Search PubMed⌕ Search

Biomedical subjects

Agnes B Fogo

Publications and source records attributed to Agnes B Fogo.

At least 55 records · Page 3Linked to original sources

The classification of glomerulonephritis in systemic lupus erythematosus revisited.

The currently used classification reflects our understanding of the pathogenesis of the various forms of lupus nephritis, but clinicopathologic studies have revealed the need for improved categorization and terminology. Based on the 1982 classification published under the auspices of the World Health Organization (WHO) and subsequent clinicopathologic data, we propose that class I and II be used for purely mesangial involvement (I, mesangial immune deposits without mesangial hypercellularity; II, mesangial immune deposits with mesangial hypercellularity); class III for focal glomerulonephritis (involving <50% of total number of glomeruli) with subdivisions for active and sclerotic lesions; class IV for diffuse glomerulonephritis (involving > or =50% of total number of glomeruli) either with segmental (class IV-S) or global (class IV-G) involvement, and also with subdivisions for active and sclerotic lesions; class V for membranous lupus nephritis; and class VI for advanced sclerosing lesions. Combinations of membranous and proliferative glomerulonephritis (i.e., class III and V or class IV and V) should be reported individually in the diagnostic line. The diagnosis should also include entries for any concomitant vascular or tubulointerstitial lesions. One of the main advantages of the current revised classification is that it provides a clear and unequivocal description of the various lesions and classes of lupus nephritis, allowing a better standardization and lending a basis for further clinicopathologic studies. We hope that this revision, which evolved under the auspices of the International Society of Nephrology and the Renal Pathology Society, will contribute to further advancement of the WHO classification.

Humans↗

Nephrotic syndrome complicating alpha-glucosidase replacement therapy for Pompe disease.

We report a patient with Pompe disease who developed reversible nephrotic syndrome during prolonged, high-dose, experimental, enzyme replacement therapy with recombinant human acid alpha-glucosidase (rhGAA). Because of the development of antibodies to rhGAA and concomitant clinical decline, escalating doses of rhGAA were administered as part of an experimental immune tolerance regimen. Histologic evaluation of kidney tissue revealed glomerular deposition of immune complexes containing rhGAA itself, in a pattern of membranous nephropathy. To our knowledge, this is the first reported case of nephrotic syndrome occurring during enzyme replacement therapy. The nephrotic syndrome gradually resolved after the rhGAA dose was decreased, indicating that decreasing the antigenic load can ameliorate glomerular immune complex deposition associated with enzyme replacement in a highly sensitized patient.

Antibodies↗

Prevention of obesity and insulin resistance in mice lacking plasminogen activator inhibitor 1.

Increased plasminogen activator inhibitor 1 (PAI-1) has been linked to not only thrombosis and fibrosis but also to obesity and insulin resistance. Increased PAI-1 levels have been presumed to be consequent to obesity. We investigated the interrelationships of PAI-1, obesity, and insulin resistance in a high-fat/high-carbohydrate (HF) diet-induced obesity model in wild-type (WT) and PAI-1-deficient mice (PAI-1(-/-)). Obesity and insulin resistance developing in WT mice on an HF diet were completely prevented in mice lacking PAI-1. PAI-1(-/-) mice on an HF diet had increased resting metabolic rates and total energy expenditure compared with WT mice, along with a marked increase in uncoupling protein 3 mRNA expression in skeletal muscle, likely mechanisms contributing to the prevention of obesity. In addition, insulin sensitivity was enhanced significantly in PAI-1(-/-) mice on an HF diet, as shown by euglycemic-hyperinsulinemic clamp studies. Peroxisome proliferator-activated receptor (PPAR)-gamma and adiponectin mRNA, key control molecules in lipid metabolism and insulin sensitivity, were maintained in response to an HF diet in white adipose tissue in PAI-1(-/-) mice, contrasting with downregulation in WT mice. This maintenance of PPAR-gamma and adiponectin may also contribute to the observed maintenance of body weight and insulin sensitivity in PAI-1(-/-) mice. Treatment in WT mice on an HF diet with the angiotensin type 1 receptor antagonist to downregulate PAI-1 indeed inhibited PAI-1 increases and ameliorated diet-induced obesity, hyperglycemia, and hyperinsulinemia. PAI-1 deficiency also enhanced basal and insulin-stimulated glucose uptake in adipose cells in vitro. Our data suggest that PAI-1 may not merely increase in response to obesity and insulin resistance, but may have a direct causal role in obesity and insulin resistance. Inhibition of PAI-1 might provide a novel anti-obesity and anti-insulin resistance treatment.

Adiponectin↗

Serial determination of glomerular filtration rate in conscious mice using FITC-inulin clearance.

