analysis of variance were 0.317~0.844,the uncertainty range was 1.01% ~2.06%.A linear regression based on stability evaluation indicated that the linear trend (within 24 weeks)was insignificant (P >0.05). The uncertainty range of long-term (within 24 weeks)stability was 0.79% ~ 1.20%.The results of the preparations on STAGO and IL system were comparable.The certificated values of the candidates were range from 12.2% to 138.9% with uncertainties were 0.06%~0.09%,respectively.The range of combined standard uncertainty was 0.03% ~ 0.16% while the expanded uncertainty was 2.2%~6.7%.Conclusion The reference materials for von Willebrand Factor antigen testing were stable and homogenous with comparability between STAGO and IL.The method of characterization was accurate and reliable.

Objective To evaluate the effect of TEG ( thromboelastography ) in the timing choose of CABG( coronary artery bypass grafting ) surgery for patients that receiving DAPT treatment .Methods This study is prospective analysis from November 2013 to May 2014 in Wuhan Asia Heart Hospital.The study cohort consisted of 1 201 patientswith acute coronary syndrome , theage range from 43 to 79 years (61.1 ±10 years) with 134 males and 79 females.and the patients undergo DAPT ( aspirin 100 mg/d, clopidogrel 75 mg/d) which were prepare to the coronary artery bypass grafting and then randomly divided into two groups according to the ratio of 1∶1.There were 101 patients, using perioperative TEG inspection , which conduct the choose of surgery timing according to the ADP-induced platelet-fibrin clot strength ( MAADP) after 24 h of the withdraw of DAPT.Distribute the patients into≥50 mm, 35-50 mm and <35 mm groups according to the MAADPresults, and theirCABG was complete within 1 day, 3-5 days and after 5 days respectively. The other 100 patientsacceptancoronary artery bypass grafting surgery and DAPT patients but without TEG examination as the control group , which were withdrawal the DAPT for 5 -7 days by routine before operation.The primary endpoint is 24 h chest tube drainage andkey secondary endpoint is the total number of transfused red blood cells .Recorded the mean intubation time , ICU stay times , Median length of hospital stayandrethoracotomyrates, 30-day mortality, and 30-day readmission rate at the same time.Using Fisher in the variables , t-test when two groups were compared , use ANCOVA for variable factor correction and Statistical analysis was performed using chi-square test in the calculate of results .Results The average chest drainage in TEG group and the control was 438.8 ml vs 487.8 ml(t=1.063,P=0.289),and the total amount of transfused red blood cell in both groups were 493.8 ml vs 551.6 ml(t=1.228,P=0.2227), there were no significant difference between the two groups .The total waiting time in the three categories of patients in TEG group is 294 days ( an average waiting time of 3.11 days per patient ) and was reduced 38%(3.1 days to 5 days)than the guidelines recommend.Conclusions Although the perioperative bleeding and red blood cell transfusion , the occurrence and postoperative adverse events were similar in two groups , but using thrombelastographto measure platelet function before CABG surgery can help to choose the timing of surgery and reduce the waiting time before surgery .

Objective: This study is to investigate the effectiveness of philtrum repairment for-microform cleft lip, using muscle tension line group reconstruction. Methods: This is a retrospective study of 19 consecutively primary microform cleft lip repairment patients, from January 2015 to December 2017. All the patients were provided muscle tension line reconstruction (group 1) or traditional muscle repairment by horizontal mattress sutures (group 2). The philtral ridge and dimple were evaluated by scores. The statistical analyses were performed using unpaired t-test by Prism 6.0 software. Results: The philtral ridge score was higher in group 1 than group 2 (4.15±0.56 vs. 2.73±0.32, P<0.01). The philtral dimple score of group 1 increased either, when compared with group 2 (2.36±0.50 vs.1.45±0.28, P=0.0004). Conclusions Functional reconstruction of philtrum, using muscle tension line group repair, has satisfactory results in philtral ridge and philtral dimple.

