title: Glomerular filtration barrier in pediatric idiopathic nephrotic syndrome.
abstract: Nephrotic syndrome (NS) is a common proteinuric disorder with defect in the perm-selectivity of the glomerular filtration barrier (GFB). Ultrastructural morphometric evaluation of the GFB in pediatric NS has been attempted in only a few studies. This study was aimed at qualitative and quantitative evaluation of the alterations involving the GFB in pediatric idiopathic NS with an attempt to correlate these alterations with the clinico-laboratory data. For this study, renal biopsies from nine patients with NS and two children with interstitial nephritis were included. Relevant clinical and laboratory data, including degree of 24-h proteinuria and renal function tests, were recorded. Renal biopsies were reviewed for morphologic and electron microscopic diagnosis. Ultrastructural morphometry of the GFB was performed using image analysis software. The age at onset of NS, duration of illness, presence of hypertension, and renal function tests were comparable between the group of patients with minimal change disease (MCD) and those with mesangioproliferative glomerulonephritis (mesPGN)/focal segmental glomerulosclerosis (FSGS). However, the latter group showed higher 24-h proteinuria compared with the group with MCD. Among the detected ultra-structural changes, glomerular basement membrane thickness and foot process width were significantly different between the MCD and the mesPGN/FSGS groups. The slit pore diameter in the glomeruli showed a positive correlation with the degree of proteinuria. We conclude that our study demonstrated remarkable differences in certain parameters and the glomerular ultrastructural alterations in the various categories of NS. These differences might underlie the observed variation in response of these entities to various therapies.
authors.affiliation: Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.
authors.forename: Alok
authors.initials: A
authors.lastname: Sharma
authors.forename: Ruchika
authors.initials: R
authors.lastname: Gupta
authors.forename: Arvind
authors.initials: A
authors.lastname: Bagga
authors.forename: Amit K
authors.initials: AK
authors.lastname: Dinda
mesh_terms: D017668:Age of Onset; D001706:Biopsy; D002675:Child, Preschool; D005260:Female; D060730:Glomerular Filtration Barrier / Q000503:physiopathology / Q000648:ultrastructure*; D015432:Glomerulonephritis, Membranoproliferative / Q000453:epidemiology / Q000473:pathology; D005923:Glomerulosclerosis, Focal Segmental / Q000453:epidemiology / Q000473:pathology; D006801:Humans; D007194:India / Q000453:epidemiology; D007223:Infant; D007677:Kidney Function Tests; D008297:Male; D046529:Microscopy, Electron, Transmission; D009402:Nephrosis, Lipoid / Q000453:epidemiology / Q000473:pathology; D009404:Nephrotic Syndrome / Q000453:epidemiology / Q000473:pathology* / Q000503:physiopathology; D011507:Proteinuria / Q000453:epidemiology / Q000473:pathology; D012189:Retrospective Studies