Epidural Hematoma in Minor Hepatic Metastasectomy.

Liver resection poses many challenges for the anesthesiologist, including intraoperative hemodynamic instability, postoperative pain, and risk of coagulopathy. We report a case of epidural hematoma after epidural catheter removal, following a minor liver single metastasectomy. The main purpose of this case report is to bring to light the false security provided by traditional coagulation parameters and whether further investigation should be considered in selected cases, before handling neuraxial catheters. Alterations in coagulation after a partial hepatectomy remain poorly understood; thus, we believe that additional hemostatic values such as viscoelastic testing might be considered to better assess these patients.

Contemporary tools for evaluation of hemostasis in neonates. Where are we and where are we headed?

The assessment of hemostatic disorders in neonates is crucial, but remains challenging for clinicians. Although the concept of developmental hemostasis is widely accepted among hemostasis specialists globally, it is probably under-recognized by clinicians and laboratory practitioners. In parallel with age-dependent hemostatic status maturation, comprehension of the differences between normal values is crucial for the accurate diagnosis of potential hemorrhagic and thrombotic disorders of the vulnerable neonatal population. This review outlines the basics of developmental hemostasis and the features of the available coagulation testing methods, with a focus on novel tools for evaluating the neonatal hemostatic profile. Common errors, issues, and pitfalls during the assessment of neonatal hemostasis are discussed, along with their impact on patient management. Current knowledge gaps and research areas are addressed. Further studying to improve our understanding of developmental hemostasis and its reflection on everyday clinical practice is warranted.

Evaluation of a diagnostic platelet aggregation test strategy for platelet rich plasma samples with low platelet counts.

INTRODUCTION: Light transmission aggregometry (LTA) is important for diagnosing platelet function disorders (PFD) and von Willebrand disease (VWD) affecting ristocetin-induced platelet aggregation (RIPA). Nonetheless, data is lacking on the utility of LTA for investigating thrombocytopenic patients and platelet rich plasma samples with low platelet counts (L-PRP). Previously, we developed a strategy for diagnostic LTA assessment of L-PRP that included: (1) acceptance of referrals/samples, regardless of thrombocytopenia severity, (2) tailored agonist selection, based on which are informative for L-PRP with mildly or severely low platelet counts, and (3) interpretation of maximal aggregation (MA) using regression-derived 95% confidence intervals, determined for diluted control L-PRP (C-L-PRP). METHODS: To further evaluate the L-PRP LTA strategy, we evaluated findings for a subsequent patient cohort. RESULTS: Between 2008 and 2021, the L-PRP strategy was applied to 211 samples (11.7% of all LTA samples) from 192 unique patients, whose platelet counts (median [range] × 109 /L) for blood and L-PRP were: 105 [13-282; 89% with thrombocytopenia] and 164 [17-249], respectively. Patient-L-PRP had more abnormal MA findings than simultaneously tested C-L-PRP (p-values <0.001). Among patients with accessible electronic medical records (n = 181), L-PRP LTA uncovered significant aggregation abnormalities in 45 (24.9%), including 18/30 (60%) with <80 × 109 platelets/L L-PRP, and ruled out PFD, and VWD affecting RIPA, in others. The L-PRP LTA strategy helped diagnose VWD affecting RIPA, Bernard Soulier syndrome, familial platelet disorder with myeloid malignancy, suspected ITGA2B/ITGB3-related thrombocytopenia, and acquired PFD. CONCLUSION: Diagnostic LTA with L-PRP, using a strategy that considers thrombocytopenia severity, is feasible and informative.

[Hemostatic disorders in neurosurgical patients: diagnostics and correction]. / Narusheniya sistemy gemostaza u neirokhirurgicheskikh bol'nykh: diagnostika i korrektsiya.

The authors analyzed the main causes of perioperative hemostatic disorders in neurosurgical patients. The problem of preoperative hemostatic screening, intraoperative and postoperative factors contributing to hemostatic disorders are considered. The authors also discuss the methods for correction of hemostatic disorders.

Reducing use of coagulation tests in a family medicine practice setting: An implementation study.

