ESPN 50th Annual Meeting

ESPN 2017


 
Idiopathic membranous nephropathy in childhood
MAR ESPINO 1 ANTONIA PEñA 2 ELVIRA IZQUIERDO 3 MERCEDES VAZQUEZ-MARTUL 4 MARIA HERRERO 5 ALEJANDRO ZARAUZA 1 JULIA VARA 1

1- PEDIATRIC NEPHROLOGY. H. 12 DE OCTUBRE. MADRID.
2- PEDIATRIC NEPHROLOGY. H. LA PAZ. MADRID.
3- PEDIATRIC NEPHROLOGY. H.GREGORIO MARAñON. MADRID.
4- PEDIATRIC NEPHROLOGY. H.NIñO JESúS. MADRID.
5- PEDIATRIC NEPHROLOGY. HOSPITAL DE CRUCES. BILBAO.
 
Introduction:

To know the clinical manifestations, treatment and prognosis of membranous nephropathy (MN) in childhood.

Material and methods:

Multicentric, retrospective study (1980-2016). Inclusion criteria: children under 16 years old with diagnosis of MN in renal biopsy report. Exclusion criteria: secondary MN (hepatitis B, C, LES, etc…) We recorded clinical manifestations at debut, age at biopsy time, pathology report and treatment. We assessed clinical situation at last review.

Results:

13 patients. Mean age at onset 9,8 years (range 6-15). Nine girls and 4 boys. At debut: eight nephrotic syndrome (NS) with microscopic hematuria (2 hypertension, 1 of them with renal failure (RF). Three nephrotic proteinuria (NP), 2 of them with macroscopic hematuria.  Two non-NP. Findings in light microscopy were MN type I in 2, type II in 5 and type III in 2. Immunofluorescence detected IgG deposits in 11/13 with C3 in 10/13. In four, there were slight deposits of IgA (2) and IgM (2) and C1q (2) with chains lambda y kappa (1). Treatment included ACEI or ARA-II in 9, with prednisone 6. One was treated with acetylsalicylic acid. Nine received prednisone (6 NS and 3 NP). Two children with RF were treated with IECA, prednisone and cyclophosphamide. At mean 9 years of follow-up (1-11years): patients with NS: three complete remission; two proteinuria >1gr; two proteinuria <1gr (1 with RF and hypertension normalized blood pressure and glomerular filtration) and one are in chronic RF G3b stage without proteinuria.  Patients with NP: two complete remission and 1 proteinuria <1gr. Patients with proteinuria <1gr: 1 developed NP and 1 proteinuria <1gr with chronic RF G2 stage.

Conclusions:

In childhood, the most typical presentation of idiopathic MN is nephrotic syndrome. The prognosis is not so bad as referred in adults and the chances of recovery renal involvement are high despite severe debut.