Objectives

Describe forms of presentation, therapy performed and evolution of patients diagnosed with IgA nephropathy (IgAN) in a Pediatric Nephrology Unit of a level III hospital.

Material and methods:

Retrospective, descriptive analysis of demographic, clinical, analytical and histological data of children with kidney biopsy proven IgAN from January 2001 to December 2020. 

Results:

Thirteen children included, 54% girls. Initial findings were one or more episodes of macroscopic hematuria in five (four linked to respiratory tract infection, two with microscopic hematuria in-between); one had persistent microscopic hematuria and nephrotic proteinuria one year before admission. Seven children had no previous complaints.

The average age at the time of biopsy was 11±4.4 years. In the six children with previous symptoms, biopsy was performed on average 25 (5-60) months. At that time, seven patients had nephritic syndrome, associated to nephrotic proteinuria (three), significative non-nephrotic proteinuria (two) and nephrotic syndrome (one); two had nephrotic syndrome with microscopic hematuria; one had macroscopic hematuria and nephrotic proteinuria; two had microscopic hematuria and nephrotic proteinuria; one was diagnosed with stage 4 chronic kidney disease.

Histopathologically, ten children had glomerular crescents and/or interstitial fibrosis. Of these, nine received corticoids and angiotensin-converting enzyme inhibitor (ACEI), five with cyclophosphamide association (persistent nephrotic proteinuria or relapse of nephritic syndrome). One child had only ACEI (spontaneous resolution).

Mean follow-up was 3.94 (0.6-11.7) years. At last evaluation, seven children (54%) had normal blood pressure, unchanged sediment, no proteinuria and normal renal function. The child with stage 4 chronic kidney disease did not worsen and five children maintained hematuria and/or proteinuria (nephrotic-three).

Conclusions:

 IgA nephropathy is the most common glomerulonephritis with a high clinical and histological variability and an important cause of end-stage renal disease.

Our results show that outcome is good, however some patients need immunosuppressive therapies and careful long-term observation is required.