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Glomerular diseases continue to be the leading cause of end-stage renal disease globally. Hence it is important to recognize the glomerular disease pattern in any given geographical area to understand the pathobiology in the region as well as the incidence and progression of the disorder. A total of 498 renal biopsies were performed on patients with proteinuria, hematuria, and mild to moderate renal impairment during a period of 13 years (between January 1990 and December 2002) at a tertiary care hospital. Primary glomerular disease accounted for two-thirds of the glomerular diseases, which was 44.8% of all renal biopsies. The most common histological lesion was minimal change disease (30%). Focal segmental glomerulosclerosis was the second most common lesion (23.8%) followed by membranoproliferative glomerulonephritis (14.3%). Secondary glomerular disease included 33.6% of glomerular diseases with 22.7% with lupus nephritis as the commonest lesion (38.9%) followed by diabetic nephropathy (31.9%) and hypertension (20.4%). Tubulointerstitial diseases accounted for 13.1% of all renal biopsies, whereas transplant diseases were noted in 12.2%. The miscellaneous group including inadequate biopsies, which constituted 7.2% of all the tissues. The results of this analysis were compared with surveys from other parts of the world.
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PMID:The spectrum of glomerular diseases in the kingdom of bahrain: an epidemiological study based on renal biopsy interpretation. 1535 Apr 79

Focal and segmental glomerulosclerosis (FSGS) is a pathologic entity that is a common and increasing cause of end-stage renal disease. Typical manifestations include proteinuria, hypertension, worsening renal insufficiency, and, frequently, renal failure. The etiology, however, remains unknown in a majority of patients. There is an estimated recurrence rate of 30% to 40% in renal transplant patients, suggesting that the pathogenesis is not solely a result of intrinsic kidney disease. Although some of its characteristics have been reported, the precise identification of a circulating factor associated with FSGS has not been made. Remarkable progress has been made in recent years regarding biologic mechanisms surrounding FSGS and proteinuria. Insight into the pathogenesis of FSGS has been gained through the study of hereditary forms of FSGS and nephrotic syndromes. Mutations in cytoskeletal proteins that affect podocyte structure have been the target until recently. Here we review the current understanding of this glomerular disease and areas for future concentration.
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PMID:Focal and segmental glomerulosclerosis: varying biologic mechanisms underlie a final histopathologic end point. 1653 Jun 1

Hepatitis B virus (HBV)-associated glomerulonephropathy (HBV-GN) has been increasingly reported, especially in adults. In the present study, the authors investigated the clinical and histopathology features of patients who suspected HBV-GN in 24 patients and age ranging from 23 to 74 years (mean 43 years). Asymptomatic hematuria was the most common presentation (54%); followed by edema and hypertension at equal percentages of 50%. The nephrotic syndrome was presented in 43%, the nephrito-nephrotic syndrome in 3.5%. Clinically suspected rapidly progressive GN was found in 14%. Renal insufficiency was determined in 30%. The most common pathologic finding was IgA nephropathy (IgAN 29%), followed by membranous nephropathy (21%), focal segmental glomerulo sclerosis (FSGS 11%), membranoproliferative GN (11%), post-infectious GN (11%). Liver disease activity also tended to be mild or had no symptoms of hepatitis. The authors remission rates both complete and partial were 75% (higher than the usual report), notwithstanding treatment. The authors achieved a sustained complete remission in half of the patients (3 in 6 cases) treated with steroid alone and 2 out of 7 cases (28.6%) treated with anti-viral therapy. Spontaneous remission was demonstrated in 2 (1 with IgAN + FSGS, and 1 with post infectious GN) out of 6 patients (33.3%). None of the patients in both treatment groups turned to ESRD that occurred in 2 cases receiving non-specific treatment. Of note, all of the patients who received anti-viral therapy or corticosteroid and had complete follow up were in remission either complete or partial.
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PMID:Hepatitis-B virus-associated nephropathies in adults: a clinical study in Thailand. 1704 67

