Thromboembolic disease seriously affects people's health, and even endangers life. Nattokinase(NK) is an alkaline serine protease with strong thrombolytic activity and low toxicity. However, when NK passes through the stomach, it is degraded by gastric acid and pepsin, and subsequently loses its thrombolytic activity. The application of preparation technology can form a protective layer and improve the high bioavailability of NK. This article briefly introduced the pharmacological properties of NK, and discussed the characteristic of different dosage forms. The development of NK preparation was prospected in order to promote its research and application.

Objective:To summarize the clinical and genetic characteristics, treatment and prognosis of four children with Steroid-resistant nephrotic syndrome (SRNS) due to variants of TRPC6 gene. Methods:Clinical data of four children with SRNS admitted to Children′s Hospital Affiliated to Zhengzhou University between May 2020 and August 2022 were collected. Peripheral blood samples were collected from the children and their parents, and whole exome sequencing was carried out. Sanger sequencing was used to verify the pathogenicity of the candidate variants among the children and their parents.Results:All of the four children were found to harbor heterozygous variants of the TRPC6 gene, including c. 523C>T (p.R175W), c. 1327T>A (p.F443I), c. 430G>C (p.E144Q) (unreported previously), and c. 523C>T (p.R175W), which were all missense variants. Two of the children have shown a simple type, whilst two have shown a nephritis type, none had extrarenal phenotype. Comprehensive renal pathology of three children revealed focal segmental glomerulosclerosis (FSGS). Two children were treated with steroids combined with calcineurin inhibitors (CNIs), among whom one showed significant improvement in symptoms. Conclusion:Discoveries of the novel c. 430G>C variant and the new SRNS phenotype of the c. 1327T>A variant have expanded the mutational and phenotypic spectrum of the TRPC6 gene, which has provided a reference for clinical diagnosis and genetic counseling for the families.

Objective:To investigate the factors affecting the time taken for B cell reconstitution after rituximab (RTX) treatment in children with steroid-sensitive nephrotic syndrome.Methods:This was a retrospective cohort study. The clinical data of 42 children with SSNS who received treatment with RTX in Department of Nephrology, Rheumatology and Immunology, Children′s Hospital Affiliated to Zhengzhou University between December 2019 and May 2023 were analyzed retrospectively. The data of demographics, immunosuppressant treatment and laboratory tests such as CD19 +B cell count, urinary protein quantification were collected. The patients were divided into 2 groups, the early B cell reconstruction group and the late reconstruction group based on the average time of B cell reconstruction. A multivariate logistic regression model was used to analyze the factors impacting the timing of B cell reconstruction, and the predictive value of these factors was assessed by plotting the receiver operating characteristic (ROC) curve. Results:There were 42 children, with 35 males and 7 females. They were aged 3.5 (2.2, 5.9) years at the onset of PNS and (8.4±3.3) years at their first RTX treatment. The time for B cell reconstitution was (152±53) d. There were 20 children in the early reconstruction group and 22 children in the late reconstruction group. There were no statistically significant differences (all P>0.05) between the 2 groups in terms of the cumulative dose of steroids within 1 year before receiving RTX infusion (0.29 (0.16, 0.50) vs. 0.29 (0.19, 0.46) mg/(kg·d)), the percentage of children using tacrolimus before RTX (65%(13/20) vs. 45%(10/22)) and cumulative doses (0.04 (0.03, 0.05) vs. 0.03 (0.03, 0.06) mg/(kg·d)), the steroid doses at the time of RTX infusion (0.73 (0.49, 0.90) vs. 0.71 (0.58, 0.89) mg/(kg·d)), the percentage of children using tacrolimus at the initial RTX infusion (50% (10/20) vs. 41% (9/22)) and the doses (0.03 (0.02, 0.04) vs. 0.02 (0.01, 0.04) mg/(kg·d)), the discontinuation time of tacrolimus post-RTX infusion (71 (42, 91) vs. 64 (42, 91) d). A multivariate analysis revealed a correlation ( OR=0.26, 95% CI 0.10-0.68, P=0.006) between B cell count following the second RTX infusion and the time taken for B cell reconstruction. The area under the ROC curve for B cell count after the RTX infusion in predicting the time to B cell reconstruction was 0.89 (95% CI 0.78-0.99, P<0.001) and the cut-off value was 0.925×10 6/L. Conclusions:The time of B cell reconstruction is not influenced by the previous or concurrent use of tacrolimus, regardless of its duration and the dosage of steroid and tacrolimus prior to the RTX infusion. Insteadly, the peripheral blood B cell count (0.925×10 6/L) following the second RTX infusion for SSNS is identified as an independent predictor of reconstruction time, allowing for a more precise prediction and early intervention to maintain disease remission.

