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Published on: September 9, 2012
Hypercoagulability risk factors in children with minimal change disease and the protective role of protein-C activity
Neşe Ozkayin1, Sevgi Mir, Kaan Kavakli
1Department of Pediatric Nephrology, Ege University Hospital, 35100 Bornova, Izmir, Turkey. ozkayin@egenet.com.tr
Insights
Children with minimal change disease exhibit increased thrombosis risk during attacks, indicated by elevated procoagulant factors and hyperviscosity. However, heightened protein-C activity and fibrinolytic factors act as protective mechanisms against this thrombotic tendency.
Area of Science:
- Pediatric Nephrology
- Hematology
- Thrombosis Research
Background:
- Nephrotic syndrome involves plasma factor imbalance, leading to thrombotic activity.
- Childhood minimal change disease (MCD) is a common cause of nephrotic syndrome.
- Understanding hypercoagulability in MCD is crucial for managing thrombotic complications.
Purpose of the Study:
- To investigate hypercoagulability risk in children with MCD.
- To identify protective hemostatic mechanisms in MCD patients.
- To analyze procoagulant and anticoagulant factor changes during MCD attacks and remission.
Main Methods:
- Study included 26 children with MCD (during attack and remission) and 33 healthy controls.
- Evaluated prothrombosis parameters, lipids, fibrinogen, platelet count, prothrombin fragments 1+2, thrombin-antithrombin complex, lupus anticoagulant, activated protein-C resistance, and D-dimer.
- Assessed protein-C activity, tissue plasminogen activator, and antiplasmin levels.
Main Results:
- During MCD attacks, prothrombosis markers, lipids, fibrinogen, and platelets significantly increased, indicating hyperviscosity and thrombosis tendency.
- Elevated prothrombin fragments 1+2 and thrombin-antithrombin complex confirmed increased thrombin formation and thrombosis risk.
- Increased D-dimer suggested activation of the fibrinolytic system in response to thrombotic activity.
- Higher protein-C activity and decreased tissue plasminogen activator/antiplasmin indicated protective roles of the fibrinolytic system.
Conclusions:
- Childhood minimal change disease is associated with significant hypercoagulability and increased thrombosis risk during attacks.
- Elevated protein-C activity and fibrinolytic system activation serve as crucial protective mechanisms against thrombosis in MCD.
- Maintaining hemostatic balance is vital for preventing thrombotic events in pediatric nephrotic syndrome.
Abstract:
It is believed that thrombotic activity in nephrotic syndrome is due to an imbalance between procoagulant/thrombotic and anticoagulant/antithrombotic factors in plasma. The aim of this study was to investigate the hypercoagulability risk in childhood minimal change disease and to find possible protective mechanisms with respect to hemostasis. Twenty-six children with minimal change disease were enrolled in this study. All patients were evaluated during an attack and on remission. The control group consisted of 33 healthy children. During the attack period, prothrombosis parameters, total lipid, cholesterol, fibrinogen levels and platelet count increased significantly compared to levels in the remission period. This denotes that hyperviscosity increases thrombosis tendency. In the attack period, the significant increase of prothrombin fragments 1 + 2 which shows thrombin formation and thrombin-antithrombin complex which causes prothrombin activation, are an indication of increased thrombosis risk. Five patients with lupus anticoagulant present and 7 patients with, activated protein-C resistance ratios carried an increased thrombosis risk. D-dimer level of fibrinolytic factors significantly increased during the attack period. These findings emphasize the existence of thrombotic activity causing the activation of the fibrinolytic system. The significant increase in protein-C activity in these patients represents one of the protective mechanisms against thrombosis. The decrease in tissue plasminogen activator and antiplasmin indicates the protective role of fibrinolytic activity. Consequently, an increase in the protein-C activity is one of the protective mechanisms. The fibrinolytic system also plays an important role in preventing thrombotic activity in these patients.
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