Hemophilia A with reduced coagulation factor Ⅺ: a case report and literature review / 中国输血杂志

【Objective】 To investigate the possible molecular pathogenesis of a child with hemophilia A accompanied by coagulation factor Ⅺ reduction by testing coagulation-related indicators and genotyping in the child and his family. 【Methods】 Peripheral blood from the patient and his parents for detection of coagulation factors Ⅷ, Ⅸ, Ⅺ, Ⅻ, VWF∶Ag, lupus anticoagulants and F VIII, F XI inhibitors were collected. All exons and flanking sequences of the genes encoding FⅧ and FⅪ were sequenced and bioinformatically analyzed. 【Results】 The child had low FⅧ and FⅪ activity and no parental abnormalities were observed. The sequencing results showed that there was a c. 1834(exon12) C>T heterozygous mutation in the FⅧ gene and a c. 1817 (exon15) G>A heterozygous mutation in the FⅪ gene, which was de novo. Bioinformatics analysis shows that the FⅪ mutation changes the original protein structure and increases the number of carboxyl groups. 【Conclusion】 For patients with prolonged APTT, in addition to excluding factors that interfere with APTT testing, all coagulation factors related to APTT should be tested to clarify the diagnosis.

Anterior cervical release and posterior fixation for the treatment of irreducible atlantoaxial dislocation with retropharyngeal internal carotid artery / 中华骨科杂志

Objective:To investigate the clinical effect of anterior cervical release and posterior fixation in the treatment of irreducible atlantoaxial dislocation with retropharyngeal internal carotid artery.Methods:Thirteen patients with irreducible atlantoaxial dislocation of retropharyngeal internal carotid artery from January 2015 to July 2019 were treated with anterior cervical release and posterior fixation. There were 8 males and 5 females, aged from 34 to 65 years with an average of 46.1±12.6 years. Positive, lateral and dynamic X-ray films, MR and CTA were performed before operation. There were 4 cases with bilateral retropharyngeal internal carotid artery and 9 cases with unilateral retropharyngeal internal pharyngeal artery. The time of operation, the amount of bleeding and intraoperative and postoperative complications were recorded. The main observations were Japanese Orthopaedic Association (JOA) score, atlantodental interval (ADI), Chamberlain line (CL), and changes in the morphology of the retropharyngeal internal carotid artery and implant fusion.Results:All the operations completed successfully. The operation time was 210-260 min, the average was 245±21 min; the blood loss was 350-600 ml, the average blood loss was 490±107 ml. There was no injury of internal carotid artery, vertebral artery, spinal cord or nerve root during the operation. All patients were followed up for 9 to 24 months, with an average of 15.1±6.2 months. Preoperative JOA score was 6.9±2.3 points, 1 month after operation was 13.5±2.5 points, and the last follow-up was 14.3±2.1 points. The difference was statistically significant ( F=30.91, P<0.001). The difference between 1 month after operation and before operation was statistically significant ( P<0.001), and the improvement rate of JOA score was 75.6%±15.2%. There was no significant difference between the last follow-up and 1 month after operation ( P>0.05). The preoperative ADI was 8.9±2.2 mm, 1 month after operation was 1.1±0.8 mm, and the last follow-up was 1.2±0.9 mm. The difference was statistically significant ( F=114.69, P<0.001). The difference between 1 month after operation and before operation was statistically significant ( P<0.001), and ADI had returned to normal level. There was no significant difference between the last follow-up and 1 month after operation ( P>0.05). The preoperative CL was 11.7±4.8 mm, 1 month after operation was 1.6±2.1 mm, and the last follow-up was 1.8±2.3 mm. The difference was statistically significant ( F=34.19, P<0.001). The difference between 1 month after operation and before operation was statistically significant ( P<0.001), and the position of odontoid process returned to normal level. There was no significant difference between the last follow-up and 1 month after operation ( P>0.05). Bone graft fusion was received at 6 to 12 months after operation, with an average of 10.2 months. Conclusion:CTA examination should be performed before anterior release of atlantoaxial dislocation to understand the position and shape of internal carotid artery. Anterior cervical release combined with posterior bone graft fusion is an effective method for the treatment of irreducible atlantoaxial dislocation with retropharyngeal internal carotid artery without increasing the risk of internal carotid artery injury.

