The study of disease pattern of various clinical classes of hemophilia in South Gujarat area, India

Background: Haemophilia is one of the most severe bleeding disorder. Seventy-five per cent of the global haemophiliacs live in developing countries, where probably only one in five cases is diagnosed, and there is little or no care available. Haemophilia as a disease and its management has a large impact on the community, including social integration and economics. Inability to be an active part of society and high cost of the medical care make this disease an important problem for all haemophilic patients.Methodology: a cross-sectional study, conducted over a period of one and half year. Institutional Ethics Committee permission was taken before starting the study. Information was gathered through personal interview of the patient's guardian with the help of predesigned questionnaires. Special emphasis was given to past history of bleeding. For each specific symptom/ specific site a detailed history and relevant details was taken and number of episodes of each symptom or number of episodes of involvement of a specific site was written.Observations: Out of 75 patients of haemophilia; 8 (10.7%) were Mild haemophilic, 17 (22.7%) were moderate haemophilic; and 50 (66.7%) were severe haemophilic. Most common site for spontaneous bleeding was muscle where 121 (25%) episodes of bleeding were reported, followed by knee joint 97(20%), and Ankle 79 (16.3%). The commonest site for traumatic bleeding was muscle where 201 (24.7%) episodes of bleeding were reported, followed by knee joint 175 (21.5%), Ankle 143 (17.6%) and Elbow joint 83 (10.2%). Site with highest duration of bleeding was intracranial for 6 days.Conclusion: Frequency of traumatic dental and nasal bleeds was significantly higher in mild class as compared to moderate and severe classes. In severe category Knee, ankle, elbow and muscle were the commonest site where traumatic bleeding. There was no significant difference in bleeding frequencies at various sites in moderate and severe cases, whether spontaneous or traumatic bleed.

Sangrado espontáneo masivo en adulto conocido sano / (Massive spontaneous bleeding)

ResumenSe reporta el caso de un paciente que presentó de manera espontánea diátesis hemorrágica, sin tener causa alguna aparente que lo justificase. Tal diátesis hemorrágica forja un amplio apartado de posibilidades diagnósticas en cuanto a trastornos de la coagulación del adulto se refiere, en el contexto de un paciente conocido sano que nunca ha presentado episodios de sangrado mayor y debuta con hemorragias de forma masiva. Entre las muchas posibilidades diagnósticas se encuentra una poco conocida: la hemofilia adquirida. La hemofilia adquirida es un trastorno infrecuente de la hemostasia, caracterizado por la presencia de inhibidores adquiridos de los factores de la coagulación, en el plasma del paciente enfermo. Los inhibidores adquiridos son anticuerpos que a su vez podrían ser de tipo aloanticuerpos o autoanticuerpos. Los aloanticuerpos se desarrollan en pacientes deficitarios per se de factores de la coagulación, en respuesta a la terapia de sustitución de factores, lo que complica su tratamiento. Por su parte, los autoanticuerpos se desarrollan en sujetos sin defectos previos; son anticuerpos específicos contra un factor de la coagulación, afectando o no su función, alterando una o varias etapas de las vías de la coagulación. El caso que aquí se presenta es de un paciente masculino de 58 años, quien se presentó con sangrado espontáneo masivo y a quien se diagnosticó hemofilia adquirida por la presencia de autoanticuerpo específico del factor VIII.

AbstractA patient that presented with spontaneous hemorrhagic diathesis, with no apparent cause is presented. Hemorrhagic diathesis presents a wide range of diagnosis possibilities as of coagulation disorders are referred, in the context of a healthy patient with no previous major bleeding episodes and that debuts with massive hemorrhages. Acquired hemophilia, a little known disease, is one that must be considered.Acquired hemophilia is an uncommon hemostasis disorder characterized by the presence of acquired inhibitors of coagulation factors in the plasma of the sick patient. These acquired inhibitors, are antibodies that could be alloantibodies or autoantibodies. Alloantibodies are developed in patients who have coagulation factor deficiency, in response to factor replacement therapy, thus complicating treatment.On the other hand, autoantibodies are developed in people without previous defects and are specific against a factor of coagulation, affecting or not their function, obstructing one or several stages of the coagulation pathways. We report a case of acquired hemophilia due to an autoantibody against factor VIII in a 58 years old male patient with spontaneous massive bleeding.

