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Frequency and clinicopathological correlations of histopathological variants of pediatric idiopathic focal segmental glomerulosclerosis.

Shakeel S, Mubarak M, Kazi JI - Indian J Nephrol (2014)

Bottom Line: Global glomerulosclerosis was found in 68 (49.3%) cases.Hyaline arteriolosclerosis was found in 13 (9.4%) cases.In conclusion, FSGS, NOS variant was the highly prevalent variant, while collapsing type was also found in small but significant number of cases.

View Article: PubMed Central - PubMed

Affiliation: Department of Histopathology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.

ABSTRACT
There is no information on the frequency and clinicopathological correlations of the histopathological variants of primary focal segmental glomerulosclerosis (FSGS) in children presenting with idiopathic nephrotic syndrome (INS) in Pakistan. All consecutive children (≤17 years) who presented with INS, and in whom the histological diagnosis of FSGS was made on renal biopsies, were included in this prospective study. Their clinical, laboratory, and histopathological features at the time of presentation were noted from the case files and the biopsy reports for analysis and clinicopathological correlations. Out of 138 children, 93 (67.4%) were males and 45 (32.6%) were females. The mean age was 8.95 ± 4.14 (range: 1.5-17) years. All had NS, with steroid dependant NS (SDNS) in 45 (32.6%) and steroid resistant NS (SRNS) in 93 (67.4%) cases. Renal dysfunction at the time of presentation was found in six (4.3%) children. Global glomerulosclerosis was found in 68 (49.3%) cases. The mean number of glomeruli involved by segmental scarring was 2.98 ± 2.44. FSGS, not otherwise specified (NOS) was the most prevalent variant, comprising 89.1% of all cases. Collapsing variant comprised 8%, tip variant 1.4%, perihilar 0.7%, and cellular 0.7%. Hyaline arteriolosclerosis was found in 13 (9.4%) cases. Mild interstitial fibrosis/tubular atrophy was found in 95 (68.6%) cases, moderate in 18 (13%), and severe in two (1.4%) cases. In conclusion, FSGS, NOS variant was the highly prevalent variant, while collapsing type was also found in small but significant number of cases. Remaining three variants were distinctly rare in our children.

No MeSH data available.


Related in: MedlinePlus

(a) Global glomerulosclerosis. Although this lesion is not required for the diagnosis of focal segmental glomerulosclerosis, it is frequently present in association with segmental glomerulosclerosis in advanced stages of the disease (Silver, ×200). (b) Segmental glomerulosclerosis involving more than half of the glomerulus with the scarring process associated with adhesion formation with Bowman's capsule in the FSGS, not otherwise specified (NOS) variant (Silver, ×200). (c) Tip variant of FSGS involving the tubular pole (periodic acid-Schiff, ×200). (d) The perihilar location of segmental sclerosis in a case of perihilar FSGS (PAS, ×200)
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Figure 1: (a) Global glomerulosclerosis. Although this lesion is not required for the diagnosis of focal segmental glomerulosclerosis, it is frequently present in association with segmental glomerulosclerosis in advanced stages of the disease (Silver, ×200). (b) Segmental glomerulosclerosis involving more than half of the glomerulus with the scarring process associated with adhesion formation with Bowman's capsule in the FSGS, not otherwise specified (NOS) variant (Silver, ×200). (c) Tip variant of FSGS involving the tubular pole (periodic acid-Schiff, ×200). (d) The perihilar location of segmental sclerosis in a case of perihilar FSGS (PAS, ×200)

Mentions: FSGS constituted 33.4% of all native renal biopsies done for INS in children during the study period. The principal histopathologic features on renal biopsies from 138 children are given in Table 2 and depicted in Figures 1 and 2. Our results indicate that FSGS, NOS is highly prevalent variant, comprising 89.1% of all cases. Collapsing variant comprised 8%, tip variant 1.4% perihilar 0.7%, and cellular 0.7%. The mean number of glomeruli included in all biopsies was 15.31 ± 8.17. Global glomerulosclerosis was found in 68 (49.3%) of all cases. No global glomerulosclerosis was found in 70 (50.7%) of cases. The number of globally sclerosed glomeruli ranged from 0 to 16 in an individual biopsy and the mean was 1.46 ± 2.34 per biopsy. The mean number of glomeruli involved by segmental scarring was 2.98 ± 2.44. The nonscarred areas of the glomeruli showed variable degrees of mesangial proliferation in 42% of the biopsies, while 58% showed minor changes of the non-sclerotic portions of the glomeruli. The vasculopathy was distinctly rare, given the young age of the cohort. Hyaline arteriolosclerosis was found in 13 (9.4%) cases, while no biopsy showed fibrointimal thickening (FITC) of arteries. The chronic parenchymal changes in the form of interstitial fibrosis/tubular atrophy (IFTA) were quite prevalent, however. Mild IFTA was found in 95 (68.6%) of cases, moderate in 18 (13%), and severe in two (1.4%) cases; while no IFTA was found in 23 (16.7%) cases. The IF examination of the biopsies showed focal to diffuse mesangial positivity of IgM in 57 (41.3%) cases associated with C3 deposition in 18 (13%) and C1q in five (3.69%) cases; while IgG and IgA were negative in all biopsies. All cases of collapsing FSGS tested negative for HCV, HBV, CMV, and HIV serology as well as autoimmune markers.


