Clinicopathologic correlation of C1q nephropathy in children.
BACKGROUND
Clinicopathologic correlation of C1q nephropathy is clarified poorly. The aim of our study is to clarify clinicopathologic correlation in childhood C1q nephropathy.


METHODS
Thirty children aged 3 to 15 years who met criteria proposed by Jennette and Hipp were enrolled in this study.


RESULTS
According to their presentation at onset, children were divided into 2 groups: the asymptomatic urinary abnormalities (asymptomatic) group (n = 18) and the nephrotic syndrome (NS) group (n = 12). Light microscopy showed minimal change disease (MCD) in 22 children (73%), mesangial proliferative glomerulonephritis in 6 children (20%), and focal segmental glomerulosclerosis (FSGS) in 2 children (7%). Four children in the asymptomatic group and all children in the NS group were administered prednisolone and/or cyclosporine. Normal urinalysis results were found in 8 children in the asymptomatic group and 3 children in the NS group during the follow-up period of 3 to 15 years. Eight children in the NS group were frequent relapsers at the latest follow-up. Two children with FSGS (1 child, asymptomatic group; 1 child, NS group) received dialysis 10 and 15 years after the diagnosis. There were no differences in histological findings and clinical outcomes between the 2 groups. Four children with MCD in the NS group underwent a second biopsy. C1q deposits disappeared in 2 children, and 1 of these 2 children showed FSGS.


CONCLUSIONS
Childhood C1q nephropathy is found in a wide clinical spectrum. Some children showed disappearance of C1q deposits through the follow-up period. A large number of children with C1q nephropathy showed MCD. However, FSGS may develop in some children on repeated biopsy. Therefore, long-term follow-up is needed in children with C1q nephropathy.
Department of Pediatrics and Pathology, School of Medicine, Fukuoka University, Japan.
Yuko
Y
Fukuma
Satoshi
S
Hisano
Yoshie
Y
Segawa
Kazuhiko
K
Niimi
Noboru
N
Tsuru
Yoshitsugu
Y
Kaku
Ken
K
Hatae
Yasuhiro
Y
Kiyoshi
Akihisa
A
Mitsudome
Hiroshi
H
Iwasaki
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015922:Complement C1q; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D006801:Humans; D008297:Male; D009402:Nephrosis, Lipoid / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D009404:Nephrotic Syndrome / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy
D015922:Complement C1q