Enfermedad von Willebrand: consideraciones prácticas para el odontólogo. Revisión de literatura / von Willebrand disease: practical considerations for the dentist. Literature review

RESUMEN La enfermedad von Willebrand es el desorden hemorrágico hereditario más común, que se origina por la deficiencia del factor von Willebrand, la cual provoca una adhesión y agregación plaquetaria defectuosa. Se caracteriza por un tiempo de sangrado y tiempo parcial de tromboplastina prolongados, con bajos valores del factor VIII, y aumento de fragilidad capilar, pero con recuento normal de plaquetas. El tratamiento odontológico en un paciente con enfermedad von Willebrand, debe ser individualizado de acuerdo con la severidad de la condición del paciente, así como coordinado con el hematólogo, quien debe de determinar el tipo de enfermedad y la necesidad de la terapia de reemplazo del factor según diagnóstico específico previo al manejo de este. Mediante esta revisión bibliográfica se desea reforzar el conocimiento al odontólogo de este trastorno hemorrágico, ya que con ello se pueden evitar o minimizar posibles complicaciones de sangrado durante el tratamiento odontológico.

ABSTRACT von Willebrand disease is the most common inherited bleeding disorder, caused by von Willebrand factor deficiency, which causes defective platelet adhesion and aggregation. It is characterized by a prolonged bleeding time and partial thromboplastin time, with low levels of factor VIII, and increased capillary fragility, but with a normal platelet count. Dental treatment in a patient with von Willebrand disease must be individualized according to the severity of the patient's condition, as well as coordinated with the hematologist, who must determine the type of disease and the need for factor replacement therapy, prior to the patient management. Through this bibliographic review, it wants to reinforce the dentist's knowledge of this bleeding disorder, since this can prevent or minimize possible bleeding complications during dental treatment.

Manejo quirúrgico del paciente con hemofilia sometido a cirugía bucal: Reporte de un caso clínico / Surgical management of the patient with hemophilia undergoing oral surgery: A case report

Resumen Introducción: La hemofilia es una deficiencia congénita de un factor de la coagulación, la cual consta en un trastorno recesivo asociado al cromosoma X, generando disminución o ausencia de actividad funcional del factor. Objetivo: Presentar una revisión narrativa de la literatura sobre pacientes hemofílicos, junto con un caso de manejo de un paciente con la condición. Metodología: Paciente de sexo femenino, 18 años, acude al postgrado de Cirugía y Traumatología bucomaxilofacial de la Universidad Andrés Bello de Santiago de Chile, derivada para realizar exodoncia de terceros molares debido al término de su mecánica ortodóntica. Al realizar la anamnesis próxima, la paciente relata padecer hemofilia A leve, y hace 6 meses presentó un 38% de factor VIII. Previo al tratamiento quirúrgico se solicitó un hemograma completo con examen de coagulación para medir el TTPA. Además, se realizó una interconsulta con el hematólogo tratante para evaluación de su patología y recomendaciones para efectuar la misma con la menor cantidad de riesgos intraquirúrgicos y postquirúrgicos, el cual sugirió la administración de factor VIII previo, y posterior al acto quirúrgico. Así mismo, se aplicaron medidas de hemostasia locales para mejor control y un correcto manejo analgésico postquirúrgico. Conclusión: La hemofilia, es un trastorno que requiere un minucioso manejo tanto pre, intra y postoperatorio de parte del odontólogo, donde los exámenes complementarios, comunicación con el hematólogo, procedimiento atraumático y un correcto manejo de la hemostasia, son fundamentales para el éxito del tratamiento.

