Abnormal ACT in a Patient with Prekallikrein Deficiency Undergoing Cardiopulmonary Bypass

Prekallikrein deficiency is a disorder that often remains undiagnosed. Prekallikrein activates factor XII, which initiates the intrinsic coagulation pathway. Prekallikrein deficiency results in prolonged Partial Thromboplastin Time and Activated Clotting Time in absence of anticoagulants or active bleeding. This case report describes the anesthesia management of a patient with Prekallikrein deficiency who underwent cardiac surgery with Cardiopulmonary Bypass for correction of a congenital cardiac malformation. We highlight the importance of understanding the different tests available for the diagnosis of coagulation factors deficiency during administration of heparin in the setting of cardiovascular procedures under general anesthesia.

Study on improving hematopoietic function of rats with blood deficiency syndrome by Shengxuebao mixture

Background:Shengxuebao mixture(SXBM)is a novel herbal drug approved by China State Food and Drug Administration for the treatment of Leukopenia and iron deficiency anemia caused by radiotherapy and chemotherapy.Methods:To explore the mechanism of SXBM in treating blood deficiency syndrome(BDS).Firstly,network pharmacology and in vivo experiments were used to screen candidate targets and important signaling pathways of SXBM,GO functional enrichment and KEGG pathway analysis were performed.Secondly,a BDS rat model was established to verify the results of the analysis of network pharmacological enrichment.Histopathology and routine peripheral blood examination were observed.The expressions of tumor necrosis factor-α,interleukin(IL)-6,HIF-1αand NF-κB were detected by Western blot,and the expressions of IL-6,IL-1βwere detected by ELISA.Results:62 bioactive components,66 potential targets and 131 signaling pathways of BDS were successfully identified by network pharmacology.Molecular docking simulation techniques showed that key targets tumor necrosis factor-α,IL-6,IL-1βcan dock well with crucial components,and the BDS-related signaling pathways HIF-1 and JAK-STAT play a vital role.The combined model experiment of acetylphenylhydrazine and cyclophosphamide showed that the model group had obvious blood deficiency,and the histopathology and blood routine were effectively restored after administration.Our findings indicate that SXBM’s therapeutic effect on BDS primarily involves the mediation of the HIF-1α/NF-κB signaling pathway and the regulation of hematopoietic factor expression.Conclusion:This study not only affirmed the protective properties of SXBM against BDS but also provided insights into a potential mechanism for blood replenishment in the treatment of BDS using SXBM.

Factor VII Deficiency: A Rare Cause of Severe Bleeding Disorder in a Newborn

Factor VII deficiency is rare. It is an autosomal recessive inherited disease with an estimated prevalence of 1/1,000,000. We report the case of a newborn male from first-degree consanguineous parents admitted at 15 days of life due to a hemorrhagic syndrome. Hemostasis tests showed low prothrombin time (PT) and normal activated partial thromboplastin time (aPTT). A coagulation panel revealed isolated factor VII deficiency. In this case, we highlight the clinical, biological, and therapeutic aspects of this condition during the neonatal period.

Relationship between Acquired Deficiency of Vitamin K-dependent Clotting Factors And Hemorrhage

This study examined the changes of activities of vitamin K-dependent clotting factors(VKDCF) under various pathological conditions and explored the relationship between acquired deficiency of VKDCFs and hemorrhage.Clinical data of 35 patients who were diagnosed as having acquired deficiency of VKDCF were retrospectively analyzed.Coagulation factors involved in the intrinsic and extrinsic pathways were detected in these patients and 41 control subjects.The results showed that the average activities of VKDCFs were decreased in the patients in comparison to the control subjects and significantly increased after treatment of these patients with vitamin K and blood products.Multivariate regression analysis indicated that decreased activity of VKDCF was not an independent risk factor for bleeding disorders owing to deficiency or metabolic disturbance of vitamin K.It was concluded that acquired deficiency of VKDCF occurs under a variety of pathologic conditions and is closely associated with hemorrhagic events.Administration of vitamin K and transfusion of blood products containing high concentrations of VKDCFs helps alleviate the hemorrhagic diseases.

