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A histopathological outlook on nephrotic syndrome: A pediatric perspective.

Arif MK, Arif M, Amjad N - Indian J Nephrol (2016 May-Jun)

Bottom Line: Focal segmental glomerulosclerosis (FSGS) was the most common histopathological subtype observed in 35 (46.8%) children followed by membranous glomerulonephritis (MGN) in 11 (14.7%), membranoproliferative glomerulonephritis (MPGN) and mesangioproliferative glomerulonephritis (MSGN) in 4 (5.3%) each and IgA nephropathy in one (1.3%).MCD was the histological lesion in 19 (25.3%) children.The histopathology established FSGS as the main underlying cause of steroid resistant NS.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Aga Khan Hospital, Karachi, Pakistan.

ABSTRACT
The developing world is observing changing histopathological patterns of idiopathic nephrotic syndrome (INS). However, the true burden of non-minimal change disease (non-MCD) presenting as INS remains unestimated owing to a paucity of data on renal biopsies. Data were collected from January 2006 to June 2014 on 75 children up to 16 years of age who underwent renal biopsies for INS. Mean age at biopsy was 11.2 ± 3.7 years. The male to female ratio was 1.5:1. A total of 25 (33.3%) children were steroid sensitive, 36 (48%) were steroid resistant, 10 (13.3%) were steroid dependent and 4 (5.3%) came with relapse of nephrotic syndrome (NS). Focal segmental glomerulosclerosis (FSGS) was the most common histopathological subtype observed in 35 (46.8%) children followed by membranous glomerulonephritis (MGN) in 11 (14.7%), membranoproliferative glomerulonephritis (MPGN) and mesangioproliferative glomerulonephritis (MSGN) in 4 (5.3%) each and IgA nephropathy in one (1.3%). MCD was the histological lesion in 19 (25.3%) children. The histopathology established FSGS as the main underlying cause of steroid resistant NS. The study highlights the emergence of non-MCD as the common cause of INS in the pediatric population and signifies the importance of renal biopsies in children with INS.

No MeSH data available.


Related in: MedlinePlus

Frequency of the different histopathological subtypes of nephrotic syndrome in relation to the clinical subtypes in children between 0-16 years of age. X-axis: histological subtypes, Y-axis: number of patients. Indications of renal biopsies related to color code on right side
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Figure 1: Frequency of the different histopathological subtypes of nephrotic syndrome in relation to the clinical subtypes in children between 0-16 years of age. X-axis: histological subtypes, Y-axis: number of patients. Indications of renal biopsies related to color code on right side

Mentions: Minimal change disease has been established as the most common cause of NS in children over the past three decades;[3] the incidence varies from 77 to 90% in different studies.[7810] However, changing trends in the histopathological patterns have been observed in the pediatric population over the past 30 years. Even in the earliest reports on renal biopsies in NS, FSGS was not uncommon. About 5–7% of renal biopsies performed for NS were consistent with this histopathological subtype.[71011] However, FSGS is becoming increasingly common in children with NS, warranting a renal biopsy on all such children for accurate diagnosis and management even in resource-limited settings like ours. A rising trend of FSGS and other non-MCD subtypes validates the need for renal biopsy in all children with NS. Our data are also consistent with the emergence of non-MCDs with FSGS as the most frequently reported histological subtype. In our study, renal biopsies on seventy-five children with NS were analyzed. There was a male predilection that is in accordance with the international data.[312] The mean age at onset was 11.2 ± 3.7 years. This is in contrast to the published data that reveal a younger age at onset of the disease.[231113] Most of the children in our study group were older than 10 years of age. A similar mean age has been described in a local study by Mubarak and Kazi.[14] Of the 75 cases of NS, 25 (33.3%) were steroid sensitive, 36 (48%) were steroid resistant, 10 (13.3%) were steroid dependent and 4 (5.3%) were with relapse. Data from Iran on children with NS reported different figures; 87% children were steroid sensitive, and 13% were found to be steroid resistant.[15] There is variation in the local data as well. Mubarak et al. also reviewed children with NS and reported 46.6% to be steroid dependent and 31% to be steroid resistant. The histopathology also varied according to the clinical subtype of NS. MCD was the most common subtype in steroid dependent NS; for all other clinical subtypes, FSGS was the leading pathological cause.[12] In our study, for all the clinical subtypes except relapse, FSGS was the most common histological lesion. MCD remained the most common underlying histological subtype for relapsed NS [Figure 1]. Studies from other countries have also reported FSGS as the leading cause of both idiopathic and difficult NS.[231617] However, data from different centers continue to report MCD as a common cause of NS in the pediatric group.[11181920] We also report a frequency of 1.3% of congenital NS that is almost similar to that reported in a local study by Mubarak et al.[12]


A histopathological outlook on nephrotic syndrome: A pediatric perspective.

