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Background: There is no study available from Pakistan on the longitudinal course of membranous nephropathy (MN). We aimed to analyse the clinicopathological profile, course, response to treatment and outcome of the disease in our setup. Methods: All consecutive adult patients (≥18 years) with MN on renal biopsy and on regular follow-up were included. Relevant data items were retrieved from case files and biopsy reports. The outcome variables included doubling of baseline serum creatinine or the development of end-stage renal disease (ESRD). Results: Of a total of 102 patients, 82 (80.4%) were males; mean age was 28.9±11.4 years. Peripheral oedema was present in 86 (84.3%), nephrotic-range proteinuria in 80 (78.4%), microscopic haematuria in 25 (24.5%), hypertension in 42 (41.2%), and renal dysfunction in 13 (12.7%) patients at the time of presentation. The mean follow-up period was 3±2 years. Overall, 22 (21.5%) subjects went into remission and a similar number experienced doubling of serum creatinine. Both conservative and immunosuppressive treatment groups had similar duration of follow-up. The proportion of patients with remission and doubling of serum creatinine was similar between the two groups (p=0.70 and p=0.91 respectively), while the proportion of patients progressing to ESRD was significantly higher in the group treated conservatively (p=0.03). Baseline proteinuria and serum creatinine were predictive of remission (p=0.04 and p=0.05 respectively). Conclusions: In conclusion, untreated MN has a higher risk of progression to ESRD. Baseline proteinuria and serum creatinine predict response to treatment. Randomised controlled trials are needed to confirm the effects of immunosuppressive treatment

Background: The lesions resembling focal segmental glomerulosclerosis (FSGS) are frequently found concurrently with IgA nephropathy (IgAN). However, there is controversy regarding the significance of this co -existence. In this study, we sought to determine the significance of FSGS-like lesions in a group of IgAN patients, especially with regard to the Oxford classification. Patients and Methods: The FSGS lesions were typed according to the Columbia classification and correlated with clinico -pathological parameters including Oxford classification criteria. Individual lesions suggesting FSGS were also evaluated. IgAN lesions were classified according to the Oxford classification. Results: Of 114 patients, 80 (70.2 %) were male. The mean age of patients was 37.7 �± 13.6 years. Of 35 patients with co-existent IgAN and FSGS, 50% had classic variant (not otherwise specified; NOS), 30% had tip and 20% perihilar variant of FSGS. None of the patients had collapsing or cellular variants. A significantly positive association of FSGS with M (p = 0.001), S (p = 0.001) and T p = 0.001) variables of Oxford classification was found. No significant difference of FSGS, hyalinosis, capsular adhesions and podocytopathy was found between males and females (p > 0.05). However, a significantly positive association of proteinuria (p = 0.037) and renal dysfunction (p = 0.026) with podocytopathy, and of serum creatinine and renal dysfunction with the presence of FSGS (p = 0.027, p = 0.001, respectively) was seen. Conclusion: In conclusion, the concurrence of FSGS -like lesions with IgAN is associated with the established poor clinical and pathological prognostic factors of this disease and the lesions appear to be of prognostic significance. The presence of such lesions should be sought diligently on renal biopsy examination.

Galloway-Mowat syndrome is a rare hereditary disorder originally described as a triad of early-onset nephrotic syndrome, microcephaly, and hiatus hernia. Subsequent reports have expanded the clinical and pathological spectrum of the disorder. We describe a patient with this syndrome, a 14-month-old girl with infantile nephrotic syndrome having a protein to creatinine ratio of 6.1, microcephaly, low-set ears, hypertelorism, beaked nose, narrow-forehead, almond-shaped eyes, arachnodactyly, pinpoint pupils and myopia. Renal biopsy revealed diffuse mesangial sclerosis and focal microcystic dilatation of the tubules and magnetic resonance imaging of the brain showed diffuse cortical atrophy. The above constellation of features favours the diagnosis of our case as Galloway-Mowat syndrome