Effect of electro-acupuncture on basic fibroblast growth factor protein and mRNA expression in hippocampal dentate gyrus of spleen deficiency rats

BACKGROUND： Spleen deficiency in traditional Chinese medicine refers to the functional disorder of spleen, pancreas, intestines, and nervous system in modern medicine. OBJECTIVE; To test whether electro-acupuncture could alter basic fibroblast growth factor （bFGF） protein and mRNA expression in the hippocampal dentate gyrus of spleen deficiency rats. DESIGN, TIME AND SETTING： The randomized, controlled, in vivo animal experiment was performed at the National LeveI-B Laboratory of Clinical Cell Molecule and Biology in Shenzhen Hospital of Traditional Chinese Medicine, between March and November in 2008. MATERIALS： Reserpine injection was produced by Guangdong Bangmin Pharmaceutical Co. Rhubarb extract granule preparation was produced by Guangdong Yifang Pharmaceutical. Huanqiu Brand sterile acupuncture pin was provided by Suzhou Acupuncture Supplies, China. Huatuo Brand electroacupuncture instrument （type SDZ-II） was purchased from Suzhou Medical Appliance Factory, China. METHODS： A total of 96 male Sprague Dawley rats were randomly assigned to control （n = 32） and induction （n = 64） groups. Spleen deficiency was induced via intraperitoneal injection of reserpine and intragastric administration of rhubarb. The successful models were randomized into two groups： model and electro-acupuncture, with 32 rats in each group. Electro-acupuncture was administered at Zusanfi （ST 36） and Sanyinjiao （SP 6） acupoints using a condensation wave and rarefaction （condensation wave 15 Hz） at a strength of 6-15 V for 20 minutes, once per day. The appearance of a slight shiver in the corresponding locus was taken as the standard. According to electro- acupuncture time points, each group was assigned to four subgroups at 7, 14, 28, and 49 days, respectively, with eight rats in each subgroup. Immunohistochemical staining, image analysis, and reverse-transcription polymerase chain reaction were performed at different time points. MAIN OUTCOME MEASURES： bFGF protein and mRNA expression in the hippocampal dentate gyrus of spleen deficiency rats. RESULTS： After 7 days of electro-acupuncture therapy, bFGF protein and mRNA expression significantly increased compared with the model and control groups （P 〈 0.05）. After 14 days, bFGF protein and mRNA expression decreased until 28 days, where levels were then equal to the model group and greater than the control group （P 〈 0.05）. After 49 days, the above indices remained increased in the electro-acupuncture group compared to the model and control groups （P 〈 0.05）. CONCLUSION： Continuous electro-acupuncture maintained a high level of bFGF protein and mRNA expression in the hippocampal dentate gyrus of spleen deficiency rats.

Transcriptome-wide identification and expression profiling of Pinus massoniana MYB transcription factors responding to phosphorus deficiency

Myeloblastosis(MYB) proteins constitute one of the largest transcription factor(TF) families in plants and play crucial roles in regulating plant physiological and biochemical processes, including adaptation to diverse abiotic stresses. These TF families contain highly conserved MYB repeats(1 R, R2 R3, 3 R and 4 R) at the N-terminus. Roles for MYB TFs have been reported in response to such stresses as dehydration, salt, cold, and drought. The characterization of Masson pine(Pinus massoniana) MYB TFs are reported, including the analysis of MYB TFs expression in seedlings under controlled conditions and two different phosphate(Pi) deficient treatments. By searching for conserved MYB motifs in full transcriptomic RNA sequencing data for P. massoniana, 59 sequences were identified as MYB TFs. Conserved domainstructures and comparative functional and phylogenetic relationships of these MYB TFs with those in Arabidopsis were assessed using various bioinformatics tools. Based on microarray analysis, P. massoniana MYB genes exhibited different expression patterns under the two Pi deficiency conditions. Genes encoding MYB TFs that showed increased expression under critical Pi deficiency were identified, and some MYBs were differentially expressed only under conditions of severe Pi starvation. These results are useful for the functional characterization of MYB TFs that may be involved in the response to Pi deficiency and play divergent roles in plants.