Introduction: Clinicians often order the international normalized ratio (INR) and activated partial thromboplastin time (APTT) to evaluate for the possibility of inherited bleeding disorders despite sensitivities and specificities of 1%-2%. The most accurate tool to evaluate for bleeding disorders is a validated bleeding assessment tool (BAT). Our aim was to reduce coagulation testing by >50% in a large family practice in Ontario, Canada. Methods: We conducted an implementation study from May 2016 to February 2020. Iterative interventions included introduction of a validated BAT into the electronic medical record (EMR); removal of the APTT as a prepopulated selection from the laboratory requisition; and education targeting family medicine teams and laboratory personnel. The primary outcome was the rate of pre- and post-APTT testing. Creatinine testing was the control. Data were analyzed via an interrupted time series analysis using Stata 13. Results: Immediately following education of the laboratory personnel on coagulation testing, the APTT rate level dropped by 1.26 tests per 100 patient visits per month (p < 0.001) and was sustained until the end of the study. Meanwhile, the PT/INR and creatinine testing rate levels did not change (rate level = -0.02 per 100 visits per month, p = 0.79 and 0.49, p = 0.22 respectively). There was good uptake of the BAT following integration and 18/88 (20%) obtained a referral to hematology after BAT completion. Conclusions: Multidisciplinary, iterative interventions reduced APTT testing and enabled the use of BATs to guide hematology referrals in a large family practice.

Comments on "Neonatal infratentorial subdural hematoma contributing to obstructive hydrocephalus in the setting of therapeutic cooling: A case report".

Although therapeutic hypothermia (TH) contributes significantly in the treatment of hypoxic ischemic encephalopathy (HIE), it could result in devastating complications such as intracranial hemorrhages. Laboratory examinations for possible coagulation disorders and early brain imaging can detect all these cases that are amenable to aggravation of HIE after the initiation of TH.

Chronic Immune Platelet Activation Is Followed by Platelet Refractoriness and Impaired Contractility.

Autoimmune diseases, including systemic lupus erythematosus (SLE), have a high risk of thrombotic and hemorrhagic complications associated with altered platelet functionality. We studied platelets from the blood of SLE patients and their reactivity. The surface expression of phosphatidylserine, P-selectin, and active integrin αIIbß3 were measured using flow cytometry before and after platelet stimulation. Soluble P-selectin was measured in plasma. The kinetics of platelet-driven clot contraction was studied, as well as scanning and transmission electron microscopy of unstimulated platelets. Elevated levels of membrane-associated phosphatidylserine and platelet-attached and soluble P-selectin correlated directly with the titers of IgG, anti-dsDNA-antibodies, and circulating immune complexes. Morphologically, platelets in SLE lost their resting discoid shape, formed membrane protrusions and aggregates, and had a rough plasma membrane. The signs of platelet activation were associated paradoxically with reduced reactivity to a physiological stimulus and impaired contractility that revealed platelet exhaustion and refractoriness. Platelet activation has multiple pro-coagulant effects, and the inability to fully contract (retract) blood clots can be either a hemorrhagic or pro-thrombotic mechanism related to altered clot permeability, sensitivity of clots to fibrinolysis, obstructiveness, and embologenicity. Therefore, chronic immune platelet activation followed by secondary platelet dysfunction comprise an understudied pathogenic mechanism that supports hemostatic disorders in autoimmune diseases, such as SLE.

Acquired hemophilia A and delta storage pool deficiency in a patient with indolent non-Hodgkin lymphoma.

B-cell lymphoproliferative diseases may be associated with acquired hemostasis disorders, such as acquired hemophilia A (AHA) caused by autoantibodies that neutralize factor VIII activity, and δ-storage pool deficiency, an abnormality of platelet function due to defective dense granules and impaired secretion. We describe the case of a 67-year-old man in whom these two acquired bleeding disorders were concomitantly present as the first clinical manifestation of an indolent non-Hodgkin lymphoma. Immunosuppressive therapy with prednisone was initially started to eradicate anti-FVIII antibodies, subsequently boosted with cyclophosphamide and rituximab, these medications being also chosen to treat the associated indolent lymphoma. Bleeding symptoms were first tackled with limited benefit by using rFVIIa and then rescued using recombinant porcine FVIII. After a 6 month's follow-up lymphoma and AHA were in remission and platelet function was improved. This case underlines the need of multiple and complex diagnostic and therapeutic approaches to rare acquired bleeding disorders associated with lymphoproliferative diseases.

