Early versus late-onset idiopathic focal segmental glomerulosclerosis

Abstract

Glomerular diseases in children, although similar in histological appearance to those in adults, may have a better prognosis. There is much controversy regarding the prognostic factors in idiopathic focal segmental glomerulosclerosis (FSGS), especially the comparative prognosis of children and adults. A comparative analysis was carried out of 36 consecutive biopsy-proven cases of idiopathic FSGS presenting early in life ['early onset' as seen in children < or =12 years (group I)] and 36 cases presenting later ['late-onset' as seen in older children >12 years and adults (group II)]. Patients were compared for clinical, biochemical, and histopathological features, as well as disease outcome. A significantly higher prevalence of hypertension (P=0.002) and microscopic hematuria was seen in group II (P=0.02). There were no differences between the two groups in glomerular filtration rates corrected for body surface area at initial presentation (92+/-11 ml/min/1.73 m2 vs. 94+/-14 ml/min/1.73 m2). Patients with 'late-onset' FSGS had a significantly higher number of glomeruli with segmental sclerosis (P=0.007), more mesangial matrix expansion (P=0.009), greater mesangial cellularity (P=0.003), and significantly higher blood vessel involvement (P=0.03) than those with 'early onset' FSGS. There was a significantly higher response to steroids in group I (82.3%) than group II (36.4%) (P<0.02). At the end of the study period, 2 patients in group I and 11 in group II had developed persistent renal failure (P=0.01). Thus 'early onset' FSGS is more common in males, has significantly lower prevalence of hypertension and microscopic hematuria, with less-severe histopathological involvement, is more often steroid responsive, and has a better prognosis than 'late-onset' FSGS.

Predictive factors of chronic kidney disease in primary focal segmental glomerulosclerosis

“…A number of cohort studies of FSGS in children [9,12,[24][25][26][27][28][29][30] and in adults [11,[31][32][33][34][35][36] has been reported in literature. Other studies have included both children and adults in the analysis [20,[37][38][39]. Nevertheless, few studies in the pediatric setting have investigated predictive factors of outcome.…”

“…The localization (vascular/tuft) and morphology (collapsing/cellular) of the changes were also determined. Other histological changes (mesangial cellularity, interstitial fibrosis, interstitial infiltration, tubular atrophy and blood vessel involvement) were scored according to a semiquantitative scoring system as follows: 0=no change, 1=focal changes, 2=mild involvement, 3=moderate changes, and 4=severe changes [20].…”

Clinical course of 110 children and adolescents with primary focal segmental glomerulosclerosis

“…For example, Yoshikawa et al [22] reported a percentage of segmentally sclerotic glomeruli ranging from 16-22% and a percentage of globally sclerotic glomeruli ranging from 8.4-14%. Series of adult patients with FSGS have shown a more severe glomerular involvement [13,32,33]. Histologic variants of FSGS have been regarded as predictors of the response to therapy and of clinical outcome in adult patients, although with conflicting results [33][34][35].…”

“…The localization (vascular/tubular) and morphology of the changes (collapsing/tip/cellular) were also determined. Other histological changes (mesangial matrix, mesangial cellularity, interstitial fibrosis, interstitial infiltration, tubular atrophy, and blood vessel involvement) were each scored according to a semi-quantitative scoring system as follows: 0=no change, 1=focal changes, 2=mild involvement, 3=moderate and 4=severe changes) [13]. Fifteen patients were submitted to two renal biopsies and previous histological findings were minimal change in six patients and mesangial proliferative changes in nine.…”

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

“…Other histopathological changes (mesangial matrix, mesangial cellularity, interstitial fibrosis, interstitial infiltration, tubular atrophy and blood vessel involvement) were scored using a semi-quantitative scoring system as follows: 0=no change; 1=focal changes (<10%); 2=mild involvement (10-25%); 3=moderate (26-50%); 4=severe changes (>50%). This system was similar to that used in an earlier study [15].…”