Dual positive cases of anti-glomerular basement membrane (GBM) antibody and anti-neutrophil cytoplasmic antibody (ANCA) associated nephritis are not rare in clinical nephrology practice. These patients often present with a clinical picture of rapidly progressive GN (RPGN). Published studies indicate that up to 30% of patients with anti-GBM antibody nephritis have associated ANCA antibodies in their sera. On the other hand, only up to 5% of ANCA-associated GN patients have anti-GBM antibodies. There are conflicting reports in the literature on the significance of double positive antibodies on the presentation, clinical course and outcome of these patients. It is possible that the predominant behaviour depends on the predominance or time of appearance of either antibody. However, all studies conclude that the recovery of renal functions is rare with dual positivity of the above antibodies. We herein present a case of a middle-aged lady presenting with non-specific features of renal failure and was diagnosed to have crescentic GN on renal biopsy and both anti-GBM and ANCA antibodies in the serum. We discuss the management of this case in the light of existing literature on this subject

Currently, the most challenging problem in the field of renal and other solid organ transplantation is the development of chronic progressive sclerosing changes in the allograft. These occur almost uniformly in all renal allografts at a rate of 2-4% per year. In addition, such changes are also quite prevalent in well functioning grafts, as revealed by protocol biopsies. The chronic changes involve all the four components of the renal graft parenchyma, i.e., the glomeruli, blood vessels, tubules and interstitium. Among these, the glomerular and vascular changes are helpful in defining the causes of chronic changes, especially chronic rejection, but are more prone to sampling error, notably the blood vessels, whereas the tubulo-interstitial changes are less specific. However, because the later are less prone to sampling error, these are used for grading the severity of chronic changes in the Banff formulation. The identification, classification and grading of not only the acute but also the chronic changes is of vital importance in guiding the management and predicting the long-term graft outcome of renal transplant recipients. Banff has addressed the issue of chronic changes in detail in its first formulation, as well as its subsequent modifications. However, the magnitude of changes in this category that have occurred over the last two decades is less than that observed in the categories of antibody-mediated and T-cell-mediated rejections. This review describes in detail the changes that have taken place in the category of chronic allograft damage, as the original Banff classification has undergone updates regularly in the last two decades

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder which commonly affects kidneys.Directory of Open Access Journals (DOAJ), Google Scholar, PubMed (NLM), LISTA (EBSCO) and Web of Science have been searched.There is sufficient epidemiological, clinical and histopathological evidence to show that antiphospholipid syndrome is a distinctive lesion caused by antiphospholipid antibodies in patients with different forms of antiphospholipid syndrome. It is now time to devise a classification for an accurate diagnosis and prognostication of the disease.Now that the morphological lesions of APSN are sufficiently well characterized, it is prime time to devise a classification which is of diagnostic and prognostic utility in this disease.

The allo-specific immune responses to transplanted tissues or organs represent one of the most formidable challenges in the field of transplantation. Traditionally, cell-mediated alloimmune responses were considered the preeminent cause of rejection and have remained the focus of immunosuppressive drugs during the last several decades. More recently, attention has also been directed to the alloantibody-mediated damage and the innate immune system in initiating and effecting the immune injury to the transplanted organs. As a corollary to the above considerations, the earlier Banff classifications focused more on diagnosing and categorizing cellular rejection. There have occurred some significant changes in the cell-mediated rejection in recent Banff updates, but the changes are not as drastic or widespread, as those of the antibody-mediated rejection. Acute/active cell-mediated rejection may occur early or considerably late after renal transplantation. In this context, the terms of acute and chronic are not synonymous to their traditional connotations in pathology in terms of speed or duration of reaction. Acute rejection may occur many years after transplantation, and conversely, chronic changes may be present in the graft from the outset, derived from donor changes. Acute/active cell-mediated rejection, although markedly reduced in recent years, still remains one of the common causes of both acute and chronic renal allograft injury and dysfunction throughout the world. Luckily, a vast majority of cases of acute/active cell-mediated rejection respond rapidly and completely to the conventional anti-rejection treatment. In spite of this, it remains one of the most important causes of graft loss, especially in the long-term. Renal allograft biopsy still remains the gold standard test for an accurate diagnosis and categorization of cell-mediated rejection. A standardized approach to renal biopsy study is necessary if the full benefits of this invasive procedure are to be realized. Prior to the early 1990s, there were no uniformly accepted criteria for the diagnosis and classification of renal allograft pathology in general and rejection in particular. During early 1990s, a group of dedicated nephropathologists, clinicians and basic scientists set out to standardize the histopathological study of renal allograft biopsies for the uniform reporting of the pathological lesions across the world. These efforts have continued since then and have resulted in marked refinements in the diagnostic criteria and categories of rejection observed on renal allograft biopsies. The present paper forms the second attempt of the series to address the evolutionary changes in the diagnostic criteria and the classification of the rejection process on renal allograft biopsies as these took place over the years since the early 1990s. An earlier paper described in detail the changes that occurred in the category of antibody-mediated rejection. In this paper, we will discuss the changes that have occurred in the diagnosis and categorization of cell-mediated rejection and the focus, as in previous paper, will be on the morphological findings as observed on renal allograft biopsies.

