Back to Search Start Over

Predictors of poor kidney outcome in children with C3 glomerulopathy.

Authors :
Pınarbaşı, Ayşe Seda
Dursun, Ismail
Gokce, Ibrahim
Çomak, Elif
Saygılı, Seha
Bayram, Meral Torun
Donmez, Osman
Melek, Engin
Tekcan, Demet
Çiçek, Neslihan
Yılmaz, Dilek
Tabel, Yılmaz
Yıldırım, Zeynep Y.
Bahat, Elif
Koyun, Mustafa
Soylu, Alper
Canpolat, Nur
Aksu, Bağdagül
Çelakıl, Mehtap Ezel
Taşdemir, Mehmet
Source :
Pediatric Nephrology. May2021, Vol. 36 Issue 5, p1195-1205. 11p. 4 Charts, 3 Graphs.
Publication Year :
2021

Abstract

Background: C3 glomerulopathy (C3G) is characterized by heterogeneous clinical presentation, outcome, and predominant C3 accumulation in glomeruli without significant IgG. There is scarce outcome data regarding childhood C3G. We describe clinical and pathological features, treatment and outcomes, and risk factors for progression to chronic kidney disease stage 5 (CKD5) in the largest pediatric series with biopsy-proven C3G. Methods: Sixty pediatric patients with C3G from 21 referral centers in Turkey were included in this retrospective study. Patients were categorized according to CKD stage at last visit as CKD5 or non-CKD5. Demographic data, clinicopathologic findings, treatment, and outcome data were compared and possible risk factors for CKD5 progression determined using Cox proportional hazards model. Results: Mean age at diagnosis was 10.6 ± 3.0 years and follow-up time 48.3 ± 36.3 months. Almost half the patients had gross hematuria and hypertension at diagnosis. Nephritic-nephrotic syndrome was the commonest presenting feature (41.6%) and 1/5 of patients presented with nephrotic syndrome. Membranoproliferative glomerulonephritis was the leading injury pattern, while 40 patients had only C3 staining. Patients with DDD had significantly lower baseline serum albumin compared with C3GN. Eighteen patients received eculizumab. Clinical remission was achieved in 68.3%. At last follow-up, 10 patients (16.6%) developed CKD5: they had lower baseline eGFR and albumin and higher frequency of nephrotic syndrome and dialysis requirement than non-CKD5 patients. Lower serum albumin and eGFR at diagnosis were independent predictors for CKD5 development. Conclusions: Children with C3G who have impaired kidney function and hypoalbuminemia at diagnosis should be carefully monitored for risk of progression to CKD5. Graphical abstract [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
0931041X
Volume :
36
Issue :
5
Database :
Academic Search Index
Journal :
Pediatric Nephrology
Publication Type :
Academic Journal
Accession number :
149550548
Full Text :
https://doi.org/10.1007/s00467-020-04799-7