The improvement of hemophilia treatment is attributed to the development of transfusion medicine, and the discovery of cryoprecipitate has opened up a new era of hemophilia treatment, laying the foundation for the development of modern hemophilia therapies. But with the further improvement of hemophilia treatment and the emergence of new treatment methods, transfusion medicine no longer plays a dominant role in hemophilia treatment. The new technologies and methods for hemophilia treatment can provide reference for the development of transfusion medicine, thereby better serving clinical practice and patients.

Objective: To investigate the effects of different storage temperatures and durations on the activities of coagulation factor Ⅷ (Factor Ⅷ, FⅧ) and coagulation factor Ⅸ (Factor Ⅸ, FⅨ) after whole blood collection, so as to provide data support for the optimal storage conditions. Methods: A total of 16 mL of whole blood was collected from each of the 20 healthy volunteers at our blood center and aliquoted into 8 sodium citrate anticoagulant tubes. Two tubes were immediately centrifuged for the measurement of FⅧ and FⅨ activity levels. The remaining 6 tubes of whole blood were respectively stored under room temperature and low-temperature conditions. At 2, 4, and 6 h, the whole blood samples were centrifuged and analyzed for FⅧ and FⅨ activity levels. The mean values of the two immediately tested tubes were used as the control group, while other tubes were designated as the experimental groups for comparison. Statistical analysis was performed using SPSS 26.0. Results: The activity of FⅧ in whole blood remained stable after 4 hours of storage at both room temperature and low temperature (116.53±25.95 vs 125.22±27.33, 109.77±23.23 vs 125.22±27.33) (P>0.05 for both). However, by 6 hours, FⅧ activity showed a statistically significant decline compared to the control group (108.65±22.92 vs 125.22±27.33, 100.46±20.19 vs 125.22±27.33) (P<0.05 for both), though the room temperature group results were closer to the control values. The activity of FⅨ in whole blood remained stable after 6 hours of storage under both conditions (97.14±19.48 vs 96.76±19.67, 97.10±17.45 vs 96.76±19.6) (P>0.05 for all comparisons). Conclusion: For whole blood samples after collection, storage at either room temperature or low temperature for up to 4 hours does not compromise the accuracy of test results. When stored for 6 hours, FⅨ activity remains stable, whereas FⅧ activity decreases significantly. Notably, FⅧ activity demonstrates better stability at room temperature than under low-temperature conditions within the 6-hour storage.

【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.

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.

Professor 's experience of acupuncture combined with medication for epilepsy is summarized, which is explained from epilepsy's etiology and pathogenesis, diagnosis and treatment of acupuncture and medication, respectively. Besides, the theoretical foundation and use instruction of acupuncture technique "-" for epilepsy are introduced. Professor highly values the adherence to etiology and pathogenesis, pays attention to syndrome differentiation and searches for the primary disease cause. He proposes the wind, phlegm, stasis and deficiency are the pathogenesis of epilepsy, and points out acupuncture could be applied during attack stage and remittent stage, but electroacupuncture should be used with caution. Regulating spirit is the key for treating epilepsy. The combination of acupuncture and medication could regulate the governor vessel and guide to the origin, which have significant curative effect.
Acupuncture Therapy ; Combined Modality Therapy ; Electroacupuncture ; Epilepsy ; therapy ; Humans

Objective To study the effect different concentrations of HDAC6 inhibitor tubacin on the proliferation,morphology and membrane surface ultrastructure of bone marrow mesenchymal stem cells(BMSCs).Methods Primary BMSCs were cultured.The P4 generation cells were taken for conducting the experiment.The different concentrations of tubacin were used to treat the cells fro 24 h.The cells survival rate was detected by MTT assay.The atomic force microscopy(AFM) was applied to observe the cellular morphology and surface ultramicrostructure and detect the mechanical property in different groups.Results The MTT results showed that low concentration of tubacin had the effect for promoting BMSC proliferation;the AFM results showed that compared with the control group,the height and width of BMSCs after treating by low concentration of tubacin,the membrane surface roughness was decreased and cellular hardness was increased.Conclusion Low concentration of tubacin can promote the BMSC proliferation,causes the changes of morphology and membrane surface ultramicrostructure,enhances the mechanical property and increases the cell implantation treatment efficiency.

OBJECTIVETo summarize the general condition, regional distribution, prevalence and clinical characteristics of Shandong province based on hemophilia case registry information.

METHODSA retrospective study was carried out on 1979 hemophilia patients registered at Shandong Hemophilia Registration Center.

RESULTSThe 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.

CONCLUSIONThe 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.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Child, Preschool ; China ; epidemiology ; Hemophilia A ; epidemiology ; Humans ; Infant ; Middle Aged ; Prevalence ; Registries

OBJECTIVETo investigate the mechanism of N- Arachidonoylethanolamine (ANA) on inhibiting platelets (PLT) apoptosis under standard blood bank storage conditions.

METHODSSamples 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.

RESULTSThe 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).

CONCLUSIONANA 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.

Apoptosis ; drug effects ; Blood Platelets ; cytology ; drug effects ; Caspase 3 ; Caspase 9 ; Cytochromes c ; Endocannabinoids ; pharmacology ; Humans ; Mitochondria ; Proto-Oncogene Proteins c-akt

In order to study the effect of seed soaking with different aluminum solution on seed germination and seedling physiological characteristics of Platycondon grandiflorum, two P. grandiflorum varieties'seed (the white flower and the purple flower) were soaked in Al3+ solution with different concentrations (0, 10, 100, 250, 500, 750 and 1000 mg x L) for 24 h, then germinated in illumination incubator. Results showed that the aluminum toxicity on the trends of the germination rate, germination index and vigor index was positive associated with its concentration, and the Al tolerance of the purple was slightly greater than that of the white. There were some relationships between the physiological indices, which were the leakage rate of electrolyte, the malonaldehyde (MDA) content, the activities of peroxidase (POD) and superoxide dismutase (SOD) , the free praline(Pro) and the soluble sugar contents, with the concentrations of Al. It was suggested that there was Al tolerance difference between the two P. grandiflorum varieties: the purple flower was greater than the white.
Aluminum ; pharmacology ; Dose-Response Relationship, Drug ; Germination ; drug effects ; Immersion ; Platycodon ; drug effects ; growth & development ; physiology ; Seedlings ; drug effects ; physiology ; Seeds ; drug effects ; growth & development

Objective To investigate the expression of monocyte chemoattractant protein-1(MCP-1)and CCR2 in peripheral blood and pleural fluid of tuberculous pleurisy patients.Methods Flow cytometry was used to detect the expression of CCR2 in the peripheral blood mononuclear cell and pleural fluid cell of tuberculous pleurisy patients;ELISA was used to detect the content of MCP-1 in serum and pleural fluid.Results MCP-1 in surem and pleural fluid and CCR2 in the peripheral blood mononuclear cell and pleural fluid cell of tuberculous pleurisy patients was significantly higher than that of the healthy normal controls[MCP-1:(340.8±220.8)and(9.0±3.8)ng/L,P＜0.01;CCR2(18.2±10.1)%and(6.9±3.5)%,P＜0.05];Both MCP-1 and CCR2 were detected in pleural fluid and both of them were positivley correlated(r=0.227,P＜0.05).Conclusion The expression of MCP-1 and CCR2 in peripheral blood of tuberculous pleurisy patient are significantly elevated which are significance molecule participating in the pathogenesis of tuberculous pleurisy.