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OBJECTIVE: Many pieces of literature have reported that inherited and acquired thrombophilia might be a risk factor for recurrent implantation failure (RIF), however, most studies have only focused on RIF patients and not their male partners. We studied the possible association of paternal thrombophilia with RIF risk. METHODS: Forty-two male partners aged 20-45 suffered from RIF compared with 42 males from couples with at least one successful pregnancy. All participants were investigated for thrombophilia markers. RESULTS: The prevalence of coagulation Factor V activity was significantly higher in the case group (42.9%) than in the control group (16.7%) (p=0.008) (OR=3.75; 95% CI, 1.38, 10.12). The prevalence of protein C and protein S deficiencies in RIF patients were 4.8% and 2.4%, respectively, and 0% in the controls. The prevalence of antithrombin III (ATIII) deficiency was significantly higher in the case group (19%) than in the control group (2.4%) (p=0.01). None of MTHFR C677T and MTHFR A1298C were statistically significant between the two groups. Combined thrombophilia was 45.2% in the men of the RIF group when compared with the control, 14.2% (p=0.001) (OR = 4.95; 95% CI, 1.75-13.86). CONCLUSIONS: Paternal thrombophilia may be related to recurrent implantation failure, so evaluation of this factor in RIF patients could be used to identify relevant risk groups and may help in the proper management of these cases to enhance the chance of implantation.
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Trombofilia , Humanos , Masculino , Trombofilia/epidemiologia , Adulto , Estudos de Casos e Controles , Feminino , Pessoa de Meia-Idade , Implantação do Embrião , Adulto Jovem , Gravidez , Fatores de RiscoRESUMO
Abstract Marfan syndrome classically presents with aortic root aneurysms. Aortic ectasia causes diverse blood flow alterations, influencing the behavior of coagulation factors and platelet activity. Heparin resistance has also been reported associated with Marfan Syndrome in a small number of patients, probably due to antithrombin III (ATIII) deficiency or various mutations. The ascending aorta and the aortic valve are replaced with prosthetic material during Bentall- de Bonno procedures. Resistance to anticoagulation during extracorporeal circulation, represents a significant challenge for both anesthesiologists and the surgical team. Resistance to heparin was observed in a patient with Marfan syndrome undergoing a Bentall procedure. ATIII concentrate was not available, and Activated Coagulation Time did not increase despite high doses of heparin. An alternate anticoagulation approach was used successfully.
Resumen El síndrome de Marfan clásicamente se presenta con aneurismas de la raíz de la aorta. La ectasia aórtica produce alteraciones en el flujo sanguíneo que influyen sobre el comportamiento de los factores de la coagulación y la actividad de las plaquetas. También se ha reportado resistencia a la heparina asociada al Síndrome de Marfan en un menor número de pacientes, probablemente debido a deficiencia de antitrombina III (ATIII) o a diversas mutaciones. La aorta ascendente y la válvula aórtica se reemplazan con material prostético en los procedimientos Bentall- de Bonno. La resistencia a la anticoagulación durante circulación extracorpórea significa un enorme desafío tanto para los anestesiólogos, como para el equipo quirúrgico. Se observó resistencia a la heparina en un paciente con Síndrome de Marfan sometido a un procedimiento de Bentall. No había disponibilidad de concentrado ATIII y no aumentó el Tiempo de Coagulación Activada a pesar de las elevadas dosis de heparina. Se utilizó exitosamente un abordaje alterno de anticoagulación.
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Resumen: La coexistencia entre el fracaso fibrinolítico y la presencia de infección es más frecuente de lo que parece; desafortunadamente muchas veces pasa por alto y es concebido como algo incidental, generando consigo catástrofes vasculares y serias disfunciones endoteliales. Presentamos el caso de un adulto joven quien debuta con choque obstructivo de acuerdo con la cardioscopia invasiva y a la información gasométrica requiere terapias tempranas dirigidas por objetivos en el contexto de sepsis severa con aislamientos de Enterococcus faecalis y púrpura fulminans postinfecciosa aguda en el escenario clínico de deficiencia de antitrombina III. De acuerdo con el perfil hemodinámico referido y manifestaciones eléctricas presentadas se documentaron marcadores de actividad fibrinolítica, por lo cual fue llevado a perfusión pulmonar documentándose enfermedad pulmonar tromboembólica. Evoluciona favorablemente y es trasladado a piso para continuar atención médica en salud por los servicios de neumología y hematología.