Abstract Introduction: Hemophilia is a congenital deficiency of a coagulation factor, associated to a recessive pattern located in the X chromosome, which induces a lower or even absent functional activity of that factor. Objective: To provide a narrative review of the literature about haemophiliac patients, as well as a case report of a patient. Methods: Female patient, 18 years old, attended in the postgraduate of Maxillofacial Surgery of the Andrés Bello University to Santiago, Chile, derived to perform extractions of wisdom teeth due to the end of its orthodontic mechanics. At the anamnesis, the patient reports to suffer from mild hemophilia A, and 6 months ago she had 38% VIII factor. Prior to surgical treatment, a complete blood count with a coagulation test was requested to measure TTPA. In addition, an interconsultation was made with the treating hematologist to perform a correct management to assess of her pathology and recommendations to carry out it with the least amount of intrasurgical and post-surgical risks. Suggested the administration of factor of freeze-dried VIII factor before and after surgery. Local hemostasis measures were also applied for better control and proper post-surgical pain management. Conclusion: Hemophilia, requires the dentist to perform a thorough management pre, intra and postoperatory, in which complementary tests, communication with the hematologist, atraumatic procedure and a precise management of hemostasis, are key for the treatment's success.

Respuesta a la prueba de desmopresina: estudio de cohorte en un hospital de Bogotá / Response to the desmopressin test: cohort study in a Bogota hospital

Introducción: La desmopresina es un análogo sintético de la vasopresina que aumenta los niveles plasmáticos del factor VIII y del factor de von Willebrand. Algunos autores señalan el tiempo de mantenimiento del efecto hemostático entre 6 y 8 h, por lo que es necesario estudiar su efecto en el tiempo. Objetivo: Determinar la variación de las variables de laboratorio de pacientes con enfermedad de von Willebrand y hemofilia tipo A posterior a la administración de desmopresina. Métodos: Estudio de cohorte retrospectivo en un hospital universitario en Bogotá. Se realizó un muestreo no aleatorio, se incluyeron 24 pacientes mayores de 18 años con diagnóstico de enfermedad de von Willebrand (67 por ciento) y hemofilia tipo A no grave (33 por ciento), a quienes se les realizó la prueba de desmopresina. Se conformaron dos grupos de pacientes, independientemente del diagnóstico: 15 pacientes con valores basales de factor VIII ; 50 UI y 13 pacientes con valores basales de antígeno von Willebrand lt; 50 UI. Se efectúo análisis estadístico descriptivo y correlacional en Stata 13. Resultados: El 87 por ciento de los pacientes del grupo I alcanzó el valor terapéutico a las 2 h de administrada la desmopresina (p= 0,000), el cual se mantuvo hasta 6 h en el 77 por ciento (p= 0,000). En el grupo II el 92 por ciento logró el valor terapéutico en 2 h (p= 0,003), que continuó hasta las 6 h en el 83 por ciento (p= 0,000). Conclusiones: La respuesta a la administración de desmopresina fue máxima a las 2 h posteriores, cuando comenzó a disminuir progresivamente, pero mantuvo el efecto terapéutico. Aunque no se encontraron efectos adversos, existe variabilidad de respuesta entre pacientes(AU)

Introduction: Desmopressin is a synthetic analog for vasopressin that increases the plasma levels of factor VIII and of von Willebrand factor. Some authors indicate maintenance time of hemostatic effect between 6 and 8 hours, so it is necessary to study its effect over time. Objective: To determine the variation of laboratory variables in patients with von Willebrand disease and type A hemophilia after desmopressin administration. Methods: Retrospective cohort study carried out in a university hospital in Bogotá. Nonrandomized sampling was used, including 24 patients older than 18 years and with a diagnosis of von Willebrand disease (67 percent) and non-severe type A hemophilia (33 percent), who underwent the desmopressin test. Two groups of patients were created, regardless of diagnosis: 15 patients with baseline values of factor-VIII 8203; #8203;lower than 50 IU and 13 patients with baseline values of von Willebrand antigen8203;8203;lower than 50 IU. Descriptive and correlational statistical analysis was performed in Stata 13. Results: 87 percent of patients in group I reached the therapeutic value two hours after desmopressin administration (p=0.000), which was maintained for up to six hours in 77 percent (p=0.000). In group II, 92 percent achieved the therapeutic value in two hours (p=0.003), which continued until six hours in 83 percent (p=0.000). Conclusions: Response to desmopressin administration was maximum at two hours, when it began to decrease progressively, but maintained the therapeutic effect. Although no adverse effects were found, there is variability of response among patients(AU)

Importancia de la orientación diagnóstica en hemofilia A adquirida / Adequate diagnosis of acquired hemophilia A

