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Nephrotic syndrome (NS) is a common renal disorder with significant tubulo-interstitial damage due to the combined effects of proteinuria and obstruction of efferent blood flow. Peritubular capillary (PTC) loss has also been correlated with interstitial fibrosis. This study included 30 pediatric cases of idiopathic NS. Clinical details, including biochemical parameters, were recorded and renal biopsy slides were reviewed for histological features. PTCs were highlighted using anti-CD34 antibody and quantified with the help of image analysis software. Postmortem kidney biopsies from seven children were taken as controls for quantification of PTCs and interstitial fibrosis. Wherever possible, as ultrastructural examination of the renal biopsy was performed. Appropriate statistical methods were applied. Patients with minimal change disease (MCD) had lower serum creatinine as compared with those with focal and segmental glomerulosclerosis (FSGS). Similarly, tubular atrophy and interstitial fibrosis were significantly lower in MCD than in FSGS. PTC density was lower in all groups of NS as compared with the controls. Biopsies with FSGS had a lower PTC density compared with both MCD and mesangioproliferative glomerulonephritis. PTC density showed a negative correlation with serum creatinine and degree of proteinuria. PTC loss appears to play an important role in the development of renal biopsy changes in pediatric NS. This aspect of the renal vasculature requires further study in idiopathic NS.

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.

Primary focal segmental glomerulosclerosis (FSGS) has recently been divided into five subtypes by the Columbia classification. However, little is known about the incidence of these subtypes in the Indian population. In addition, there are very few studies evaluating the clinico-pathologic features with morphometric parameters in these subtypes. This study was aimed at evaluating the clinical, histopathological and morphometric parameters in various subtypes of FSGS at our referral center. Sixty-five (65) cases of idiopathic FSGS, diagnosed over two years (2006-2007), were included in the study. Detailed clinical and biochemical investigations were noted. Histological sections were reviewed and cases classified according to the Columbia classification and various glomerular and tubulo-interstitial features were noted. Glomerular morphometry on digitized images was performed using image analysis software. Renal biopsies with minimal change disease were used as controls for morphometric evaluation. In this study, FSGS not otherwise specified (NOS) was the most common subtype (44.6%), followed by perihilar FSGS (24.6%), collapsing (13.8%), tip (12.3%) and cellular FSGS (4.6%). Collapsing subtype showed significantly shorter duration of symptoms and higher degree of proteinuria, mean serum urea and creatinine compared with the other subtypes. On histologic analysis, features like glomerular hyalinosis, capsular adhesion, mesangial proliferation and visceral epithelial cell prominence (VEP) were frequently seen. The cases with VEP had a shorter duration of symptoms and a higher mean serum creatinine and 24-h urine protein excretion compared with those without VEP. The morphometric study revealed a significant higher mean glomerular area in the NOS, perihilar and collapsing variants as compared with the control biopsies. The present study highlights the differences in the prevalence in the FSGS subtypes in our population compared with the western data. Also, the significant differences in the clinical, biochemical and histological parameters reaffirm the utility of the Columbia classification of FSGS in routine reporting of renal biopsies. We found VEP (without causing collapse of the tuft) to be associated with higher serum creatinine at presentation. This feature needs to be evaluated in further studies for its potential significance.

Background: Crescentic glomerulonephritis (CrGN), defined as crescents involving more than 50% of the glomeruli, includes pauci-immune, immune complex-mediated and anti-glomerular basement membrane disease. Objectives: The present study was aimed at evaluating the various clinical, biochemical and histological parameters in CrGN with respect to these categories and clinical outcome. Materials and Methods: Renal biopsies diagnosed as CrGN between Jan 2008 and Feb 2010 were included. Clinical and laboratory parameters were retrieved along with the therapeutic approach and clinical outcome, wherever available. Renal biopsy slides were evaluated for various glomerular, tubulo-interstitial and arteriolar features. Appropriate statistical tests were applied for significance. Results: A total of 46 cases of CrGN were included; majority (71.7%) of cases were pauci-immune (PI) while 28.3% were immune complex-mediated (IC). Among clinical features, gender ratio was significantly different between PI and IC groups (P = 0.006). The various histological parameters, including proportion of cellular crescents, tuft necrosis and Bowman′s capsule rupture, were similar in both the groups. Four unusual associations, including idiopathic membranoproliferative glomerulonephritis (MPGN), multibacillary leprosy, acute lymphoblastic leukemia and C1q nephropathy were detected. Adequate follow-up information was available in 21 (46%) of the patients. Of these, 11 (52.4%) were dialysis-dependent at the last follow-up. Adult patients required renal replacement therapy more frequently than pediatric cases (P = 0.05). Presence of arteriolar fibrinoid necrosis also showed association with poor clinical outcome (P = 0.05). Conclusions: Crescentic glomerulonephritis remains one of the main causes of acute renal failure with histological diagnosis. Immunohistologic examination is essential for accurate classification into one of the three categories. This condition should be considered in rare causal associations like leprosy or MPGN with renal failure, to allow for timely performed renal biopsy and appropriate aggressive therapy.