Focal Segmental Proliferative Glomerulonephritis Research Articles

Glomerulonephritis Following Russell’s Viper Bite – 1st Case Report in Bangladesh

Russell’s viper bite causing coagulopathy, hypotension, DIC, rhabdomyolysis and acute kidney injury. Nephropathy usually is caused by RV having hemotoxic or myotoxic venom components. Multiple mechanism causes renal pathologic changes including tubular necrosis, cortical necrosis, interstitial nephritis, and glomerulonephritis. But above all glomerulonephritis is very rare. We report a case of Russell’s viper bite who died of renal failure due to focal segmental proliferative glomerulonephritis admitted int the Rajshahi Medical College Hospital, Rajshahi, Bangladesh.
 TAJ 2022; 35: No-2: 169-172

Histopathological Pattern of Glomerulonephritis: Experience from BIRDEM General Hospital, Dhaka, Bangladesh

Background: Glomerulonephritis (GN) remains the most probable underlying cause of end stage renal disease of uncertain aetiology in many developing countries including Bangladesh. The pattern of glomerular disease varies widely from country to country. In Bangladesh, the incidence and histological pattern of GN is inadequately described. We performed a study, aiming to determine the pattern of GN in a diabetic hospital of our country.Methods: It was a cross-sectional hospital based prospective study conducted at BIRDEM General Hospital starting from July 2013 to December 2014. It included all patients with suspected GN who underwent native kidney biopsy.Results: Total 57 biopsies were performed and four cases other than primary or secondary GN (renal cortical necrosis 1, tubulointertsitial nephritis 2,chronic GN 1) were excluded i.e total number of PGN was 37 and secondary GN was 16. number of patients with were 53. M:F was 1.2:1.Mean age was 42.35±15(14-72) years. Thirty one (58.49%) of the study subjects had diabetes mellitus (DM). Mesangialproliferative GN (15/37,40.5%) and diabetic nephropathy (9/16,56%) were the commonest histopathological pattern found among primary and secondary GN respectively. Membranoproliferative GN (10/37,27%),was the second commonly observed pattern followed by focal segmental proliferative GN (8%), membranous nephropathy (8%), focal segmental glomerulosclerosis (5.4%) in primary GN and lupus nephritis (6/16,38%) and Wegeners granulomatosis (1/16) were other varieties in secondary group. Among 53 cases, 37 had proliferative variety. Nephrotic range proteinuria (41.5%) was the commonest indication of biopsy and 22% had post biopsy bleeding and 3.7% required blood transfusion.Conclusion: In conclusion, mesangial proliferative and membranoproliferative GN are the two common causes of primary GN. Diabetic nephropathy is the commonest cause of secondary GN. Nephrotic range proteinuria was the main indication of biopsy. Post biopsy complication was negligible. Creation of a national renal registry is essential for obtaining more specific epidemiological data.Birdem Med J 2016; 6(2): 67-73

Relationship between Fractional Excretion of Magnesium and Renal Histology in Glomerulonephritis

Progressive pediatric glomerularnephroses can lead to tubulointerstitial fibrosis (TIF) and chronic kidney disease (CKD). Fractional excretion of magnesium (FEMg) is a sensitive tubular function test (Ref). FEMg correlates positively with TIF (Ref). The purpose of the study was to correlate FEMg with renal histolopathology in pediatric glomerular nephrosis (GN). Sixty three cases, between 1-12 years, of biopsy diagnosed GN were included purposively in this cross sectional study from November 2008 to April 2010. Cases were taken from Department of Paediatric Nephrology, Dhaka Shishu (Children) Hospital and Department of Paediatric Nephrology, National Institute of Kidney Diseases & Urology, Sher-e-Bangla Nagar, Dhaka, Bangladesh. Minimal change disease (MCD) was most common at 27% and remaining 73% were other than MCD. The mean FEMg in MCD was normal (1.81 0.34%) but in other than MCD as a group it was significantly elevated (7.60 ± 19.23%; p<0.001). and significantly elevated separately in diffuse mesangial proliferative glomerulonephritis (3.96 ± 1.06%; p<0.001), focal segmental glomerulosclerosis (9.99± 4.45%; p<0.01), focal segmental proliferative glomerulonephritis (4.63 ± 2.29%; p<0.05) and crescentic glomerulonephritis (17.74 4.93%; p<0.01).

Correlation of fractional excretion of magnesium with steroid responsiveness in children with nephrotic syndrome.