Acquired hemophilia A (AHA) is a rare and life-threatening autoimmune hemorrhagic disorder where autoantibodies are developed against factor VIII. An early diagnosis is challenging and mandatory: an immediate hemostatic control is required to reduce morbidity and mortality. Laboratory features of AHA are: presence of autoantibodies against factor VIII, prolonged activated partial thromboplastin time (with normal prothrombin time and thrombin time) and decreased factor VIII levels. In some cases, the results of laboratory tests may be incorrect due to errors in analysis, blood extraction or manipulation of samples; also worth of consideration are limitations in the measurement range and low sensitivity of the tests. This review highlights the importance of adequate screening in patients with suspected AHA to make an adequate diagnosis and reduce overall fatal outcomes.

Development and current status of research on thrombosis and hemorrhagic diseases in China / 白血病·淋巴瘤

Thrombosis and hemostasis are clinical interdisciplinary subjects involving multiple specialties. Related thrombus and hemorrhagic diseases seriously endanger health. Since the founding of the People's Republic of China, the diagnosis and treatment level of thrombus and hemorrhagic diseases in China have been continuously improved, the theoretical research has been continuously deepened, and a series of fruitful results have been achieved in the basic and clinical research on platelets, coagulation factors, anticoagulant and fibrinolytic systems. This paper summarizes the current situation and future development direction of related representative work.

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)

Cuidado odontológico de pacientes con trastornos hereditarios de la coagulación / Dental care for coagulation hereditary disorder patients

Resumen Los trastornos hereditarios de la coagulación como la hemofilia que es una enfermedad genética ligada al cromosoma X que se manifiesta por la deficiencia de los factores de la coagulación VIII, IX y XI -Hemofilia A, B y C respectivamente. , de acuerdo a la cantidad de deficiencia de estos se clasifican en leve, moderada o severa. Otra de enfermedad que se ve relacionada al trastorno de la coagulación es conocida como enfermedad de Von Willebrand cuya proteína con el mismo nombre se encuentra ausente o disminuido, otro aspecto a resaltar es que esta enfermedad no se encuentra ligada al sexo. El correcto diagnóstico clínico y exámenes de laboratorio, hace parte de un número de pasos que debe tener en cuenta el odontólogo para realizar normas de atención adecuadas según el tratamiento de cada paciente, ya sea consulta programada para realizar procedimientos como: operatoria, endodoncia, periodoncia, exodoncias, procedimientos de cirugía oral; O que se deba realizar tratamientos de urgencias como: heridas de la mucosa, laceraciones en boca, trauma facial, abscesos o celulitis, trauma dentoalveolar, enfocándose no solo en la parte clínica sino en el adecuado manejo del dolor de cada paciente.

Abstract Hereditary disorders of coagulation are genetic disease, among them are hemophilia that is attached to the X chromosome, so it manifests itself in men; In them there is deficiency of coagulation factors VIII, IX and XI -Hemofilia A, B and C respectively-, according to the amount of deficiency of these are classified as mild, moderate or severe. Another disease that is related to the coagulation disorder is known as Von Willebrand disease whose protein with the same name is absent or diminished, another aspect to emphasize is that this disease is not linked to sex. The correct clinical diagnosis and laboratory tests are part of a number of steps that the dentist must take into account to make adequate standards of care according to the treatment of each patient, whether scheduled consultation to perform procedures such as: surgery, endodontics, periodontics , dental extractions, oral surgery procedures; Or that it is necessary to carry out emergency treatments such as: mucosal wounds, lacerations in the mouth, facial trauma, abscesses or cellulitis, dentoalveolar trauma, focusing not only on the clinical part but also on the adequate management of the pain of each patient.