Arif MK, Arif M, Amjad N - Indian J Nephrol (2016 May-Jun)

Frequency of the different histopathological subtypes of nephrotic syndrome in relation to the clinical subtypes in children between 0-16 years of age. X-axis: histological subtypes, Y-axis: number of patients. Indications of renal biopsies related to color code on right side
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4862264&req=5

Figure 1: Frequency of the different histopathological subtypes of nephrotic syndrome in relation to the clinical subtypes in children between 0-16 years of age. X-axis: histological subtypes, Y-axis: number of patients. Indications of renal biopsies related to color code on right side
Mentions: Minimal change disease has been established as the most common cause of NS in children over the past three decades;[3] the incidence varies from 77 to 90% in different studies.[7810] However, changing trends in the histopathological patterns have been observed in the pediatric population over the past 30 years. Even in the earliest reports on renal biopsies in NS, FSGS was not uncommon. About 5–7% of renal biopsies performed for NS were consistent with this histopathological subtype.[71011] However, FSGS is becoming increasingly common in children with NS, warranting a renal biopsy on all such children for accurate diagnosis and management even in resource-limited settings like ours. A rising trend of FSGS and other non-MCD subtypes validates the need for renal biopsy in all children with NS. Our data are also consistent with the emergence of non-MCDs with FSGS as the most frequently reported histological subtype. In our study, renal biopsies on seventy-five children with NS were analyzed. There was a male predilection that is in accordance with the international data.[312] The mean age at onset was 11.2 ± 3.7 years. This is in contrast to the published data that reveal a younger age at onset of the disease.[231113] Most of the children in our study group were older than 10 years of age. A similar mean age has been described in a local study by Mubarak and Kazi.[14] Of the 75 cases of NS, 25 (33.3%) were steroid sensitive, 36 (48%) were steroid resistant, 10 (13.3%) were steroid dependent and 4 (5.3%) were with relapse. Data from Iran on children with NS reported different figures; 87% children were steroid sensitive, and 13% were found to be steroid resistant.[15] There is variation in the local data as well. Mubarak et al. also reviewed children with NS and reported 46.6% to be steroid dependent and 31% to be steroid resistant. The histopathology also varied according to the clinical subtype of NS. MCD was the most common subtype in steroid dependent NS; for all other clinical subtypes, FSGS was the leading pathological cause.[12] In our study, for all the clinical subtypes except relapse, FSGS was the most common histological lesion. MCD remained the most common underlying histological subtype for relapsed NS [Figure 1]. Studies from other countries have also reported FSGS as the leading cause of both idiopathic and difficult NS.[231617] However, data from different centers continue to report MCD as a common cause of NS in the pediatric group.[11181920] We also report a frequency of 1.3% of congenital NS that is almost similar to that reported in a local study by Mubarak et al.[12]

Bottom Line: Focal segmental glomerulosclerosis (FSGS) was the most common histopathological subtype observed in 35 (46.8%) children followed by membranous glomerulonephritis (MGN) in 11 (14.7%), membranoproliferative glomerulonephritis (MPGN) and mesangioproliferative glomerulonephritis (MSGN) in 4 (5.3%) each and IgA nephropathy in one (1.3%).MCD was the histological lesion in 19 (25.3%) children.The histopathology established FSGS as the main underlying cause of steroid resistant NS.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Aga Khan Hospital, Karachi, Pakistan.

ABSTRACT
The developing world is observing changing histopathological patterns of idiopathic nephrotic syndrome (INS). However, the true burden of non-minimal change disease (non-MCD) presenting as INS remains unestimated owing to a paucity of data on renal biopsies. Data were collected from January 2006 to June 2014 on 75 children up to 16 years of age who underwent renal biopsies for INS. Mean age at biopsy was 11.2 ± 3.7 years. The male to female ratio was 1.5:1. A total of 25 (33.3%) children were steroid sensitive, 36 (48%) were steroid resistant, 10 (13.3%) were steroid dependent and 4 (5.3%) came with relapse of nephrotic syndrome (NS). Focal segmental glomerulosclerosis (FSGS) was the most common histopathological subtype observed in 35 (46.8%) children followed by membranous glomerulonephritis (MGN) in 11 (14.7%), membranoproliferative glomerulonephritis (MPGN) and mesangioproliferative glomerulonephritis (MSGN) in 4 (5.3%) each and IgA nephropathy in one (1.3%). MCD was the histological lesion in 19 (25.3%) children. The histopathology established FSGS as the main underlying cause of steroid resistant NS. The study highlights the emergence of non-MCD as the common cause of INS in the pediatric population and signifies the importance of renal biopsies in children with INS.

No MeSH data available.


Related in: MedlinePlus