Abstract: Coexistence between fibrinolytic failure and the presence of infection is more common than it seems; unfortunately it often is not recognized and is conceived as incidental; leading to vascular catastrophes and serious endothelial dysfunctions. We present the case of a young adult who debuts with purpura fulminans related to Enterococcus faecalis isolation in the clinical setting of antithrombin III deficiency and thromboembolic pulmonary disease. According to the hemodynamic profile referred and electrical manifestations presented, markers of fibrinolytic activity were documented, for which it was taken to pulmonary perfusion documenting thromboembolic lung disease. He evolves favorably and is transferred to continue medical health care by the services of pulmonology and hematology.
Resumo: A coexistência entre falência fibrinolítica e presença de infecção é mais frequente do que parece; Infelizmente, muitas vezes é negligenciado e concebido como algo incidental, gerando catástrofes vasculares e graves disfunções endoteliais. Apresentamos o caso de um adulto jovem que apresenta choque obstrutivo de acordo com cardioscopia invasiva e informações gasométricas, requer terapias precoces direcionadas por objetivos no contexto de sepse grave com isolamento de Enterococcus fecalis e púrpura fulminante pós-infecciosa aguda no cenário clínico de deficiência de antitrombina III. De acordo com o perfil hemodinâmico referido e manifestações elétricas apresentadas, foram documentados marcadores de atividade fibrinolítica, para o qual foi encaminhado para perfusão pulmonar, documentando doença pulmonar tromboembólica. O paciente progride favoravelmente e é transferido para o leito para continuar o atendimento médico nos serviços de pneumologia e hematologia.
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Antithrombin III (AT III) is a critical component of the coagulation cascade that functions primarily to inhibit activated coagulation factors IIa and Xa. AT III deficiency is a disorder that predisposes patients to thromboemboli. Antiphospholipid syndrome (APS) is an autoimmune disorder that predisposes patients to vascular and microvascular thrombosis, which can often be devastating and lead to multiorgan involvement. The mainstay of treatment for both conditions involves the use of lifetime vitamin K antagonists. Recent studies suggest that patients with APS refractory to warfarin therapy may benefit from the addition of aspirin, statin, or hydroxychloroquine; low weight molecular heparin; or a combination regimen. Studies have also suggested that patients with AT III deficiency refractory to warfarin therapy may see improvement with use of a novel oral anticoagulant. This case report describes the recurrent hospitalizations of a 45-year-old patient who presented with multiorgan thrombosis involving the descending aorta, deep lower extremity veins, superior mesenteric artery and artery of the brain. This led to mesenteric ischemia, limb necrosis and a subacute frontal cortex infarct. Initial anticoagulation therapy was refractory to the use of warfarin. Enoxaparin therapy was initiated, resulting in no further thrombotic events. Clinicians should consider poor gastrointestinal absorption of warfarin in patients who fail to reach therapeutic anticoagulation goals. In addition, a thorough workup for hereditary and acquired thrombophilias should be performed in patients who present with recurrent thromboemboli, as these disorders increase the risk of poor patient outcomes if left untreated.
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OBJECTIVE: To clarify the incidence and genetic risk of neonatal-thromboembolism, we conducted a nationwide study exploring the impact of thrombophilia on neonatal-thromboembolism in Japan. STUDY DESIGN: A questionnaire survey was conducted for perinatal centers in Japan, focusing on the clinical expression, genotype, treatment, and outcome of patients who developed thromboembolism within 28 days of birth from 2014 to 2018. RESULTS: The estimated incidence of neonatal-thromboembolism was 0.39 cases per 10 000 live births. Intracranial lesions and purpura fulminans occurred in 66 and 5 of 77 patients, respectively. Fifty-eight (75.3%) infants presented within 3 days after birth. Four (5.2%) died, and 14 (18.2%) survived with disability. At the diagnosis, <20% plasma activity of protein C was noted in 16 infants, protein S (in 2), and antithrombin (in 1). Thirteen genetic tests identified 4 biallelic and 5 monoallelic protein C-variants but no protein S- or antithrombin-variants. Protein C-variants had purpura fulminans (P < .01), ocular bleeding (P < .01), positive-family history (P = .01), and death or disability (P = .03) more frequently than others. Protein C-variants were independently associated with disability (OR 5.74, 95% CI 1.16-28.4, P = .03) but not death. Four biallelic variants had serious thrombotic complications of neurologic disability, blindness, and/or amputation. Three monoallelic variants survived without complications. The only protein C-variant death was an extremely preterm heterozygote infant. CONCLUSIONS: Monoallelic protein C-variants had a higher incidence of neonatal-thromboembolism than biallelic variants. Thrombophilia genetic testing should be performed in the setting of neonatal-thromboembolism and low protein C to identify the underlying genetic defect.
