RESUMO
Factor XI deficiency is a rare disease found predominantly in Ashkenazi Jews. There is a poor correlation between factor XI level and bleeding in patients with factor XI deficiency. Individuals with severe factor XI deficiency are usually at risk of excessive bleeding after surgery and injury, particularly when trauma involves tissues rich in fibrinolytic activity. Women with partial or severe deficiency are at risk of excessive uterine bleeding during labor. The unpredictable nature of factor XI deficiency complicates management during pregnancy and delivery. This review gives an overview of the management of pregnant women with factor XI deficiency.
Assuntos
Deficiência do Fator XI , Complicações Hematológicas na Gravidez/terapia , Adulto , Anestesia Epidural , Anestesia Obstétrica/métodos , Raquianestesia , Perda Sanguínea Cirúrgica , Cesárea , Contraindicações , Desamino Arginina Vasopressina/uso terapêutico , Fator XI/genética , Fator XI/uso terapêutico , Deficiência do Fator XI/tratamento farmacológico , Deficiência do Fator XI/etnologia , Deficiência do Fator XI/genética , Feminino , Humanos , Recém-Nascido , Judeus/genética , Mutação , Plasma , Hemorragia Pós-Operatória/prevenção & controle , Hemorragia Pós-Parto/prevenção & controle , Gravidez , Complicações Hematológicas na Gravidez/tratamento farmacológico , Complicações Hematológicas na Gravidez/genética , Hemorragia Uterina/prevenção & controleRESUMO
The development of antibodies that inhibit or neutralize replacement therapy with factor VIII or factor IX is today the most serious complication of haemophilia and its treatment. Inhibitor patients have more severe joint morbidity than patients without inhibitors, and older adults experience significant orthopaedic disabilities. Because of the serious and disabling consequences of persistent inhibitors, there is considerable clinical and research interest in establishing effective bypassing agent regimens to prevent bleeding in inhibitor patients in much the same way as prophylaxis procedure works in noninhibitor patients. In the majority of these patients, the bypass agent was used as a secondary prophylactic. In this report, the use of recombinant factor VIIa prior to any clinically evident joint bleed in a patient with haemophilia A and high-titre inhibitor is described.
Assuntos
Fator VIIa/uso terapêutico , Hemofilia A/tratamento farmacológico , Pré-Escolar , Humanos , Masculino , Estudos Prospectivos , Proteínas Recombinantes/uso terapêutico , Resultado do TratamentoRESUMO
Until recently, orthopedic surgery was strongly contraindicated in patients with hemophilia and inhibitors. However, recent advances in our knowledge of bypassing agents (particularly recombinant activated factor VII [rFVIIa]) that provide effective surgical hemostasis have allowed us to successfully perform major orthopedic procedures in these patients. Adequate hemostasis during surgery and postoperative rehabilitation is crucial, as development of a wound hematoma may jeopardize long-term outcomes. It also should be noted that success depends not only on appropriate drug therapy but also on preoperative preparations and adequate perioperative surveillance. Preoperative assessment of vascular status is very important, and strong motivation--on the part of the patient, the surgeon, and the hematologist--is needed to ensure a satisfactory result. Although inhibitor patients undergoing surgery face a higher risk of bleeding and other complications than their non-inhibitor counterparts, outcomes are generally good if a multidisciplinary team approach is applied.
Assuntos
Inibidores dos Fatores de Coagulação Sanguínea/sangue , Hemofilia A/sangue , Deformidades Articulares Adquiridas/cirurgia , Procedimentos Ortopédicos , Fatores de Coagulação Sanguínea/uso terapêutico , Procedimentos Cirúrgicos Eletivos , Fator VIIa/uso terapêutico , Hemofilia A/complicações , Humanos , Deformidades Articulares Adquiridas/etiologia , Proteínas Recombinantes/uso terapêuticoRESUMO
Reactivation of hepatitis B infection is an increasing problem for patients with lymphoma, even in resolved infections, who were treated with rituximab-based regimens. Our cases point out the need of prolonged prophylaxis in HBsAg-negative patients due to the high risk of developing fatal reactivation.
Assuntos
Anticorpos Monoclonais/efeitos adversos , Hepatite B/induzido quimicamente , Ativação Viral/efeitos dos fármacos , Idoso , Anticorpos Monoclonais Murinos , Feminino , Guanina/análogos & derivados , Guanina/uso terapêutico , Hepatite B/tratamento farmacológico , Humanos , Linfoma de Células B/complicações , Linfoma de Células B/tratamento farmacológico , Pessoa de Meia-Idade , Rituximab , Resultado do TratamentoRESUMO
Hematopoietic stem cell transplantation is a procedure necessitating the administration of high-dose chemoradiotherapy. This therapy may induce aggressive disruptions that can lead to special nutritional and metabolic conditions. These patients are at an increased risk for malnutrition in the phase before transplantation and afterward. Artificial nutrition, total parenteral nutrition in particular, is provided to patients undergoing hematopoietic stem cell transplantation to help minimize adverse nutritional consequences.
