Hui Wang1, Shuzhen Sun2, Weiran Zhou3, Ying Shen4, Xuemei Liu3, Junhui Zhen5, Hongxia Zhang3, Fan Duan4, Yanyan Pan3, Linlin Dong3. 1. Department 2 of Nephrology, Beijing Children's Hospital Affiliated to Capital Medical University, National Center for Children's Health, Key Laboratory of Major Diseases in Children's Ministry of Education, Beijing Key Laboratory of Pediatric Chronic Kidney Diseases and Blood Purification, Beijing, China. wanghui@bch.com.cn. 2. Department of Pediatric Nephrology, Rheumatology and Immunology, Shandong Provincial Hospital, Shandong University, Jinan, China. 3. Department of Pediatric Nephrology, Rheumatology and Immunology, Children's Hospital Affiliated to Shandong University, Jinan Children's Hospital, Jinan, Shandong Province, China. 4. Department 2 of Nephrology, Beijing Children's Hospital Affiliated to Capital Medical University, National Center for Children's Health, Key Laboratory of Major Diseases in Children's Ministry of Education, Beijing Key Laboratory of Pediatric Chronic Kidney Diseases and Blood Purification, Beijing, China. 5. Department of Pathology, Shandong University, Jinan, China.
Abstract
BACKGROUND: C4d may be used as a marker to evaluate the condition and prognosis of adults with IgA nephropathy, but there have been few studies of children with IgA nephropathy. METHODS: C4d immunohistochemical staining was performed on samples from children with IgA nephropathy with C1q-negative immunofluorescence. The clinical and pathological treatment and prognostic characteristics of children in the C4d-positive and -negative groups were compared. RESULTS: A total of sixty-five children with IgA nephropathy were included in the study and were followed up for an average of 37 months. C4d was mainly deposited along the capillary loops. The urinary protein-to-creatinine ratio (UPCR) in the C4d-positive group was significantly higher than that in the C4d-negative group (3.97 vs. 0.81, P < 0.001), and the average integrated optical density value of each child was positively correlated with the UPCR (r = 0.441, P < 0.001). There was a significant difference in the proportions of children with mesangial hypercellularity (M1) (68.97% vs. 44.44%, P = 0.048) and segmental glomerulosclerosis (S1) (65.52% vs. 33.33%, P = 0.010) between the C4d-positive group and the C4d-negative group. The proportion of children who received immunosuppressants in the C4d-positive group was higher than that in the C4d-negative group (86.21% vs. 36.11%, P < 0.001). There was no significant difference in the proportion of children developing kidney failure between the two groups. CONCLUSION: C4d was found to be associated with proteinuria, segmental lesions, and immunosuppressant treatment. Activation of the lectin pathway may reflect the severity of clinical and pathological manifestations of IgA nephropathy in children. A higher resolution version of the Graphical abstract is available as Supplementary information.
BACKGROUND: C4d may be used as a marker to evaluate the condition and prognosis of adults with IgA nephropathy, but there have been few studies of children with IgA nephropathy. METHODS: C4d immunohistochemical staining was performed on samples from children with IgA nephropathy with C1q-negative immunofluorescence. The clinical and pathological treatment and prognostic characteristics of children in the C4d-positive and -negative groups were compared. RESULTS: A total of sixty-five children with IgA nephropathy were included in the study and were followed up for an average of 37 months. C4d was mainly deposited along the capillary loops. The urinary protein-to-creatinine ratio (UPCR) in the C4d-positive group was significantly higher than that in the C4d-negative group (3.97 vs. 0.81, P < 0.001), and the average integrated optical density value of each child was positively correlated with the UPCR (r = 0.441, P < 0.001). There was a significant difference in the proportions of children with mesangial hypercellularity (M1) (68.97% vs. 44.44%, P = 0.048) and segmental glomerulosclerosis (S1) (65.52% vs. 33.33%, P = 0.010) between the C4d-positive group and the C4d-negative group. The proportion of children who received immunosuppressants in the C4d-positive group was higher than that in the C4d-negative group (86.21% vs. 36.11%, P < 0.001). There was no significant difference in the proportion of children developing kidney failure between the two groups. CONCLUSION: C4d was found to be associated with proteinuria, segmental lesions, and immunosuppressant treatment. Activation of the lectin pathway may reflect the severity of clinical and pathological manifestations of IgA nephropathy in children. A higher resolution version of the Graphical abstract is available as Supplementary information.
Authors: Anja Roos; Maria Pia Rastaldi; Novella Calvaresi; Beatrijs D Oortwijn; Nicole Schlagwein; Danielle J van Gijlswijk-Janssen; Gregory L Stahl; Misao Matsushita; Teizo Fujita; Cees van Kooten; Mohamed R Daha Journal: J Am Soc Nephrol Date: 2006-05-10 Impact factor: 10.121