Current Status and Discussion of Hemophilia Aid System / 罕见病研究

Hemophilia is a hereditary bleeding disorder where the only effective treatment is factor replacement therapy. The main contradiction of hemophilia in China is the contradiction between the increasing demand for quality of life of hemophilia patients and insufficient medical insurance, medical treatment and medicine. Expensive treatment costs are the main reason for patients to "be impoverished due to illness" and "return to poverty due to illness". This article analyzes the current status of the hemophilia aid system through the existing literature data and existing patient aid projects. Cooperation of social insurance, social relief and high-quality medical services is critical to a complete hemophilia comprehensive medical aid system, so that hemophilia patients can receive standardized diagnosis and treatment, and try to achieve the close quality of life as normal.

Predictive value of C-reactive protein to lymphocyte ratio for stroke-associated pneumonia / 国际脑血管病杂志

Objective:To investigate the predictive value of C-reactive protein to lymphocyte ratio (CLR) for stroke-associated pneumonia (SAP).Methods:Consecutive patients with acute ischemic stroke admitted to the Department of Neurology, Guangming Ophthalmic Hospital from September 2018 to December 2020 were enrolled retrospectively. Fasting venous blood was taken the next morning after admission to detect the levels of C-reactive protein and lymphocytes. The baseline clinical data of the patients in the SAP group and the non-SAP group were compared. Multivariate logistic regression analysis was used to identify the independent predictors of SAP. The predictive value of CLR for SAP was evaluated by receiver operator characteristic (ROC) curve analysis. Results:A total of 479 patients with acute ischemic stroke were included. The median CLR was 3.69×10 -9, 81 patients (16.9%) had SAP. The incidence of SAP in the high CLR group was significantly higher than that in the low CLR group (26.7% vs. 7.1%; P=0.001). Univariate analysis showed that there were significant differences in age, atrial fibrillation, dysphagia, baseline National Institutes of Health Stroke Scale score, stroke etiology type, leukocyte count, fasting blood glucose, total cholesterol and CLR between the SAP group and the non-SAP group (all P<0.05). Multivariate logistic regression analysis showed that after adjusting for confounding factors, CLR was an independent predictor of SAP (odds ratio 3.472, 95% confidence interval 1.431-14.706; P=0.013). ROC curve analysis showed that the area under the curve of CLR predicting SAP was 0.735 (95% confidence interval 0.693-0.774; P<0.001). The optimal cut-off value was 6.14 ×10 -9. The sensitivity and specificity were 69.1% and 72.6% respectively. Conclusion:Higher baseline CLR can predict SAP risk in patients with acute ischemic stroke.

Practice and reflection on international cooperation and exchange in hospital discipline construction / 中华医学科研管理杂志

Objective Discipline construction is the core for the development of a hospital and international cooperation and exchange play an important role in promoting discipline construction.This paper summarized the experiences of carrying out international cooperation and exchange in the hospital discipline construction.Methods Analyzed the exploration,practice and its effect in promotion of hospital discipline construction regarding to international cooperation in recent years.Results The international cooperation and exchange have effectively promoted the development of hospital disciplines,for instance,introduction and training of talents,organizing and holding of high-level international conferences,establishment of international academic journals and the joint construction of academic platforms.Conclusions In accordance with the requirements of discipline development strategy,the hospital can improve the overall level of discipline construction rapidly by carrying out all-dimensional cooperation with international top medical institutions.

