There is no detailed information on clinical and immunopathologic features of immunoglobulin M nephropathy (IgMN) in children with idiopathic nephrotic syndrome (INS) in Pakistan. We reviewed our native renal biopsies over 15 years (July 1995-July 2010) and identified 135 cases of IgMN in nephrotic children (≤17 years). Their demographic, clinical and immunopathologic data were retrieved from biopsy reports and case notes. Mean age of this cohort was 7.6 ± 4.2 years. Males were 92 (68.1%) and females were 43 (31.9%). Steroid-dependent NS was seen in 88 (65.2%) cases and steroid-resistant NS in 47 (34.2%). Hematuria was found in 42 cases (31.2%) and hypertension in 27 (19.5%). The most common morphologic change was glomerular mesangial proliferation, found in 89 (65.9%) biopsies. Minor changes were seen in 46 (34.1%) cases and focal segmental glomerulosclerosis (FSGS) in 37 (27.4%). Immunofluorescence microscopy showed diffuse mesangial positivity of IgM in all cases. C3 and C1q were found in 72 (53.3%) and 40 (29.7%) cases, respectively. Our results show that IgMN is a fairly common cause of INS in children in Pakistan. It shows a spectrum of morphologic changes ranging from minor changes to FSGS.

Steroid-resistant nephrotic syndrome (SRNS) is a common problem in pediatric nephrology practice. There is currently little information in the literature on the spectrum of histopathologic lesions in children presenting with SRNS in Pakistan. This study was designed to determine the histopathologic lesions in children presenting with SRNS at our center. The study was conducted at the Histopathology Department, Sindh Institute of Urology and Transplantation (SIUT) from January 2009 to August 2011. All children (≤16 years) presenting with SRNS, in whom renal biopsies were performed, were included. Their demographic, clinical, laboratory, and histopathological data were retrieved from files and original renal biopsy forms. The results were analyzed by SPSS version 10.0. A total of 147 children were included. Of these, 91 (61.9%) were males and 56 (38.1%) females, with male-to-female ratio of 1.6 : 1. The mean age was 7.03 ± 4.0 years (range: 6 months–16 years). The histopathological lesions seen on renal biopsies comprised of focal segmental glomerulosclerosis (FSGS) (38.5%), followed by minimal change disease (MCD) (23.2%), IgM nephropathy (IgMN) (13.6%), idiopathic mesangial proliferative GN (10.2%), membranous GN (8.2%), and mesangiocapillary GN (4.8%). Our results indicate that FSGS is the predominant lesion in children with SRNS, followed by MCD and IgMN.

Background: There is no information on the frequency and clinicopathological presentation of the variants of primary focal segmental glomerulosclerosis (FSGS) in adults presenting with idiopathic nephrotic syndrome (INS) in Pakistan. Objectives: The aim of this study was to determine the frequencies of different histologic variants of primary FSGS with INS at our center and to compare our findings with those published in literature. Patients and Methods: All consecutive adults (≥18 years) with INS, and diagnosis of FSGS on renal biopsies, were included. Their clinicopathological features at the time of presentation were retrieved and compared among the variants. Results: There were 120 (65.2%) males and 64 (34.8%) females. The mean age was 30.62±12.02 years. The mean 24-hr urinary protein excretion was 4.69±2.36 grams. Microscopic hematuria was found in 30 (16.3%) patients. The mean serum creatinine was 1.58±0.87 mg/dL. At presentation, 128 (69.6%) patients were normotensive, while 56 (30.4%) exhibited hypertension. FSGS, not otherwise specified (NOS) was the predominant variant, comprising 76.6% of all; collapsing variant comprised 12%, tip variant, 9.8%, perihilar, 1.1%, and cellular, 0.5%. The mean number of glomeruli involved by segmental scarring was 3.41±2.87 and there was significant difference among the variants (p= 0.001). Arteriolopathy was found in 23.4 % cases and fibrointimal thickening of arteries in 18.5%. Tubular atrophy and interstitial fibrosis (IF/TA) was noted in 93% of cases. There was no significant difference in vasculopathy and IF/TA among the variants. Conclusions: Collapsing variant was the second most common variant following NOS and these findings are different from other regional studies. Implication for health policy/practice/research/medical education:Focal segmental glomerulosclerosis (FSGS) is a heterogeneous lesion with different etiologies, pathogenesis and clinical outcome. Recent classification of FSGS on histological grounds into five variants reflects this heterogeneity of the lesion. A study of these variants in different populations is of utmost importance in understanding the varied etiopathogenetic mechanisms underlying this common lesion in nephrology practice.Please cite this paper as: Shakeel Sh, Mubarak M, Kazi JI, Jafry N, Ahmed E. Frequency and clinicopathological characteristics of variants of primary focal segmental glomerulosclerosis in adults presenting with nephrotic syndrome.

IgMN is a common cause of paediatric SRNS and is significantly different from MCD. There is also a significant difference in clinical and laboratory parameters among responders and non-responders to CNI in IgMN.

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 abstract

Focal segmental glomerulosclerosis (FSGS) is a conglomerate of glomerular pathological lesions unified by the segmental distribution of proliferative, sclerosing or collapsing lesions in less than 50% of glomeruli (i.e., focal distribution) in the early stages of the process and can only be diagnosed on renal biopsy. Epidemiologically, FSGS has acquired the top position among the causes of nephrotic syndrome (NS) in adults in many parts of the world and has emerged as the main cause of chronic kidney disease (CKD) and end-stage renal disease (ESRD) worldwide. Numerous approaches have been used to classify this lesion, but all have merits and demerits and no one system is entirely satisfactory. One of the popular schemes for the classification is based on morphological features and distribution of the lesions in the glomeruli, the so-called Columbia classification of FSGS. In this review, we briefly summarize the relevant epidemiology and pathology of FSGS variants in the light of our own experience with the use of this classification.

Banff classification of renal allograft pathology has evolved considerably over the past 30 years and has undergone significant changes in many of the original diagnostic categories. With this evolution, it has increased in complexity and level of difficulty in routine clinical application. To make it user friendly, this series of tutorials has been planned. In this pictorial, we aim to provide an illustrated presentation of the Banff classification categories and practical tips and tricks to identify and report the lesions. This will be useful for better understanding of renal transplant pathology for trainees and residents of nephrology and histopathology as well as for practicing pathologists and nephrologists. Banff category 4. Acute rejection (Acute/active T-cell-mediated rejection [TCMR])-Part II (Plasma cell-rich acute rejection)The main lesion of acute/active T-cell-mediated rejection (TCMR) was discussed in the previous issue of PJKD.In this issue, a special type or variant of active rejection, i.e., plasma cell-rich acute/active rejection (PCAR) is discussed. Although, it is not included as a formal category in the synoptic table of the Banff classification, it is discussed in text in Banff classifications under the TCMR category. Herein, we discuss this entity along with a few representative images and explanation of the lesions.