Intraoperative thromboelastography-guided transfusion in a patient with factor XI deficiency: A case report

BACKGROUND Factor XI(FXI)deficiency,also known as hemophilia C,is a rare bleeding disorder of unpredictable severity that correlates poorly with FXI coagulation activity.This often poses great challenges in perioperative hemostatic management.Thromboelastography(TEG)is a method for testing blood coagulation using a viscoelastic hemostatic assay of whole blood to assess the overall coagulation status.Here,we present the successful application of intraoperative TEG monitoring in an FXI-deficient patient as an individualized blood transfusion strategy.CASE SUMMARY A 21-year-old male patient with FXI deficiency was scheduled to undergo reconstructive surgery for macrodactyly of the left foot under general anesthesia.To minimize his bleeding risk,he was scheduled to receive fresh frozen plasma(FFP)as an empirical prophylactic FXI replacement at a dose of 15-20 mL/kg body weight(900-1200 mL)before surgery.Subsequent FFP transfusion was to be adjusted according to surgical need.Instead,TEG assessment was used at the beginning and toward the end of his surgery.According to intraoperative TEG results,the normalization of coagulation function was achieved with an infusion of only 800 mL FFP,and blood loss was minimal.The patient showed an uneventful postoperative course and was discharged on postoperative day 8.CONCLUSION TEG can be readily applied in the intraoperative period to individualize transfusion needs in patients with rare inherited coagulopathy.

Diagnosis and treatment discussion of congenital factor VII deficiency in pregnancy:A case report

BACKGROUND Congenital factor VII deficiency(FVIID)is a rare autosomal recessive genetic disorder.The clinical manifestations of this deficiency vary greatly.Predicting the risk of bleeding during and after childbirth of pregnant women with congenital FVIID is difficult.Recombinant factor VIIa is the most common replacement therapy for FVIID.However,no unified diagnosis and treatment plan for pregnant women with congenital FVIID has been established.CASE SUMMARY We report the clinical history of a pregnant woman who was considered to have congenital FVIID.Recombinant factor VIIa was prophylactically administered to the pregnant woman at the time of cervical fully opening.She successfully delivered a live infant without any complications,such as postpartum hemorrhage,neonatal abnormalities,and so on.CONCLUSION Prophylaxis of recombinant factor VIIa during delivery can effectively reduce the incidence of postpartum hemorrhage among pregnant women with congenital FVIID associated with a high risk of bleeding.

Iron-deficiency anemia. A study of risk factors among adult population of Quetta Valley

Risk factors for iron deficiency anemia among the adult population of the Quetta valley have been investigated. Anemic adult patients, both males and females, who were admitted in the Sandeman Provincial Hospital, Quetta, were invited to participate in this study. After detailed history and examination, preliminary blood tests including full blood counts, platelets count, retics count, absolute blood values and blood film examination were done. A clinical diagnosis was made based upon the findings of history, examination and blood tests. In patients suspected to have iron deficiency anemia, serum iron studies (i.e. serum iron, Total iron binding capacity (TIBC) and serum ferritin) were done to confirm the diagnosis. Among the selected anemic patients, 60% were iron deficiency anemic, while 40% were non-iron deficiency anemic. Iron deficiency anemia was more common among females than males, as 70% patients were female and 30% were male. The risk factors were found to be pregnancy (40%), nutritional inadequacy (17%), menorrhagia (9%), hemorrhoids (9%), hook worms (8%), hematuria (2%) and blood loss due to various gastro-intestinal pathologies (15%).

Acquired coagulation dysfunction resulting from vitamin Kdependent coagulation factor deficiency associated with rheumatoid arthritis: A case report

