Humans ; Myocardial Infarction/complications* ; Thrombophilia/complications*

This article summarizes the pathogenesis of hypercoagulability in β thalassemia patients, including platelet activation, alteration of red blood cell membranes, abnormal expression of adhesion molecules on vascular endothelial cells and iron overload. Clinical evidence, clinical manifestations of hypercoagulable state and thrombosis in β thalassemia and the effect of splenectomy on hypercoagulable state were reviewed. Strategies to prevent and treat the thromboembolic events in β-thalassemia intermedia are also discussed, including transfusion therapy to raise hemoglobin levels, avoidance or delay of splenectomy and a number of treatments in the exploration.
Erythrocyte Aggregation ; Humans ; Thrombophilia ; etiology ; prevention & control ; beta-Thalassemia ; complications

OBJECTIVES: To analyze the risk factors for postoperative deep vein thrombosis (DVT) in neurosurgical patients to provide the basis for the prevention of postoperative DVT. METHODS: A total of 141 patients underwent neurosurgery were enrolled. Thrombelastography (TEG) test was performed before and at the end of surgery. According to whether there was DVT formation after operation, the patients were divided into a thrombosis group and a non-thrombosis group. -test and rank sum test were used to compare the general clinical characteristics of the 2 groups, such as age, gender, intraoperative blood loss, -dimer, intraoperative crystal input, colloid input, blood product transfusion, operation duration, length of postoperative hospitalization. The application of chi-square test and rank-sum test were used to compared TEG main test indicators such as R and K values between the 2 groups. Logistic regression was used to analyze the possible risk factors for postoperative DVT in neurosurgical patients. RESULTS: There were significant differences in postoperative TEG index R, clotting factor function, intraoperative blood loss, hypertension or not, length of postoperative hospital stay, and postoperative absolute bed time (all <0.05). Logistic regression analysis showed hypercoagulability, more intraoperative blood loss and longer postoperative absolute bed time were risk factors for DVT formation after craniotomy. CONCLUSIONS Hypercoagulability in postoperative TEG test of patients is an important risk factor for the formation of postoperative DVT after neurosurgery, which can predict the occurrence of postoperative DVT to some extent.
Craniotomy ; adverse effects ; Humans ; Postoperative Complications ; epidemiology ; Postoperative Period ; Risk Factors ; Thrombophilia ; Venous Thrombosis ; epidemiology ; etiology

OBJECTIVE: To investigate the coagulation function indicators and identify influence factors of hypercoagulability in patients with adrenocorticotropic hormone (ACTH) independent Cushing syndrome (CS). METHODS: In our retrospective study, the electronic medical records system of Peking University First Hospital was searched for the patients diagnosed with ACTH independent CS on discharge from January 2014 to June 2019. Nonfunctional adrenal adenoma patients were chosen as control group and matched 1 ∶1 by body mass index (BMI), gender, and discharge date. Clinical features and coagulation function indicators were compared between the two groups. RESULTS: In the study, 171 patients were included in each group. Compared with control group, activated partial thromboplastin time (APTT), and prothrombin time (PT) in ACTH independent CS group were significantly lower [(29.22±3.39) s vs. (31.86±3.63) s, P < 0.001; (29.22±3.39) s vs. (31.86±3.63) s, P < 0.001], and both D-dimer and fibrin degradation products (FDP) levels were significantly higher (P < 0.05). Percentage of APTT levels under the lower limit of reference range in the CS patients was significantly higher than that in nonfunctional group (21.6% vs. 3.5%, P < 0.001). Percentage of D-dimer levels over the upper limit of reference range in the CS patients was significantly higher than that in nonfunctional group (13.5% vs. 6.6%, P=0.041). There were three patients with deep venous thrombosis and one patient with pulmonary embolism in CS group, however none was in control group. The area under curve (AUC) of serum cortisol rhythm (8:00, 16:00 and 24:00) levels was negatively associated with the levels of PT (r=-0.315, P < 0.001) and APTT (r=-0.410, P < 0.001), and positively associated with FDP (r=0.303, P < 0.001) and D-dimer levels (r=0.258, P < 0.001). There were no differences in coagulation function indicators among different histopathologic subgroups (adrenocortical adenoma, adrenocortical hyperplasia, oncocytic adenoma, adrenocortical carcinoma). With Logistic regression analysis, the AUC of cortisol and glycosylated hemoglobin A1c (HbA1c) levels were independent risk factors for hypercoagulability in the ACTH independent CS patients (P < 0.05). CONCLUSION ACTH independent CS patients were more likely in hypercoagulable state compared with nonfunctional adrenal adenoma, especially in ACTH independent CS patients with higher levels of cortisol AUC and HbA1c. These patients should be paid attention to for the hypercoagulability and thrombosis risk.
Humans ; Cushing Syndrome/complications* ; Adrenocortical Adenoma/complications* ; Adrenocorticotropic Hormone ; Hydrocortisone ; Retrospective Studies ; Glycated Hemoglobin ; Adrenal Cortex Neoplasms/diagnosis* ; Adenoma/diagnosis* ; Thrombophilia/complications*

INTRODUCTIONPregnancies in women with thrombophilia are associated with a higher risk of obstetric complications. We systematically reviewed the findings of relevant randomised controlled trials (RCTs) with the aim of investigating the effectiveness of low-molecular-weight heparins (LMWHs) in pregnant women with inherited thrombophilic disorders and its effect on the incidence of live births in these patients.

