C1q nephropathy and minimal change nephrotic syndrome.
C1q nephropathy (C1qN) is an uncommon disorder seen in children and adults with nephrotic syndrome and non-specific urinary findings. It has been described with minimal change nephrotic syndrome (MCNS), focal segmental glomerulonephritis and isolated mesangial proliferative glomerulonephritis. We describe nine children with MCNS and mesangial C1q deposition. These children had a median age of 2.7 years at diagnosis (range 1.3-15 years), 56% were male and 78% were Hispanic. We compared these children to concurrent patients with nephrotic syndrome and biopsy-proven MCNS. We found that the C1qN patients were more likely than MCNS children to require chronic immunosuppression with calcineurin inhibitors or mycophenolate mofetil to maintain remission. However, all children were able to achieve and sustain clinical remission of nephrotic syndrome. Children with C1qN and minimal change histology have an increased frequency of frequently relapsing and steroid-unresponsive disease, but they can attain prolonged remission and stable renal function with calcineurin inhibitor or mycophenolate mofetil therapy.
Wong
Craig S
CS
Department of Pediatrics, Division of Nephrology, University of New Mexico School of Medicine, MSC-10 5590, 1 University New Mexico, Albuquerque, NM 87131, USA.
Fink
Christopher A
CA
Baechle
Jane
J
Harris
Alexis A
AA
Staples
Amy O
AO
Brandt
John R
JR
D000293:Adolescent; D065095:Calcineurin Inhibitors; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D015922:Complement C1q / Q000378:metabolism*; D004359:Drug Therapy, Combination; D005260:Female; D015432:Glomerulonephritis, Membranoproliferative / Q000188:drug therapy / Q000378:metabolism*; D005923:Glomerulosclerosis, Focal Segmental / Q000188:drug therapy / Q000378:metabolism*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D008297:Male; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D009402:Nephrosis, Lipoid / Q000188:drug therapy / Q000378:metabolism*; D011239:Prednisolone / Q000494:pharmacology; D012189:Retrospective Studies
D065095:Calcineurin Inhibitors; D007166:Immunosuppressive Agents; D015922:Complement C1q; D011239:Prednisolone; D009173:Mycophenolic Acid