SINDROME NEFROSICA ED IPERCOAGULABILITÀ EMATICA. NUOVE PROSPETTIVE DIAGNOSTICHE

BACKGROUND

Several studies showed that an imbalance of prothrombotic and antithrombotic factors and impaired thrombolytic activity contribute to the thrombophilia of the Nephrotic Syndrome (NS). However, it is not clear whether blood cell injury and/or activation is involved in hypercoagulability in NS patients.

AIM OF THE STUDY

Evaluate haemostasis and the main genetic mutations associated with thrombotic risk in children with SN.

PATIENTS AND METHODS

We have studied 20 SN children aged 1 to 14 years, matched with 40 health-subjects. Patients with Lupus Nephritis were not admitted in the present study. Were evaluated the following paramethers: PT, aPTT, platelet count, fibrinogen, antithrombin III, C- and S-proteins, plasminogen and homocysteine. Furthermore we have performed the molecular study for thrombotic risk (Factor V Leiden, MTHFR C677T, A1298C and Prothrombin G20210A) by the means of the analysis of subclasses of antiphospholipid antibodies (anticardiolipin, anti-Beta2GPI and anti Z-protein) and by the means of the thrombin generation test with thromboelastometer.

RESULTS

In the studied population with SN, PT aPTT and homocysteine were into the normal range. We found high levels of fibrinogen, high values of thrombin generation test and high prevalence of anticardiolipin, anti-Beta2GPI and anti Z-protein antibodies in children with SN (sea table 1-4). Finally, there was not any signifant difference in the incidence of mutations related to the thrombotic risk between children with SN and health-subjects group (sea table 5).

CONCLUSIONS

In SN the coagulation cascade is greatly activated, as previously demonstrated by low levels of Antithrombin III and plasminogen and high levels of fibrinogen, and is now confirmed by high values of thrombin generation test. More studies about haemostasis in SN are needed, to improve prognosis and therapeutic management in pediatric population with this widespread disease.