METHODSThe MEDLINE-PubMed and Cochrane CENTRAL databases from 2000 to 2010 were searched using a combination of keywords, including low-molecular-weight heparin, enoxaparin, pregnancy, live birth and thrombophilia. Studies were included if they were RCTs assessing the effect of anticoagulant treatment on live birth rates in women with a history of miscarriage without apparent causes other than thrombophilic disorder. Interventions included LMWH, with or without aspirin, aspirin alone or placebo controlled for the prevention of adverse pregnancy outcome.

RESULTS43 articles with seven RCTs were retrieved following the initial search, of which four studies had to be excluded as they assessed thromboembolic events as the final outcome (n = 1), focused on idiopathic recurrent miscarriages (n = 1), compared efficacy and safety of two doses of enoxaparin (n = 1), and examined patients with or without thrombophilic disorder (n = 1). Pooled data from the remaining three RCTs showed no significant difference in the improvement of live birth rates following LMWH interventions (p = 0.15).

CONCLUSIONAt present, the use of LMWH in women with inherited thrombophilia with recurrent pregnancy loss is not indicated. Large randomised placebo-controlled trials are further needed to prove the effectiveness of LMWH in these patients.

Female ; Heparin, Low-Molecular-Weight ; therapeutic use ; Humans ; Pregnancy ; Pregnancy Complications, Hematologic ; drug therapy ; Pregnancy Outcome ; Thrombophilia ; complications ; drug therapy ; Treatment Outcome

The prevalences of deficiencies in antithrombin III (AT III), protein C (PC), protein S (PS) and in the activated protein C (APC) resistance in the thrombotic population of the Trakya region, Turkey were investigated. 37 patients with venous thrombosis (VT) and 17 patients with arterial thrombosis (ArT) were included in this study. The mean ages of the patients with VT and ArT were 46 years (range 20-70) and 38 years (range 32-40), respectively. The activity of AT III was measured by commercially available immuno-turbidimetric assay. The activities of PC and PS were determined by coagulometric assay. The APC resistance was measured using a modified APTT-based clotting assay. Among the VT patients, there were 2 cases (5.4%) with AT III, 5 (13.51%) with PC deficiency, 5 (13.51%) with PS deficiency and 2 (5.4%) with APC resistance. In the ArT patient group, there was 1 patient (5.88%) with AT III, 3 (17.64%) with PC deficiency, 1 (5.88%) with PS deficiency and no APC resistant patients, while there was one (2.08%) with PC deficiency and one (2.08%) with APC resistance in the control group (49 persons, mean age 41 years). The relative risk of thrombosis (odds ratio) was 1.7 in the deficiency of PC and 5.6 in the deficiency of PS. The data presented suggests that the prevalences of AT III, PC and PS deficiencies causing thrombophilia in the Trakya region of Turkey are higher than in other reported studies while the APC resistance is lower than in others. Further studies including more patients would be required to clarify these discrepancies.
Activated Protein C Resistance/complications ; Adult ; Antithrombin III Deficiency/complications ; Human ; Middle Age ; Prevalence ; Protein C Deficiency/complications ; Protein S Deficiency/complications ; Risk Factors ; Thrombophilia/epidemiology* ; Thrombosis/etiology ; Turkey/epidemiology

Diabetic ketoacidosis (DKA), a fatal acute diabetic complication, is characterized by severe metabolic decompensation and intravascular volume depletion. These conditions may result in hypercoagulability and prothrombic state. Pulmonary thromboembolism (PTE) could be presented as an uncommon and life-threatening complication of DKA. Reported herein is a case involving a 54-year-old male patient who was admitted with DKA due to chronic alcohol consumption and stopping the intake of oral antidiabetic drugs. After low-molecular-weight heparin and warfarin treatment because of PTE during the DKA treatment, the patient's condition improved over the week that he was discharged on insulin and warfarin.
Alcohol Drinking ; Anticoagulants ; Diabetes Complications ; Diabetic Ketoacidosis* ; Heparin, Low-Molecular-Weight ; Humans ; Hypoglycemic Agents ; Insulin ; Male ; Middle Aged ; Pulmonary Embolism* ; Thrombophilia ; Warfarin

