Antithrombin III assay using thrombin in disseminated intravascular coagulation (DIC), other thromboembolic disorders and hepatic diseases.

Fifty cases comprising 11 cases of disseminated intravascular coagulation (DIC), 16 cases of venous thromboses and 23 cases of hepatic diseases were studied for AT III levels using clotting assay. Twelve samples were subjected to ATIII estimation by the commercially available synthetic chromogenic assay. Twenty age and sex matched controls were also analysed to find out the reference value for the techniques. Low AT III levels, if present, were correlated with other markers of DIC, viz FDP and D-dimer assays. There was a decrease in the AT III levels in all the three disease categories with a significant difference between the AT III levels of the three disease categories. In DIC, lowest levels were observed which correlated well with FDP and D-dimer levels. There was no significant difference between the average AT III levels measured by both the clotting and synthetic chromogenic assay with the former procedure being relatively inexpensive.

Deficiência de proteína C da anticoagulaçåo: estudo de 5 casos / Protein C deficiency: a study of 5 cases

O artigo descreve os achados clínicos e laboratoriais de cinco portadores de deficiência heterozigótica de Proteína C (PC). Três pacientes eram do sexo feminino e a idade média ao diagnóstico era de 48 anos. Em apenas um dos portadores pôde-se identificar fator clínico predisponente de tromboembolismo venoso. Nenhum dos pacientes apresentou sinais clínicos ou laboratoriais de insuficiência hepática, coagulopatia de consumo persistente, hipovitaminose K ou uso de anticoagulantes orais que sugerissem a presença da forma adquirida da deficiência. Em todos os casos pôde-se realizar os testes imunológicos para PC, caracterizando-os como portadores de deficiência tipo I (quantitativa). Os estudos familiares foram possíveis em quatro destes pacientes, detectando-se novos portadores assintomáticos em três famílias, com padråo de herança autossômico dominante. A pesquisa de inibidores antiPC no plasma destes pacientes foi negativa. Os pacientes foram acompanhados clinicamente após diagnóstico hematológico. Uma paciente apresentou necrose subcutânea durante a fase inicial do uso de anticoagulante oral. Outra recusou esta forma de terapia, tendo sido implantado o filtro de Greenfield em sua veia cava. Quatro pacientes receberam anticoagulaçåo oral prolongada (média de 36 meses de uso continuado). Houve um episódio de recediva com tromboflebite superficial genital e um de hematúria, sem sequelas graves

Protein C and protein S deficiency in thalassemic patients.

To investigate the status of the protein C-protein S anticoagulant pathway in thalassemic patients, we measured protein C and protein S levels of plasma of 30 adults and 18 children with beta-thalassemia/HbE disease, beta-thalassemia major and HbE disease. Mean +/- 1 SD values of protein C, protein S and other coagulant proteins produced by the liver were as follows: protein C 50.4 +/- 17.2%; protein S 58.8 +/- 25.5%; antithrombin III 78.1 +/- 12.8%; PLG 86.4 +/- 18.4%; prothrombin 71.0 +/- 13.1%; factor VII 72.7 +/- 21.5%; and factor X 79.2 +/- 15.6%. Protein C and protein S levels of thalassemic patients were significantly lower than those of other coagulant proteins produced by the liver. Decrease in protein C level was stronger than that of proteins S. gamma-Carboxylated protein C levels of splenectomized patients were significantly lower than those of nonsplenectomized patients. Severe decrease of protein C and protein S may be responsible for occurrence of thrombosis in thalassemic patients.

Deficiência de proteína C da coagulaçäo e antitrombina III em pacientes com tromboembolismo de repetiçäo / Protein C and antithrombin III deficiency in patients with recurrent thromboembolic episodes

A pesquisa de deficiência de antitrombina III e proteína C deve ser feita em casos individuais de resistência ao uso de anticoagulantes, de tromboses recidivantes ou em jovens (especialmente na ausência de outros fatores de risco) e em famílias com fenômenos tromboembólicos de repetiçäo. Nesta série altamente selecionada de 18 pacientes, detectaram-se seis casos de deficiência de proteína C e um de antitrombina III. Nos pacientes deficientes sintomáticos, a anticoagulaçäo a longo prazo é recomendada e a triagem familiar, desejävel

Thrombo-embolism in minimal change nephrotic syndrome.

A case report of thrombo-embolism in minimal change nephrotic syndrome was presented. Autopsy revealed massive bilateral pulmonary emboli, edema and congestion, bilateral adrenocortical atrophy and left renal vein thrombosis. Thrombo-embolic phenomenon should be borne in mind during management and treatment of nephrotic syndrome.