Assuntos
Transplante de Células-Tronco Hematopoéticas , Distúrbios Nutricionais/prevenção & controle , Necessidades Nutricionais , Estado Nutricional , Nutrição Parenteral/métodos , Proteínas Alimentares/administração & dosagem , Ingestão de Energia/fisiologia , Nutrição Enteral , Humanos , Distúrbios Nutricionais/etiologiaAssuntos
Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Ciclofosfamida/administração & dosagem , Leucemia Mieloide Aguda/induzido quimicamente , Vidarabina/análogos & derivados , Anticorpos Monoclonais/administração & dosagem , Anticorpos Monoclonais Murinos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Feminino , Humanos , Linfoma Folicular/tratamento farmacológico , Pessoa de Meia-Idade , Rituximab , Vidarabina/administração & dosagemRESUMO
No disponible
Assuntos
Humanos , Vitamina B 12/administração & dosagem , Anemia Megaloblástica/tratamento farmacológico , Qualidade de VidaRESUMO
An accurate initial staging of patients with non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL) is critical for the selection of an appropriate treatment. Computed tomography (CT) remains the standard imaging technique, although it is based on anatomic criteria. Positron emission tomography (PET) with 2-deoxy-2-[fluorine-18]fluoro-d-glucose (FDG) provides useful functional information but requires anatomical correlation to localise lesions accurately. We have prospectively compared the accuracy of combined PET/CT with that of CT and PET alone at initial staging in lymphoma patients. Forty-seven newly diagnosed patients were evaluated. PET/CT was superior compared with CT and PET in nodal evaluation and detection of extranodal disease. Using a staging algorithm with PET/CT resulted in the disease stage being increased in 11 of 47 patients (10 NHL and 1 HL) (McNemar test P = 0.012). Therefore, a different treatment strategy based on PET/CT findings was suggested for seven patients (14.8%). PET/CT markedly improves accuracy in the diagnostic work-up of patients with lymphoma.
Assuntos
Linfoma/diagnóstico por imagem , Tomografia por Emissão de Pósitrons/métodos , Tomografia Computadorizada por Raios X/métodos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Algoritmos , Medula Óssea/patologia , Feminino , Fluordesoxiglucose F18 , Doença de Hodgkin/diagnóstico por imagem , Doença de Hodgkin/tratamento farmacológico , Humanos , Linfoma/tratamento farmacológico , Linfoma não Hodgkin/diagnóstico por imagem , Linfoma não Hodgkin/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Estudos Prospectivos , Compostos Radiofarmacêuticos , Resultado do TratamentoAssuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Doença de Hodgkin/tratamento farmacológico , Carmustina/uso terapêutico , Citarabina/uso terapêutico , Esquema de Medicação , Etoposídeo/uso terapêutico , Humanos , Melfalan/uso terapêutico , Recidiva , Terapia de SalvaçãoRESUMO
No disponible
Assuntos
Humanos , Anemia Megaloblástica/diagnóstico , Anemia Megaloblástica/tratamento farmacológico , Anemia Megaloblástica/etiologia , Vitamina B 12/uso terapêuticoAssuntos
Injúria Renal Aguda/complicações , Sarcoma Histiocítico/complicações , Dermatopatias Papuloescamosas/complicações , Injúria Renal Aguda/terapia , Idoso , Antineoplásicos/uso terapêutico , Sarcoma Histiocítico/diagnóstico , Sarcoma Histiocítico/tratamento farmacológico , Humanos , Imageamento por Ressonância Magnética , Masculino , Dermatopatias Papuloescamosas/diagnóstico , Resultado do TratamentoRESUMO
No disponible
Assuntos
Humanos , Células-Tronco Hematopoéticas , Transplante de Células/ética , Consentimento Livre e Esclarecido/ética , Inquéritos e Questionários , Satisfação do PacienteRESUMO
No disponible
Assuntos
Masculino , Idoso , Humanos , Sarcoma Histiocítico/diagnóstico , Diagnóstico Diferencial , Plexo Braquial/patologia , Úmero/patologia , Sarcoma Histiocítico/fisiopatologiaAssuntos
Hemofilia A , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Antibacterianos/efeitos adversos , Anticonvulsivantes/efeitos adversos , Autoanticorpos/imunologia , Doenças Autoimunes/complicações , Doenças Autoimunes/imunologia , Criança , Fator VIII/imunologia , Feminino , Neoplasias Hematológicas/complicações , Neoplasias Hematológicas/imunologia , Hemofilia A/diagnóstico , Hemofilia A/imunologia , Hemofilia A/terapia , Hemostáticos/uso terapêutico , Humanos , Imunoglobulinas Intravenosas , Técnicas de Imunoadsorção , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Plasmaferese , Gravidez , Complicações Hematológicas na Gravidez/diagnóstico , Complicações Hematológicas na Gravidez/imunologia , Complicações Hematológicas na Gravidez/terapia , PrognósticoRESUMO
OBJECTIVE: To assess the efficacy, safety, and complications of otolaryngologic surgery in children with von Willebrand disease (vWD) undergoing surgery. DESIGN: A prospective, controlled study of 41 children with vWD who underwent surgery between June 1, 1999, and January 31, 2001. SETTING: A tertiary care, university-based children's hospital. INTERVENTIONS: All children had a preoperative diagnosis of vWD. The patients were treated with either a protocol that includes the use of desmopressin acetate and tranexamic acid (37 children) or factor VIII concentrate in children with a positive history of seizures (4 children). MAIN OUTCOME MEASURES: Immediate and delayed postoperative bleeding, hyponatremia, seizures, and urine output. RESULTS: Two adenotonsillectomy patients (5%) had an immediate postoperative hemorrhage. Delayed postoperative bleeding was not detected in our patients. Severe hyponatremia occurred in 2 patients (1 of them with clinical manifestations). CONCLUSIONS: Our management of children with vWD was efficacious in otolaryngologic surgery. One child had important adverse effects with the use of desmopressin (seizure). Thus, the use of desmopressin should be weighed and closely monitored.