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Deficiência de Proteína C/complicações , Tromboembolia/epidemiologia , Feminino , Predisposição Genética para Doença , Humanos , Recém-Nascido , Japão , Masculino , Deficiência de Proteína C/genética , Estudos Retrospectivos , Fatores de Risco , Inquéritos e Questionários , Tromboembolia/genéticaRESUMO
OBJECTIVE: Thrombotic events are potential complications in patients receiving extracorporeal membrane oxygenation (ECMO) necessitating the use of systemic anticoagulation with heparin. Heparin works by potentiating the effects of antithrombin (AT), which may be deficient in critically ill patients and can be replaced. The clinical benefits and risks of AT replacement in children on ECMO remain incompletely understood. METHODS: This single-center, retrospective study reviewed 28 neonatal and pediatric patients supported on ECMO at a tertiary care hospital between April 1, 2013, and October 31, 2014, who received at least 1 dose of AT during their ECMO course. The primary outcome of the study was the change in anti-factor Xa levels after pooled human AT supplementation. Secondary outcomes included the percentage of anti-factor Xa levels within the therapeutic range surrounding AT administration; survival to decannulation; 30 days after cannulation and discharge; time to first circuit change; and incidence of bleeding and thrombotic events. RESULTS: A total of 78 doses of AT were administered during the study period. The mean increase in anti-factor Xa level following AT administration in patients without a ≥10% concurrent change in heparin was 0.075 ± 0.13 international units/mL. A greater percentage of anti-factor Xa levels were therapeutic for the 48 hours following AT administration (64.2% vs 38.6%). Survival and adverse events were similar to Extracorporeal Life Support Organization averages, with the exception of a higher incidence of intracranial hemorrhage. CONCLUSIONS: Patients experienced a small but significant increase in anti-factor Xa level and a greater percentage of therapeutic anti-factor Xa levels following AT supplementation.
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In this study, sulfated polysaccharide-rich extracts were isolated from 22 tropical seaweeds (4 red, 11 brown, and 7 green) found in northeastern Brazil, and evaluated for the role of anticoagulant agents. Fifteen of the extracts showed anticoagulant activity, including all the extracts from green seaweeds. Udotea flabellum (a green seaweed) extract was the most potent, requiring an amount of only 3 µg to double the plasma coagulation time in the activated partial thromboplastin time test. A similar result was obtained with 1 µg of heparin. Two sulfated homogalactans with anticoagulant activity, F-I (130 kDa) and F-II (75 kDa), were isolated from this extract using several bio-guided purification steps. Their anticoagulant activity, as well as properties related to antitumor activity (anti-proliferative, anti-adhesive, and anti-migratory), were accessed. Their anticoagulant activities were close to that of heparin. We found that F-I and F-II (0.5â»10 µg/mL) were not able to directly inhibit thrombin. In the presence of anti-thrombin, F-I (0.5 µg/mL) was more effective than heparin (0.5 µg/mL) in inhibiting thrombin, while F-II showed similar effects as heparin. F-I and F-II also inhibited B16-F10 (murine melanoma cells) adhesion, migration, and proliferation on a fibronectin-coated surface, but not on laminin- or collagen I-coated surfaces. Except for the antiproliferative activity, the other effects of F-I and F-II were eliminated upon their desulfation (~50%), indicating that the degree of sulfation is not as important for F-I and F-II anti-proliferative activity as the sulfation position. Taken together, the results provide strong evidence for the potential utility of sulfated galactans from U. flabellum, making these compounds an interesting option for future investigations that aim to design new anticoagulant/antitumor agents.
