Asunto(s)
Síndrome de Secreción Inadecuada de ADH/complicaciones , Insomnio Familiar Fatal/complicaciones , Síndromes Mielodisplásicos/complicaciones , Anciano , Sustitución de Aminoácidos , Anemia Macrocítica/etiología , Deleción Cromosómica , Cromosomas Humanos Par 20/ultraestructura , Codón/genética , Femenino , Predisposición Genética a la Enfermedad , Humanos , Insomnio Familiar Fatal/genética , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Mutación Missense , Síndromes Mielodisplásicos/genética , Mutación Puntual , Proteínas Priónicas , Priones/genética , Hermanos , Tálamo/patología , Vietnam/etnologíaRESUMEN
A 32 year old Caucasian male presented with fever, massive hepatosplenomegaly and lymphadenopathy. Peripheral blood and bone marrow findings were consistent, both morphologically and immunophenotypically, with a natural killer cell leukemia/lymphoma. The disease was rapidly progressive and was fatal within five days after presentation.
Asunto(s)
Células Asesinas Naturales , Leucemia Linfoide/patología , Linfoma no Hodgkin/patología , Adulto , Resultado Fatal , Humanos , Inmunofenotipificación , Células Asesinas Naturales/inmunología , Leucemia Linfoide/inmunología , Linfoma no Hodgkin/inmunología , MasculinoRESUMEN
Full dose heparin therapy is monitored by a variety of laboratory methods, of which the activated partial thromboplastin time (APTT) is the most popular. A large number of APTT reagents are currently available, with different sensitivities to heparin evident in many. Within the literature it is apparent that there is a lack of consensus, and indeed some confusion, regarding the therapeutic ranges for the APTT for standard heparin therapy in the treatment of venous thromboembolic disease. Accordingly we conducted an Australasian survey to evaluate current laboratory and clinical practices in monitoring heparin therapy, to determine the extent of variation in the approach and to stimulate the process of standardisation of acceptable procedures and methodology. Results of the survey demonstrate that currently there is no uniform practice used to establish therapeutic ranges for monitoring standard heparin therapy. Furthermore, results suggest that current practice may lead to subtherapeutic anticoagulation in many laboratories.
Asunto(s)
Monitoreo de Drogas , Heparina/uso terapéutico , Laboratorios/normas , Tiempo de Tromboplastina Parcial , Australia , Humanos , Nueva ZelandaRESUMEN
The present study summarizes the results of 12 cardiac surgical procedures performed in a carrier of Haemophilia B and in six patients with Haemophilia A at a single centre from 1979 to 1998. The median age of the patients at the time of intervention was 56 years ranging from 18 years to 73 years. The six patients with Haemophilia A ranged in severity from moderately to mildly affected. Three patients were hepatitis C antibody positive. No patients were HIV antibody or hepatitis B surface antigen positive. The cardiac procedures included cardiac catheterization (n=4), coronary artery bypass surgery (n=2), percutaneous transluminal coronary angioplasty (n=1), cardiac valve replacement (AVR n=1 and AVR/MVR n=2), and closure of an atrial septal defect and subsequent drainage of a pericardial effusion (n=1). No patients had demonstrable inhibitors at the time of surgery. Haemostasis was achieved with AHF in 10/11 procedures and high purity factor IX (Immunine) in one procedure. The initial procedures involved intermittent bolus factor therapy while more recently, AHF was administered by continuous intravenous infusion. All patients demonstrated excellent intra- and post-operative haemostasis. These results, although from a small and varied group of patients, demonstrate that cardiac surgical procedures can be performed safely in patients with Haemophilia.