Objective:To determine the relative correction factor of pre-vitamin D and simplify the calculation method of vitamin D assay.Methods:By studying the calculation method of vitamin D content in drug standards of various countries,HPLC was used to determine the relative correction factor of pre-vitamin D,and the influencing factors of determination were investigated.Results:The relative correction factors of pre-vitamin D at 254 nm and 265nm wavelength were determined by statistical analysis of 7 laboratories in China.Conclusion:Using the pre-vi-tamin D relative correction factor method to calculate the total amount of vitamin D simplified the experimental steps can be simplified by the pre-vitamin D relative correction factor method to calculate the total amount of vitamin D and the random operating errors can be avoided.The method is rapid and accurate,and lay a solid foundation for further improving the standard of vitamin D preparations.

Objective:To compare the positioning errors in tracing the body surface markers between radiotherapy placement with or without using the laser positioning coordination system in post-breast conservative surgery patients, and to verify the clinical value of the laser positioning coordination system.Methods:A total of 45 post-breast-conservative surgery patients who underwent radiotherapy in Department of Radiation Oncology of the Affiliated Hospital of Inner Mongolia Medical University from January 2022 to September 2023 were prospectively collected. In the experimental group 1 ( n=15), the initial version of the laser positioning coordination system was employed to trace the body surface markers. In the experimental group 2 ( n=15), the upgraded version of the laser positioning coordination system was adopted to draw the body surface markers. In the control group ( n=15), the body surface markers were traced with conventional approach. All patients were treated with spiral tomotherapy (TOMO), and the error values in the left and right directions ( X), head and foot directions ( Y), ventral and dorsal directions ( Z), and rotation angles (ROLL) before each radiotherapy were recorded. The differences in the positioning errors among the three groups were analyzed by t-test. Results:The positioning errors in the X, Y, Z directions and ROLL in the experimental group 1 were (3.10±2.43) mm, (4.36±3.45) mm, (2.29±2.49) mm and 0.95°±0.88°, and (2.88±2.28) mm, (3.58±2.95) mm, (2.40±2.54) mm, and 0.70°±0.70° in the experimental group 2, and (4.32±3.48) mm, (5.49±4.74) mm, (2.61±3.38) mm and 1.22°±1.16° in the control group, respectively. Statistical significance was observed in the differences of positioning errors in the X, Y directions and ROLL between the experimental group 1 and control group ( t=4.32, 2.89, 2.78, P < 0.001, =0.004, =0.006), respectively. Statistical significance was detected in the differences of positioning errors in the X, Y directions and ROLL between the experimental group 2 and control group ( t=5.20, 5.14, 5.82, all P<0.001). Statistical significance was noted in the differences of positioning errors in the Y direction and ROLL between the experimental group 1 and 2 ( t=2.58, 3.41, P=0.010, 0.001). Conclusion:The laser positioning coordination system-assisted tracing the body surface marking line can significantly reduce the positioning errors in the X and Y directions and ROLL, and the upgraded version of the laser positioning coordination system can further reduce the positioning errors in the Y direction and ROLL compared with the initial version, which is of high clinical application value.

