Disturbances in hemostatic and hemorheological parameters have been investigated in a group of 29 children with nephrotic syndrome: 23 children classified as steroid-sensitive and 6 as steroid-resistant. Studies were performed before prednisone treatment and 3 weeks later, after initiation of steroid therapy. Before treatment, the alterations in hemostatic system involved moderate thrombocytosis with spontaneous aggregation in 19 patients. High levels of fibrinogen, factor VIII, Willebrand factor, protein C, protein S and α2-macroglobulin (α2M) were observed. Factor XII and α1-antitrypsin (α1AT) were lower than normal. Antithrombin III (ATIII) level was normal in the majority of patients. A plasma and blood hyperviscosity syndrome was also observed as well as an increase in erythrocyte aggregation. During treatment, an improvement in the hemostatic parameters was observed in the patients who responded to prednisone. The expected increase in factor VIII (frequently described in the literature) was not observed, while there was a significant increase in protein C. In the steroid-resistant patients, the only significant changes observed were decreased fibrinogen and increased protein C. The hemorheological parameters showed a tendency towards normality regardless of whether or not the treatment provided remission of NS. The relationship between hemorheological and hemostatic factors changes are discussed.
In a nonrelated white family, the Fletcher factor level in the father was 0.41 U/ml and in the mother, 0.30 U/ml (controls, 0.75-1.25 U/ml). One sibling with recurrent epistaxis had a level of 0.012 U/ml, whereas the other without tendency to spontaneous bleeding had levels between 0.75 and 0.32 U/ml. This suggests autosomal recessive transmission with clinical symptoms when the defect is homozygous. HLA antigens were studied to determine whether characters of the histocompatibility system and this defect are linked: we determined that the gene(s) of the disease is (are) not shared on the HLA complex.
Several members of the same family underwent examinations on the coagulation and fibrinolysis systems after recurring thrombo-embolic disease was observed in one of the members of the family. The results of biological examinations revealed normal coagulation factors, but a high level of plasminogen activation inhibitors accompanied by an increase in platelet aggregation was observed. These results are compared with the few similar cases reported by other authors. The exact part played in this particular case by the platelets and the inhibitor in the patient's predisposition to venous thrombosis and the spread of the disease is discussed. It would seem that the platelet abnormality merely accompanys the increase in inhibitors which is passed on by recessive autosomal transmission. The authors consider that in all cases of thrombosis, particularly recurring thrombosis, an investigation, comprising a study of the fibrinolysis factors and particularly the inhibitors, must be carried out on the patient's close family.
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