Manejo quirúrgico del paciente con hemofilia sometido a cirugía bucal: Reporte de un caso clínico

Introducción: La hemofilia es una deficiencia congénita de un factor de la coagulación, la cual consta en un trastorno recesivo asociado al cromosoma X, generando disminución o ausencia de actividad funcional del factor. Objetivo: Presentar una revisión narrativa de la literatura sobre pacientes hemofílicos, junto con un caso de manejo de un paciente con la condición. Metodología: Paciente de sexo femenino, 18 años, acude al postgrado de Cirugía y Traumatología bucomaxilofacial de la Universidad Andrés Bello de Santiago de Chile, derivada para realizar exodoncia de terceros molares debido al término de su mecánica ortodóntica. Al realizar la anamnesis próxima, la paciente relata padecer hemofilia A leve, y hace 6 meses presentó un 38% de factor VIII. Previo al tratamiento quirúrgico se solicitó un hemograma completo con examen de coagulación para medir el TTPA. Además, se realizó una interconsulta con el hematólogo tratante para evaluación de su patología y recomendaciones para efectuar la misma con la menor cantidad de riesgos intraquirúrgicos y postquirúrgicos, el cual sugirió la administración de factor VIII previo, y posterior al acto quirúrgico. Así mismo, se aplicaron medidas de hemostasia locales para mejor control y un correcto manejo analgésico postquirúrgico. Conclusión: La hemofilia, es un trastorno que requiere un minucioso manejo tanto pre, intra y postoperatorio de parte del odontólogo, donde los exámenes complementarios, comunicación con el hematólogo, procedimiento atraumático y un correcto manejo de la hemostasia, son fundamentales para el éxito del tratamiento.

Introduction: Hemophilia is a congenital deficiency of a coagulation factor, associated to a recessive pattern located in the X chromosome, which induces a lower or even absent functional activity of that factor. Objective: To provide a narrative review of the literature about haemophiliac patients, as well as a case report of a patient. Methods: Female patient, 18 years old, attended in the postgraduate of Maxillofacial Surgery of the Andrés Bello University to Santiago, Chile, derived to perform extractions of wisdom teeth due to the end of its orthodontic mechanics. At the anamnesis, the patient reports to suffer from mild hemophilia A, and 6 months ago she had 38% VIII factor. Prior to surgical treatment, a complete blood count with a coagulation test was requested to measure TTPA. In addition, an interconsultation was made with the treating hematologist to perform a correct management to assess of her pathology and recommendations to carry out it with the least amount of intrasurgical and post-surgical risks. Suggested the administration of factor of freeze-dried VIII factor before and after surgery. Local hemostasis measures were also applied for better control and proper post-surgical pain management. Conclusion: Hemophilia, requires the dentist to perform a thorough management pre, intra and postoperatory, in which complementary tests, communication with the hematologist, atraumatic procedure and a precise management of hemostasis, are key for the treatment's success.

Differential diagnosis of childhood hemorrhagic disorders

Bruising and bleeding are common events in children. The pediatrician must be able to determine whether a child's symptoms are normal or perhaps indicative of hemorrhagic disorders. A thorough medical history and physical examination should enable the pediatricians to identify those patients warranting further evaluation. This review describes the characteristics of the medical history, physical examination, and clinical laboratory testing that are important in recognizing clinically significant bleeding disorders. This review will help the pediatrician to perform the initial laboratory evaluation, differentiate for patients with bleeding tendency and make the correct diagnosis for a variety of cases.

Clinical and laboratory characteristics of patients with dengue hemorrhagic fever manifestations and their transfusion profile

Background: Dengue is an infectious disease with a recurring incidence, especially in developing countries. Despite recent economic growth, success in disease control has not been achieved, and dengue has evolved from cyclic epidemic outbreaks to a lack of seasonality. The lack of scientific basis for the proper management of cases with hemorrhagic manifestations, especially regarding transfusion procedures, might contribute to the high death rate in potentially avoidable cases. Objective: The aim of the study was to identify the clinical and laboratory manifestations in hemorrhagic dengue fever treated at the emergency services in Rio Branco, AC, Brazil, as well as to describe transfusion characteristics of patients and identify possible prognostic factors. Methods: A retrospective descriptive study was performed to analyze the distribution of relative frequencies of clinical and laboratory variables. The study was carried out in Rio Branco with confirmed dengue fever cases. Secondary data were obtained by Acre Epidemiological Surveillance teams of cases with bleeding or platelet counts under 100.0 × 109/L. The patients' clinical, laboratory and transfusion data were obtained from hospital records. Results: A total of 90,553 dengue cases were reported of which 7,447 had serologic confirmation; 267 cases had hemorrhagic manifestations and 193 patients were located. Nearly half of the patients had anemia and the mean of the lowest platelet count of these patients was 26.4 × 109/L. Platelet concentrate was transfused in 22.3% of cases with a mean of 7.5 IU/patient, fresh frozen plasma in 21.2% with a mean of 5.2 IU/patient and just 2.6% of patients received concentrated red blood cells with a mean of 3.2 IU/patient. Bleeding led to transfusions. Signs of plasma leakage and cardiopulmonary dysfunction were correlated to unfavorable outcomes...

