Fibrinolytic study in a homozygous protein C deficient patient

Aznar, Justo; Dasí, A. Real; España, Francisco; Estellés, Amparo

doi:10.1016/0049-3848(86)90260-4

Cited by 11 publications

(1 citation statement)

References 32 publications

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“…Similar results have been obtained by others. Aznar et al (48) found normal fibrinolytic activity in a patient with homozygous protein C deficiency and in heterozygous relatives. Conard et al (49) also found normal fibrinolytic activity in patients with heterozygous protein C deficiency.…”

Section: Discussionmentioning

confidence: 96%

Thromboembolic Disease – Critical Evaluation of Laboratory Investigation

et al. 1992

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SummaryPrevious studies of patients with thromboembolic disease have revealed an association either with hereditary anticoagulant protein deficiencies or with defects in the fibrinolytic system. To obtain a more comprehensive picture and to investigate which analyses are useful in the evaluation of such patients, we have performed an extensive laboratory investigation in 439 individuals with thromboembolic disease. Anticoagulant protein deficiencies were found in 24 patients. Deficiencies of protein C (n = 10) and protein S (n = 9) were most common followed by deficiencies of antithrombin III (n = 3) and plasminogen (n = 2). Six of the nine protein S deficient patients demonstrated a selective deficiency of free protein S with normal total protein S concentrations. To diagnose protein C and S deficiencies among the 201 patients receiving oral vitamin K antagonists, the concentrations of protein C and S were compared with the mean concentration of several other vitamin K-dependent proteins. One protein C and three protein S deficiencies were identified among the treated patients. The number of protein C deficiencies found in this group was significantly lower than the number found among untreated patients. Although fewer protein S deficiencies were also identified among the treated patients, than in the untreated group, the difference was not statistically significant. The results suggest that protein C deficiencies went undetected in the treated group and that oral anticoagulant therapy should be discontinued before efforts to diagnose protein C deficiency are made. We found no cases with heparin cofactor II deficiency. Lupus anticoagulant was present in 10 patients. Evaluation of the fibrinolytic system revealed that the patient group had slightly lower mean euglobulin fibrinolytic activity (EFA) after venous occlusion than controls and a subgroup (approximately 15%) of patients with EFA below the level of the 5th percentile of controls, could be distinguished. Repeated analysis demonstrated a substantial individual day-to-day variation in both patients and controls and the combined EFA results did not clearly distinguish patients from controls. There was a significant negative correlation between EFA and plasminogen activator inhibitor (PAI) levels in both patients and controls and the patient group had significantly higher levels of PAI than the control group. In contrast, there was no difference between controls and patients in tissue plasminogen activator (tPA) release after venous occlusion and no correlation between EFA and tPA was observed. These results suggest that although a statistically significant difference between patients and controls in values of fibrinolytic parameters was found, an extensive laboratory evaluation of the fibrinolytic system in individual patients may not be warranted. The association between patients with thrombosis and deficiencies of anticoagulant proteins suggests that the investigation of individual patients should focus on these components.

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Section: Discussionmentioning

confidence: 96%

Thromboembolic Disease – Critical Evaluation of Laboratory Investigation

et al. 1992

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Fibrinolysis

Bachman¹

1989

Clinical Chemistry

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Clinical disorders of fibrinolysis: A critical review

Francis

1989

Blut

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Much progress has recently been made in understanding the biochemistry and physiology of endogenous fibrinolysis. As a result, a better understanding of the mechanisms and clinical consequences of disordered fibrinolysis has emerged. Increased fibrinolytic activity is an uncommon but important cause of hemorrhagic disease. Congenital disorders of fibrinolysis which cause bleeding include increased plasma plasminogen activator activity and deficiency of alpha-2 antiplasmin. Acquired disorders associated with increased fibrinolytic activity and bleeding include liver cirrhosis, amyloidosis, acute promyelocytic leukemia, some solid tumors, and certain snake envenomation syndromes. Increased fibrinolysis is important to recognize because epsilon-aminocaproic acid (EACA) may be required to prevent or control bleeding. Diminished fibrinolytic activity has been associated with a variety of thrombotic disorders, but a direct cause-and-effect relationship has yet to be established. Congenital abnormalities of fibrinolysis associated with thrombosis include plasminogen deficiency, decreased endothelial generation of plasminogen activator activity, and certain abnormal fibrinogens. Thrombosis in these disorders is effectively managed with warfarin. Diminished fibrinolysis has also been reported in "idiopathic" venous thrombosis, oral contraceptive-induced and post-operative venous thrombosis, coronary artery disease, cerebrovascular disease, systemic lupus erythematosus, and thrombotic thrombocytopenic purpura, but the significance of abnormal fibrinolysis in these disorders is uncertain. Large, prospective studies of fibrinolytic variables as risk factors for vascular and thrombotic disease are needed to determine whether pharmacologic augmentation of impaired fibrinolysis could be useful in the prevention or treatment of these disorders.

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Fibrinolytic study in a homozygous protein C deficient patient

Cited by 11 publications

References 32 publications

Thromboembolic Disease – Critical Evaluation of Laboratory Investigation

Thromboembolic Disease – Critical Evaluation of Laboratory Investigation

Fibrinolysis

Clinical disorders of fibrinolysis: A critical review

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