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3.
Blood Coagul Fibrinolysis ; 27(5): 517-25, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-26829362

RESUMO

Pulmonary embolism is a complication of deep vein thrombosis. It occurs in the population with a normal clotting mechanism, but it may also occur in patients with congenital bleeding conditions. Here, we report on all cases of pulmonary embolism in congenital hemorrhagic disorders. All reported cases of pulmonary embolism in congenital coagulation disorders have been gathered by a time-unlimited PubMed search. Cross-checking of the references listed at the end of the single papers was carried out to avoid omissions. Seventy-two patients had an objectively demonstrated pulmonary embolism. The event occurred in patients with fibrinogen, factor V, factor VIII (FVII), FVIII, FIX, and FXI deficiency, and in those with von Willebrand's disease. No embolism was reported in FII, factor X, and FXIII deficiency. Thirty were women and 28 were men, whereas in the remaining 14 cases, sex was not reported. Age varied from 6 to 81 years (mean age 34.3 years). The management varied from only supportive to the administration of unfractionated heparin, low-molecular-weight heparin, and anti-vitamin K medications, accompanied by adequate replacement therapy. Evolution was fair or good in the majority of cases, but there were 10 fatalities. Risk factors were present in 61 patients. The most frequent of these were replacement therapy (35 cases), surgery (34), and old age (13). Some patients had more than one risk factor. Eleven patients had no risk factors. There are discrepancies in the prevalence of pulmonary embolism among different clotting disorders. The conditions most frequently affected are FVII deficiency and fibrinogen defects. The significance of the findings is discussed.


Assuntos
Transtornos de Proteínas de Coagulação/sangue , Transtornos de Proteínas de Coagulação/tratamento farmacológico , Fibrinolíticos/uso terapêutico , Embolia Pulmonar/sangue , Embolia Pulmonar/tratamento farmacológico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Fatores de Coagulação Sanguínea/uso terapêutico , Criança , Transtornos de Proteínas de Coagulação/complicações , Transtornos de Proteínas de Coagulação/congênito , Feminino , Heparina de Baixo Peso Molecular/uso terapêutico , Humanos , Masculino , Pessoa de Meia-Idade , Embolia Pulmonar/complicações , Embolia Pulmonar/congênito
6.
Am J Clin Pathol ; 100(6): 701-3, 1993 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-8249920

RESUMO

Intrauterine pulmonary infarction is extremely rare. In a series of 775 stillborn and newborn infants, only a few cases of acute pulmonary infarction were reported. The authors present the findings from a full-term infant who died at 9 hours of age of a chronic pulmonary infarction. Several pathogenic mechanisms, including the entry of placental thromboplastin into the fetal circulation, are discussed.


Assuntos
Embolia Pulmonar/congênito , Embolia Pulmonar/patologia , Evolução Fatal , Humanos , Recém-Nascido , Masculino , Placenta/patologia , Embolia Pulmonar/diagnóstico
7.
Am J Med Genet ; 36(2): 167-71, 1990 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-2368803

RESUMO

We report an infant with a bronchiolitis-like illness and rapid deterioration who developed a cor pulmonale-like picture with a dilated right ventricle. Urinary organic acid assays established a probable diagnosis of Cbl-C-type methylmalonic aciduria, later confirmed by complementation studies. Despite medical intervention and cyanocobalamin treatment the patient died on his tenth hospital day. Postmortem examination showed the presence of thromboemboli in the pulmonary circulation. We hypothesize that acute cor pulmonale developed in this infant secondary to thromboembolism of his pulmonary circulation. A review of the literature shows that thromboembolism may be a part of this disease process.


Assuntos
Homocistinúria/complicações , Malonatos/sangue , Ácido Metilmalônico/sangue , Doença Cardiopulmonar/complicações , Ventrículos do Coração/patologia , Homocistinúria/patologia , Humanos , Lactente , Masculino , Mudanças Depois da Morte , Embolia Pulmonar/complicações , Embolia Pulmonar/congênito , Embolia Pulmonar/patologia , Doença Cardiopulmonar/congênito
8.
An Esp Pediatr ; 26(2): 115-7, 1987 Feb.
Artigo em Espanhol | MEDLINE | ID: mdl-3565951

RESUMO

Authors report a case of newborn with thrombosis of the pulmonary artery, probably caused by a maternal deficit of antithrombin III. Deficit of antithrombin III (AT III) as the possible cause of thrombosis is discussed.


Assuntos
Deficiência de Antitrombina III , Troca Materno-Fetal , Embolia Pulmonar/congênito , Feminino , Humanos , Recém-Nascido , Masculino , Gravidez
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