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Anticoagulantes/farmacologia , Antineoplásicos/farmacologia , Clorófitas/química , Extratos Vegetais/farmacologia , Alga Marinha/química , Animais , Anticoagulantes/química , Anticoagulantes/isolamento & purificação , Antineoplásicos/química , Antineoplásicos/isolamento & purificação , Brasil , Adesão Celular/efeitos dos fármacos , Linhagem Celular Tumoral , Movimento Celular/efeitos dos fármacos , Proliferação de Células/efeitos dos fármacos , Ensaios de Seleção de Medicamentos Antitumorais , Galactanos/química , Galactanos/isolamento & purificação , Galactanos/farmacologia , Heparina/farmacologia , Camundongos , Extratos Vegetais/química , Extratos Vegetais/isolamento & purificação , Sulfatos/química , Sulfatos/isolamento & purificação , Sulfatos/farmacologia , Trombina/antagonistas & inibidoresRESUMO
Antithrombin (AT) is a serpin that inhibits mainly thrombin and fXa after being activated by binding to glycosaminoglycans as heparin and heparan sulfate. Upon binding, the native AT conformation, relatively inactive as a protease inhibitor, is converted to an activated form. Recently, a new compound, named TMI, was discovered in our group with nanomolar affinity to antithrombin, and shown to be able to induce a partial activation of antithrombin. As TMI represents an original scaffold for structural optimizations aiming the development of new antithrombotic drugs, the present work demonstrated, through a series of molecular dynamics simulations, that TMI is able to modulate AT reactive center loop flexibility similarly to what is observed to heparin, as well as exposing AT P1 residue, Arg393. These results represent the first atomic level indication of AT conformational activation by TMI, and may offer a predictive basis for future studies aiming TMI structural optimization.
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Antitrombinas/química , Ativadores de Enzimas/química , Fibrinolíticos/química , Heparina/química , Heparitina Sulfato/química , Fosfatos de Inositol/química , Regulação Alostérica , Antitrombinas/metabolismo , Sítios de Ligação , Desenho de Fármacos , Ativadores de Enzimas/metabolismo , Fator Xa/química , Fibrinolíticos/metabolismo , Heparina/metabolismo , Heparitina Sulfato/metabolismo , Humanos , Fosfatos de Inositol/metabolismo , Cinética , Simulação de Dinâmica Molecular , Ligação Proteica , Conformação Proteica em alfa-Hélice , Conformação Proteica em Folha beta , Domínios e Motivos de Interação entre Proteínas , Termodinâmica , Trombina/químicaRESUMO
INTRODUCTION: Hemostasis protects upon the occurrence of vascular endothelial damage, with involving of different factors. The interaction of these factors in older adults is poorly known, and has been associated with different disorders. Therefore, we determined the activity of coagulation factors (CF), anticoagulant proteins (AP), and plasminogen (Plg), as well as the frequency of deficiencies of these proteins in a population of healthy Mexican older adults (OA). METHODS: CF (I, II, V, VII, VIII, IX, X, and XI y XII), AP [protein C (PC), protein S (PS), and antithrombin (AT)], and Plg were determined from 244 plasma samples of OA using commercial kits in a coagulometer ACL Elite Pro. RESULTS: A total of 139 women and 105 men were under study. They were divided into age range groups (50-59, 60-69, 70-79, and Ë80 years). Activity of CF, AP, and Plg was determined. Frequencies of CF, AP, and Plg activity values were obtained for each age group according to gender. Differences were found between both frequencies for each protein. CONCLUSION: Significant differences were found, so it is recommended to establish reference values (RV) for the activity of fibrinogen and FX by decade and gender, FVII and FXII by gender, FII, FV, FVIII, PC, PS, and Plg by decade, whereas for FIX, FXI, and AT, they are not modified by age or gender, so the RV described for adult Mexican population can be used. It is important to integrate these results into established diagnostic algorithms, which can be taken into account to provide an accurate diagnosis and treatment for patients with suspected hemorrhagic or thrombotic processes, as well as suggest those habits that improve their quality of life, to maintain optimal health and prevent thrombotic and hemorrhagic events.
