ESPN 50th Annual Meeting

ESPN 2017


 
Collapsing Focal Segmental Glomerulosclerosis in the Paediatric Cohort: a Single Centers Experience
YONG HONG NG 1 SIEW LE CHONG 1

1- KK WOMENS AND CHILDRENS HOSPITAL, SINGAPORE
 
Introduction:

The collapsing variant of focal segmental glomerulosclerosis (FSGS) has been associated with poor treatment response with rapid progression to renal failure. There is relative paucity of knowledge on the clinical outcome of idiopathic collapsing FSGS in the paediatric population. We describe the units experience with 4 cases of idiopathic collapsing FSGS in the past 6 years.

Material and methods:

The case records of 4 paediatric patients with histological diagnosis of collapsing FSGS were reviewed with regards to patient demographics, clinical presentation, treatment, clinical course and eventual renal outcome. The primary endpoint for this descriptive review was the progression to end stage renal failure (ESRF).

Results:

The 4 patients were predominantly males (3:1). Age of presentation varies from 2.3-12.3 years. The degree of proteinuria at presentation ranges from 2.90g/L to massive proteinuria (13.9 – 20.7g/L) and did not have a correlation to renal outcome. At the time of the review, 3 patients had developed ESRF. All patients had a full virology and immunological work-up done and results returned negative. All were started on prednisolone therapy. Two were late non-responders while the remaining two patients demonstrated steroid resistance right from the start. One patient demonstrated extensive tubulointerstitial injury with more than 50% global sclerosis on the initial renal biopsy performed three weeks into the presentation and developed rapid progression to ESRF. All patients were treated with prednisolone in combination with other immunosuppressants (cyclosporine, mycophenolate mofetil or rituximab). Two patients achieved partial remission. The remaining two who presented with elevated serum creatinine at initial diagnosis remained refractory to therapy and progress to ESRF within 3 years from initial presentation (0.08-2.25 years).

Conclusions:

An elevated initial serum creatinine, lack of remission and tubulointerstitial involvement have been identified as poor prognostic factors in previous studies. The same observations were made in this case series with a male predominance.