Laboratory diagnostic pathways of bleeding and thrombosis diseases / 中华检验医学杂志

The important works for blood coagulation laboratory are providing evidences for diagnosing bleeding and thrombosis diseases.Besides to establish enough test items,the doctors of laboratory should follow pathways of lab test and clinical analysis to find the reasons for bleeding and thrombosis.In generally,screening test should be done first,such as prothrombin time,activated partial thromboplastin time,fibrinogen,fibrin/fibrinogen degradation product,D dimer,platelet count and platelet function test.Then the diagnostic tests are selected according to the screening test results and clinical condition.The last diagnosis can be two forms,one is diagnosis of disease,another one is diagnosis of targets such as malfunction of endothelial cell,blood coagulation factor,anticoagulation factors,fibrinolysis proteins and platelet count and function.

The promotion of a common view between laboratory tests and clinical diagnosis of hemorrhagic and thrombotic diseases / 中华检验医学杂志

A number of common view from Chinese experts with regard to diagnosis and treatment of hemorrhagic and thrombotic diseases had been recently released by Chinese Medical Association,in which is of importance about some items and its clinical application of thrombosis and hemostasis tests which have a significant effect on the decision-making of clinical diagnosis and treatment.It will be discussed that diagnostic standard,the key to the question and strategies of solution which based the common view in laboratory diagnosis for several kinds of hemorrhagic and thrombotic diseases,and it is very useful to the experts working on laboratory diagnosis of hemorrhagic and thrombotic diseases.

Distúrbios da hemostasia: doenças hemorrágicas / Disorders of homeostasis: bleeding disorders

As doenças hemorrágicas abrangem diversas condições clínicas, sendo caracterizadas por hemorragias de gravidade variável em diferentes locais do corpo. Podem ser de causa hereditária ou adquirida, relacionadas a doenças hematológicas ou a outras condições sistêmicas. Para o diagnóstico e tratamento adequados dessas doenças éfundamental a realização de anamnese detalhada e de testes laboratoriais, que podem ser complexos. Neste artigo serão abordadas as principais condições hemorrágicas, classificadas em doenças vasculares/doenças plaquetárias, coagulopatias e doenças hemorrágicas secundárias a doenças sistêmicas e uso de anticoagulantes.

The bleeding disorders include several clinical conditions, being characterized by bleeding of varying severity in different body sites. They can be either inherited or acquired disease - related to hematological diseases or other systemic conditions. For the diagnosis and treatment of these diseases, it is essential to conduct a detailed clinical history and laboratory tests, which may be complex. This article deals with the major hemorrhagic conditions, classified as vascular diseases/platelet diseases, coagulopathy and bleeding disorders secondary to systemic diseases and use of anticoagulants.

Uso del factor VII activado recombinante como agente hemostático en trastornos hemorrágicos / Use of recombinant-activated factor VIIa as a hemostatic agent in hemorrhagic disorders