A phase Ⅲ multi-center clinical trial on safety and efficacy of a domestic plasma derived factor Ⅸ for the treatment of patients with hemophilia B / 中华血液学杂志

Objective@#To evaluate the efficacy and safety of a domestic human plasma derived coagulation Factor Ⅸ concentrate (pd-FⅨ) in patients with hemophilia B.@*Methods@#The study was a multicenter, open-label and single-arm study. The efficacy of pd-F Ⅸ was evaluated by objective performance criteria. The doses of pd-FⅨ were calculated according to the bleeding symptom and disease severity. The infusion efficiency of pd-FⅨ and improvement of bleeding symptoms were measured at 30 minutes and (24±4) h after the first infusion, respectively. Adverse events were recorded. Viral infection and FⅨ inhibitor were detected 90 d after the first infusion.@*Results@#All 36 subjects with hemophilia B were enrolled in the study. The median age of these patients was 31 years old and the median injection doses were 4 (1-17) times. The hemostatic effect of 27/36 (75.00%) and 9/36 (25.00%) acute bleeding events were rated as "excellent" and "better" , respectively. The recovery rate was 111.92% (65.55%-194.28%) at 30 minutes after infusion of FⅨ. There was no adverse event related to FⅨ. No reactivation of HBV, HCV or HIV and FⅨ inhibitor was detected at 90-104 d after the first FⅨ infusion.@*Conclusion@#This domestically made human plasma derived FⅨ concentrate is safe and effective in the treatment of acute bleeding in patients with hemophilia B.@*Clinical trial registration@#China food and Durg Administration, 2016L08027.

Analysis of hemophilia case information of Shandong province / 中华医学遗传学杂志

<p><b>OBJECTIVE</b>To summarize the general condition, regional distribution, prevalence and clinical characteristics of Shandong province based on hemophilia case registry information.</p><p><b>METHODS</b>A retrospective study was carried out on 1979 hemophilia patients registered at Shandong Hemophilia Registration Center.</p><p><b>RESULTS</b>The 1979 cases have included 1704 hemophilia A and 275 hemophilia B patients. Hemophilia A was characterized as severe in 1021 patients (59.9%), moderate in 483 patients (28.4%), and mild in 200 patients (11.7%); while hemophilia B was characterized as severe in 125 patients (45.4%), moderate in 116 patients (42.2%), and mild in 34 patients (12.4%). The median age was 23 years (ranging from 1 month to 81 years), and most were young patients. Joint deformity occurred in 963 patients, and 948 patients had a family history of hemophilia. All counties of Shandong province had patients except for Changdao county and Fushan district of Yantai city. The prevalence of Heze city and Dongying city (3.39/100 000 and 3.05/100 000, respectively) were relatively higher.</p><p><b>CONCLUSION</b>The above data revealed epidemiological and clinical characteristics of Shandong Province. Patient-centered registry system allowed a more detailed and accurate patient information, and promoted the comprehensive care of hemophilia, which also suggested the necessity for the establishment and improvement of the National Hemophilia Registry System.</p>

Correlation between the MRI-based grading system and F wave as well as H-reflex in patients with lumbar disc herniation / 中国组织工程研究

BACKGROUND:Lumbar spine MRI and electrophysiological test are reliable methods for evaluating nerve root injury caused by lumbar disc herniation. OBJECTIVE:To analyze the correlation between the MRI-based grading system and the latency and frequency of F wave as wel as latency and amplitude of H-reflex in patients with lumbar disc herniation. METHODS:MRI imaging of the lumbar spine was performed with a 3.0-T imager and a dedicated TCL coil to classify lumbar disc herniation and nerve root compression. F wave and H reflex were detected on the patient bilateral tibial nerves using Oxford myoelectricity evoked potential instrument. RESULTS AND CONCLUSION:Spearman correlation analysis showed that the MRI-based grading of patients with lumbar disc herniation had a negative correlation with F wave frequency (r=-0.594 0, P<0.000 1), and a positive correlation with F wave latency (r=0.825 6, P<0.000 1) and H-reflex latency (r=0.875 0, P<0.000 1), but no correlation with H-reflex amplitude (R=0.117 4, P=0.257 3). With MRI grading increased, F wave frequency was decreased, and F wave and H-reflex latency were prolonged gradual y, indicating aggravating nerve root compression.