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Envelhecimento/sangue , Anticoagulantes/sangue , Fatores de Coagulação Sanguínea/metabolismo , Hemostasia , Plasminogênio/metabolismo , Adulto , Idoso , Idoso de 80 Anos ou mais , Testes de Coagulação Sanguínea/métodos , Endotélio Vascular/lesões , Endotélio Vascular/metabolismo , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Human antithrombin is a blood derivative widely used in the treatment of coagulation dysfunction. Affinity chromatography using heparin (HEP) derivatives is usually used for antithrombin purification. In this study, an affinity procedure based on a magnetic Dacron-HEP composite is proposed. Dacron was firstly converted to Dacron-hydrazide and magnetised by co-precipitation with of Fe2+/Fe3+ (mDAC). HEP was activated by carbodiimide and N-hydroxysuccinimide and covalently linked to mDAC (mDAC-HEP). EDX and infrared spectra analyses confirmed each synthesis step of mDAC-HEP. This composite exhibited superparamagnetism behaviour. Human plasma was incubated with mDAC-HEP (fresh and stored over a long period) and washed with phosphate buffer containing increasing concentrations of NaCl. Human plasma antithrombin activity was reduced by approximately 20% in the presence of the 1.0M NaCl fraction, and this eluate was able to prolong coagulation time (aPTT) using both preparations. Electrophoresis of the eluates revealed bands corresponding to the expected size of antithrombin (58kDa). The mDAC-HEP particles are reusable. This method presents the following advantages: easy, low-cost synthesis of the composite, magnet-based affinity purification steps, and reusability.
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Antitrombinas/sangue , Antitrombinas/isolamento & purificação , Cromatografia de Afinidade/métodos , Heparina/química , Imãs/química , Polietilenotereftalatos/química , Humanos , Tempo de Tromboplastina ParcialRESUMO
La deficiencia de antitrombina III hereditaria es una rara enfermedad que afecta al 0.02-0.2 por cento de la población. Puede presentar mayor frecuencia de complicaciones y resultados adversos tanto en la madre como en el feto. Se presenta el manejo obstétrico de dos gestaciones consecutivas en una mujer con deficiencia de antitrombina III. Descripción: en ambos embarazos la madre realiza profilaxis de la enfermedad tromboembólica con heparina de bajo peso molecular para evitar la aparición de esta patología tanto en el embarazo como en el puerperio y mejorar el flujo útero-placen-tario. Con respecto a las complicaciones obstétricas, sólo existe un enlentecimiento del crecimiento fetal que obliga a un control obstétrico estricto. En ambas gestaciones los estudios eco-Doppler están dentro de la normalidad lo que permite una conducta expectante, consiguiendo llegar a término. Discusión: la profilaxis con heparina de bajo peso molecular en las gestantes con esta trombofilia y las intervenciones preventivas de factores de riesgo de enfermedad tromboembólica, junto con un control obstétrico adecuado, ha conseguido evitar la apari-ción de complicaciones derivadas de esta patología en el embarazo y en el puerperio. Por otra parte, el control del crecimiento fetal y el estudio Eco-Doppler han permitido asegurar el bienestar fetal no adelan-tando el parto, consiguiendo partos a término...
Hereditary antithrombin III deficiency is a rare disease that affects 0.02-0.2 percent of the population. It may be associated with a higher rate of complications and adverse outcomes in both mother and fetus. The present study describes the management of a woman with antithrombin III deficiency and two consecutive pregnancies. Description: in both pregnancies, the woman under went prophylaxis with low molecular weigh heparin, to prevent thromboembolic disease and improve the utero-placental flow during pregnancy and the postpartum period. The only obstetric compli-cation was fetal growth retardation requiring strict obstetric control. In these two cases the eco-Doppler studies offeto-placentalflow were normal, leading to the expectation of managing a term birth. Discussion: low molecular weigh heparin prophylaxis in pregnant women with thrombophilia and preventive interventions for risk factors for throm-boembolic disease, together with appropriate obstetric care managed to avoid the emergence of complications of this disease in pregnancy and puer-perium. Fetal growth control and a Doppler study also help to ensure the well-being of the fetus and avoid a preterm birth...
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Humanos , Feminino , Gravidez , Deficiência de Antitrombina III/prevenção & controle , Gravidez de Alto Risco , Retardo do Crescimento Fetal , Tromboembolia Venosa/prevenção & controleRESUMO
The structural characterization and the anticoagulant potential of a novel heparin/heparan sulfate-like compound from the heads of Litopenaeus vannamei shrimp are described. While it is distinct from either heparin or heparan sulfate, enzymatic depolymerization and nuclear magnetic resonance spectroscopy analyses revealed that this molecule does share some structural features with heparin, such as the high degree of N- and 6-O-sulfation and minor N-acetylation, and with heparan sulfate, in the glucuronic acid content. Its ability to stabilize human antithrombin explains its significant anticoagulant activity in aPTT and Factor-Xa inhibition assays. Interestingly, in contrast to mammalian heparin, the shrimp compound displayed negligible hemorrhagic effect. Together, these findings have particular interest since they reveal a novel molecule with significant anti-Xa activity coupled with low bleeding effects which make the shrimp heparin/HS-like compound a potential alternative for mammalian heparin.
