ESPN 50th Annual Meeting

ESPN 2017


 
Long follow-up of IgA Nephropathy with severe debut in childhood
MAR ESPINO 1 JULIA VARA 2 SILVIA ALVAREZ DIAZ 3 CARMEN GALLEGO 5 ANGEL SEVILLANO 4 EDUARDO GUTIERREZ 4 MIGUEL ANGEL MARTINEZ 3 TANIA CARBAYO 2

1- PEDIATRIC NEPHROLOGY. H. 12 DE OCTUBRE. MADRID. UCM.
2- PEDIATRIC NEPHROLOGY. H. 12 DE OCTUBRE. MADRID.
3- PATHOLOGY. H. 12 DE OCTUBRE. MADRID.
4- NEPHROLOGY. H. 12 DE OCTUBRE. MADRID.
5- PEDIATRIC RADIOLOGY. H. 12 DE OCTUBRE.MADRID
 
Introduction:

We try to know the prognosis in adulthood of IgA nephropathy (IgAN) with onset in infancy with severe nephritic or nephrotic syndrome

Material and methods:

Retrospective study. Inclusion criteria: patients with diagnosis of IgAN in renal biopsy report. We recorded age at biopsy, criteria to carry it out, number of glomeruli, pathology report and glomerular filtration rate (GFR), presence of hypertension (HTA), proteinuria and treatments, at debut, at one year of follow-up, at the time of transition to adults and at the last review.

Results:

14 patients with a mean age at onset of 10 years. Percutaneous biopsies were performed with radiological control except one. The average number of glomeruli was 16. Light microscopy: mesangial hypercellularity (11), cellular crescent (3), relevant interstitial fibrosis with tubular atrophy (3). In Immunofluorescence was detected IgA (14); C3 (11) and C4d (2/2). Initial treatment: ACEI or ARAII in all patients, we associated mycophenolate in 2; prednisone, and cyclophosphamide bolus in 2; prednisone and azathioprine in one. One year after debut: 2 patients had nephrotic proteinuria, 1 more than 1 gr and 1 lower. One had HTA. GFR was less than 90 ml/min/1.73m2 in a patient. The average pediatrics follow-up was 71 months. In the transition to adults: 2 had microalbuminuria; 2 proteinuria> 1 gr. Two patients had HTA. 1 patient had been transplanted, 1 had CKD stage 2 DOQI and the others normal GFR. In the last review, in adult units, no more patient had CKD; 2 had nephrotic proteinuria. The remaining patients received ACEI and had proteinuria <1 gr or were untreated.

Conclusions:

The chances of preserving a normal GFR of patient with IgAN, despite debut with a severe nephrotic or nephritic syndrome in childhood, are high. Although proteinuria is considered one of the most important prognostic factors, in our series, patients with high proteinuria maintained normal renal function.