ESPN 51th Annual Meeting

ESPN 2018


 
Congenital and infantile nephrotic syndromes: Tunisian experience
ABIR BOUSSETTA MANEL JELLOULI MAHA TAGORTI MERIEM FERJANI CHOKRI ZARROUK ONS NAIJA TAHER GARGAH

1- Pediatric nephrology department Charles Nicolle Hospital, Tunis, Tunisia
 
Introduction:

 

The congenital and infantile nephrotic syndromes are rare and heterogeneous.  The aim of our study is to investigate clinical and outcomes characteristics of Tunisian patients with congenital and nephrotic syndrome.  

Material and methods:

 

This is a retrospective study, in Pediatric Nephrology Department, in Charles Nicolle Hospital over seventeen years between 1998 and 2015.

Results:

 

 Twenty-five patients, with congenital and infantile nephrotic syndrome, showed interest in participating in the study. Twelve patients had congenital nephrotic syndrome and thirteen had an infantile nephrotic syndrome. Median age at diagnosis was 128 days. Parental consanguinity was 64% in 16 cases. The antenatal diagnosis of congenital nephrotic syndrome was evocated in one case with an elevated level of amniotic fluid alpha-fetoprotein, 41170µmol/l and without neural defects.

Edemas were noted in 20 patients (80%). The proteinuria was ranged between 50 and 2720 mg/kg/24h during 24h. The histological studies for nine patients showed the presence of congenital nephrotic syndrome of Finnish type in three cases, four cases with mesangial proliferative glomerulonephritis and two cases with mesangial diffuses sclerosis. The genetic studies for two patients noted an NPHS1 mutation. 

The corticosteroids treatment interested five patients with an infantile nephrotic syndrome.     A complete remission was achieved in one case. A peritoneal dialysis was performed in one patient with infantile nephrotic syndrome.

Four patients underwent medical follow-up in 2014 and 2015; one case with congenital nephrotic syndrome and preserved renal function and three cases with infantile nephrotic syndrome in completed remission, after cortico-therapy for one patient and spontaneously for the two others patients.

Conclusions:

 

The management of congenital and infantile nephrotic syndrome must be improved in our country.