Arthroscopic partial anterior synovectomy of the knee on patients with haemophilia.

PURPOSE: This study assessed the results of two-portal knee arthroscopic synovectomy in terms of bleeding recurrence, knee function, quality of life (QOL), and radiographic staging in a prospective case series of patients with haemophilia. METHODS: Nine knees from eight patients (median age 16.1 years; range 9.6-25 years) with haemophilia and recurrent knee haemarthrosis were prospectively evaluated. Yearly recurrence of bleeding was evaluated once a year for 5 years postoperatively. Range of motion (ROM) and radiographic staging, as well as results of the short form (SF)-36 and subjective knee form of the International Knee Documentation Committee (IKDC) and Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) questionnaires, were evaluated before surgery and at the end of follow-up. RESULTS: Mean bleeding recurrence was significantly reduced during the 5-year follow-up period. Questionnaire results showed significant improvements (IKDC P = 0.015, WOMAC P = 0.011, and SF-36 P = 0.023), whereas ROM was not significantly affected. Arthropathy progressed from Arnold-Hilgartner radiographic stage III to stage IV (P = 0.0082). CONCLUSIONS: Two-portal knee arthroscopic synovectomy was effective at reducing bleeding recurrence and improving knee function and QOL in patients with haemophilia, but did not interrupt the progression of radiographic changes.

Successful management of neonatal alloimmune thrombocytopenia in the second pregnancy: a case report / Sucesso no manejo da trombocitopenia aloimune neonatal na segunda gestação: relato de caso

Neonatal alloimmune thrombocytopenia is a serious disease, in which the mother produces antibodies against fetal platelet antigens inherited from the father; it is still an underdiagnosed disease. This disease is considered the platelet counterpart of the RhD hemolytic disease of the fetus and newborn, yet in neonatal alloimmune thrombocytopenia the first child is affected with fetal and/or neonatal thrombocytopenia. There is a significant risk of intracranial hemorrhage and severe neurological impairment, with a tendency for earlier and more severe thrombocytopenia in subsequent pregnancies. This article reports a case of neonatal alloimmune thrombocytopenia in the second pregnancy affected and discusses diagnosis, management and the clinical importance of this disease.

A púrpura trombocitopênica neonatal aloimune é uma doença grave, na qual a mãe produz anticorpos contra antígenos plaquetários fetais herdados do pai, e é ainda subdiagnosticada na prática clínica. É considerada o equivalente plaquetário da doença hemolítica do recém-nascido, com a diferença que o primeiro filho é afetado, apresentando trombocitopenia fetal e/ou neonatal. Há risco significativo de hemorragia intracraniana e sequelas neurológicas graves, com tendência a trombocitopenia mais grave e mais precoce nas gestações subsequentes. Este artigo relata um caso de trombocitopenia aloimune neonatal na segunda gestação afetada e discute diagnóstico, manejo e importância clínica dessa doença na prática clínica.

Aspirin responsiveness safely lowers perioperative cardiovascular risk.

INTRODUCTION: Vascular surgeries are related to high cardiac morbidity and mortality, and the maintenance of aspirin in the perioperative period has a protective effect. The purpose of this study was to evaluate the association between preoperative platelet aggregability and perioperative cardiovascular (CV) events. METHODS: A preoperative platelet aggregation test was performed on an impedance aggregometer in response to collagen and to arachidonic acid (AA) for 191 vascular surgery patients under chronic use of aspirin. We analyzed the following CV events: acute myocardial infarction, unstable angina, isolated troponin elevation, acute ischemic stroke, reoperation, and cardiac death. Hemorrhagic events were also evaluated and classified according to the Thrombolysis In Myocardial Infarction criteria. RESULTS: The incidence of CV events was 22% (n = 42). Higher platelet response to AA was associated with CV events, so that patients in the fourth quartile (higher than 11Ω) had almost twice the incidence of CV events when compared with the three lower quartiles: 35% vs 19%; P = .025. The independent predictors of CV events were hemodynamic instability during anesthesia (odds ratio [OR], 4.12; 95% confidence interval [CI], 1.87-9.06; P < .001), dyslipidemia (OR, 3.9; 95% CI, 1.32-11.51; P = .014), preoperative anemia (OR, 2.64; 95% CI, 1.19-5.85; P = .017), and AA platelet aggregability in the upper quartile (OR, 2.48; 95% CI, 1.07-5.76; P = .034). Platelet aggregability was not associated with hemorrhagic events, even when we compared the lowest quartile of AA platelet aggregability (0-1.00 Ω) with the three upper quartiles (>1.00 Ω; OR, 0.77; 95% CI, 0.43-1.37; P = .377). CONCLUSIONS: The degree of aspirin effect on platelet aggregability maybe important in the management of perioperative CV morbidity, without increment in the bleeding toll.