Frequency and clinicopathological correlations of histopathological variants of pediatric idiopathic focal segmental glomerulosclerosis.

Shakeel S, Mubarak M, Kazi JI - Indian J Nephrol (2014)

(a) Global glomerulosclerosis. Although this lesion is not required for the diagnosis of focal segmental glomerulosclerosis, it is frequently present in association with segmental glomerulosclerosis in advanced stages of the disease (Silver, ×200). (b) Segmental glomerulosclerosis involving more than half of the glomerulus with the scarring process associated with adhesion formation with Bowman's capsule in the FSGS, not otherwise specified (NOS) variant (Silver, ×200). (c) Tip variant of FSGS involving the tubular pole (periodic acid-Schiff, ×200). (d) The perihilar location of segmental sclerosis in a case of perihilar FSGS (PAS, ×200)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4127833&req=5

Figure 1: (a) Global glomerulosclerosis. Although this lesion is not required for the diagnosis of focal segmental glomerulosclerosis, it is frequently present in association with segmental glomerulosclerosis in advanced stages of the disease (Silver, ×200). (b) Segmental glomerulosclerosis involving more than half of the glomerulus with the scarring process associated with adhesion formation with Bowman's capsule in the FSGS, not otherwise specified (NOS) variant (Silver, ×200). (c) Tip variant of FSGS involving the tubular pole (periodic acid-Schiff, ×200). (d) The perihilar location of segmental sclerosis in a case of perihilar FSGS (PAS, ×200)
Mentions: FSGS constituted 33.4% of all native renal biopsies done for INS in children during the study period. The principal histopathologic features on renal biopsies from 138 children are given in Table 2 and depicted in Figures 1 and 2. Our results indicate that FSGS, NOS is highly prevalent variant, comprising 89.1% of all cases. Collapsing variant comprised 8%, tip variant 1.4% perihilar 0.7%, and cellular 0.7%. The mean number of glomeruli included in all biopsies was 15.31 ± 8.17. Global glomerulosclerosis was found in 68 (49.3%) of all cases. No global glomerulosclerosis was found in 70 (50.7%) of cases. The number of globally sclerosed glomeruli ranged from 0 to 16 in an individual biopsy and the mean was 1.46 ± 2.34 per biopsy. The mean number of glomeruli involved by segmental scarring was 2.98 ± 2.44. The nonscarred areas of the glomeruli showed variable degrees of mesangial proliferation in 42% of the biopsies, while 58% showed minor changes of the non-sclerotic portions of the glomeruli. The vasculopathy was distinctly rare, given the young age of the cohort. Hyaline arteriolosclerosis was found in 13 (9.4%) cases, while no biopsy showed fibrointimal thickening (FITC) of arteries. The chronic parenchymal changes in the form of interstitial fibrosis/tubular atrophy (IFTA) were quite prevalent, however. Mild IFTA was found in 95 (68.6%) of cases, moderate in 18 (13%), and severe in two (1.4%) cases; while no IFTA was found in 23 (16.7%) cases. The IF examination of the biopsies showed focal to diffuse mesangial positivity of IgM in 57 (41.3%) cases associated with C3 deposition in 18 (13%) and C1q in five (3.69%) cases; while IgG and IgA were negative in all biopsies. All cases of collapsing FSGS tested negative for HCV, HBV, CMV, and HIV serology as well as autoimmune markers.

Bottom Line: Global glomerulosclerosis was found in 68 (49.3%) cases.Hyaline arteriolosclerosis was found in 13 (9.4%) cases.In conclusion, FSGS, NOS variant was the highly prevalent variant, while collapsing type was also found in small but significant number of cases.

View Article: PubMed Central - PubMed

Affiliation: Department of Histopathology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.

ABSTRACT
There is no information on the frequency and clinicopathological correlations of the histopathological variants of primary focal segmental glomerulosclerosis (FSGS) in children presenting with idiopathic nephrotic syndrome (INS) in Pakistan. All consecutive children (≤17 years) who presented with INS, and in whom the histological diagnosis of FSGS was made on renal biopsies, were included in this prospective study. Their clinical, laboratory, and histopathological features at the time of presentation were noted from the case files and the biopsy reports for analysis and clinicopathological correlations. Out of 138 children, 93 (67.4%) were males and 45 (32.6%) were females. The mean age was 8.95 ± 4.14 (range: 1.5-17) years. All had NS, with steroid dependant NS (SDNS) in 45 (32.6%) and steroid resistant NS (SRNS) in 93 (67.4%) cases. Renal dysfunction at the time of presentation was found in six (4.3%) children. Global glomerulosclerosis was found in 68 (49.3%) cases. The mean number of glomeruli involved by segmental scarring was 2.98 ± 2.44. FSGS, not otherwise specified (NOS) was the most prevalent variant, comprising 89.1% of all cases. Collapsing variant comprised 8%, tip variant 1.4%, perihilar 0.7%, and cellular 0.7%. Hyaline arteriolosclerosis was found in 13 (9.4%) cases. Mild interstitial fibrosis/tubular atrophy was found in 95 (68.6%) cases, moderate in 18 (13%), and severe in two (1.4%) cases. In conclusion, FSGS, NOS variant was the highly prevalent variant, while collapsing type was also found in small but significant number of cases. Remaining three variants were distinctly rare in our children.

No MeSH data available.


Related in: MedlinePlus