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Anticoagulantes/química , Hemorragia/prevenção & controle , Heparina/química , Heparitina Sulfato/química , Penaeidae/química , Acetilação , Animais , Anticoagulantes/isolamento & purificação , Anticoagulantes/farmacologia , Antitrombinas/antagonistas & inibidores , Antitrombinas/química , Antitrombinas/isolamento & purificação , Bovinos , Cromatografia por Troca Iônica , Fator Xa/química , Inibidores do Fator Xa , Ácido Glucurônico/química , Cabeça , Heparina/isolamento & purificação , Heparina/farmacologia , Heparitina Sulfato/isolamento & purificação , Heparitina Sulfato/farmacologia , Humanos , Intestinos/química , Pâncreas/química , Tempo de Tromboplastina Parcial , Ratos , Suínos , Cauda/irrigação sanguínea , Cauda/efeitos dos fármacosRESUMO
OBJECTIVE: To investigate Port-A-Cath (PAC)-related thrombosis and postthrombotic syndrome (PTS) in children with cancer. STUDY DESIGN: The study population was a consecutive cohort of children diagnosed with cancer and a PAC implanted at diagnosis. Children were evaluated for the presence of PAC-related thrombosis by magnetic resonance venography and the presence of congenital prothrombotic risk factors and PTS. RESULTS: A total of 114 children (median age, 6.04 years) were included. Of these children, 48 (42%) were treated for solid tumors and 66 (58%) were treated for hematopoietic tumors, including 38 for acute lymphoblastic leukemia. At the time of magnetic resonance venography, 42 children (37%) had the PAC still in place, and 72 (63%) had the PAC removed. Overall, PACs were in place for a total of 324.92 PAC-years. PAC-related thrombosis was detected in 45 children (39.5%) with a current or previous PAC. Of these, 21 (47%) had a solid tumor, 14 (31%) had acute lymphoblastic leukemia, and 10 (22%) had another hematopoietic tumor. Younger age at diagnosis, female sex, duration of PAC use, and left-side PAC placement were independently associated with an increased risk of thrombosis, whereas asparaginase therapy and the presence of inherited prothrombotic risk factors were not. Mild PTS (ie, presence of prominent collateral vessels in the skin) was present in 5.6% of the children. CONCLUSION: PAC-related thrombosis is common in pediatric oncology patients. In some children, thrombotic complications can lead to the development of PTS.
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Síndrome Pós-Trombótica/diagnóstico , Trombose/diagnóstico , Dispositivos de Acesso Vascular/efeitos adversos , Adolescente , Fatores Etários , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Neoplasias/complicações , Neoplasias/terapia , Flebografia , Síndrome Pós-Trombótica/etiologia , Fatores de Risco , Trombose/etiologiaRESUMO
Introducción: Virshow definió el síndrome de hipercoagulabilidad como parte de la triada causal de trombosis venosa (VTE) y la primera causa identificable de esta condición fue el déficit de antitrombina (AT). Objetivo: Mostrar nuestra experiencia sobre la conducta de una paciente con trombofilia como causa de embolismo pulmonar perioperatorio. Material y Método: Paciente programada para histerectomía abdominal electiva por severa menometrorragia que desarrolló un embolismo pulmonar (EP) en el momento de la inducción anestésica. Discusión: Los estudios hechos para identificar la causa de esta complicación demostraron bajos niveles de AT. La presencia de un fenómeno embolico asociado o no a este diagnóstico aumenta el riesgo de recurrencia. La anticoagulación usando un puente entre los antagonistas de la vitamina K y la heparina es mandatoria. Conclusión: La sospecha clínica, el diagnóstico precoz y la participación multidisciplinaria fueron elementos claves para el manejo exitoso de esta enferma.
Introduction: Virshow defined the hypercoagulability syndrome as part of causal triad of venous thromboembolism (VTE), and the first identifiable cause of this condition was a antithrombin deficit (AT), Objective: To demonstrate our experience on the behavior of a patient presenting with thrombophilia as cause of Perioperative pulmonary embolism. Material and Methods: Patient programmable for elective abdominal hysterectomy from a severe menometrorrhagia provoking a pulmonary embolism (PE) from the moment of anesthesia induction. Discussion: Studies performed to identify the cause of this complication showed low levels of AT. Presence of an embolic phenomenon associated or not to this diagnosis, increases the recurrence risk. Anticoagulant therapy using a bridge among vitamin K antagonist and heparin is mandatory. Conclusion: Clinical suspicion, an early diagnosis and multidisciplinary participation were key elements to successful management of this patient.