Camundongos como modelos de doenças hemostáticas

Mice initiated to be used for experimental purposes in laboratories in laboratories in the early 20 th century, simultaneously with the rediscover of Mendel’s Laws. Ever since, they are considered excellent models for genetic studies, as they have high biological similarity with humans -between 70 and 90%-, and have a genome easily handled by means of molecular techniques and breeding procedures. Furthermore, mice have a short gestation period, shorter life cycle in comparison to other animals and easy handling in laboratory facilities. Around the 1990s, genetically modified animals were produced as models for mimicking hemostatic diseases. These genetically modified animals showed hemostatic alterations characteristic of human diseases, as hemophilia A -caused by mutations in coagulation factor VIII-, hemophilia B -caused by mutations in coagulation factor IX- and von Willebrand disease -vWD- caused by mutations in von Willebrand factor-. In this context, the animal models of hemostatic disorders have become important tools for evaluating new treatments and studying rare disease conditions.

Os camundongos foram introduzidos nos laboratórios com fins experimentais no início do século XX juntamente com a redescoberta da Lei de Mendel. Desde então, são considerados excelentes modelos para o estudo de genética, por apresentarem alta similaridade biológica com o ser humano, compreendida entre 70 e 90% e um genoma facilmente manipulado por meio de técnicas moleculares e sistemas de acasalamentos. Além disso, os camundongos apresentam um período de gestação curto, seu ciclo de vida é menor se comparado a outros animais e são de fácil manuseio em laboratório. Por volta dos anos 1990, foi introduzida a utilização de animais geneticamente modificados como modelos para reprodução de doenças hemostáticas. Esses animais geneticamente modificados apresentavam alterações hemostáticas características de doenças humanas, como as hemofilias do tipo A -causada por mutações no fator VIII da coagulação-, hemofilia do tipo B -causada por mutações no fator IX da coagulação- e doença de von Willebrand -vWD- causada por mutações no fator de von Willebrand -vWF-. Os modelos de animais com distúrbios hemostáticos se tornaram importantes ferramentas para testar novas formas de tratamento e estudar condições raras das doenças.

The impact of prehospital tranexamic acid on mortality and transfusion requirements: match-pair analysis from the nationwide German TraumaRegister DGU®.

BACKGROUND: Outcome data about the use of tranexamic acid (TXA) in civilian patients in mature trauma systems are scarce. The aim of this study was to determine how severely injured patients are affected by the widespread prehospital use of TXA in Germany. METHODS: The international TraumaRegister DGU® was retrospectively analyzed for severely injured patients with risk of bleeding (2015 until 2019) treated with at least one dose of TXA in the prehospital phase (TXA group). These were matched with patients who had not received prehospital TXA (control group), applying propensity score-based matching. Adult patients (≥ 16) admitted to a trauma center in Germany with an Injury Severity Score (ISS) ≥ 9 points were included. RESULTS: The matching yielded two comparable cohorts (n = 2275 in each group), and the mean ISS was 32.4 ± 14.7 in TXA group vs. 32.0 ± 14.5 in control group (p = 0.378). Around a third in both groups received one dose of TXA after hospital admission. TXA patients were significantly more transfused (p = 0.022), but needed significantly less packed red blood cells (p ≤ 0.001) and fresh frozen plasma (p = 0.023), when transfused. Massive transfusion rate was significantly lower in the TXA group (5.5% versus 7.2%, p = 0.015). Mortality was similar except for early mortality after 6 h (p = 0.004) and 12 h (p = 0.045). Among non-survivors hemorrhage as leading cause of death was less in the TXA group (3.0% vs. 4.3%, p = 0.021). Thromboembolic events were not significantly different between both groups (TXA 6.1%, control 4.9%, p = 0.080). CONCLUSION: This is the largest civilian study in which the effect of prehospital TXA use in a mature trauma system has been examined. TXA use in severely injured patients was associated with a significantly lower risk of massive transfusion and lower mortality in the early in-hospital treatment period. Due to repetitive administration, a dose-dependent effect of TXA must be discussed.

