Hemorheological and Hemostatic Parameters in Children with Nephrotic Syndrome Undergoing Steroid Therapy

Abstract

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.

Coagulopathy of Childhood Nephrotic Syndrome – A Reappraisal of the Role of Natural Anticoagulants and Fibrinolysis

“…The elevation in the levels of the natural anticoagulants protein C and S in the relapse of nephrosis is in line with earlier studies [1,7,[12][13][14], During remission the level of protein C increased further in some patients, re mained unchanged or decreased in others; almost all values were in the upper normal range, at a time when the levels of other hae mostatic factors normalized.…”

“…Andre et al [14], in a recent report, re viewed the conflicting data on the protein C level in nephrotic syndrome, and concluded, on the basis of their own data, that corticoste roid therapy may have a direct effect in caus ing the markedly elevated protein C levels in the remission phase of nephrosis. The data obtained in our study does not support this suggestion.…”

“…In a previous study [23] fibrinolytic activity induced by ve nous occlusion was found to be higher in adult nephrotic patients than healthy controls. Ear lier investigators measured plasminogen, cuantiplasmin, ou-macroglobulin, ai-antitrypsin [1,3,5,7,9,11,14], but reported very con flicting results.…”

“…Despite the ex tensive research, the thrombophilie changes in childhood nephrosis are not fully under stood and there is still a need for more charac terisation of these abnormalities. This re mains to be of interest in view of the fact that the thromboembolic complications associat ed with nephrotic syndrome occur with a much lower frequency in children than in adults, despite the more pronounced prothrombotic coagulation abnormalities in the former [1], There is therefore a need for more haemostatic studies to supplement the alrea dy available literature [1][2][3][4][5][6][7][8][9][10][11][12][13][14]. This is facili tated by the currently available more specific and sensitive assays of small haemostatic frag ments especially those believed to be respon sible for preventing thrombosis, i.e.…”

Thalamic stroke secondary to straight sinus thrombosis in a nephrotic child

“…Decreased antithrombin level may be a major factor in the hypercoagulability status and attributed to venous thrombosis. During relapse of NS, an increase in the concentrations of protein C (148±59%) and protein S (109±41%) were reported in steroid-resistant nephrotic children [9]; in contrast, a deficiency of protein C and protein S was reported in cases of CST [5]. As we missed his antithrombin level in the initial coagulation study, we could not ascertain its actual role in this patient.…”