BACKGROUND: Patients undergoing brain surgery have a high risk of developing a number of perioperative coagulation disorders. Anesthesia and surgical stress may affect blood coagulation and fibrinolysis. The aim of this study was to evaluate perioperative changes in hemostatic parameters of patients undergoing clipping of cerebral aneurysms with a thromboelastograph (TEG) in combination with simple laboratory tests. METHODS: Twenty adult patients who had cerebral aneurysms and no history of coagulation disorders were studied. Isoflurane and N2O were used for all anesthetic proceedings. Preanesthetic, intraoperative (after skin incision and after clipping of cerebral aneurysms) and postanesthetic measurements included a TEG and simple laboratory tests. The TEG variables included r time (reaction time for clot formation), k time (clot formation time), alpha angle (rate of clot growth), MA (maximal amplitude of clot strength) and LY30 (fibrinolytic index). RESULTS: In simple laboratory tests, prothrombin time (PT) and partial thromboplastin time (PTT) at intraoperation and postanesthesia were longer than those at preanesthesia (p < 0.05). In the TEG, r and k time at intraoperation and postanesthesia were shorter than those at preanesthesia (p < 0.05). However the alpha angle at intraoperation and postanesthesia was longer than that at preanesthesia (p < 0.05). There was no significant difference in MA and LY30 except an increase in MA after the skin incision (p < 0.05) compared to the MA at preanesthesia. CONCLUSIONS: These results indicate a general hypercoagulability during and after a cerebral aneurysms operation in terms of TEG, although, the level of the PT and PTT can be at the upper limits within normal. Therefore perioperative use of coagulants in cerebral aneurysms may increase the risk of a thromboembolism because of accelerating blood coagulability. By early intraoperative and postoperativeevaluation of the hemostatic abnormality with a TEG, appropriate measures might be initiated to prevent postoperative complications due to hypercoagulability.
Adult ; Anesthesia ; Blood Coagulation* ; Brain ; Coagulants ; Fibrinolysis ; Humans ; Intracranial Aneurysm* ; Isoflurane ; Partial Thromboplastin Time ; Postoperative Complications ; Prothrombin Time ; Skin ; Thromboembolism ; Thrombophilia

Chronic thromboembolic pulmonary hypertension (CTEPH) is considered to be an aberrant outcome of acute pulmonary thromboembolism, due to inadequate thrombus dissolution. However, the mechanism of thrombi dissolution failure remains unclear. With respect to inherited thrombophilia, the co-occurrence of natural anticoagulant deficiencies with CTEPH was found to be rare. Pulmonary thromboendarterectomy (PTE) is a potentially curative surgical procedure for CTEPH, but it is associated with considerable mortality due to postoperative complications, such as reperfusion pulmonary edema and right heart failure. The postoperative course after PTE poses a unique series of ventilatory care and hemodynamic management challenges. We present the case of a 42-year-old woman with unilateral CTEPH combined with thrombophilia (Protein S deficiency). Successful PTE was followed by independent lung ventilation with unilateral nitric oxide (NO) inhalation, which resulted in functional improvement without postoperative complications.
Adult ; Endarterectomy* ; Female ; Heart Failure ; Hemodynamics ; Humans ; Hypertension, Pulmonary* ; Inhalation ; Lung ; Mortality ; Nitric Oxide ; Postoperative Complications ; Protein S Deficiency ; Pulmonary Edema ; Pulmonary Embolism ; Reperfusion ; Thrombophilia ; Thrombosis ; Ventilation

Thrombophilia refers to inherited or acquired hemostatic disorders that result in a predisposition to blood clot formation. When combined with the hypercoagulable state that is characteristic of pregnancy, there is an increased risk of severe and recurrent pregnancy complications. Activated protein C resistance caused by factor V Leiden (FVL) mutation is known to be the most common cause of inherited thrombophilia in Caucasian population. FVL mutation has been related to pregnancy complications associated with hypercoagulation, e.g. miscarriage, intrauterine fetal demise, placental abruption, and intrauterine growth retardation. Although the FVL mutation is easily detected using molecular DNA techniques, patients who are heterozygous for this disorder often remain asymptomatic until they develop a concurrent prothrombotic condition. Because there are potentially serious effects of FVL mutation for pregnancy, and because effective treatment strategies exist, early detection and treatment of this condition might be considered.
Abortion, Spontaneous ; Abruptio Placentae ; Activated Protein C Resistance ; DNA ; Factor V ; Female ; Fetal Death ; Fetal Growth Retardation ; Hemostatic Disorders ; Humans ; Pregnancy Complications ; Pregnancy ; Pregnant Women ; Thrombophilia