Pathogenesis, Diagnosis, and Treatment of Hemostatic Disorders in COVID-19 Patients.

The novel coronavirus infection named COVID-19 was first detected in Wuhan, China, in December 2019, and it has been responsible for significant morbidity and mortality in scores of countries. At the time this article was being written, the number of infected and deceased patients continued to grow worldwide. Most patients with severe forms of the disease suffer from pneumonia and pulmonary insufficiency; in many cases, the disease is generalized and causes multiple organ failures and a dysfunction of physiological systems. One of the most serious and prognostically ominous complications from COVID-19 is coagulopathy, in particular, decompensated hypercoagulability with the risk of developing disseminated intravascular coagulation. In most cases, local and diffuse macro- and microthromboses are present, a condition which causes multiple-organ failure and thromboembolic complications. The causes and pathogenic mechanisms of coagulopathy in COVID-19 remain largely unclear, but they are associated with systemic inflammation, including the so-called cytokine storm. Despite the relatively short period of the ongoing pandemic, laboratory signs of serious hemostatic disorders have been identified and measures for specific prevention and correction of thrombosis have been developed. This review discusses the causes of COVID-19 coagulopathies and the associated complications, as well as possible approaches to their early diagnosis, prevention, and treatment.

Provider Attitudes and Practices Regarding Intrauterine System (IUS) Insertion in Adolescents With and Without Bleeding Disorders for Management of Heavy Menstrual Bleeding.

STUDY OBJECTIVE: Heavy menstrual bleeding (HMB) may be the sentinel event for identifying a patient with a bleeding disorder (BD). The levonorgestrel intrauterine system (LNG IUS) has been proposed as a treatment for HMB in adolescents with and without BDs; however, no standard protocols for LNG IUS insertion in these populations exist. Providers were surveyed regarding the use of the LNG IUS in adolescents with HMB, with and without BD. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: An institutional review board-approved survey assessing provider attitudes, LNG IUS insertion practices, and patient outcomes in adolescents with HMB, with and without BD, was electronically distributed to 3523 providers in the fields of hematology, adolescent medicine, and obstetrics and gynecology. Descriptive analysis was performed. RESULTS: A total of 312 respondents across all 3 specialties completed the survey. Nearly 100% of respondents considered the LNG IUS safe and effective treatment for adolescents with HMB, both with and without BD. Additionally, 66% of providers chose LNG IUS as the ideal treatment for HMB in patients with BD. Differences were noted in clinical setting for LNG IUS insertion, peri-procedural medication use, and post-procedure follow-up among specialties. Providers across all specialties reported low complication rates related to IUS insertion and use in both patient groups. CONCLUSION: Providers considered the LNG IUS safe and effective treatment for HMB in adolescents with and without a diagnosed BD. Practice patterns regarding LNG IUS insertion in this population vary. Further research is necessary to explore IUS outcomes in adolescent patients with HMB, with and without BD, and to inform evidence-based protocols for LNG IUS insertion.

A case-control study indicates that coagulation imbalance is associated with arteriosclerosis and markers of endothelial dysfunction in kidney failure.

Endothelial dysfunction, one of many causes of arterial changes in end-stage kidney disease (kidney failure), is a likely link between early vascular aging and the risk of thrombosis or bleeding in this condition. To evaluate this, we compared links between arterial stiffness and endothelial/coagulation factors in 55 patients receiving hemodialysis therapy and 57 age-/sex-matched control individuals. Arterial stiffness was assessed from carotid-femoral pulse wave velocity, and coagulation status from the endogenous thrombin generating potential. Markers of endothelial dysfunction (von Willebrand factor, tissue factor pathway inhibitor), neutrophil extracellular traps and tissue factor-positive extracellular vesicles were higher in patients with kidney failure. Prothrombin fragments 1 and 2, and D-dimer markers of in vivo coagulation activation were also higher. However, in vitro in the presence of platelets, endogenous thrombin generating potential was lower and its downregulation by activated protein C impaired. Antiplatelet drugs did not affect these parameters. In multiple regression analysis, prothrombin fragments 1 and 2, D-dimer, factor VIII and monocyte-derived tissue factor-positive extracellular vesicles correlated with higher carotid-femoral pulse wave velocity. In patients with kidney failure, in vivo hypercoagulability occurred with reduced thrombin generation in platelet-rich plasma, likely explaining the opposing thrombotic and bleeding tendencies in patients with kidney failure. Importantly, arteriosclerosis is more closely related to a prothrombotic state. Thus, coagulation changes plus arterial stiffness highlight a major therapeutic challenge for anticoagulant and antiplatelet drug use.