Growth and secretion features of microencapsulated human adrenal pheochromocytoma cells in artificial cerebrospinal fluid / 中国组织工程研究

BACKGROUND:Microencapsulated cels are commonly used as a tool to overcome immune rejection after subarachnoid transplantation. However, the effect of microencapsulation on the secretion of human pheochromocytoma cels is unclear. OBJECTIVE:To observe the growth and secretion of primarily microencapsulated cultured human pheochromocytoma cels in artificial cerebrospinal fluid. METHODS: The human pheochromocytoma tissues were digested successively to isolate human pheochromocytoma cels that were then cultured in artificial cerebrospinal fluid. Primary cels were covered with alginate-polylysine-alginate microcapsules, and then the cel morphology was observed with inverted phase contrast microscope. Levels of met-enkephalin and norepinephrine in cel culture medium were detected by enzyme-labeled immunosorbent assay (ELISA). We used cel counting kit-8 colorimetric assay to obtain the growth curve of human pheochromocytoma cels in artificial cerebrospinal fluid. RESULTS AND CONCLUSION:Microcapsulated human pheochromocytoma cels were in suspension and the process outgrowth increased slowly. Compared with non-microcapsulated cels, the proliferation rate of microcapsulated cels increased significantly. ELISA results revealed a significant increase in the levels of met-enkephalin and norepinephrine secreted from the microencapsulated cels compared to the non-microcapsule group. There was a wide variation in contents of met-enkephalin and norepinephrine from different tumors. These findings indicate that microencapsulated human pheochromocytoma cels can survive wel and have good secretion function in artificial cerebrospinal fluid, and human pheochromocytoma cels from different tumor tissues have stable secretory function.

Effects of Salidroside on expression of substance P and neurokinin-1 receptor of bone marrow cells in bone marrow depressed anemia mice / 中国免疫学杂志

Objective:To observe the effects of salidroside on the expression of substance P (SP)and neurokinin-1 receptor (NK-1R)of bone marrow cells(BMCs)in bone marrow(BM)depressed anemia mice,and to explore its roles for hematopoietic regulation.Methods:Automatic blood cell analysator was used to detected the changes of peripheral blood cells in each group ,immuno-histochemistry and reverse transcription-polymerase chain reaction ( RT-PCR) were used to analyze the expressions of SP and its receptor NK-1R of BMCs in each group respectively.Results: Peripheral blood testing results showed that the number of white blood cells (WBC),red blood cells(RBC)and hemoglobin(HB)of model group were significantly decreased when compared with control group.Compared with model group ,low-dose,middle-dose and high-dose salidroside obviously elevated the number of white blood cells ,and middle-dose salidroside obviously elevated the number of platelets.Immunohistochemistry studies showed that the expression of SP and its receptor NK-1R of BMCs was found in each group.Compared with control group , the expression of SP of BMCs was decreased obviously in model group(P0.05),and the expression of SP and its receptor NK-1R of BMCs was increased significantly in low-dose,middle-dose and high-dose salidroside groups (P<0.05).RT-PCR data showed that the expression of SP mRNA of BMCs was increased obviously in model group ,low-dose,middle-dose and high-dose salidroside groups when compared with control group (P<0.05).The expression of NK-1R mRNA of BMCs was un-detected in control group and model group.The expression level of NK-1R mRNA of BMCs was elevated gradually with the increase of salidroside dosage in low-dose,middle-dose and high-dose salidroside groups.Conclusion: The mRNA and protein expressions of SP and its receptor NK-1R of BMCs were up-regulated significantly by salidroside in dose-dependent manner.These data suggest that salidroside could participate in the recovery of hematopoietic function of BM depressed anemia mice by increasing the expression of SP and its receptor NK-1R of BMCs.

Models and mechanisms for international cooperation and exchange in the disciplines development / 中华医学科研管理杂志

International cooperation and exchanges plays an important role in the disciplines development in hospital,and can indirectly improve the overall capacity and quality of healthcare..This paper draw on 10 years of international cooperation and exchange in the practice of discipline development,,analyzed working patterns and mechanisms to strengthen international scientific cooperation.