Objective:To provide reference for establishing the testing method for quality control of neutron beam in boron neutron capture therapy (BNCT) equipment in China by testing the radiation characteristic parameters and dosimetry characteristic parameters of epithermal neutron beam in hospital neutron irradiator (IHNI).Methods:By comparing the uncertainties in the result of various test items with the deviation values recommended by the European Joint Research Center (EC-JRC), the feasibility of the relevant of testing method was analyzed and evaluated.Results:The uncertainty in epithermal neutron fluence rate was 2.7%. The uncertainty in ratio of thermal to epithermal neutron fluence rate was 3.1%. The uncertainty in ratio of fast neutron air kerma to epithermal neutron fluence rate was 9.3%. The uncertainty in ratio of gamma air kerma to epithermal neutron fluence rate was 8.7%. The uncertainty in spatial distribution of neutron fluence rate was 2.7%. The uncertainty in thermal neutron fluence rate in phantom was 1.8%. The uncertainty in neutron and gamma-ray dose rate in phantom was 17.1% and 4.0%, respectively.Conclusions:The uncertainty in neutron dose rate measurement result in phantom is higher, and further research is needed to improve the accuracy of the testing method. The uncertainty in the measurement result of other test items is lower, and the accuracy of the test result is expected to meet the allowable deviation value recommended by the European Joint Research Center, and the test method is feasible.

Objective:To summarize the clinicopathologic features and clinical diagnosis and treatment experience of retroperitoneal paraganglioma.Methods:This study retrospectively analyzed the clinical, pathological and follow-up data of 39 patients admitted to the First Affiliated Hospital of Xi'an Jiaotong University from 1 Oct 2012 to 1 Oct 2022 for retroperitoneal paragangliomas undergoing resection.Results:There were 19 males and 20 females with tumor being functional in 11 cases (28%) and non-functional in 28 cases (72%). CT angiography showed that the tumors were distributed around the abdominal aorta and inferior vena cava in most cases. All 39 patients underwent tumor rescetion.Patients in laparoscopic group had shorter operation time and postoperative hospital saty compared with open sugery [(135±66)min vs. (194±67)min, t=-2.529, P=0.016; (6.6±2.2)d vs.(9.6±4.8)d, t=-2.096, P=0.043], while there was no statistically significant difference between the two groups in terms of intraoperative blood loss [(152±151)ml vs. (361±608)ml, t=-1.169, P=0.250]. There were no major postoperative complications in the laparoscopic group, and pulmonary infection in 1 case and intestinal obstruction in 1 case in the open group. Thrity-six cases were followed up, ranging from 2 to 115 months, 1 patient in the laparoscopic group died 1 year after surgery due to recurrence and metastasis. In the open group, 1 case recurred 2 years later and was discharged after the second operation, and 1 case died of recurrence 2 years after surgery. Conclusions:Surgery is indicated for retroperitoneal paraganglioma. Adequate perioperative management is the key to the success of the operation. Laparoscopic surgery is superior to open surgery in terms of operation time and postoperative recovery .

Objective:To construct an evaluation index system for continuous nursing outcomes of patients undergoing knee arthroplasty.Methods:From January to May 2022, a preliminary draft of the index system was developed based on literature research, semi-structured interviews, and group discussions. We developed a questionnaire and conducted two rounds of consultation with 15 experts using the Delphi expert consultation method to construct the final index system. The positivity of experts was expressed by the effective recovery rate of the questionnaire. The expert authority coefficient was represented by the arithmetic mean of the expert's judgment basis and familiarity with the indicator. The coordination of expert opinions was represented by the Kendall's W. Results:Around two rounds of consultation, the effective recovery rates of the questionnaire were 100.0% (15/15) and 93.3% (14/15), respectively, and the expert authority coefficients were 0.815 and 0.860, respectively, and the expert Kendall's W were 0.297 and 0.121 ( P<0.01). The final evaluation index system for continuous nursing outcomes of patients undergoing knee arthroplasty included 3 primary indicators, 7 secondary indicators, and 47 tertiary indicators. Conclusions:The evaluation index system for continuous nursing outcomes of patients undergoing knee arthroplasty is strongly scientific and reliable, and has guiding significance for standardizing the continuous nursing of knee arthroplasty patients.

