ESPN 51th Annual Meeting

ESPN 2018


 
TOLVAPTAN IN A PEDIATRIC NEPHROTIC SYNDROME WITH MASSIVE EDEMA
EMEL KABAKOĞLU ÜNSÜR 1 ZÜBEYDE GÜNDÜZ 2 RUHAN DÜŞÜNSEL 2 İSMAİL DURSUN 2 KİBRİYA FİDAN 3

1- ACIBADEM UNIVERSITY
2- ERCIYES MEDICAL FACULTY
3- GAZİ MEDICAL FACULTY
 
Introduction:

       Edema is a common clinical feature of nephrotic syndrome (NS). It is often massive (up to 30 % of body weight) and leads to functional problems. The non-osmotic stimulation of arginine vasopressin  has been implicated as one of the important factors in abnormal water retention in patients with NS .

   Tolvaptan is currently the only oral selective vasopressin V2receptor antagonist  is known as an  ‘aquaretic’ due to its ability to increase urinary water excretion. Thus, tolvaptan is primarily indicated for dilutional hyponatraemia such as syndrome of inappropriate antidiuresis like congestive heart failure and liver cirrhosis.

Material and methods:

 There have been a few  reports describing clinical experience regarding tolvaptan therapy for NS especially in children. In this report, we present a case of massive edema caused by NS (WT1 mutation) which was effectively treated with tolvaptan.

Results:

 A four year- old girl was admitted to our department with massive edema, proteinuria and hypertension as diagnosed NS . She had  initially prednisolone, antihypertensive treatment. However, she subsequently became unresponsive to immunsupresive therapy (cyclosporine,azothioprine). In renal biopsy focal segmental glomerulosclerosis was seen,  in genetic test WT 1 mutation was detected and immunsuppressants were stopped. Despite all the treatment including paracentesis massive edema did not resolve and she became immobilize and had severe dyspnea; so that after informed consent from the patient’s family tolvaptan was  used (3.75mg/day) for 15 days. With tolvaptan her urine volume and the urine sodium levels also increased during the treatment. Her edema dramatically resolved . Serum levels of sodium did not change throughout the tolvaptan therapy. No side effects including liver dysfunction were observed during treatment. Her proteinuria decreased slightly, but remained.

Conclusions:

 In our case, tolvaptan was promisingly effective in reducing massive edema with nephrotic syndrome, it was well tolareted and had no side effects.