Mechanism investigation of platelet apoptosis inhibition by N-Arachidonoylethanolamine / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the mechanism of N- Arachidonoylethanolamine (ANA) on inhibiting platelets (PLT) apoptosis under standard blood bank storage conditions.</p><p><b>METHODS</b>Samples taken from collected apheresis PLT by the Amicus instrument were split into three parts. An aliquot of 0.5 μmol/L ANA were added to one part of storage PLT as the ANA group; an aliquot of 0.5 μmol/L ANA and 1 μmol/L SR141716 was added to the another part as the ANA + SR141716 group; and the third part without ANA and SR141716 as the control group. These samples were stored on a flat-bed shaker at (22 ± 2) ⁰C for 7 days. The expression of phosphatidyl serine (PS) positive, phospho (p)-Akt, Akt, p-Bad, Bad, caspase-3, caspase-9, cytochrome C (Cyt-C) and BCL-XL interaction with Bak were detected.</p><p><b>RESULTS</b>The rate of PLT PS positive in ANA group decreased significantly than that in control group[ (8.29 ± 1.44) % vs (14.24 ± 2.47) %, P<0.05]. The release of Cyt-C from mitochondria to cytosol in ANA group decreased significantly compared with control group[ (3.29 ± 1.44) % vs (15.24 ± 3.40) %, P<0.05]. Also the expressions of p-Akt and p-Bad in ANA group increased significantly than those in control group[ (71.33 ± 10.26) % vs (35.00 ± 6.00) %, P<0.05; (39.00 ± 9.64) % vs (10.33 ± 1.53) %, P<0.05, respectively]. Higher amounts of Bak protein were co-precipitated with BCL-XL in ANA group than that in control group (about 2.6 fold, P<0.05). The expressions of cleaved caspase- 9 and caspase- 3 in ANA group decreased significantly than those in control group[ (9.63 ± 1.47) % vs (23.24 ± 2.47) %, P<0.05; (6.30 ± 1.40) % vs (13.20 ± 2.50) %, P<0.05, respectively]. There were no significantly changes between ANA+SR141716 and control groups (P>0.05).</p><p><b>CONCLUSION</b>ANA protected PLTs from apoptosis as a result of inhibiting the release of Cyt-C from mitochondria to cytosol by modifying the expressions of apoptosis-relative proteins.</p>

Effects of salidroside on bone marrow matrix metalloproteinases of bone marrow depressed anemic mice / 生物医学工程学杂志

To examine the effect of salidroside on the expression and activities of matrix metalloproteinase-2 (MMP-2) and matrix metalloproteinase-9 (MMP-9) in bone marrow (BM) of BM depressed anemic mice by immunohistochemistry and gelatin zymography respectively, and to explore its roles in hematopoietic regulation. Immunohistochemistry showed that the expression of MMP-2 and MMP-9 of bone marrow cells (BMCs) was found in each group. Compared with control group, the expression of MMP-2 and MMP-9 was obviously increased in the model group, low-dose, middle-dose and high-dose salidroside. At day 4 after treatment of radiation and chemotherapy, the peak of the expression of MMP-2 and MMP-9 was found in middle-dose salidroside . At day 8 after treatment of radiation and chemotherapy, the peak of the expression of MMP-2 and MMP-9 was found in low-dose and middle-dose salidroside respectively. Gelatin zymography revealed that 66 kD proMMP-2, 62 kD MMP-2, 86 kD MMP-9 and 94 kD proMMP-9 were detected in control group, and the activity of MMP-9 was stronger among them. After treatment of radiation and chemotherapy, the activity of gelatinases of hemopoietic microenviroment (HM) was obviously decreased, but low-dose, middle-dose and high-dose salidroside could significantly increase the activities of proMMP-9 and MMP-9, attenuate the activity of proMMP-2. These results suggest that salidroside could promote the recovery of hematopoietic function of BM depressed anemic mice by increasing the expression and activity of MMPs, releasing the cytokines from ECM or cell membrane, repairing impaired microvessels of HM and promotion proliferation, migration and differentiation of HSCs.