Automonitorização e automanejo da anticoagulação oral / Oral anticoagulation self-monitoring and automanejoda

Esta revisão de 18 trabalhos mostrou que houve redução significante dos eventos trombóticos e da mortalidade com o emprego dos equipamentos portáteis. Embora tenha sido demonstrada diferença entre o autocontrole (quando o próprio paciente determinava a conduta terapêutica) e a automonitorização (quando o paciente somente quantificava o INR e transmitia o resultado para seu médico) quanto à redução de novos eventos tromboembólicos e sangramentos maiores, demonstrou-se melhora da qualidade de vida dos pacientes sob uso de dicumarínicos. Contudo, por vários motivos, essa auto avaliação não pode ser aplicada por todos os pacientes. Porém, sabe-se que existem vários aparelhos portáteis, com diferentes sensibilidades e custos diversos, e os autores não esclarecem quais os equipamentos usados e se foram observadas diferenças entre eles. De qualquer maneira, esta revisão mostra que o uso dos equipamentos portáteis é uma condição que pode alterar e melhorar a evolução clínica dos pacientes sob tratamento com drogas antagonistas da vitamina K e abre perspectivas para outros trabalhos avaliando diferenças de impacto econômico entre a avaliação tradicional e a realizada com aparelhos portáteis e também entre os diferentes aparelhos atualmente disponíveis no mercado.

Comparative study of platelet aggregation by turbidimetric and impedance methods in patients under acetylsalicylic acid antiplatelet therapy

INTRODUÇÃO: A hiperagregação (agregação excessiva) das plaquetas pode causar a formação de um trombo e a posterior oclusão dos vasos sanguíneos levando à isquemia. Esse fenômeno é responsável por doenças isquêmicas cardiovasculares, como angina pectoris e aterosclerose, bem como outras formas de isquemia, como o acidente vascular cerebral. Visando diminuir a função das plaquetas para reduzir a formação de trombos, o ácido acetilsalicílico vem sendo utilizado para tratamento antitrombótico, com diversos estudos mostrando sua eficácia. Dessa forma faz-se mister o uso de uma ferramenta laboratorial para o monitoramento da efetividade do tratamento, o que é feito por meio do teste de agregação plaquetária. O objetivo desse estudo foi comparar duas metodologias para esse exame (impedância elétrica e turbidimetria) em relação a trinta pacientes adultos de ambos os sexos em uso do fármaco. CONCLUSÃO: Os resultados mostraram uma boa correlação entre os métodos, possibilitando o uso concomitante de ambas as técnicas em laboratórios clínicos de rotina.

INTRODUCTION: Hyperaggregation of platelets can cause the formation of thrombi and subsequent occlusion of blood vessels leading to ischemia. This phenomenon can be responsible for ischemic cardiovascular diseases such as angina pectoris and atherosclerosis as well as other forms of ischemia such as stroke. To decrease platelet function and reduce the formation of thrombi, acetylsalicylic acid has been used for antithrombotic treatment, with several studies showing its effectiveness. Therefore it is necessary to use a laboratory tool to monitor the effectiveness of treatment, which is achieved through laboratory testing of platelet aggregation. The aim of this study was to compare two different methods (impedance and turbidimetry) to test platelet aggregation in 30 adult patients of both genders taking acetylsalicylic acid. CONCLUSION: The results show that there is a good correlation between these two methods and so both these techniques can be used in the clinical routine.

Does the evaluation of coagulation factors contribute to etiological diagnosis of pleural effusions?

OBJECTIVE: The aim of this study was to identify the participation of the coagulation system in the differential diagnosis of pleural effusions. INTRODUCTION: Imbalance between immunologic and metabolic factors triggers a sequence of events resulting in pleural reactions and accumulation of fluid. The coagulation system, which is fundamental for the maintenance of homeostasis, contributes to the inflammatory process responsible for pleural effusions, and participates in cellular proliferation and migration as well as in the synthesis of inflammatory mediators. METHODS: We evaluated the laboratory profile of coagulation and fibrinolysis in 54 pleural fluids (15 transudates and 39 exudates). RESULTS: The coagulation system acts according to the pathophysiologic mechanisms involved in the development of pleural effusions. In inflammatory effusions (exudates), there is activation of coagulation with increased levels of fragment 1+2 and thrombin-antithrombin complex in addition to reduction of fibrinogen levels due to fibrinolysis and fibrin tissue incorporation. As a consequence, there is activation of the fibrinolytic system with increased levels of fibrin degradation products, including the D-dimer. These changes are not sufficient for differentiation of different subgroups of exudates. In transudates, these events were observed to a lesser degree. CONCLUSION: The coagulation system plays an important role in the development of pleural diseases. Coagulation tests show differences between transudates and exudates but not among exudate subgroups. Understanding the physiopathological mechanisms of pleural disorders may help to define new diagnostic and therapeutic approaches.