Objective: To explore the effect of managing syndromic craniosynostosis using posterior vault distraction osteogenesis. Methods: The authors conducted a retrospective cohort study of four children with syndromic craniosynostosis treated between 2015 January to 2016 March using posterior vault distraction osteogenesis. The posterior craniotomy was performed from vertex, biparietally to a point above the occipital protuberance. Two distraction devices were fixed in the parasagittal, collinear position. After a latency of 3 days, the device was activated at 0.5 mm/day. After the distraction, the consolidation period was about 6 months. Results: The average distraction distance was 27.3 mm(range, 25 to 30 mm). Cerebrospinal fluid leak happened in one patient. After taken the 3D CT scan, all of them were undertaken the second operation of removing the distraction devices. All the patients were followed up at a mean of 12.8 months (range, 7 to 20 months). Conclusions It is effective to enlarge the posterior cranial vault using distraction osteogenesis for the syndromic craniosynostosis.

Objective: In clinical, cephalic index is the most common method to evaluate scaphocephaly. However, cephalic index alone is not sufficient to describe the nuances of skull shape. The authors hypothesize that the location of euryon could be a valuable and practical index as the supplement of cephalic index to better describe the skull. Methods: This is a retrospective study. CT scans of 17 scaphocephaly patients and 17 normal controls were included. Eight of 17 scaphocephaly patients have 6 months postoperative CT scan. The preoperative location of euryon of scaphocephaly patients was compared to that of the controls and postoperative. The horizontal location of euryon was defined as the ratio of the distance, from the most prominent point of forehead to euryon, to anteroposterior cranial length. The vertical location of euryon was defined as the ratio of the vertical distance, from the nasion to euryon, to cranial height. Results: Both the horizontal and vertical locations of euryon of preoperative scaphocephaly patients were significantly decreased compared to normals (46.50%±4.02% vs. 50.39%±2.21%, t=3.49, P=0.0014; 35.78%±8.62% vs. 52.6%±8.08%, t=5.86, P<0.001). Both the postoperative horizontal vertical locations of euryon were significantly increased, when compared to preoperative (47.25%±4.23% vs. 44.02%±3.72%, t=2.42, P=0.045; 42.39%±9.06% vs. 35.18%±5.36%, t=2.554, P=0.0379). Conclusions The location of euryon could be a practical and valuable index to describe the nuances of skull shape.

Objective: The purpose of this study was to measure intracranial volumes in normal children of different ages and to explore the growth of intracranial volumes. Methods: A total of 151 normal children (male, 96; female, 55) were included in this study who were subjected to computed tomography (CT) for head trauma in the pediatric emergency room from June 1, 2015 to July 31, 2015. All of them had no congenital deformities and depressed fracture of the skull. Total, anterior, middle and posterior intracranial volumes were measured using a DICOM viewer. The Graphad Prism 6.0 was applied to analyze the data and plot the best fit logarithmic growth curve and liner regression and the comparison of the growth curves between different groups was done. Results: The intracranial volumes had a rapid growth within 2 years old. The girls′ total intracranial volumes were smaller than the boys′ of the same age. There was a significant difference of linear regression slope between anterior intracranial volumes and posterior intracranial volumes within 2 years old (P<0.01). Conclusions There is a rapid growth of intracranial volumes of normal children within 2 years old. The posterior intracranial volumes are expanded faster than the anterior intracranial volumes in the first two years. The girls′ intracranial volumes are smaller than the boys′.

