ESPN 50th Annual Meeting

ESPN 2017


 
THROMBOEMBOLISM IN CHILDREN WITH CONGENITAL NEPHROTIC SYNDROME – LESSONS FROM AN ESPN SURVEY
STEPHANIE DUFEK 1 ELISA YLINEN 5 AGNES TRAUTMANN 2 ANDREA PASINI 3 GEMA ARICETA 4 TUULA HOLTTA 5 ALBERTO EDEFONTI 6 RUKSHANA SHROFF 1

1- GREAT ORMOND STREET HOSPITAL FOR CHILDREN NHS FOUNDATION TRUST, LONDON, UK
2- CENTER FOR PAEDIATRIC & ADOLESCENT MEDICINE, HEIDELBERG, GERMANY
3- AZIENDA OSPEDALIERA UNIVERSITARIA SANTORSOLA-MALPIGHI, BOLOGNA, ITALY
4- HOSPITAL MATERNOINFANTIL DE LA VALL D’HEBRON, BARCELONA, SPAIN
5- UNIVERSITY OF HELSINKI AND HELSINKI UNIVERSITY HOSPITAL, HELSINKI, FINLAND
6- on behalf of the ESPN Dialysis Working Group
 
Introduction:

Thromboembolism is a serious and potentially fatal complication in children with congenital nephrotic syndrome (CNS). 

Material and methods:

We conducted a 6-year survey across members of the ESPN Dialysis Working Group to evaluate thrombosis risk, treatment and outcome in children with CNS across Europe.

Results:

Eighty-two children (52% male) were included from 19 tertiary nephrology units in 11 European countries. Median age at presentation with CNS was 9.5 (IQR 0-164) days with S-albumin 11 (4-29) g/L and S-creatinine 27 (2-480) μmol/L.

Prophylactic antithrombotic medications were given in 47 (57%); Warfarin being the most common (51%).

Ten (12%) patients developed thrombosis: 5 not on prophylaxis (14.3%) versus 5 on prophylaxis (10.6%) (p=0.618); At thrombosis median age was 1.5 (0.7 – 5.5) months and median S-albumin 15 (10 – 22) g/L. Nine patients received regular albumin infusions with diuretic treatment and 5 were on ACE inhibitors. No patient had a history of preceding dehydration.

Nine patients had central lines in situ, and one patient was on haemodialysis at the time of thrombosis. In 5 thrombus formation was at the site of the central line (infection related in one) and 4 needed line removal. Symptoms were catheter malfunction in 3, asymmetric extremity symptoms in 2, convulsion in 1 and fever with malaise in 1. Three were asymptomatic and diagnosed by routine heart ultrasound.  

All patients received therapeutic anticoagulation (heparin in 7 or warfarin in 3) and one additional thrombolysis. At final follow-up (median 22 months), complete resolution of thrombus was achieved in 4, partial in 2. One patient had ongoing convulsions and 2 died (17 days and 7.5 months after thrombosis). 

Conclusions:

In infants with CNS the risk of thrombotic episodes is high and the benefit of prophylaxis needs to be considered. Treating clinicians need a high level of vigilance, particularly in infants with central lines.