Objective:To explore the curative effects of individualized surgical method for treating pyriform sinus fistula (PSF) in children.Methods:A total of 43 PSF children treated in the Department of Pediatric Surgery of Henan Provincial People′s Hospital from June 2015 to November 2019 were selected, with 41 cases on the left side and 2 cases on the right side.There were 27 cases in the quiescent inflammatory infection stage and 16 cases in the acute inflammatory infection stage.According to patients′ condition, low-temperature plasma radiofrequency ablation, fistulectomy, abscess incision and drainage or combined operation were conducted.The postoperative complications and recurrence were observed.Results:Among 43 cases, there were 19 cases treated with low-temperature plasma radiofrequency ablation, 11 cases treated with low-temperature plasma radiofrequency ablation combined with abscess incision and drainage, 5 cases treated with low-temperature plasma radiofrequency ablation combined with fistulectomy, and 8 cases treated with fistulectomy.Four cases had postoperative hoarseness and recovered within 1-5 months.One case occurred pseudo healing after abscess incision and drainage, and healed after incision and debridement.Totally, 4 cases recurred, and the follow-up time was from 3 months to 57 months.Conclusions:Low-temperature plasma radiofrequency ablation has the advantages of minimally invasive, safe and effective, which provides a new treatment for PSF in children.Based on the condition of children, the choice of individualized treatment can significantly improve the curative effects.

Objective:To explore the strategy of selecting the second stage operation after neonatal enterostomy.Methods:Clinical data of 35 cases admitted to the Department of Pediatric Surgery of Henan Provincial People′s Hospital for enterostomy in neonatal period and radical operation in the second stage from May 2014 to December 2019 were subject to retrospective analysis.They were inquired about the medical history in detail before the operation, and received barium enema and colonoscopy, 24-hour delayed radiography, anorectal manometry and other examinations.During the operation, a multi-point intestinal biopsy and rapid frozen pathological examination were performed accor-ding to the individual′s condition, and the specific surgical method was determined in combination with intraoperative exploration.Postoperative observations were conducted for such complications as anastomotic leakage, intestinal obstruction, and abdominal infection, as well as such medium and long-term outcomes as intestinal function recovery, defecation, and nutritional status.Results:Nine cases were diagnosed with neonatal necrotizing enterocolitis (NEC) and received ostomy closure.Sixteen cases were diagnosed with NEC concomitant with intestinal stenosis and received the narrow bowel enterectomy and anastomosis combined with ostomy closure; 10 cases were diagnosed with Hirschsprung′s disease, including 5 cases of total colonic aganglionosis treated with Soave radical operation, 3 cases of long-segment Hirschsprung′s disease treated with subtotal colectomy, inverted ascending coloanal anastomosis with Deloyers method and second enterostomy, and 2 cases of normal-segment Hirschsprung′s disease treated with pull-through operation combined with ostomy closure.After the operation, 1 case had anastomotic leakage and septic shock, 1 case had incision dehiscence, 3 cases had adhesive intestinal obstruction and 1 case occurred left lung consolidation.During a follow-up period of 3-36 months, their recovery condition was acceptable.Conclusions:For those neonates receiving enterostomy, detailed medical history should be enquired before the second stage operation.The combination of barium enema and colonoscopy, 24-hour delayed radiography and multi-point intestinal biopsy with intraoperative exploration conduced to selecting appropriate individualized surgical schemes and reducing surgical complications, thus improving the long-term quality of life of children.