La eficacia del tratamiento con factor VII activado recombinante (FVIIar) durante episodios hemorrágicos en pacientes hemofílicos con inhibidores y el conocimiento de su mecanismo de acción, determiná que en los últimos años se ampliara rápidamente su uso en pacientes con hemorragia de diversas causas no controladas con la terapéutica habitual; entre otras, defectos congénitos de la coagulación, trastornos plaquetarios, hepatopatías, cirugía, hemorragia intracraneal, sangramientos digestivos. Aunque un grupo importante de estas comunicaciones se han realizado en forma de casos reportados y serie de casos, se considera que los resultados obtenidos son importantes y que la administración de FVIIar es una alternativa en pacientes con hemorragia grave no controlada. A pesar de su potente acción procoagulante, el riesgo de complicaciones tromboembólicas es bajo y esté relacionado en un grupo importante de pacientes con la presencia de otros factores protrombóticos. En la actualidad se considera que el FVIIar esta indicado en aquellos pacientes con hemorragia masiva que no responden a la terapia con componentes sanguíneos ni a medidas quirúrgicas apropiadas

Effectiveness of the treatment with recombinant activated factor VII (raVII) during the hemorrhagic episodes in hemophilic patients using inhibitions and the knowledge of its action mechanism determined that in pas years its use will be expanded in patients with hemorrhage from non-controlled diverse causes using the usual therapeutics among other, congenital coagulation defects, platelet disorders, liver diseases, surgery, intracranial hemorrhage, digestive bleedings. Although a significant group of these communications have been carried out in reported cases and in cases series, it is considered that the results obtained are important and that the administration of raVII is an alternative in patients presenting with non-controlled severe hemorrhage. Despite its potent pro-coagulant action, thromboembolism complications risk is low and it is related to a significant group of patients with other prothrombotic factors. Nowadays, it is considered that raVII is prescribed in those patients with massive hemorrhage without response either to therapy using blood components or appropriate surgical measures

Idiopathic thrombocytopenic purpura and oral surgery: case report / Púrpura trombocitopênica idiopática e cirurgia bucal: relato de caso

OBJECTIVES: To review the function of platelets in the blood clotting mechanism. To address,conceptualize and classify thrombocytopenic purpura, especially idiopathic thrombocytopenic purpura (ITP),emphasizing the immunological aspects involved in the etiology of the disease. To address the clinicalmanifestations of the disease and the appropriate therapy. To present the case study of a patient with ITPwho requires oral surgical intervention. DISCUSSION: Leukopenia in ITP can be subclinical. The firstmanifestation of the disease can be severe hemorrhaging due to small lacerations or minor medical anddental surgical procedures. The cause of the platelet reduction is idiopathic; an autoimmune reaction in whichthe antibodies destroy the platelets appears to participate in the process. A variety of situations can occur,leading to mild to severe thrombocytopenia. One frequent aspect is the instability of the platelet count,which oscillates inconsistently and may be related to infections and other factors that have not been clearlydetermined, including stress. In the case presented here, the leukopenia in the patient was mild in presentation.However, it was decided that the patient should be referred to a hematologist for preparation and clearance.Oral surgery that compromises the bone, such as exodontia, can present significant difficulties in localizingand clamping intraosseous vessels, which does not only occur in small soft tissue surgeries. CONCLUSIONS:Platelet destruction in ITP occurs from a complex process that is comprised of multiple components of theimmune system. The platelets are prematurely destroyed by antibodies that are aimed at the platelet glycoprotein,which can results in serious, even fatal consequences. It is important to emphasize the significance of themedical history and the appropriate physical examination during the diagnostic process, as well as collaborationwith the patient’s medical...