Objective: To evaluate the contribution of electron microscopy (EM) to the accurate diagnosis of glomerulopathies in childhood nephrotic syndrome (NS) in a developing country. Methods: The study was carried out at the Histopathology Department, Sindh Institute of Urology and Transplantation (SIUT) from April 2007 to March 2008. All children (≤18 years) presenting with NS were included. Patients' demographic, clinical, laboratory, and biopsy data were retrieved from case records and biopsy reports. Renal biopsies were studied by light microscopy, immunoflourescence, and EM. Results: The mean age of 74 children was 11.34±4.85 years. EM was useful in 97.2% of cases, being essential in 31% and helpful in 66.2% cases. Conclusion: The results demonstrate that the ultrastructural study is both helpful and essential to a correct classification of glomerular diseases underlying NS in children in nearly all cases and whenever feasible this should be used in the pathologic evaluation of renal biopsies. Objetivo: Evaluar la contribución de la microscopía electrónica (ME) al diagnóstico preciso de las glomerulopatías en el síndrome nefrótico (SN) infantil en un país en vías de desarrollo. Método: El estudio se realizó en el Departamento de Histopatología del Instituto Sindh de Urología y de Trasplante (SIUT) desde abril de 2007 a marzo de 2008. En él se incluyeron todos los niños (≤ 18 años) que presentaban SN. Los datos demográficos, clínicos, de laboratorio y de la biopsia de los pacientes se obtuvieron a partir de expedientes de casos e informes de la biopsia. Las biopsias renales se estudiaron mediante microscopía de luz, inmunofluorescencia y ME. Resultados: La media de edad de 74 niños era de 11,34 ± 4,85 años. La ME resultó útil en el 97,2 % de los casos, siendo esencial en el 31 % y constituyendo una ayuda en el 66,2 % de los casos. Conclusión: Los resultados demuestran que el estudio ultraestructural es de utilidad y resulta esencial para la correcta clasificación de las enfermedades glomerulares subyacentes al SN infantil en casi todos los casos; por lo tanto, debería utilizarse en la evaluación patológica de biopsias renales siempre que fuera posible.

Objective: To evaluate the contribution of electron microscopy (EM) to the accurate diagnosis of glomerulopathies in childhood nephrotic syndrome (NS) in a developing country. Methods: The study was carried out at the Histopathology Department, Sindh Institute of Urology and Transplantation (SIUT) from April 2007 to March 2008. All children (≤18 years) presenting with NS were included. Patients' demographic, clinical, laboratory, and biopsy data were retrieved from case records and biopsy reports. Renal biopsies were studied by light microscopy, immunoflourescence, and EM. Results: The mean age of 74 children was 11.34±4.85 years. EM was useful in 97.2% of cases, being essential in 31% and helpful in 66.2% cases. Conclusion: The results demonstrate that the ultrastructural study is both helpful and essential to a correct classification of glomerular diseases underlying NS in children in nearly all cases and whenever feasible this should be used in the pathologic evaluation of renal biopsies.

Collapsing Glomerulopathy: The Expanding Etiologic Spectrum of a Shrinking Glomerular Lesion