Standardizing care to manage bleeding disorders in adolescents with heavy menses-A joint project from the ISTH pediatric/neonatal and women's health SSCs.

BACKGROUND: Bleeding disorders (BD) are under-recognized in adolescents with heavy menstrual bleeding (HMB). OBJECTIVES: The lack of clinical guidelines and variable symptomatic management of HMB created the imperative to standardize HMB care to identify and manage BD in adolescents. METHODS: We convened an international working group (WG), utilized the results of a literature review to define knowledge gaps in HMB care, and used the collective clinical experience of the WG to develop care considerations for adolescents with BD and HMB. We then solicited input on the appropriateness of HMB care considerations from expert stakeholders representing hematology, adolescent medicine, and obstetrics-gynecology. We conducted an expert panel online, using the ExpertLens platform. During a three-round online modified-Delphi process, the expert panel rated the appropriateness of 21 care considerations using a 9-point scale to designate care as appropriate (7-9), uncertain (4-6), or inappropriate (1-3) covering screening for BD, the laboratory work-up, and management of adolescents with BD that present with HMB. We used the RAND/UCLA appropriateness method to determine the existence of consensus among the interdisciplinary panel of experts. RESULTS: Thirty-nine experts participated in the panel. The experts rated fifteen HMB care considerations as appropriate, six as uncertain, and none as inappropriate. CONCLUSIONS: The HMB care statements represent the first set of HMB care considerations in adolescents with BD, developed with broad expert input on appropriateness. Although likely to be of interest to a range of clinicians who routinely manage adolescents with HMB, additional research is required in many key areas.

Clinical and laboratorial description of the differential diagnoses of hemostatic disorders in the horse.

The process of fibrin clot formation is a series of complex and well-regulated reactions involving blood vessels, platelets, procoagulant plasma proteins, natural inhibitors, and fibrinolytic enzymes. Vasculitis can be caused by a variety of different agents as bacteria, viruses, protozoal, rickettsial organisms, toxic, drugs, medications, and neoplasms. The most common cause of vasculitis is the purpura hemorrhagica, which is associated with exposure to Streptococcus equi ssp. equi or less commonly, equine influenza. Deficiencies or defects of the hemostatic components may result in bleeding and/or thrombosis. Inherited alterations of primary hemostasis (von Willebrand disease: vWD and Glanzmann's thrombasthenia: GT) and of secondary hemostasis (hemophilia A and prekallikrein: PK deficiency) are scarcely reported in equine clinic. On the contrary, acquired alterations of primary and secondary hemostasis are commonly found. They include thrombocytopenia, platelet dysfunction due to the administration of some drugs and targeted antiplatelet agents, decreased factor synthesis (liver disease or deficiency of vitamin K), release of inactive factors, inhibition of factor activity, or excessive consumption and depletion of factors (platelets, coagulation factors, and anticoagulants factors as antithrombin (AT) and protein C). Disseminated intravascular coagulation (DIC) is the most common and complex hemostatic disorder in horses and appears to be associated with sepsis, inflammatory and ischemic gastrointestinal tract disorders and other systemic severe diseases. These alterations are commonly found in patients in intensive care units.