Objective:To summarize the surgery effect of tongue volume reduction for macroglossia patients with Beckwith-Wiedemann syndrome (BWS).Methods:The clinical data of children diagnosed as BWS based on clinic symptoms and gene tests, who received operation in Department of Plastic Surgery, Children’s Hospital of Fudan University from July 2012 to December 2019 were analyzed retrospectively. All the patients underwent dorsal tongue bell-shaped resection and ventral V-shaped resection. By pulling tongue tip, the tongue body was cut and dissected along the marking line followed by suturing the muscle layer of the central part of the tongue tissue. Intermittent mattress suture of the dorsal and ventral mucous wounds of the tongue. After operation, all patients kept the trachea intubation and were transferred to PICU for further treatment and received early nasal feeding. The short-term postoperative complications, and long-term follow-up of the tongue body shape and clinical symptoms were recorded.The clinical effect was evaluated by onesurgeon and the parents.The effect was graded into four levels: dissatisifaction, average, satisifaction and great satisifaction.Results:A total of 61 children were included in this study, including 30 males and 31 females, with an average age of 8.5 months. The length of the tongue exposed outside at the resting state was 1.34-2.62 cm, with a median of 1.87 cm. Symmetric hypertrophy was found in 52 cases (85.2%), lateral hypertrophy in 9 cases (14.8%). There were 3 cases with respiratory obstruction (4.9%), 35 cases with salivation (57.4%), 4 cases suffering difficulty of feeding and dysphagia (6.6%). All children underwent the operation successfully, and the average operation time was 35.8 min. Among the short-term complications, there were 3 ventral wounds dehiscence, none of which needed sutured again. By prolonging nasal feeding time, the wounds were all healed in the secondary stage. Respiratory obstruction occurred in 1 patient after operation, and the symptoms were relieved after tongue retraction. The mean follow-up time was 3.3 years (2 months to 7 years), and 1 patient was inadequately corrected and good condition was got after second aryoperation. The remaining children obtained satisfactory resting tongue position and appearance, and the postoperative clinical symptoms were improved. In the long-term follow-up, the comprehensive evaluation results of the resting tongue position and clinical symptoms of the children by surgeon and parents were satisfied in 10 cases (16.4%), very satisfied in 50 cases (82.0%), and average in 1 case (insufficient correction after the first operation).Conclusions:Central resection and volume reduction of tongue body is a simple, effective and safe surgical technique in children with giant tongue diagnosed as BWS.

Objective:To investigate the effect of tranexamic acid on perioperative hemorrhage of cranioplasty for craniosynostosis.Methods:A retrospective analysis was performed on the clinical data of children who received cranial remodeling from the Children’s Hospital of Fudan University from February 2015 to October 2018. The patients were divided into two groups: tranexamic acid treatment group (TXA group) and no tranexamic acid treatment group (control group). The average intraoperative blood loss, the average amount of blood transfusion, postoperative average amount of blood transfusion, average drainage volume, and changes of postoperative hemoglobin and blood coagulation function were observed. The measurement data were described by M ( Q1, Q3) and analyzed with Mann-Whitney U test. Results:A total of 41 patients were included in this study. There were 26 patients in TXA group, 18 males and 8 females, aged 9.5 (7.0, 38.8) months; in the control group, there were 15 patients, 10 males and 5 females, aged 9.2 (6.6, 18.3) months. The results showed that there was no significant difference in preoperative hemoglobin level between the two groups ( P>0.05). The hemoglobin level of TXA group was higher than that of control group immediately after operation and 24 h and 72 h after operation ( P<0.05). Total intraoperative blood loss and blood loss per kilogram of body weight in TXA group were lower than those in control group [150.0 (57.5, 225.0) ml vs. 200.0(80.0, 300.0) ml, P<0.05; 13.6(6.8, 18.5) ml/kg vs. 15.8(7.0, 27.0) ml/kg, P<0.01]. The amount of intraoperative transfusion volume per kilogram of body weight in TXA group was less than that in control group [15.0(8.8, 17.7) ml/kg vs.17.1 (13.0, 23.3) ml/kg, P<0.05]. There were no significant differences between the two groups in postoperative total transfusion volume, transfusion volume per kilogram of body weight, total drainage volume and drainage volume per kilogram of body weight ( P>0.05). There was no significant difference in prothrombin time (PT), activated partial thromboplastin time (APTT) and fibrinogen between the two groups before and after surgery ( P<0.05). Conclusion:TXA can reduce the amount of blood loss and blood transfusion in craniosynostosis in children.