Steroid-resistant nephrotic syndrome (SRNS) patients are candidates for other alter-native drug regimes, and the non-responsiveness to steroid is more common among glomerulo-nephritides other than minimal change disease. Without performing biopsy and proper renal histology, progression of the disease cannot be assessed. Fractional excretion of magnesium (FE Mg) has been found to correlate directly with various renal histologies. The aim of this study is to evaluate the relationship of FE Mg in children with the histological pattern in SRNS. In this prospective observational study, 40 children of nephrotic syndrome, both with the first episode as well as relapse, aged 1-12 years were included in the study. Of them, 20 were steroid-responsive cases and 20 were steroid-resistant cases. FE Mg was determined in all the patients and renal histology was performed in the steroid-resistant cases. A correlation was found between FE Mg and renal histology. Data were analyzed in SPSS program version-16. Comparison of two groups was performed by the Fisher exact test and unpaired t test. P-value less than 0.05 were considered to be significant. The results of histo-pathology showed that the mean difference in FE Mg was significant (P <0.001), as FE Mg was 7.0 ± 2.3% in mesangiocapillary glomerulonephritis, 6.9 ± 1.3% in focal segmental glomerulosclerosis, 4.7 ± 0.6% in immunoglobulin M nephropathy, 4.5 ± 1.2% in focal segmental proliferative glomerulo-nephritis, 4.4 ± 1.6% in minimal change disease, 4.2 ± 0.4% in diffuse mesangial proliferative glome-rulonephritis and 3.8 ± 1.3% in mesangial proliferative glomerulonephritis. There was a statistically significant difference between FE Mg in steroid-resistant nephrotic syndrome (4.9 ± 1.9) and steroid-responsive syndrome (1.2 ± 0.3). FE Mg is a simple, minimally invasive screening marker for SRNS, and is an early predictor of clinical outcome. It can be considered as an initial investigation where biopsy cannot be performed or indications are not clear.

Acute glomerulonephritis in a donor as a side effect of allogeneic peripheral blood stem cell mobilization with granulocyte colony-stimulating factor

Following on from the recently published articles reported side effects occurring due to donation of stem cells, we describe a case of a donor with transient, biopsy-proved acute focal segmental proliferative glomerulonephritis (GN) due to peripheral blood stem cells (PBSC) mobilization with granulocyte colony-stimulating factor (G-CSF). A 44-year-old woman with no relevant past medical history suffering from obesity and hypertension well controlled with metoprolol without hypertensive retinopathy was admitted to our hospital as a donor of PBSC. She received G-CSF subcutaneously-filgrastim (Amgen)-at a dose of 5 microg/kg twice a day for 6 days. The macroscopic hematuria and proteinuria occurred on 5th day of G-CSF administration. Due to mobilization and collection of stem cells, proteinuria was becoming more intense and reached the nephrotic range. The immunological, infectious, urological and gynecological causes of such complication were excluded. The final histological recognition was early stage of focal segmental proliferative GN. To our knowledge this a first report of GN in a donor due to mobilization of PBSC confirmed with renal biopsy. These findings suggest that filgrastim may induce transient urinary excretion of protein and hematuria in PBSC donors as the symptoms of acute GN without adversely affecting renal function.

Histomorphometric study in children with idiopathic nephrotic syndrome.

From 1981 to 1987, renal specimens obtained from 91 children with idiopathic nephrotic syndrome were categorized into 5 histologic subgroups, according to the WHO classification. We analyzed differences in the ratios of the size of the mesangial stalks and attached capillary, including the Bowman's space between the capillary tufts (E), mesangial matrix (Mx), and approximate capillary lumens (E-Mx) to the whole glomerular area of these subgroups using the histomorphometer-IBAS. Focal segmental glomerulosclerosis with diffuse mesangial proliferation (FSGS + DP) had the largest matrix area (Mx/G > 0.5), and smallest capillary lumen size (E/G-Mx/G) (0.3474 +/- 0.0702). On the contrary, FSGS with minimal change (FSGS + MC) had the smallest mesangial matrix (Mx/G), 0.2834 +/- 0.07726, but preserved a larger capillary lumen (0.427 +/- 0.1215). The approximate size of capillary lumens, from the smallest to the largest, was in the following order: FSGS + DP, diffuse mesangial proliferative glomerulonephritis (DPGN), focal segmental proliferative glomerulonephritis (FSPGN), FSGS + MC and minimal change nephrotic syndrome (MCNS). The ratio of the total number of mesangial cells to the area of the whole glomerulus (MCN/G) derived from the light microscopic examination was parallel to the value of Mx/G obtained by the histomorphometric technique. In summary, we introduced the histomorphometric technique using the histomorphometer-IBAS for quantitative measurement of various areas in the glomeruli. The data derived from the system is compatible with those obtained by experienced nephrologists, suggesting that the histomorphometric technique is helpful in histopathology. It is hoped that this new methodology will be used more extensively in the near future.