Análise dos valores hematológicos de camundongos com distúrbios hemostáticos ao longo do seu desenvolvimento / Analysis of hematological values ​​of mice with hemostatic disorders during their development

The mouse was chosen as an animal model because of the possibility of being genetically manipulated, having lineages originated through induced or spontaneous mutations. The mutant pearl mice have reduced levels of adenine and serotonin nucleotides in the dense platelet granules and they are a model for Hermansky- Pudlak syndrome. The Vwf-/- mice were genetically modified to present mutations in the Vwf gene, to study von Willebrand disease. Other mice are genetically modified to have hemostatic changes, such as type A hemophilia (deficiency of coagulation factor VIII) and B (deficiency of coagulation factor IX). This work aimed to analyze the blood count of pearl mice, hemophiliac A and B and von Willebrand disease and test collection techniques. Twenty mice from each strain were used, ten males and ten females that were weighed weekly until the age of two months, after which they were weighed monthly. Three blood samples were collected from each animal in three different ways: caudal vein, saphenous vein and retro-orbital plexus. Regarding the growth curve, males are heavier than females, less HB males who are smaller than children up to the fifth week of age. And there was little difference in weight between the lines compared to the genetic background. The analyzed data of the hemograms were the white blood count (WBC), red blood cell count (RBC), hematocrit (HCT) and platelet count (PLT). It was concluded that the best collection method was the retro- orbital for all strains and the saphenous vein in the case of pearls, the least recommended method was the caudal vein. The weight curve is important to obtain a weight x age relationship over the physical development of the animals. It was noticed that hemophiliacs A really have high mortality, probably caused by internal bleeding. With hemogram data, there were also some differences between strains, such as the WBC of pearls and HA being higher than that of C57BL/6 and Vwf-/- and C57BL/6 also have a higher RBC value than the other strains and the HB, the lowest. The HCT had very similar values, varying between 41% and 51%. And PLT tend to have a higher value among males than females of all strains. It was possible to see some differences between lineages, gender and age, but more collections are still needed.

O camundongo foi escolhido como modelo animal pela possibilidade de ser manipulado geneticamente, tendo linhagens originadas através de mutações induzidas ou espontâneas. Os camundongos pérolas mutantes possuem níveis reduzidos de nucleotídeos de adenina e serotonina nos grânulos densos de plaquetas e são um modelo para a síndrome de Hermansky-Pudlak. Os camundongos Vwf-/- foram modificados geneticamente para apresentarem mutações no gene Vwf, para estudo da doença de von Willebrand. Outros camundongos são modificados geneticamente para apresentarem alterações hemostáticas, como as hemofilias do tipo A (deficiência do fator VIII da coagulação) e B (deficiência do fator IX da coagulação). Este trabalho teve como objetivo analisar o hemograma de camundongos pérolas, hemofílicos A (HA) e B (HB) e Vwf-/- testar técnicas de coleta. Foram usados 20 camundongos de cada linhagem, sendo dez machos e dez fêmeas que foram pesados semanalmente até os dois meses de idade, após isso foram pesados mensalmente. Foram coletadas três amostras de sangue de cada animal de três vias distintas: veia caudal, veia safena e plexo retro-orbital. Com relação à curva de crescimento, os machos são mais pesados do que as fêmeas, menos os machos HB que eram menores que as fêmeas até a quinta semana de idade. E houve pouca diferença de peso das linhagens comparadas com o fundo genético. Os dados analisados dos hemogramas foram a contagem leucocitária (WBC), contagem de hemácias (RBC), hematócrito (HCT) e contagem de plaquetas (PLT). Conclui-se que o melhor método de coleta foi a retro-orbital para todas as linhagens e a veia safena no caso dos pérolas, e o método menos recomendado foi pela veia caudal. Percebeu-se que os hemofílicos A realmente possuem alta mortalidade, provavelmente causada por hemorragias internas. Com os dados dos hemogramas, houve também algumas diferenças entre as linhagens, como o WBC dos pérolas e HA ser mais alto do que dos C57BL/6 e Vwf-/- e os C57BL/6 também possuem um valor de RBC mais alto do que as outras linhagens e os HB, o mais baixo. O HCT teve valores bem parecidos, variando entre 41% e 51%. E as PLT tendem a ter um valor maior entre os machos do que das fêmeas de todas as linhagens. Foi possível ver algumas diferenças entre as linhagens, gênero e idade, porém ainda é necessário mais coletas.

Factors Associated with Systemic Bleeding in Bothrops Envenomation in a Tertiary Hospital in the Brazilian Amazon.