BACKGROUND Rheumatoid arthritis(RA)is a common chronic inflammatory autoimmune disease with the main clinical feature of progressive joint synovial inflammation,which can lead to joint deformities as well as disability.RA often causes damage to multiple organs and systems within the body,including the blood hemostasis system.Few reports have focused on acquired coagulation dysfunction resulting from vitamin K-dependent coagulation factor deficiency associated with RA.CASE SUMMARY A 64-year-old woman with a history of RA presented to our hospital,complaining of painless gross hematuria for 2 wk.Blood coagulation function tests showed increased prothrombin time,international normalized ratio,and activated partial thromboplastin time.Abnormal blood coagulation factor(F)activity was detected(FII,7.0%;FV,122.0%;and FX,6.0%),indicating vitamin K-dependent coagulation factor deficiency.Thromboelastography and an activated partial thromboplastin time mixed correction experiment also suggested decreased coagulation factor activity.Clinically,the patient was initially diagnosed with hematuria,RA,and vitamin K-dependent coagulation factor deficiency.The patient received daily intravenous administration of vitamin K120 mg,etamsylate 3 g,and vitamin C 3000 mg for 10 d.Concurrently,oral leflunomide tablets and prednisone were administered for treatment of RA.After the treatment,the patient's symptoms improved markedly and she was discharged on day 12.There were no hemorrhagic events during 18 mo of follow-up.CONCLUSION RA can result in vitamin K-dependent coagulation factor deficiency,which leads to acquired coagulation dysfunction.Vitamin K1 supplementation has an obvious effect on coagulation dysfunction under these circumstances.

Factor XII (Hageman Factor) Deficiency: A Very Rare Coagulation Disorder

Background: Factor XII (Hageman Factor) is the initiating factor for the Intrinsic Pathway of Coagulation. Very low levels of Factor XII have been associated with increased levels of activated Partial Thromboplastin Time (aPTT). Association of Factor XII deficiency is more with thromboembolic disorders rather than bleeding tendencies. Aim: To learn more about the relationship of factor XII (Hageman Factor) deficiency and high levels of activated Partial Thromboplastin Time. Case Presentation: The Patient was admitted with complains of recurrent headaches and loin pain. Patient was incidentally found to have prolonged activated Partial Thromboplastin Time. This led to investigations which ultimately provided the evidence of severely low levels of Hageman Factor. Conclusion: Hageman Factor deficiency causes prolonged activated Partial Thromboplastin Time. However, most of the patients are asymptomatic for many years despite Hageman Factor deficiency.

MT_1-MMP和Factor Ⅷ在人脑胶质瘤中表达差异及其意义

目的探讨膜型基质金属蛋白酶-1(MT1-MMP)和FactorⅧ在人脑胶质瘤中的表达及两者之间的关系。方法用免疫组织化学SP法检测45例人脑胶质瘤组织和10例正常人脑组织中MT1-MMP和FactorⅧ的表达。结果正常人脑组织中无MT1-MMP表达,高级别脑胶质瘤组织(Ⅲ、Ⅳ)中MT1-MMP和FactorⅧ的阳性表达率显著高于低级别胶质瘤组织(Ⅰ、Ⅱ),并且两者的表达呈显著正相关性。结论MT1-MMP在高级别脑胶质瘤组织中高表达,其表达与脑胶质瘤的进展和侵袭密切相关,可作为脑胶质瘤恶性表型的有用指标。MT1-MMP可能在脑胶质瘤的血管生成中发挥重要的调控作用。

Ki-67和Factor Ⅷ在人脑胶质瘤中表达的相互关系及意义

目的探讨Ki-67和FactorⅧ在人脑胶质瘤中的表达及两者之间的关系。方法用免疫组织化学S-P法检测38例人脑胶质瘤组织和7例正常人脑组织中Ki-67和FactorⅧ的表达。结果正常人脑组织中无Ki-67表达,高度恶性胶质瘤(Ⅲ～Ⅳ级)中Ki-67和FactorⅧ的阳性表达率显著高于低级别胶质瘤组织(I～Ⅱ级),并且两者的表达呈显著正相关性。结论Ki-67在恶性胶质瘤组织中高表达,与胶质瘤的进展和侵袭密切相关,可作为胶质瘤恶性表型的有用指标。Ki-67可能在胶质瘤的血管生成中发挥重要的调控作用。

Milk fat globule epithelial growth factorⅧ(MFG-E8)sustains survival of cancer cells by prompting tumor angiogenesis and suppressing host immunities