Paulista registry of glomerulonephritis: 5-year data report

The Paulista Registry of Glomerulopathies was created in May 1999 and comprises several centres of São Paulo, the most populous Brazilian State, that concentrates people from all regions of the country who look for health care. This report includes data from 2086 patients from Brazil submitted to renal biopsy due to the presumed diagnosis of glomerular diseases, registered prospectively since May 1999 until January 2005. Data were collected by the integrants of the 11 centres involved, utilizing a standardized questionnaire. The mean age of the patients was 34.5+/-14.6 years. Primary glomerular diseases were more frequent in males (55.1%) than in females; on the other hand, secondary glomerular diseases were more frequent in females (71.8%). The most common clinical presentation was nephrotic syndrome and the frequency of hypertension, at this time, was 55.5%. There was a predominance of indication of biopsies in the third, fourth and fifth decades of life. The most common primary glomerular diseases were focal and segmental glomerulosclerosis (29.7%), followed by membranous nephropathy (20.7%), IgA nephropathy (17.8%), minimal change disease (9.1%), membranoproliferative glomerulonephritis (7%), crescentic glomerulonephritis (4.1%), advanced chronic glomerulopathy (4%), non-IgA mesangial glomerulonephritis (3.8%), diffuse proliferative glomerulonephritis (2.5%), focal segmental proliferative glomerulonephritis (1%) and others (0.3%). The most frequent secondary glomerular disease was lupus nephritis, corresponding to 66.2% of the cases, followed by post-infectious glomerulonephritis (12.5%), diabetic nephropathy (6.2%), diseases associated to paraproteinaemia (4.9%), hereditary diseases (4.6%), vasculitis (3.2%), malignancies (0.9.%), secondary focal segmental glomerulosclerosis (0.6%) and others (0.9%). Focal segmental glomerulosclerosis was the most frequent primary glomerular disease, followed by membranous nephropathy and IgA nephropathy. Lupus nephritis predominated over all the other secondary glomerular diseases.

Antineutrophil Cytoplasmic Antibody–Associated Glomerulonephritis in Chronic Graft-Versus-Host Disease After Allogenic Hematopoietic Stem Cell Transplantation

Graft-versus-host disease (GVHD) is one of the most frequent complications that occur after hematopoietic stem cell transplantation (HSCT). Recently, renal involvement, including membranous nephropathy, focal segmental glomerulosclerosis, and minimal change disease, has been described as a manifestation of chronic GVHD. This case report describes a patient who developed antineutrophil cytoplasmic antibody (ANCA)–associated glomerulonephritis after HSCT. Following preparation with chemotherapy, a 29-year-old man with chronic myeloid leukemia underwent allogenic peripheral blood stem cell (PBSC) transplantation, after which first acute and then chronic GVHD developed. Treatment with prednisone resulted in improvement in the patient’s GVHD. After the termination of steroid therapy and about 10 months after PBSC transplantation, nephritic syndrome appeared and the patient’s serum creatinine value increased to 1.7 mg/dL. Laboratory evaluation revealed perinuclear antineutrophilic cytoplasmic antibody (p-ANCA) in the serum. Histological examination of renal biopsy tissue showed focal segmental proliferative glomerulonephritis with glomerulosclerosis in 20% of available glomeruli, large cellular crescents in 6% of glomeruli, and no staining of immunoglobulins or complement along the capillary walls. Electron microscopy revealed no immune deposits. After treatment with prednisone 60 mg/d, diltiazem 120 mg/d, and enalapril 10 mg/d, the proteinuria gradually decreased, and p-ANCA was undetectable. These findings suggest that in this patient the ANCA-associated glomerulonephritis was associated with renal involvement that occurred during the course of chronic GVHD.

