Assuntos
Síndrome de Secreção Inadequada de HAD/complicações , Insônia Familiar Fatal/complicações , Síndromes Mielodisplásicas/complicações , Idoso , Substituição de Aminoácidos , Anemia Macrocítica/etiologia , Deleção Cromossômica , Cromossomos Humanos Par 20/ultraestrutura , Códon/genética , Feminino , Predisposição Genética para Doença , Humanos , Insônia Familiar Fatal/genética , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Mutação de Sentido Incorreto , Síndromes Mielodisplásicas/genética , Mutação Puntual , Proteínas Priônicas , Príons/genética , Irmãos , Tálamo/patologia , Vietnã/etnologiaRESUMO
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.
Assuntos
Cateterismo Cardíaco , Hemofilia A/cirurgia , Procedimentos Cirúrgicos Torácicos , Adolescente , Adulto , Idoso , Valva Aórtica , Aspirina/efeitos adversos , Aspirina/uso terapêutico , Cateterismo Cardíaco/efeitos adversos , Angiografia Coronária/efeitos adversos , Ponte de Artéria Coronária/efeitos adversos , Fator IX/administração & dosagem , Fator IX/imunologia , Fator IX/metabolismo , Fator VIII/administração & dosagem , Fator VIII/imunologia , Fator VIII/metabolismo , Anticorpos Anti-HIV/sangue , Implante de Prótese de Valva Cardíaca , Hemofilia A/complicações , Hemofilia A/tratamento farmacológico , Hemofilia B/complicações , Hemofilia B/tratamento farmacológico , Hemofilia B/cirurgia , Hemorragia/tratamento farmacológico , Hemorragia/etiologia , Hemostasia/efeitos dos fármacos , Antígenos de Superfície da Hepatite B/sangue , Anticorpos Anti-Hepatite C/sangue , Humanos , Isoanticorpos/sangue , Pessoa de Meia-Idade , Isquemia Miocárdica/complicações , Procedimentos Cirúrgicos Torácicos/efeitos adversos , Varfarina/efeitos adversosRESUMO
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.
Assuntos
Células Matadoras Naturais , Leucemia Linfoide/patologia , Linfoma não Hodgkin/patologia , Adulto , Evolução Fatal , Humanos , Imunofenotipagem , Células Matadoras Naturais/imunologia , Leucemia Linfoide/imunologia , Linfoma não Hodgkin/imunologia , MasculinoRESUMO
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.