OBJETIVOS: Revisar a função das plaquetas no mecanismo de coagulação sanguínea. Conceituare classificar a púrpura trombocitopênica, especialmente a púrpura idiopática, enfatizando osaspectos imunológicos envolvidos na etiologia da doença. Enfocar as manifestações clínicas dadoença e a terapia apropriada. Apresentar um caso de manifestação clínica da doença e a terapiaapropriada. Apresentar o caso de uma paciente com púrpura que necessitava de intervençãocirúrgica bucal. DISCUSSÃO: A leucopenia na púrpura trombocitopênica idiopática pode sersubclínica. A primeira manifestação da doença pode ser uma hemorragia severa por causa depequenas lacerações ou pequenos procedimentos médicos e odontológicos. A causa da reduçãodas plaquetas é idiopática; uma reação autoimune na qual os anticorpos destroem as plaquetasparece participar do processo. Uma variedade de situações pode ocorrer, levando àtrombocitopenia discreta a severa. Um aspecto frequente é a instabilidade da contagem plaquetária,que oscila inconsistentemente e pode relacionar-se com infecções e outros fatores ainda nãoclaramente determinados, incluindo estresse. No presente caso, o paciente apresentava discretaleucopenia. Entretanto, decidiu-se que o paciente deveria ser avaliado por um hematologista,para preparação e liberação para cirurgia, pois a cirurgia bucal que compromete osso, como aexodontia, pode apresentar dificuldades significativas na localização e pinçamento de vasosintraósseos, o que não acontece em pequenas cirurgias de tecidos moles. CONCLUSÕES: Adestruição plaquetária na púrpura trombocitopênica ocorre por meio de um complexo processo,com múltipos componentes do sistema imune. As plaquetas são destruídas prematuramente poranticorpos dirigidos contra as glicoproteínas plaquetárias, o que pode resultar em sérias – emesmo fatais – consequências. É importante enfatizar o significado da história médica e o examefísico adequado no processo diagnóstico...

Intoxicação aguda por samambaia (Pteridium aquilinum) em bovinos na Região Central do Rio Grande do Sul / Acute poisoning by bracken fern (Pteridium aquilinum) in cattle in central Rio Grande do Sul, Brazil

Foram revisados casos de intoxicação por samambaia (Pteridium aquilinum) em bovinos de 10 pequenas propriedades rurais de sete municípios da Região Central do Rio Grande do Sul. O estudo abrangeu 6.256 necrospias de bovinos realizadas num período de 43 anos e incluiu 15 necropsias de bovinos que morreram em conseqüência da intoxicação aguda por samambaia. As taxas de morbidade e mortalidade foram 17,9 por cento e a letalidade era virtualmente de 100 por cento. Em 40 por cento das propriedades a doença ocorreu em pequenos surtos afetando vários bovinos; em 60 por cento das propriedades apenas um bovino era afetado. Os principais sinais clínicos incluíam febre (40-42ºC), apatia, salivação e hemorragias, principalmente pelas gengivas, pela cavidade nasal e pelo trato gastrintestinal. Múltiplas petéquias eram observadas nas mucosas e na pele. Ocasionalmente observou-se hematúria e sangue no leite. A doença era invariavelmente fatal após um curso clínico de dois dias. Alterações hematológicas consistiam de leucopenia por neutropenia, anemia normocítica normocrômica e trombocitopenia arregenerativa. Os achados de necropsia incluíam hemorragias de intensidade variável em vários órgãos e infartos no fígado. Tanto as hemorragias quanto os infartos foram confirmados histologicamente; agregados de bacilos e vasos trombosados foram observados em associação com os infartos. Aplasia da medula óssea era um achado consistente nos quatro casos em que esse órgão foi examinado.(AU)

Cases of acute poisoning by bracken fern (Pteridium aquilinum) in cattle from 10 small farms of seven municipalities of the Central Region of Rio Grande do Sul State, Brazil, were reviewed. The study encompassed 6,256 necropsies of cattle considering a 43-year period and included 15 necropsies of cattle dying from acute bracken poisoning. Morbidity and mortality were 17.9 percent and lethality was virtually 100 percent. In 40 percent of the farms the disease occurred in small outbreaks affecting several cattle per farm and in 60 percent only one bovine was affected in each farm. Main clinical signs include fever (40-42ºC), apathy, drooling, and hemorrhages; the latter were mainly from the gums, nostrils, and gastrointestinal tract. Multiple petechiae were observed in several mucosae and in the skin. Occasionally hematuria and blood in the milk were reported. The disease was invariably fatal after a course of approximately two days. Hematological changes included severe neutropenia, non-regenerative normocytic normochromic anemia and thrombocytopenia. Necropsy findings include hemorrhages o varying degrees in several organs and infarcts in the liver. Both hemorrhages and hepatic infarcts were confirmed histologically; clusters of bacterial rods and thrombosed vessels were associated with the infarcts. Marked bone marrow aplasia was a consistent finding in the four cases in which the marrow was evaluated.(AU)