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.

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.

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.

Necrotizing Fasciitis in the Puerperium of a Woman with Complement Deficiency: Case Report and Review Literature

Complement deficiencies are uncommon types of primary immunodeficiency. Necrotizing fasciitis is a rare complication in pregnancy characterized by soft tissue invasion and necrosis of the subcutaneous and other adjacent tissues, leading to high mortality rates. We report a case of a 29-year-old pregnant woman with functional deficiency of the C4 complement component and short uterine cervix. Admitted at the hospital with preterm labor, she received multiple doses of immunoglobulin. After 8 weeks, she had a premature membrane rupture, and due to pelvic presentation she had a cesarean. The patient presented multiple obstetric complications, such as operative wound infection, endometritis, sepsis, necrotizing fasciitis and pelvic septic thrombophlebitis. She underwent multiple antimicrobial schemes, a hysterectomy and 4 extensive debridements of the abdominal wall because of significant necrosis. She stayed at the hospital for 101 days (32 of those in ICU in immediate postpartum). 41 days after cesarean, patient was discharged in good conditions. Our case emphasizes individual handling and high multiple doses of immunoglobulin for favorable outcome of the case.

Folate level and prevalence of folate deficiency in population from agriculture and stock-raising regions and its correlation with hypertension

Objective:To compare the plasma folate level,prevalence of folate deficiency and the related influencing factors of hypertension in Xinjiang agricultural and pastoral areas.Methods:We enrolled 1926 study subjects with 447 hypertensive and non-hypertensive 1479 ones aged≥15 years old using multi-stage stratified sampling in Emin Xinjiang between January and December 2014,conducted the health behavior questionnaire and physical examination,and collected blood samples.The plasma folate level were measured by chemiluminescence method.Results:(1)There were significant differences(P<0.05)in age,gender,ethnic composition,marital status,education level,smoking consumption,drinking data,overweight and obesity and hyperhomocysteinemia(HHcy)between hypertension group and non hypertension group.(2)The folate deficiency rate of hypertension group was higher than that of non hypertension group(P=0.003);the folate deficiency rate of systolic and diastolic hypertension was the highest(P=0.008);the folate level of hypertension with HHcy was the lowest(P<0.001)and the folate deficiency rate was the highest(P=0.023).(3)Partial correlation analysis showed that folate was negatively correlated with systolic blood pressure and pulse pressure(P<0.05).(4)Multivariate logistic regression analysis showed that age 45-59(OR=3.78,95%CI:2.45-5.82),age≥60(OR=6.87,95%CI:4.35-5.10.87),male gender(OR=6.96,95%CI:3.86-12.54),Kazakhs(OR=2.12,95%CI:1.52-2.96),Mongolian(OR=2.38,95%CI:1.25-4.53),smoking(OR=4.21,95%CI:2.05-8.63),drinking(OR=6.36,36%CI:3.00-13.48),overweight and obesity(OR=2.11,95%CI:1.58-2.81),and folate deficiency(OR=1.72,95%CI:1.05-2.80)were the related factors of hypertension.Conclusions:The prevalence of folate deficiency in the population with hypertension in Xinjiang is higher than that in the non hypertension group,and the highest prevalence of folate deficiency is in hypertension with HHcy.Therefore,folate supplementation in the prevention of hypertension may be helpful,especially for the elderly,men and Kazakhs,and at the same time,healthy life style has an important reference value for reducing the level of hypertension and preventing and controlling cardiovascular diseases.

七味白术散治疗老年肺炎肺脾气虚证的疗效及对胃肠功能的影响

目的 观察七味白术散对老年肺炎肺脾气虚证的疗效及对胃肠功能的影响。方法 将60例老年肺炎肺脾气虚证患者按照随机数字表法分为2组,对照组30例予常规基础治疗,治疗组30例在对照组治疗基础上予七味白术散加减治疗。2组均治疗2周。比较2组治疗前后中医证候评分、外周血炎症因子[白细胞介素6(IL-6)、白细胞计数(WBC)、C反应蛋白(CRP)、降钙素原(PCT)]水平、胃肠功能指标(腹内压、肠鸣音次数)及肠道菌群情况,并统计2组疗效及安全性。结果 治疗组总有效率93.33%(28/30),对照组总有效率73.33%(22/30),治疗组临床疗效优于对照组(P<0.05)。2组治疗后中医证候各项评分均较本组治疗前降低(P<0.05),且治疗组治疗后均低于对照组(P<0.05)。2组治疗后IL-6、WBC、CRP、PCT水平均较本组治疗前降低(P<0.05),且治疗组治疗后均低于对照组(P<0.05)。2组治疗后腹内压均较本组治疗前降低(P<0.05),肠鸣音次数均增加(P<0.05),且治疗组治疗后腹内压低于对照组(P<0.05),肠鸣音次数高于对照组(P<0.05)。2组治疗后肠道乳酸杆菌、双歧杆菌菌落数均较本组治疗前增加(P<0.05),肠球菌、肠杆菌菌落数均降低(P<0.05),且治疗组治疗后乳酸杆菌、双歧杆菌菌落数均高于对照组(P<0.05),肠球菌、肠杆菌菌落数均低于对照组(P<0.05)。2组治疗期间未出现肝肾功能异常。结论 七味白术散治疗老年肺炎肺脾气虚证疗效显著,可有效降低炎症因子水平,调节肠道菌群,改善胃肠功能,安全性高。