Five Pathogen Mixed Infection in STDs of Female Genitourinary Tract:Analysis and Status / 中华医院感染学杂志

OBJECTIVE To study the status of five pathogen mixed infection in sexually transmitted diseases(STDs) and analyze the clinical meaning.METHODS We detected five common pathogens by fluorescence polymerase chain reaction,which were Neisseria gonorrhoeae(NG),Chlamydia trachomatis(CT),Ureaplasma urealyticum(Uu),human papilloma virus(HPV),Candida albicans(Ca),and herpes simplex virus.RESULTS We analyzed the status of infection among 4 601 patients,got 279 mixed infection cases,accounted for 6.1% in all cases;and the population with ages from 21 to 40 years was accounted for 89.6%. CONCLUSIONS Fluorescence polymerase chain reaction is a simple,rapid,high sensibility technique for quantitation testing of STDs pathogens,and we should pay great heed to its effective control.

The immunomodulatory effect of polysaccharide from brown seaweed / 中国生化药物杂志

Purpose　The aim is to investigate the effects of BSP on immunological function in normal and immunosuppressed mices.Methods　Thymus and spleen indexes, peripheral blood WBC number,the lymphocyte proliferation response, IL-2 production and serum and splenocyte hemolysin contents were measured after intraperitoneal injection of BSP in normal and immunosuppressed mices.Results　(1)BSP 100 mg/（kg*d）×10d significantly increased the thymus and spleen indexes and peripheral blood WBC number in immunosuppressed mice.The thymus and spleen indexes in normal mice was also increased. In addition,BSP markedly improved T,B lymphocyte proliferation responses and IL-2 production in normal and immunosupressed mices.(2) BSP improved the serum and splenocyte hemolysin contents in normal and immunosuppressed mice. Conclusion　It was suggested that BSP was a kind of immunomodulator, and could improve the immunological function of normal and immunosuppressed mices.

POSTOPERATIVE PULMOMNARY EMBOLISM: ITS DIAGNOSIS AND TREATMENT / 解放军医学杂志

14 cases of postoperative pulmonary embolism were reported. The incidence of this postoperative complication was 0.47‰ during a 5-year period. The main source of embolus was ileo-fomoral venous thrombosis (confirmed in 9 cases). 10 patients died of this complication, autopsy in 6 of them revealed the embolism occurred in a main pulmonary artery on one or both sides. With a review of recent literatures, the authors discussed the causes, diagnosis, treatment and prevention of this fatal complication, and pointed out the diagnostic value of a comprohensive study of clinical manifestations, arterial blood gas analysis, EKG and plain chest film findings. A more aggressive attitude towards the acceptance of pulmonary embolectomy was suggested in those patients who failed to respond rapidly to medical managment.

EFFECTS OF BROWN SEAWEED POLYSACCHARIDE ON LYMPHOCYTE FUNCTION AND CYTOKINES PRODUCTION / 营养学报

Objective: To investigate the effects of BSP on lymphocyte function and cytokines production in normal and immunosuppressed mice.Methods: The lymphocyte proliferation response, T lymphocyte subpopulations and IL 2 production were measured after intraperitoneal injection of BSP in normal and immunosuppressed mice, and the activity of IL 1 and TNF secreted by mouse peritoneal macrophages were detected after BSP supplementation in vitro.Results: (1)BSP〔(100 mg/(kg?d)?10 d)〕markedly improved T,B lymphocyte proliferation responses and IL 2 production in normal and immunosuppressed mice, and significantly increased the number of L 3T + 4 cells in thymus and spleen of immunosuppressed mice, and the ratio of L 3T + 4/Lyt 2 + cells was also increased.(2)BSP distinctly enhanced the activity of IL 1 and TNF production by mouse peritoneal macrophages in vitro.Conclusion: BSP can improve the immunological function of normal and immunosuppressed mice.