HIV-associated nephropathy (HIVAN) is characterized by a collapsed glomerular capillary tuft with hyperplasia and hypertrophy of podocytes. Recently generated were conditional transgenic mice (podocin/Vpr) that express one of the HIV-1 accessory genes, vpr, selectively in podocytes using podocin promoter and Tet-on system. These transgenic mice developed renal injury similar to HIVAN when treated with doxycycline for 8 to 12 wk. This study demonstrated that nephron reduction by heminephrectomy markedly enhanced phenotypic changes of podocytes and led to severe FSGS within 4 wk. Nephrotic-range proteinuria was observed already at 2 wk, together with dedifferentiation and dysregulation of podocytes, indicated by decreased expression of nephrin, synaptopodin, and Wilms' tumor 1 protein and increased expression of Ki-67. The acceleration of phenotypic changes of podocytes, proteinuria, and subsequent glomerulosclerosis by heminephrectomy was almost completely inhibited by angiotensin II type 1 receptor (AT1R) blocker olmesartan. In contrast, the renoprotective effect of the calcium channel antagonist azelnidipine was minimal, although it lowered systemic BP to the same level as olmesartan, demonstrating that the inhibitory effect of AT1R blocker was independent of systemic BP. Olmesartan also reduced proteinuria and prevented glomerulosclerosis even by the delayed treatment, which was initiated after the podocyte injury appeared. These data suggest that nephron reduction exaggerates podocyte injury and subsequent glomerulosclerosis, possibly through glomerular hypertension, in the mouse model of HIVAN. AT1R blockade could be beneficial in the treatment of HIVAN by ameliorating podocyte injury by avoiding the vicious cycle of nephron reduction and glomerular hypertension.
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PMID:Angiotensin II type 1 receptor blockade inhibits the development and progression of HIV-associated nephropathy in a mouse model. 1722 13

Focal segmental glomerulosclerosis is a form of chronic kidney disease that causes 3.6% of end stage renal disease cases. While the cause of this disease is multifactoral, the Human Genome Project has identified some specific chromosome sites linked to this disease process. Treatment for focal segmental glomerulosclerosis may include corticosteroids, managing hypertension, and controlling proteinuria. This article discusses the pathophysiology of focal segmental glomerulosclerosis, treatments, and resources for patient and professional education.
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PMID:Focal segmental glomerulosclerosis: a case study with review of pathophysiology. 1748 48

Familial hypercholesterolemia (FH) is a common autosomal dominant inherited disorder characterized by increased levels of circulating plasma low-density lipoprotein cholesterol (LDL-C), tendon xanthomas, and premature atherosclerotic cardiovascular disease. Homozygous FH occurs in only one in a million people. Focal segmental glomerulosclerosis (FSGS) is clinically characterized by proteinuria, which is marked in the majority of cases and accompanied by nephrotic syndrome, high incidence of hypertension, and progression to renal failure. To our knowledge, we herein report for the first time a case of homozygous FH associated with FSGS. A seven-and-a-half-year-old boy was referred to our hospital due to cutaneous xanthomata and growth retardation. He had multiple nodular yellowish cutaneous xanthomatous lesions each 1 cm in size over his knees and sacral region. Laboratory data included cholesterol level of 1,050 mg/dl, low density lipoprotein cholesterol (LDL-C) 951 mg/dl, high-density lipoprotein cholesterol (HDL-C) 29 mg/dl, triglycerides 168 mg/dl, total protein 6.3 g/dl, and albumin 3.2 g/dl. Urinary protein excretion was 78 mg/m(2) per hour. A percutaneous renal biopsy was performed, and histological findings showed FSGS. Treatment with cholestyramine and atorvastatin was unsuccessful in terms of lowering lipids, and he was placed on weekly sessions of plasmapheresis. Total cholesterol was reduced from 1,050 mg/dl to 223 mg/dl, LDL-C from 951 mg/dl to 171 mg/dl, and urinary protein excretion from 78 mg/m(2) per hour to 42 mg/m(2) per hour after eight sessions of plasmapheresis. It is our belief that plasmapheresis is a treatment of choice in patients with FSGS associated with FH.
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PMID:A case of homozygous familial hypercholesterolemia with focal segmental glomerulosclerosis. 1763 41

Glomerulonephritis (GN) is a major cause of chronic renal failure (CRF). To evaluate the trends and outcome with modern improved treatment strategies, we retrospectively reviewed the clinical records of 120 patients with biopsy proven primary GN at our center from January 1990 to June 2001. All the biopsy specimens were subjected to light, electron and immunofluorescent microscopy. The recorded clinical parameters included the presenting symptoms, blood pressure readings, complete blood count, urinalysis, 24-hr urinary protein excretion, creatinine clearance besides rendered therapy and the outcome. Focal segmental glomerulosclerosis was the most common GN and accounted for 56 (47.6%) cases. The frequency of other GN cases in our study included IgA GN in 21 (17.5%) patients, membranous GN in 20 (16.7%), minimal change disease (MCD) in 13 (10.8%), membranoproliferative GN in 4 (3.3%), post infection in 4 (3.3%) and rapidly progressive glomerulonephritis (RPGN) in 2 (1.7%). The type of nephropathy had great influence on outcome and response to therapy. The deterioration of patients with FSGS was the fastest of the glomerulopathies, and nine (16.1%) patients developed end-stage renal failure (ESRD). MCD and post infection GN had the best outcome. Corticosteroids alone along with supportive medication conferred good results in MCD, while combined therapies of mycophenolate mofetil (MMF) and/or cyclophosphamide with corticosteroids provided better outcomes in the rest of the GN. RPGN responded well to the cyclophosphamide and the patients did not develop ESRD. Hyperuricemia, high serum creatinine and hypertension predicted worse outcomes. The control of blood pressure and glucose, and treatment of hyperuricemia and hypoalbuminemia had salutary effect on the outcome. We conclude that due to the better delivered care the outcome of primary GN has improved over the years. However, FSGS is still the most frequently encountered primary GN and has the worst outcome. In the present study, combined therapies with corticosteroids and cytotoxic drugs and supportive therapy were associated with better outcome.
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PMID:Spectrum and outcome of primary glomerulonephritis. 1764 79

