ESPN 51th Annual Meeting

ESPN 2018


 
CLINICAL COURSE OF C3 GLOMERULOPATHY IN TURKİSH CHILDREN: A MULTICENTER STUDY
Ismail DURSUN 1 Ayşe Seda PINARBASI 1 Ibrahim Gokce 2 Elif Comak 3 Seha Saygılı 4 Meral Torun Bayram 5 Osman Donmez 6 Engin Melek 7 Demet Tekcan 8 Dilek Yılmaz 9 Sevcan Azime Bakkaloglu Ezgü 10 Yılmaz Tabel 11 Zeynep Y Yıldırım 12 Elif Bahat 13 Mustafa Koyun 3 Alper Soylu 5 Salim Calıskan 4 Bagdagul Yavas 14 Mehtap Ezel Celakıl 15 Mustafa Tasdemir 16 Meryem Benzer 17 Gül Ozcelik 18 Ruhan DUSUNSEL 1

1- ERCIYES UNIVERSITY FACULTY OF MEDICINE, DEPARTMENT OF PEDIATRIC NEPHROLOGY
2- MARMARA UNIVERSITY, MEDICAL FACULTY DEPARTMENT OF PEDIATRIC NEPHROLOGY
3- AKDENIZ UNIVERSITY, MEDICAL FACULTY DEPARTMENT OF PEDIATRIC NEPHROLOGY
4- ISTANBUL UNIVERSITY, CERRAHPAŞA SCHOOL OF MEDICINE, DEPARTMENT OF PEDIATRIC NEPHROLOGY
5- 9 EYLUL UNIVERSITY, MEDICAL FACULTY, DEPARTMENT OF PEDIATRIC NEPHROLOGY
6- ULUDAĞ UNIVERSITY, MEDICAL FACULTY, DEPARTMENT OF PEDIATRIC NEPHROLOGY
7- CUKUROVA UNIVERSITY, MEDICAL FACULTY, DEPARTMENT OF PEDIATRIC NEPHROLOGY
8- 19 MAYIS UNIVERSITY, MEDICAL FACULTY, DEPARTMENT OF PEDIATRIC NEPHROLOGY
9- ADNAN MENDERES UNIVERSITY, MEDICAL FACULTY, DEPARTMENT OF PEDIATRIC NEPHROLOGY
10- GAZI UNIVERSITY, MEDICAL FACULTY, DEPARTMENT OF PEDIATRIC NEPHROLOGY
11- INÖNÜ UNIVERSITY, MEDICAL FACULTY, DEPARTMENT OF PEDIATRIC NEPHROLOGY
12- ISTANBUL UNIVERSITY, ÇAPA SCHOOL OF MEDICINE, DEPARTMENT OF PEDIATRIC NEPHROLOGY
13- KARADENIZ TEKNIK UNIVERSITY, MEDICAL FACULTY, DEPARTMENT OF PEDIATRIC NEPHROLOGY
14- SAGLIK BILIMLERI UNIVERSITY, HASEKI TRAINING AND TEACHING HOSPITAL, DEPARTMENT OF PEDIATRIC NEPHROLOGY
15- KOCAELI UNIVERSITY, FACULTY OF MEDICINE DEPARTMENT OF PEDIATRIC NEPHROLOGY
16- KOÇ UNIVERSITY, FACULTY OF MEDICINE, DEPARTMENT OF PEDIATRIC NEPHROLOGY
17- SAGLIK BILIMLERI UNIVERSITY, SADI KONUK TRAINING VE TEACHING HOSPITAL, DEPARTMENT OF PEDIATRIC NEPHROLOGY
18- SAGLIK BILIMLERI UNIVERSITY, SISLI ETFAL CHILDREN HOSPITAL, DEPARTMENT OF PEDIATRIC NEPHROLOGY
 
Introduction:

 C3 glomerulopathy (GP) is considered to be a primary complement pathway disease. Patients with C3GP also have a high risk of progressing into end-stage renal disease. In this multicenter study (18 center), we retrospectively evaluated the clinical course and outcome of C3 GP

Material and methods:

 Fifty-six children from 18 pediatric nephrology center were enrolled in to our study. Demographic features, clinical presentation, kidney biopsy findings on the LM, IF and when possible, EM, and treatment modalities, and outcome were evaluated. The diagnosis of C3 GP was made on the basis of the 2013 consensus guidelines. Outcome was divided into two groups as outcome 1 (complete recovery with no proteinuria, a normal C3 level, and an eGFR of >90 mL/min/1.73 m2) and others (incomplete recovery). Then, incomplete recovery were divided into four groups as follow; outcome 2: proteinuria only, with a normal C3 level and an eGFR of >90 mL/min/1.73 m2; outcome 3: no proteinuria, with a low C3 level and an eGFR of >90 mL/min/1.73 m2; outcome 4: proteinuria and a low C3 level and an eGFR of >90 mL/min/1.73 m2; outcome 5: an eGFR of <90 mL/min/1.73 m

Results:

The mean age at diagnosis was 10.4 years and the mean follow-up duration was 44.1 months The major clinical presentations of our patients were edema and macroscopic hematuria. 76.3 % of children had low C3 level on admission. Nephrotic range proteinuria was detected in 33 (59%). Mesengial proliferation and MPGN pattern of injuries were commonly seen LM findings. We were able to get EM results in 20 children. Mesangial and partially distributed intramembranous deposits were seen most of the cases. Five children had DDD. Eculizumab were used in 16 children. Only 13 children reached at outcome 1. Outcome 5 was detected in 16 (7 ESRD) /min/1.73 m2; outcome 4: proteinuria and a low C3 level and an eGFR of >90 mL/min/1.73 m2; outcome 5: an eGFR of <90 mL/min/1.73 m

 

Conclusions:

 C3GP is associated with the high risk for CKD in children