The Prognosis of Lupus Nephritis in African-Americans: A Retrospective Analysis

To fully describe the clinical course of lupus nephritis in an African-American population, we report our experience with 54 patients seen at a large inner-city hospital over a period of 14 years. The patients were divided into five histopathologic groups. Group MES (n = 3) represented mesangial nephritis (World Health Organization [WHO] class II) and group FOC (n = 11) represented mild and moderate focal segmental proliferative glomerulonephritis (WHO class III). Group DIF (n = 9) included patients with severe segmental proliferative, diffuse proliferative, membranoproliferative, and membranous and severe superimposed proliferative lesions (WHO classes III, IV, and Vd). Group CRES (n = 9) combined all the patients with cellular crescents in more than 40% of the glomeruli and included patients in WHO classes III (severe), IV, and Vc and d. Group MEM (n = 22) represented membranous nephritis occurring alone or with superimposed mesangial or mild segmental proliferative lesions (WHO class Va and b). Groups DIF and CRES received intensive treatment with high-dose prednisone and cytotoxic drugs. Groups FOC and MEM received lower doses of prednisone, but half of the patients later received intensive treatment largely for severe systemic manifestations. The three patients in group MES remained well. End-stage renal failure (ESRF) developed in 11 of 18 patients in groups DIF and CRES combined, and in two of 22 patients in group MEM. Three of 11 patients in group FOC, five in groups DIF and CRES, and one in group MEM died. The actuarial 5- and 10-year survival rates were, respectively, 78% and 78% for FOC, 80% and 0% for DIF and CRES, and 100% and 100% for MEM (P < 0.03 v DIF/CRES). Five- and 10-year survival rates without ESRF were, respectively, 78% and 78% for FOC, 52% and 0% for DIF and CRES (P < 0.05), and 94% and 85% for MEM (P = 0.002 v DIF/CRES). Univariate proportional hazards regression analysis, uncontrolled for histopathologic groups, showed a significant association between ESRF and severe thrombocytopenia (P = 0.003), serum creatinine above 1.4 mg/dL at entry (P = 0.04), and severe systemic manifestations (P = 0.05). After controlling for histopathologic groups, only thrombocytopenia remained strongly associated with ESRF, both by univariate (P = 0.01) and multivariate (hazard ratio = 14.19, P = 0.05) analyses. We conclude that severe proliferative lupus nephritis in African-Americans has a poor prognosis. For mild and moderate focal proliferative nephritis and uncomplicated membranous lupus nephritis the prognosis is as good as in white patients. Severe thrombocytopenia predicts ESRF.

Autoantibodies to leucocyte antigens in hydralazine-associated nephritis.

Clinical and laboratory findings and drug history were studied in 17 patients with suspected hydralazine-associated nephritis, five of whom only had renal disease, while twelve also had extrarenal manifestations. Renal biopsies revealed extracapillary proliferative or focal segmental proliferative glomerulonephritis in 10 patients, and tubulo-interstitial nephritis in five patients. Antinuclear antibody (ANA) was found in 16 patients, but none of the 14 patients tested had antibodies to DNA. Tests for antibodies to myeloperoxidase (anti-MPO) and antibodies to neutrophil cytoplasm antigen (ANCA) were performed by ELISA. Twelve of the 14 patients tested had anti-MPO; five of these 14 patients had ANCA, while one had borderline levels. These findings suggest that hydralazine facilitates the induction of a systemic disease with multiple autoantibody production.

Glomerular Nephropathy Associated With Chronic Q Fever

Of three patients with Coxiella burnetii endocarditis, two developed focal segmental proliferative glomerulonephritis (GN), and the third developed diffuse intracapillary proliferative glomerulonephritis. In one case, a good therapeutic response was followed by partial remission of the renal alterations, but 10 months later there were clinical and histological signs of active glomerular nephropathy, suggesting that the antigenic stimulus persisted. In another case, poor evolution of the infection was accompanied by clinically and histologically aggressive glomerular nephropathy, and advanced renal failure. The third patient, who had diffuse proliferative glomerulonephritis, underwent renal biopsy earlier than the other two cases, and the behavior of the nephropathy has not been aggressive to date. Immunohistopathologic study revealed a diffuse granular deposit of IgM and C3 in all three cases; the first two also presented a discrete linear IgG deposit in the capillary loops. Attempts to identify C burnetii antigen at the glomerular level by immunohistologic techniques failed in two patients. The literature on the association of chronic Q fever with glomerulonephritis is briefly reviewed.