Bothrops snakebites usually present systemic bleeding, and the clinical⁻epidemiological and laboratorial factors associated with the development of this manifestation are not well established. In this study, we assessed the prevalence of Bothrops snakebites with systemic bleeding reported at the Fundação de Medicina Tropical Dr. Heitor Vieira Dourado, in Manaus, Amazonas State, Brazil, and the clinical⁻epidemiological and laboratorial factors associated with systemic bleeding. This is an observational, cross-sectional study carried out between August, 2013 and July, 2016. Patients who developed systemic bleeding on admission or during hospitalization were considered cases, and those with non-systemic bleeding were included in the control group. Systemic bleeding was observed in 63 (15.3%) of the 442 Bothrops snakebites evaluated. Bothrops snakebites mostly occurred in males (78.2%), in rural areas (89.0%) and in the age group of 11 to 30 years old (40.4%). It took most of the patients (59.8%) less than 3 h to receive medical assistance. Unclottable blood (AOR = 3.11 (95% CI = 1.53 to 6.31; p = 0.002)) and thrombocytopenia (AOR = 4.52 (95% CI = 2.03 to 10.09; p < 0.001)) on admission were independently associated with systemic bleeding during hospitalization. These hemostatic disorders on admission increase the chances of systemic bleeding during hospitalization. Prospective studies are needed to clarify the pathophysiology of systemic bleeding in Bothrops snakebites in the Amazon region.

Factors associated with systemic bleeding in Bothrops envenomation in a tertiary hospital in the Brazilian Amazon

Bothrops snakebites usually present systemic bleeding, and the clinical–epidemiological and laboratorial factors associated with the development of this manifestation are not well established. In this study, we assessed the prevalence of Bothrops snakebites with systemic bleeding reported at the Fundação de Medicina Tropical Dr. Heitor Vieira Dourado, in Manaus, Amazonas State, Brazil, and the clinical–epidemiological and laboratorial factors associated with systemic bleeding. This is an observational, cross-sectional study carried out between August, 2013 and July, 2016. Patients who developed systemic bleeding on admission or during hospitalization were considered cases, and those with non-systemic bleeding were included in the control group. Systemic bleeding was observed in 63 (15.3%) of the 442 Bothrops snakebites evaluated. Bothrops snakebites mostly occurred in males (78.2%), in rural areas (89.0%) and in the age group of 11 to 30 years old (40.4%). It took most of the patients (59.8%) less than 3 h to receive medical assistance. Unclottable blood (AOR = 3.11 (95% CI = 1.53 to 6.31; p = 0.002)) and thrombocytopenia (AOR = 4.52 (95% CI = 2.03 to 10.09; p < 0.001)) on admission were independently associated with systemic bleeding during hospitalization. These hemostatic disorders on admission increase the chances of systemic bleeding during hospitalization. Prospective studies are needed to clarify the pathophysiology of systemic bleeding in Bothrops snakebites in the Amazon region.

Hemostatic abnormalities in patients with Ehlers-Danlos syndrome.

Essentials Ehlers-Danlos Syndrome (EDS) is a rare heterogeneous group of inherited collagen disorders. A cohort of EDS patients was investigated for bleeding tendency and hemostatic abnormalities. EDS is associated with an increased risk of bleeding. EDS patients have platelet function abnormalities, whose severity correlates with bleeding risk. SUMMARY: Background Ehlers-Danlos syndrome (EDS) includes a heterogeneous group of connective tissue disorders affecting skin, bones, vessels, and other organs. Patients with EDS have an increased risk of bleeding, but a comprehensive study of hemostasis in EDS patients is lacking. Objective To investigate the bleeding tendency of a cohort of patients with EDS by using the Bleeding Assessment Tool of the ISTH, the bleeding severity score (BSS). Methods The BSS was defined as abnormal when it was ≥ 4 in men and ≥ 6 in women. Patients with a bleeding tendency were compared with those without in terms of type and number of hemostatic abnormalities. Results Fifty-nine of 141 patients with EDS (41.7%) had an abnormal BSS. Prothrombin time and activated partial thromboplastin time were slightly prolonged in 10 patients (7.1%) because of mild coagulation factor deficiencies, which were not responsible for the bleeding diathesis. von Willebrand factor antigen, ristocetin cofactor, endogenous thrombin potential and platelet count were normal in all patients. At least one platelet function abnormality was found in 53 patients (90%) with an abnormal BSS and in 64 (78%) with a normal BSS (adjusted odds ratio [OR] 2.55, 95% confidence interval [CI] 0.87-7.48). The risk of bleeding progressively increased with the number of platelet function abnormalities, reaching an OR of 5.19 (95% CI 1.32-20.45) when more than three abnormalities were detected. Conclusions Our results show that nearly half of patients with EDS have an abnormal BSS, which, in 90% of cases, appear, at least in part, to be attributable to platelet function abnormalities. Abnormalities of primary hemostasis may contribute to the risk of bleeding in patients with EDS.