Spontaneous Lumbar Artery Bleeding and Retroperitoneal Hematoma in a Patient Treated with Continuous Renal Replacement Therapy / 대한구급학회지

Rupture of the lumbar artery is usually associated with trauma but rarely has been reported in association with anticoagulation. We present a 71-year-old man who developed spontaneous rupture of the lumbar artery leading to a retroperitoneal hematoma while receiving continuous renal replacement therapy (CRRT). The bleeding was confirmed by computed tomography and angiography and was controlled successfully using selective angiographic embolization. We suggest that spontaneous retroperitoneal bleeding should be considered in a case of sudden decrease in hemoglobin in a CRRT patient.

Spontaneous Lumbar Artery Bleeding and Retroperitoneal Hematoma in a Patient Treated with Continuous Renal Replacement Therapy / 대한중환자의학회지

Rupture of the lumbar artery is usually associated with trauma but rarely has been reported in association with anticoagulation. We present a 71-year-old man who developed spontaneous rupture of the lumbar artery leading to a retroperitoneal hematoma while receiving continuous renal replacement therapy (CRRT). The bleeding was confirmed by computed tomography and angiography and was controlled successfully using selective angiographic embolization. We suggest that spontaneous retroperitoneal bleeding should be considered in a case of sudden decrease in hemoglobin in a CRRT patient.

Prueba de torniquete como predictiva de sangrado espontáneo en casos de dengue clásico por den-2 / Tourniquet test as predictive of spontaneous bleeding in classic dengue, den-2, cases

Objetivo: Determinar el valor de la prueba de torniquete como predictiva de sangrado espontáneo en pacientes afectados por dengue clásico en un brote por el virus DEN-2. Materiales y métodos: Se realizó un estudio caso-control con una población de 267 pacientes egresados de la Unidad de Atención del Dengue del Hospital Monseñor Sanabria en Puntarenas, con diagnóstico de dengue clásico, en el periodo de julio de 1999 a junio de 2002, durante el brote de virus DEN-2 en la Región Pacífico Central de Costa Rica. Hubo un total de 61 pacientes con sangrado espontáneo, casos, y 181 pacientes sin el evento, controles. Se calculó la capacidad predictiva de la prueba de torniquete mediante una tabla de contingencia con la que se determinaron las características diagnósticas de la prueba así como la razón de posibilidades, Odds ratio y la fracción etiológica, tomando como prueba de oro la condición de sangrado espontáneo. Resultados: La sensibilidad y especificidad de la prueba fueron 41.0 por ciento y 75.1 por ciento, respectivamente; asimismo, el valor predictivo positivo fue de 35.7 por ciento, mientras que el valor predictivo negativo fue de 79.1 por ciento. Los pacientes con diagnóstico de dengue por DEN-2 con la prueba de torniquete positivo tuvieron el doble de posibilidad de sufrir de sangrado espontáneo que aquellos con resultado negativo a la prueba, OR igual 2.1; IC 95 por ciento: 1.1-3.9. Conclusión: La prueba de torniquete no es confiable para indicar fragilidad capilar y no debería utilizarse de manera definitiva para clasificar al paciente que requiere hospitalización por DEN-2.

Objective: To determine the significance of the tourniquet test as predictive of spontaneous bleeding in classic dengue fever patients in an DEN-2 virus outbreak. Materials and methods: A casecontrol study on 267 patients at the Dengue Unit of the Monseñor Sanabria Hospital in Puntarenas, diagnosed as classic dengue fever, between July 1999 and June 2002, during the Central Pacific Area DEN-2 virus outbreak. There were a total of 61 patients with spontaneous bleeding (cases) and 181 controls. The predictive ability of the tourniquet test, the odds ratio and the etiologic fraction were assessed using a 2x2 table, using the spontaneous bleeding condition as golden standard. Results: Sensitivity and specificity of the positive tourniquet were 41.0% and 75.1%, respectively; besides, the predictive positive value was 35.7% while the predictive negative value was 79.1%. Patients diagnosed with dengue by DEN-2 virus, with a tourniquet positive test had two times the chance of spontaneous bleeding than those with a negative test (OR= 2.1, 95% IC: 1.1-3.9). Conclusion: The tourniquet test is not reliable to predict capillary fragility; hence it should not be used to classify patients that require hospitalization in cases of dengue fever caused by DEN-2 virus.

Spontaneous Rupture of Lumbar Artery & Inferior Epigastric Artery in a Hemodialysis Patient / 대한신장학회잡지

We report a case of retroperitoneal hemorrhage and abdominal wall hemorrhage due to spontaneous lumbar artery and inferior epigastric artery rupture, in a patient with chronic renal failure treated with hemodialysis. There was no history of specific trauma. The bleeding was confirmed by CT and angiography, and controlled successfully by selective angiographic embolization. We suggest that a possibility of spontaneous retroperitoneal bleeding should be considered in a case of abdominal pain or mass in a hemodialysis patient.