HIV/AIDS肺脾气虚证相关心脑血管疾病风险因素探析

目的通过检测血脂四项、血管内皮损伤因子、炎症相关因子,并结合前期的基因芯片结果,初步探讨人类免疫缺陷病毒(HIV)/艾滋病(AIDS)肺脾气虚证相关心脑血管疾病风险因素及机制,为中医药早期干预心脑血管疾病提供依据。方法2020年9月—2020年11月选取河南省某地区确诊的20例HIV/AIDS肺脾气虚证患者作为研究对象,同地区20例HIV抗体阴性作为健康对照组。检测CD_(3)^(+)、CD_(4)^(+)、CD_(8)^(+)计数,并计算CD_(4)^(+)/CD_(8)^(+)比值;检测血脂四项甘油三酯(TG)、胆固醇(TC)、高密度脂蛋白胆固醇(HDL-C)、低密度脂蛋白胆固醇(LDL-C);检测氧化低密度脂蛋白(Ox-LDL)、载脂蛋白A-I(ApoAⅠ)、载脂蛋白A-Ⅱ(ApoAⅡ)、载脂蛋白B(ApoB)、锌-α2-糖蛋白1(AZGP1)、可溶性血栓调节蛋白(sTM)、血管性血友病因子(vWF)、脂联素、瘦素(LEP)、超敏C反应蛋白(Hs-CRP)、单核细胞趋化因子1(MCP-1)、肿瘤坏死因子α(TNF-α)。结果HIV/AIDS肺脾气虚证患者与健康对照组比较CD_(4)^(+)降低,CD_(4)^(+)/CD_(8)^(+)比值倒置,TC降低,sTM、AZGP1、Ox-LDL、Hs-CRP升高。结论HIV/AIDS肺脾气虚证患者存在炎性反应、血脂异常的表现,罹患心脑血管疾病风险增加。为中医药早期干预心脑血管疾病的发生提供新思路。

针药并用治疗气阴两虚兼瘀型糖尿病性勃起功能障碍的疗效观察及对血管内皮功能及相关生长因子的影响

目的 观察针刺联合益气养阴活血方治疗气阴两虚兼瘀型糖尿病性勃起功能障碍的临床疗效及对血管内皮功能及相关生长因子的影响。方法 将90例气阴两虚兼瘀型糖尿病性勃起功能障碍患者随机分为治疗组和对照组,每组45例。对照组予糖尿病基础治疗及他达拉非片口服治疗,治疗组在对照组基础上采用针刺联合益气养阴活血方。比较两组国际勃起功能指数-5(international index of erectile function-5, IIEF-5)评分和中医证候积分变化,比较两组血清内皮素-1(endothelin-1, ET-1)、血管紧张素-Ⅱ(angiotensin Ⅱ, Ang-Ⅱ)、一氧化氮(nitric oxide, NO)和血管活性肠肽(vasoactive intestinal peptide, VIP)含量变化,比较两组血清内皮生长因子(vascular endothelial growth factor, VEGF)、转化生长因子-β(transforming growth factor-β, TGF-β)、成纤维细胞生长因子-21(fibroblast growth factor 21, FGF-21)和胰岛素样生长因子-1(insulin-like growth factor 1, IGF-1)含量变化,并比较两组临床疗效。结果 治疗后,两组IIEF-5评分高于治疗前(P<0.05),对照组勃起无力、腰膝酸软、阴部刺痛及中医证候积分总分低于治疗前(P<0.05),治疗组中医证候各项积分及总分低于治疗前(P<0.05);且治疗组IIEF-5评分高于对照组(P<0.05),治疗组中医证候各项积分及总分低于对照组(P<0.05)。治疗后,两组血清ET-1和AngⅡ低于治疗前,NO和VIP高于治疗前(P<0.05);且治疗组血清ET-1和AngⅡ低于对照组,NO和VIP高于对照组(P<0.05)。治疗后,两组血清VEGF和IGF-1高于治疗前,TGF-β和FGF-21低于治疗前(P<0.05);且治疗组血清VEGF和IGF-1高于对照组,TGF-β和FGF-21低于对照组(P<0.05)。治疗组总有效率为90.7%,高于对照组的73.8%(P<0.05)。结论 在西药治疗的基础上,针刺联合益气养阴活血方能够改善气阴两虚兼瘀型糖尿病性勃起功能障碍患者临床症状,提高治疗效果,可能与改善血管内皮功能、调节相关生长因子有关。