Conhecimento dos cirurgiões-dentistas em atendimento de pacientes com coagulopatias hereditárias / Knowledge of dentists in the care of patients with hereditary coagulopathies

Objetivo: verificar o conhecimento dos cirurgiões-dentistas que trabalham nas Unidades Básicas de Saúde (UBS) com Equipes de Saúde Bucal (ESB) modalidade I, no município de Campina Grande, sobre o atendimento odontológico de pacientes com coagulopatias hereditárias. Sujeitos e método: por meio de questionário autoaplicável, foi feita uma entrevista com os cirurgiões-dentistas do município em questão para analisar os seus conhecimentos. Participaram da pesquisa 24 profissionais que se encaixaram nos critérios de inclusão e responderam de forma adequada o questionário com questões objetivas, sendo que os participantes foram orientados a assinalar somente uma alternativa para cada questionamento. Resultados: a maioria dos profissionais é formada há mais de 10 anos. A média de acertos das respostas foi de 50%. Em relação aos achados clínicos que determinam a possível presença de distúrbio hemorrágico, 44% afirmaram que são: púr-pura, sangramento gengival espontâneo e hemartrose. Os pacientes considerados de risco elevado para o tratamento odontológico foram: pacientes sem distúrbios hemorrágicos revelados, mas com exames complementares alterados; pacientes em tratamento com AAS; e pacientes em tratamento com anticoagulante por via oral. A maioria (68%) não considera a utilização de sugadores de saliva como risco para sangramento bucal. Os procedimentos odontológicos que os profissionais não se sentem seguros a executar, nesse tipo de paciente, foram: exodontias (88%); tratamento periodontal cirúrgico (76%); raspagem e alisamento coronoradicular (RACR) (28%); anestesia do nervo alveolar inferior ou outros (24%); tratamento endodôntico (20%); e anestesia infiltrativa (8%). Conclusão: os dados obtidos na pesquisa mostraram que o conhecimento dos cirurgiões-dentistas das UBS do município de Campina Grande não é satisfatório, havendo dúvidas sobre a maioria dos tratamentos odontológicos direcionados aos pacientes com coagulopatias hereditárias. (AU)

Objective: this study aimed to determine the knowledge of dentists working in UBS with ESB mode I in the city of Campina Grande on Patients with hereditary Coagulopathies. Through self-administered questionnaire, own and without exclusion criteria, an interview was conducted with dental surgeons of the municipality concerned to analyze their knowledge of the relevant topic. By itself and without exclusion criterion an interview was made with the dentists concerning to analyze their knowledge about hereditary coagulopathies. Subjects and method: the participants were 24 dentists which fit the inclusion criteria and responded adequately to the questionnaire which contained objective questions, and the respondents were asked to point out only one alternative of each questioning. Results: most of them were graduated over 10 years. The mean score was 50% of the questionnaire. In relation to clinical findings that determine the possible presence of bleeding disorder, 44% said they are: purple spontaneous gingival bleeding and hemarthrosis; patients considered at high risk for dental treatment were patients without bleeding disorders disclosed but with altered exams; patients being treated with ASA; and patients treated with anticoagulant orally. Most dentists (68%) do not consider the use of saliva-sucking as a risk for oral bleeding. Dental procedures that professionals do not feel safe running in those patients were: extractions (88%); surgical periodontal treatment (76%); RACR (28%); anesthesia of nerve alveolar inferior or other (24%); endodontic treatment (20%); and infiltrative anesthesia (8%). Conclusion: the data obtained from the survey showed that knowledge of dentists from Campina Grande municipality is not satisfactory and there is doubt on most dental treatments targeted to patients with inherited bleeding disorders. (AU)