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Moyamoya disease is a unique chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation. It can be primary or secondary to genetic syndromes such as Down syndrome. We report a seven year-old girl with a Down syndrome that presented with a disturbance of consciousness, seizures and a right hemiparesia at the age of five. Magnetic resonance imaging showed old cortical ischemic lesions in both cerebral hemispheres and a recent infarction in the territory of the ¡eft middle cerebral artery. Brain angiography showed a proximal stenosis of both medial cerebral arteries and a net of collateral vessels, consistent with the diagnosis of moyamoya syndrome. The patient had also an antithrombin III deficiency. Aspirin was indicated and a surgical correction was recommended. However, prior to the procedure, the patient had a new infarction in the territory of the right middle cerebral artery, which caused a severe disability.
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Criança , Feminino , Humanos , Síndrome de Down/complicações , Doença de Moyamoya/diagnóstico , Deficiência de Antitrombina III/diagnósticoRESUMO
OBJECTIVE: To evaluate antithrombin III (AT), thrombin (Fragment 1+2 [F1+2] and thrombin-antithrombin [TAT]) generation markers, as well as other coagulation parameters, such as prothrombin time, partial activated thromboplastin time, thrombin time, fibrinogen, euglobulin lysis time, and platelet count, in postmenopausal women after hormonal therapy. STUDY DESIGN: Forty-five patients who received either 0.625 mg/day unopposed oral conjugated equine estrogen (CEE), 0.625 mg/day oral CEE plus medroxyprogesterone acetate (MP), or 50 µg/day transdermal 17beta-estradiol plus MP, were included. Tests were performed before (T0) and after 3 (T3), 6 (T6) and 12 (T12) months of treatment. AT was determined by an amidolytic method, whereas F1+2 and TAT complex were measured by ELISA. RESULTS: There was a significant reduction in the AT level of patients who received oral CEE plus MP at T3. There was no AT reduction in patients taking either oral CEE alone or transdermal 17beta-estradiol plus MP. F1+2 increased in all patients, but it reached statistical significance only in patients receiving transdermal 17beta-estradiol MP at T3. CONCLUSIONS: The CEE associated with MP treatment may reduce AT levels, whereas unopposed CEE or transdermal 17beta-estradiol plus MP does not change AT. These changes might not be clinically relevant in the general population; however, hormonal replacement therapy may increase the risk of thrombosis in women with congenital or acquired thrombophilia.
OBJETIVO: Avaliar os marcadores antitrombina III (AT), fragmento 1 + 2 da trombina (F1+2) e complexo trombina-antitrombina (TAT), bem como outros parâmetros da coagulação, como tempo de pró-trombina, tempo parcial de tromboplastina ativado, tempo de trombina, fibrinogênio e tempo de lise da euglobulina em mulheres na pós-menopausa após terapia hormonal. DESENHO DO ESTUDO: Foram incluídas 45 voluntárias que receberam estrogênios conjugados eqüinos (ECE) 0,625 mg/dia, isoladamente ou associado ao acetato de medroxiprogesterona (AMP) ou usaram o 17beta-estradiol (50 µg/dia) transdérmico com AMP. Os exames foram realizados antes do tratamento (T0) e após três (T3), seis (T6) e doze (T12) meses após o início do tratamento. AT foi avaliada pelo método amidolítico, enquanto que o F1+2 e o complexo TAT por ELISA. RESULTADOS: Houve redução significante nos níveis de AT em pacientes que receberam ECE associado ao AMP no T3. Não houve redução na AT em mulheres que usaram ECE isoladamente ou aquelas com 17beta-estradiol transdérmico e AMP. O F1+2 aumentou em todos os grupos, mas apenas o grupo com 17beta-estradiol transdérmico e AMP apresentou diferença significante durante o T3. CONCLUSÕES: A associação de ECE e AMP pode reduzir os níveis de AT, enquanto ECE isoladamente ou 17beta-estradiol transdérmico com AMP não modificam-o acentuadamente. Essas alterações poderiam ser mais relevantes clinicamente na análise populacional. Todavia, a terapia de reposição hormonal aumentaria o risco de trombose em mulheres com trombofilia prévia congênita ou adquirida.