Milk fat globule epithelial growth factor VIII(MFG-E8) is a novel adhesion protein mainly produced by macrophages and dendritic cells; it is expressed in most of the human tissues and functions to prompt cancer progression and survival. MFG-E8 contains a signal sequence for secretion, two epidermal growth factor(EGF)-like domains at the NH2 terminus and two discoidin domains with blood-clotting factor V/factor Ⅷ(C1 and C2) at the COOH terminus. The second EGF domain contains an arginine-glycine-aspartic(RGD) integrin-binding motif that engages α_vβ_5 integrins to facilitate cell adhesion and induce integrinmediated signal transduction. Integrin α_vβ_3 associates with VEGF receptor 2, engagement of integrins can promote angiogenesis, which plays key roles in growth, proliferation, and survival of cancer cells. VEGF stimulates the expression of α_vβ_3 and α_vβ_5 integrins on angiogenic vasculature, thereby potentiating effects of VEGF receptor engagement. Mice expressing a mutant form of α_vβ_3 integrin are unable to undergo tyrosine phosphorylation, confirming the important role that this integrin plays in pathological angiogenesis and providing important mechanistic insights. The C-terminus discoidin-like domains promote binding to membrane phospholipids, functioning close to VEGF like angiogenesis. MFG-E8 is an opsonin for apoptotic cells, and it acts as a bridging protein between apoptotic cells and phagocytes. It also influences cell immunities by altering CD4^+ and/or CD8^+ cells. Antibody or small peptide works with MFG-E8 at different functional sites or interacts with EGF-like domains and/or discoidin-like domains may play an important role in anti-angiogenesis or immune restoration. Altering the structures and/or functions of MFG-E8 and/or its domains is promising for development of novel anti-cancer strategies.

Change of Coagulation Factor Ⅷ and Antithrombin Ⅲ Activity in Bank-Stored Blood

Coagulation factor Ⅷ and antithrombin Ⅲ activity were detected in 15 health donors. It was found that antithrombin Ⅲ activity decreased obviously 12 h after blood drawing. It lost 56 % of the activity at the 3rd day, and 70 % of the activity at the 7th day. FⅧ:c showed no obvious change after 24 h, until the 3rd day. It lost 40 %-60 % of the activity after 36 h and was reduced to the 30 % of the original activity at the 5th day. Our results suggested that at the 3rd day coagulation factor Ⅷ of bank stored blood can be used to replenish antithrombin Ⅲ, while bank stored blood in one day can be used to replenish FⅧ.

Nutrients Deficiency and Excess Phenomena of Cut Rose and Their Physiological Significances

On the basis of a brief introduction of essential major dements and trace dements for cut rose, the physiological effect and deficiency and excess phenomena of major elements including nitrogen, phosphorus, potassium, magnesium, calcium, sulfur and trace dements including zinc, molybdenum, boron, iron, manganese, copper were mainly described; the main factors influencing nutrients absorption of cut rose were summarized, in order to provide a reference for the reasonable fertilization of cut rese.

血友病A患者凝血因子Ⅷ抑制物产生机制及相关遗传因素研究进展

血友病A是一种发病率为1/5000的出血性疾病,目前主要的治疗方式仍为替代治疗,治疗过程中产生凝血因子Ⅷ(FⅧ)的中和性抗体(FⅧ抑制物)是本病最大的并发症之一,将大大影响疾病治疗效果。抑制物的产生是多因素共同作用的结果,包括遗传因素和非遗传因素,遗传因素是主要因素,非遗传因素为次要因素。遗传因素包括FⅧ基因突变、免疫应答基因多态性、种族、家族史、血型。本文综述血友病A患者FⅧ抑制物产生机制及相关遗传因素的最新进展。

Growth Hormone Replacement Therapy in Patients without Adult Growth Hormone Deficiency: What Answers Do We Have So Far?

Growth hormone (GH) and insulin-like growth factor 1 (IGF-1) have been suggested as “anti-aging” therapies, or for improving quality of life with aging. In this study, we focus on the actions of GH in the main organs and organ systems of the human body, like skeletal muscle, bones and brain, particularly in regard to data and research on the use of GH replacement therapy in adults without growth hormone deficiency, especially elderly patients. Several different studies have been carried out to show what the effects and side effects of GH replacement in healthy people and what would be the impact in quality of life and life span. In this review, we demonstrate what answers we have so far about the effects of GH replacement in many organs and systems in healthy people.