Idiopathic collapsing glomerulopathy in children.
BACKGROUND
Collapsing glomerulopathy (CG) is a clinically and pathologically distinct variant of focal segmental glomerulosclerosis (FSGS). Pathologically similar lesions have been reported in adults and children with human immuno-deficiency virus (HIV) infection. However, there is a recent interest in the recognition of this variant in the absence of HIV infection.


OBJECTIVE
To evaluate the clinical presentation and outcome of our pediatric patients with idiopathic CG.


METHODS
A sum of six children with idiopathic CG, aged 1-7 years at presentation, were retrospectively identified. Clinical data and renal biopsy were reviewed for all patients. Serum creatinine and estimated GFRs at presentation and last follow-up were compared using the Wilcoxon signed rank test and the risk factors for occurrence of ESRD analyzed using the Cox proportional hazard models.


RESULTS
Steroid-resistant nephrotic syndrome with or without azotemia was the presenting clinical finding in all the cases. The median serum creatinine values at onset and last follow-up were 1.05 and 1.25 mg/dl, respectively (p = 0.128). Following immunosuppressive therapy one patient achieved complete remission of proteinuria, and four were in partial remission. The remaining one patient did not show any change in proteinuria at 6 months of therapy. Two of the six patients progressed to end-stage renal disease within a median follow-up period of 27 months (range 14-96 months).


CONCLUSIONS
Collapsing glomerulopathy is an aggressive variant of focal segmental glomerulosclerosis. All patients with CG should be screened for the underlying etiology, and patients with idiopathic CG should be offered a trial of immunosuppressive therapy.
Gulati
Ashima
A
Department of Pediatrics, All India Institute of Medical Sciences, Teaching block, Room number 3053#, Ansari Nagar, New Delhi, 110029, India.
Sharma
Alok
A
Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
Hari
Pankaj
P
Department of Pediatrics, All India Institute of Medical Sciences, Teaching block, Room number 3053#, Ansari Nagar, New Delhi, 110029, India.
Dinda
Amit K
AK
Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
Bagga
Arvind
A
Department of Pediatrics, All India Institute of Medical Sciences, Teaching block, Room number 3053#, Ansari Nagar, New Delhi, 110029, India. arvindbagga@hotmail.com.
D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000097:blood; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005919:Glomerular Filtration Rate / Q000502:physiology; D005923:Glomerulosclerosis, Focal Segmental / Q000175:diagnosis* / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D007668:Kidney / Q000473:pathology*; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone; D003404:Creatinine