Focal and segmental glomerulosclerosis in children: a longitudinal assessment

Abstract

Recent data suggest that the histologic finding of focal and segmental glomerulosclerosis (FSGS) is increasing among children. There are, however, limited longitudinal pediatric data on prevalence, demographics, and steroid responsiveness in FSGS. We identified 201 consecutive nephrotic children diagnosed between 1977 and 2002 with 2 years follow-up; 51% had undergone renal biopsy due to steroid sequelae or resistance; 48 children with FSGS were diagnosed. Compared with non-FSGS children, FSGS children were older at diagnosis (6.9 years vs 4.4 years, P<0.02), more likely girls (54% vs 28%, P<0.02), Black or Hispanic (42% vs 16%, P<0.001), and the FSGS was more likely to be steroid resistant (73% vs 10%, P<0.001). To assess for longitudinal differences, we grouped children by presentation: pre-1985, between 1985 and 1995, and post-1995. There was no difference in proportion of children biopsied or diagnosed with FSGS during each interval. Among FSGS children, there was no difference in racial or gender composition in each period, but there was a difference in age at diagnosis (2.6 vs 5.7 vs 8.5 years; P=0.01), also observed in the non-FSGS children (2.2 vs 3.9 vs 4.9 years; P=0.02). In contradistinction to non-FSGS children, there was a marked increase in steroid resistance with FSGS (43% vs 62% vs 86%; P=0.03).

Idiopathic Nephrotic Syndrome in Childhood: A Retrospective Analysis of Two Hundred and Eighty Nine Patients

“…In our study, FSGS was detected in 16.6% of patients who underwent kidney biopsy and 75% of them had steroid resistance. Our findings were compatible with that of the study by Boyer et al (16) which showed increased steroid resistance over the past two decades. FSGS is a histopathological finding of poor prognosis leading to the development of ESRD in patients with NS (17).…”

Circulating suPAR in Two Cohorts of Primary FSGS

“…[1][2][3] It can occur as a primary disorder, consequent to genetic mutations in specific podocyte proteins, secondary to a wide range of medical problems, or as a consequence of adaptive changes to reduced kidney mass. 4,5 Intensive research for .50 years has focused mainly on genetic, immunologic, and systemic causes of FSGS but the cause of primary disease remains elusive.…”

Discrepancy between Medical Evidence Form 2728 and Renal Biopsy for Glomerular Diseases

“…Epidemiologic research of glomerular diseases is limited due to the rarity of individual disease types, the difficulty in correctly diagnosing them, and the lack of accurate disease data. Most epidemiologic studies rely upon biopsy results for accurate disease information (2)(3)(4).…”