Glomerular diseases continue to be the leading cause of end-stage renal disease globally. Hence, it is important to recognize the pattern of these diseases in any given geographical area. A total of 498 renal biopsies performed on patients with proteinuria, hematuria and mild to moderate renal impairment during a period of 13 years (between January 1990 and December 2002) at the Salmaniya Medical Complex (a tertiary care hospital of the Kingdom of Bahrain), were reviewed and categorized. Primary glomerular disease accounted for two-third of the glomerular diseases, which in turn constituted 44.8% of all renal biopsies. The most common histological lesion was minimal change disease (30%). Focal and segmental glomerulosclerosis was the second most common lesion (23.8%) followed by membranoproliferative glomerulonephritis (14.3%). Secondary glomerular disease comprised 33.6% of glomerular diseases (22.7% of all the renal biopsies) with lupus nephritis forming the commonest lesion (38.9%) followed by diabetic nephropathy (31.9%) and hypertension (20.4%). Tubulointerstitial diseases accounted for 13.1% of all renal biopsies whereas transplant diseases were noted in 12.2%. The miscellaneous group including inadequate biopsies constituted 7.2% of all the biopsies. The results of this analysis were compared with surveys from other parts of the World.
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PMID:Renal biopsy findings in the kingdom of bahrain: a 13-year retrospective study. 1764 89

Identification of the profile of glomerular disease in a particular geographical region is of fundamental academic, clinical and epidemiological importance. It helps in the recognition of specific risk factors and subsequent planning for adequate prevention. In the present study, 1234 consecutive renal biopsies referred to the nephropathology team of Cairo University over two years were retrospectively analyzed. The main indications for biopsy included nephrotic syndrome, persistent sub-nephrotic proteinuria, recurrent hematuria, suspected secondary hypertension, lupus nephritis and acute and chronic renal failure of undetermined etiology. Proliferative forms of glomerulonephritis [GN] (32.1%) and focal and segmental glomerulosclerosis [FSGS] were the most prevalent lesions among patients with the nephrotic syndrome (22.6%). In subjects with sub-nephrotic proteinuria, FSGS was the principal lesion followed by proliferative lesions. Although all forms of GN were encountered in those presenting with recurrent hematuria, mesangioproliferative GN and FSGS were significantly more frequent. IgA glomerular deposits were detected in 9.8% of all GNs and 15% of those presenting with hematuria. One half of the biopsies obtained for the assessment of suspected secondary hypertension showed only changes compatible with the effect of hypertension per se, i.e. nephroangiosclerosis. On the other hand, a parenchymal renal lesion was found in 52.9% of biopsies. The common glomerular pathologies in patients with lupus nephritis were Classes III and IV. Among patients with chronic renal failure, the predominant lesion was chronic interstitial nephritis (32.6%). An acute interstitial inflammatory element was also detected in 8.4% of cases. About one third of the biopsies obtained for acute renal failure showed acute tubular (11%) or cortical (13.2%) necrosis. Another third showed vasculitis (17.6%) or acute interstitial nephritis (14.3%), and the remaining showed chronic lesions in which the rapid deterioration was probably functional.
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PMID:Spectrum of glomerulonephritis in egypt. 1820 34

We studied the glomerular architecture in renal biopsies of 95 patients undergoing bariatric surgery for extreme obesity but whose renal function was normal. The comparison group was 40 control patients having protocol biopsies. These latter patients had normal weight and renal function, were non-diabetic, non-hypertensive, and were undergoing nephrectomy or donating a kidney. Logistic regression models determined associations between the clinical and biochemical variables and glomerular lesions. Arterial hypertension, sleep apnea syndrome (SAS), and microalbuminuria were prevalent in the obese patients, as was hyperglycemia to a lesser extent. Focal and segmental glomerulosclerosis was present in only five extremely obese (EO) patients but absent in controls. Increased mesangial matrix, podocyte hypertrophy, mesangial cell proliferation, and glomerulomegaly were more frequent in the obese cohort than in the control group. Body mass index was a significant independent risk factor associated with glomerular lesions in all 135 patients and in the 95 EO patients, whereas SAS was associated with glomerulomegaly only in the EO. Our study shows that EO patients who lack overt clinical renal symptoms have a variety of glomerular abnormalities that correlate with body mass.
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PMID:Renal injury in the extremely obese patients with normal renal function. 1885 52


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