Antiplatelet activity of Croton celditifolius / Atividade antiplaquetária do Croton celtidifolius

Croton celtidifolius Baill is a tree found in the Atlantic Forest South of Brazil, mainly in Santa Catarina. The bark and leaf infusions of this medicinal plant have been popularly used for the treatment of inflammatory diseases. The anti-aggregant activity of C. celtidifolius crude extract (CE) and the column chromatography (CC) isolated compounds flavonoids, catechin and gallocatechin were evaluated in human blood platelets. The platelet-rich plasma (PRP) was incubated with different concentrations of flavonóides (50 - 200 µg/mL) to be tested before platelet aggregation was induced by the agonists adenosine 5'diphosphate (ADP) and collagen. At 200 µg/mL the CE, catechin and gallocatechin markedly inhibited platelet aggregation with the aggregant agents. Using ATP production as an index of platelet secretory capacity, we observed a decreased production of ATP in platelets treated with flavonoids when stimulated by collagen. On the other hand, the flavonoids did not promote inhibitory effect on prothrombin time (PT), thromboplastin time (APTT) and thrombin time (TT). In conclusion, these observations suggest that C. celtidifolius is likely to exert an inhibitory action on platelets in vitro by suppressing secretion and platelet aggregation.

Croton celtidifolius Baill é uma árvore encontrada na Mata Atlântica, no sul do Brasil, principalmente no estado de Santa Catarina. A infusão da casca e folhas dessa planta medicinal é utilizada na medicina popular para o tratamento de doenças inflamatórias. A atividade antiagregante do extrato bruto de C. celtidifolius (CE) e de seus flavonóides isolados por coluna cromatográfica (CC), catequina e galocatequina, foi avaliada em plaquetas humanas. O plasma rico em plaquetas (PRP) foi incubado com diferentes concentrações dos flavonóides testados (50 - 200 µg/mL) e posteriormente a agregação foi induzida pelos agonistas adenosina 5'difosfato (ADP) e colágeno. Na concentração de 200 µg/mL o CE, a catequina e a galocatequina inibiram a agregação plaquetária induzida pelos agonistas. A produção de ATP foi utilizada como um índice de capacidade de secreção plaquetária e observamos uma diminuição na produção de ATP nas plaquetas tratadas com os flavonóides e estimuladas com o colágeno. Por outro lado, os flavonóides não promoveram um efeito inibitório no tempo de protrombina (PT), tempo de tromboplastina parcial ativada (APTT) e tempo de trombina (TT). Essas observações sugerem que o C. celtidifolius exerce, in vitro, uma ação inibitória nas plaquetas através da inibição da secreção e agregação plaquetária.

Hematological findings in Noonan syndrome

OBJECTIVE: Noonan syndrome is a multiple congenital anomaly syndrome, and bleeding diathesis is considered part of the clinical findings. The purpose of this study was to determine the frequency of hemostatic abnormalities in a group of Noonan syndrome patients. METHOD: We studied 30 patients with clinical diagnosis of Noonan syndrome regarding their hemostatic status consisting of bleeding time, prothrombin time, activated partial thromboplastin time and thrombin time tests, a platelet count, and a quantitative determination of factor XI. RESULTS: An abnormal laboratory result was observed in 9 patients (30 percent). Although coagulation-factor deficiencies, especially factor XI deficiency, were the most common hematological findings, we also observed abnormalities of platelet count and function in our screening. CONCLUSIONS: Hemostatic abnormalities are found with some frequency in Noonan syndrome patients (30 percent in our sample). Therefore, we emphasize the importance of a more extensive hematological investigation in these patients, especially prior to an invasive procedure, which is required with some frequency in this disorder

Anestesia em pacientes hemofílicos sem necessidade de reposiçäo de hemoderivados: utilizaçäo da técnica anestésica CA-ZOE com injetor eletrônico / Hemophilic patient anesthesia with out hemoderivativas reposition: CA-ZOE anesthias technic with eletronic injector

Os pacientes que apresentam distúrbios da coagulaçäo como a hemofilia e necessitam de tratamento restaurador odontológico em dentes posteriores inferiores (molares e pré-molares), apresentam contra-indicaçäo para a anestesia pterigo-mandibular e a utilizaçäo da técnica anestésica Ca-Zoe consiste na restauraçäo destes dentes sem a necessidade de reposiçäo de hemoderivados aos pacientes hemofílicos

Platelet aggregation and lipoprotein leves in a patient with familial hypercholesterolemia after sective LDL-apheresis

Platelet aggregation was studied in a patient with familial hypercholesterolemia immediately after aphereis selective for low-density lipoprotein (LDL), a lipid-lowering procedure. This treatment reduced plasmatic levels of total and LDL-cholesterol, apo B, and triglyceride. Increased platelet aggregation was reduced immediately after the apheresis in whole blood as well as in platelet-rich plasma. However, aggregation in washed platelets remained unchanged after LDL-apheresis. In conclusion, in this patient reduction of LDL-cholesterol improved platelet function in the very short term.

Ativaçäo plaquetária na doença hipertensiva específica da gestaçäo / Platelet activation in pregnancy-induced hypertension

Realizou-se estudo seqüencial em 72 gestantes prímigestas, com a finalidade de observar o número, a agregaçao e a adesividade plaquetáría naquelas que desenvolviam a doença hipertensiva específica da gestaçao. Entre as gestantes estudadas, 12 desenvolveram pré-eclâmpsia e, comparadas às gestantes normais, apresentaram os seguintes resultados: diminuiçao da contagem de plaquetas e da agregaçao plaquetáría com adrenalina e aumento da adesividade plaquetária. Os resultados obtidos sugerem a ativaçao das plaquetas na doença hipertensiva específica da gestaçao.