Malgorzata Mizerska-Wasiak

Risk factors for progression in children and young adults with IgA nephropathy: an analysis of 261 cases from the VALIGA European cohort

BackgroundThere is a need for early identification of children with immunoglobulin A nephropathy (IgAN) at risk of progression of kidney disease.MethodsData on 261 young patients [age <23xa0years; mean follow-up of 4.9 (range 2.5–8.1) years] enrolled in VALIGA, a study designed to validate the Oxford Classification of IgAN, were assessed. Renal biopsies were scored for the presence of mesangial hypercellularity (M1), endocapillary hypercellularity (E1), segmental glomerulosclerosis (S1), tubular atrophy/interstitial fibrosis (T1-2) (MEST score) and crescents (C1). Progression was assessed as end stage renal disease and/or a 50xa0% loss of estimated glomerular filtration rate (eGFR) (combined endpoint) as well as the rate of renal function decline (slope of eGFR). Cox regression and tree classification binary models were used and compared.ResultsIn this cohort of 261 subjects aged <23xa0years, Cox analysis validated the MEST M, S and T scores for predicting survival to the combined endpoint but failed to prove that these scores had predictive value in the sub-group of 174 children aged <18xa0years. The regression tree classification indicated that patients with M1 were at risk of developing higher time-averaged proteinuria (pu2009<u20090.0001) and the combined endpoint (pu2009<u20090.001). An initial proteinuria of ≥0.4xa0g/day/1.73xa0m2 and an eGFR of <90xa0ml/min/1.73xa0m2 were determined to be risk factors in subjects with M0. Children aged <16xa0years with M0 and well-preserved eGFR (>90xa0ml/min/1.73xa0m2) at presentation had a significantly high probability of proteinuria remission during follow-up and a higher remission rate following treatment with corticosteroid and/or immunosuppressive therapy.ConclusionThis new statistical approach has identified clinical and histological risk factors associated with outcome in children and young adults with IgAN.

Serum GDIgA1 levels in children with IgA nephropathy and Henoch-Schönlein nephritis

Introduction GDIgA1 (galactose deficient IgA1) plays a significant role in the pathogenesis of IgA nephropathy (IgAN) and Henoch-Schönlein nephritis (HSN). Aim of the study The aim of this study was to assess the relevance of serum GDIgA1 level as a prognostic marker in children with IgAN and HSN. Material and methods 41 children were included to the study group (15 IgAN, 26 HSN) and 22 to the control group. The following parameters were evaluated at baseline and endpoint: proteinuria, erythrocyturia, serum creatinine, serum IgA, GFR. A kidney biopsy was performed in all patients and evaluated according to the Oxford Classification (1 – present, 0 – absent: M – mesangial hypercellularity; E– endocapillary hypercellularity; S – segmental sclerosis/adhesion; T – tubular atrophy/interstitial fibrosis), and was calculated as the total score (sum of M, E, S, T). At the end of follow-up, the serum GDIgA1 concentration was measured. Results The serum GDIgA1 concentration in patients with IgAN and HSN was significantly higher than in the control group. No significant differences in mean proteinuria, erythrocyturia, GFR, MEST score, or GDIgA1 in serum, as well as the duration of follow-up between IgAN and HSN were observed. Baseline serum IgA concentration and time to kidney biopsy were significantly higher in children with IgAN than in children with HSN. We observed a positive correlation between GDIgA1 and IgA levels (r = 0.53), and GDIgA1 and serum creatinine levels (r = 0.5), as well as negative correlation between GDIgA1 and GFR (r = –0.37). Conclusions Serum GDIgA1 level may have a prognostic value in children with IgAN and HSN; however, to fully elucidate its clinical potential further studies performed in larger patient cohorts are required.

Erratum to: Risk factors for progression in children and young adults with IgA nephropathy: an analysis of 261 cases from the VALIGA European cohort

Rosanna Coppo & Danilo Lofaro & Roberta R. Camilla & Shubha Bellur & Daniel Cattran & H. Terence Cook & Ian S. D. Roberts & Licia Peruzzi & Alessandro Amore & Francesco Emma & Laura Fuiano & Ulla Berg & Rezan Topaloglu & Yelda Bilginer & Loreto Gesualdo & Rosaria Polci & Malgorzata Mizerska-Wasiak & Yasar Caliskan & Sigrid Lundberg & Giovanni Cancarini & Colin Geddes & Jack Wetzels & Andrzej Wiecek & Magdalena Durlik & Stefano Cusinato & Cristiana Rollino & Milena Maggio & Manuel Praga & Hilde K. Smerud & Vladimir Tesar & Dita Maixnerova & Jonathan Barratt & Teresa Papalia & Renzo Bonofiglio & Gianna Mazzucco & Costantinos Giannakakis & Magnus Soderberg & Diclehan Orhan & Anna Maria Di Palma & JadwigaMaldyk &YaseminOzluk &Birgitta Sudelin &Regina Tardanico &DavidKipgen & Eric Steenbergen & Henryk Karkoszka & Agnieszka Perkowska-Ptasinska & Franco Ferrario & Eduardo Gutierrez & Eva Honsova

Usefulness of urinary collagen IV excretion for predicting the severity of Henoch-Schönlein nephropathy children

The aim of the study The aim of the study was to evaluate the usefulness of urinary collagen IV (Col IV) excretion for predicting the severity of autoimmune renal inflammation in children with HSN (Henoch-Schönlein nephritis). Material and methods We studied 26 children, in whom HSN was diagnosed based on kidney biopsy. In all patients, urinalysis was performed and 24-hour urinary protein excretion was measured at the onset of the disease. All kidney biopsies were also scored using the Oxford classification: M – mesangial hypercellularity score (M0 absent, M1 present); E – presence of endocapillary proliferation (E0 absent, E1 present), S – segmental glomerulosclerosis/adhesion (S0 absent, S1 present), T – tubular atrophy/interstitial fibrosis (T0 ≤ 25%, T1 26-50%, T2 > 50%). The MEST score was calculated as the sum of M + E + S + T. Results Urinary Col IV level was significantly higher in the study group than in control group. Urinary Col IV level was insignificantly higher in group A (nephrotic proteinuria) compared to the B (non-nephrotic proteinuria) and C (without proteinuria).We found no significant differences in the age at the disease onset, severity of proteinuria, and Col IV between groups 1 (S0, T0) and 2.(S1,T1/T2). The MEST score was significantly higher in group 2 than group 1. Conclusions Urinary Col IV excretion in children with HSN may be related to the lesions severity by the Oxford classification but seems to be associated with the mean value (the MEST score). In younger children, a more aggressive disease course is observed, and thus earlier and more aggressive treatment should be considered in this group.