Idiopathic collapsing focal segmental glomerulosclerosis in pediatric patients

Abstract

The aim of this study was to evaluate the clinical outcome of our patients with idiopathic collapsing focal segmental glomerulosclerosis (FSGS) as compared to those with non-collapsing FSGS. The study included a total of 39 patients with idiopathic FSGS. Of these, 11 had collapsing FSGS and the remaining 28 were collectively grouped as non-collapsing FSGS. The mean ages, gender ratio (M:F), and percentage of African-American patients in collapsing versus non-collapsing FSGS groups were 12.7+/-3.1 and 8.9+/-5.1 years, 1.2:1 and 4.6:1, and 90.9 and 53.6%, respectively. After a mean followup period of 31.5+/-22.3 months, 8 patients (73%) with collapsing FSGS had chronic renal impairment as compared to 8 (29%) patients with non-collapsing FSGS group after a mean follow-up period of 18.7+/-12.9 months. However, the cumulated renal survival at 30 months did not reveal a significant difference. In comparison to non-collapsing FSGS, collapsing FSGS in our study was equally common in females as in males and occurred predominantly in African Americans. The outcome of our patients with collapsing FSGS at 30 months was better than in previous reports.

Collapsing Focal Segmental Glomerulosclerosis in a Young Child

“…Collapsing FSGS has been described by many authors from different parts of the world as a cause of SRNS in children [9,12,13,14]. Our patient had high blood pressure at presentation, and this is similar to previous reports of initial high blood pressure in affected children [13].…”

“…In general, there is a poor response to steroids [12,13,14]. However, remission is achievable in some patients with other immunosuppressive therapy [14,15,16].…”

“…In general, there is a poor response to steroids [12,13,14]. However, remission is achievable in some patients with other immunosuppressive therapy [14,15,16]. While there have been reports of efficacy for rituximab in FSGS (for example, Kaito et al [17] reported a case of a 2-year-old boy with steroid- and CsA-resistant collapsing FSGS who went into complete remission with a combination of intravenous rituximab and MP pulse therapy while receiving oral CsA), we were unable to offer rituximab in this case.…”

Idiopathic collapsing glomerulopathy in children

“…Patients with CG typically present with steroid-resistant nephrotic syndrome, and a significant proportion shows features of renal insufficiency with rapid progression to renal failure [11]. While CG was initially described in association with HIV-infection, subsequent reports have suggested an association with other infections (parvovirus), medications (interferon, pamidronate), acute ischemia associated with thrombotic microangiopathy, and mitochondrial dysfunction [4,12,13]. Although FSGS is known as one of the major renal complications of mitochondrial cytopathies, further investigations to clarify the pathogenic role of mitochondrial mutations could not be done in the present study.…”

Primary focal segmental glomerulosclerosis in Egyptian children: a 10-year single-centre experience

“…Steroid resistance defined as lack of improvement in proteinuria after 1 month of prednisolone therapy of 60 mg/m 2 /day plus three doses of MP (30 mg/kg maximum 1 g). Steroid dependence is defined as patient relapse while on alternate-day steroid therapy or within 14 days of stopping steroid therapy [16,17].…”