Two nonradioactive methods for determining glomerular filtration rate (GFR) in conscious mice using FITC-labeled inulin (FITC-inulin) were evaluated. The first method measured GFR using clearance kinetics of plasma FITC-inulin after a single bolus injection. Based on a two-compartment model, estimated GFR was 236.69 +/- 16.55 and 140.20 +/- 22.27 microl/min in male and female C57BL/6J mice, respectively. Total or (5/6) nephrectomy reduced inulin clearance to 0 or 32.80 +/- 9.32 microl/min, respectively. Conversely, diabetes mellitus induced by streptozotocin was associated with increased GFR. The other approach measured urinary inulin clearance using intraperitoneal microosmotic pumps to deliver FITC-inulin and metabolic cages to collect timed urine samples. This approach yielded similar GFR values of 211.11 +/- 26.56 and 157.36 +/- 20.02 microl/min in male and female mice, respectively. These studies demonstrate the feasibility of repeated nonisotopic measurement of inulin clearance in conscious mice.

Animals↗

Recurrent lupus nephritis in renal transplant recipients revisited: it is not rare.

BACKGROUND: Although recurrent lupus nephritis (RLN) after kidney transplantation is reported to be rare (1%-4%), recent studies suggest a higher incidence. The purpose of this study was to determine the incidence of RLN in a large cohort of renal transplant recipients with systemic lupus erythematosus (SLE). METHODS: The records of 54 renal transplant recipients with SLE were reviewed. Thirty-one patients underwent biopsy because of worsening renal function and proteinuria. All biopsy specimens were evaluated by light microscopy, immunofluorescence (IF), and electron microscopy (EM). RESULTS: Among the 50 patients with at least 3 months of follow-up, RLN was present in 15 (52% of patients who underwent biopsy, 30% of total patients): mesangial lupus nephritis (LN) (class II) in eight, focal proliferative LN (class III) in four, and membranous LN (class Vb) in three patients. One patient had graft loss because of RLN (class II) at 10.5 years. The duration of dialysis before transplantation was not different between patients with RLN compared to patients without RLN (P=0.40). Overall patient survival (n=50) was 96% at 1 year and 82% at 5 years, and graft survival was 87% at 1 year and 60% at 5 years. Graft survival was worse in patients who underwent biopsy compared with patients who never underwent biopsy (P<0.01). CONCLUSIONS: RLN is more common than previously reported, but in our series, graft loss because of RLN was rare. Aggressive use of allograft biopsies and morphologic evaluation with IF and EM are important factors in the diagnosis of RLN. The impact of new immunosuppressive agents on the incidence of RLN remains to be seen.

Adult↗

COX2 activity promotes organic osmolyte accumulation and adaptation of renal medullary interstitial cells to hypertonic stress.

The mechanism by which COX2 inhibition decreases renal cell survival is poorly understood. In the present study we examined the effect of COX2 activity on organic osmolyte accumulation in renal medulla and in cultured mouse renal medullary interstitial cells (MMICs) and its role in facilitating cell survival. Hypertonicity increased accumulation of the organic osmolytes inositol, sorbitol, and betaine in cultured mouse medullary interstitial cells. Pretreatment of MMICs with a COX2-specific inhibitor (SC58236, 10 micromol/liter) dramatically reduced osmolyte accumulation (by 79 +/- 9, 57 +/- 12, and 96 +/- 10% for inositol, sorbitol, and betaine respectively, p < 0.05). Similarly, 24 h of dehydration increased inner medullary inositol, sorbitol, and betaine concentrations in vivo by 85 +/- 10, 197 +/- 28, and 190 +/- 24 pmol/microg of protein, respectively, but this increase was also blunted (by 100 +/- 5, 66 +/- 15, and 81 +/- 9% for inositol, sorbitol, and betaine, respectively, p < 0.05) by pretreatment with an oral COX2 inhibitor. Dehydrated COX2-/- mice also exhibited an impressive defect in sorbitol accumulation (88 +/- 9% less than wild type, p < 0.05) after dehydration. COX2 inhibition (COX2 inhibitor-treated or COX2-/- MMICs) dramatically reduced the expression of organic osmolyte uptake mechanisms including betaine (BGT1) and sodium-myo-inositol transporter and aldose reductase mRNA expression under hypertonic conditions. Importantly, preincubation of COX2 inhibitor-treated MMICs with organic osmolytes restored their ability to survive hypertonic stress. In conclusion, osmolyte accumulation in the kidney inner medulla is dependent on COX2 activity, and providing exogenous osmolytes reverses COX2-induced cell death. These findings may have implications for the pathogenesis of analgesic nephropathy.

Adaptation, Physiological↗

Shigatoxin-1 binding and receptor expression in human kidneys do not change with age.

Postdiarrheal hemolytic-uremic syndrome (D+HUS) occurs predominantly in young children. The rarity of D+HUS in adults has been ascribed to aging-associated loss of glomerular globotriaosylceramide (Gb3) expression, the major cognate receptor for shigatoxin. This belief, however, is based on relatively little data. The current study was undertaken to examine renal shigatoxin-1 (Stx-1) binding and Gb3 expression by human kidneys from varying aged subjects. Immunofluorescent staining and thin layer chromatography of neutral lipid extracts were performed. Abundant Stx-1 binding to both glomeruli and tubules was observed in frozen renal sections from all subjects of all ages (6 months to 85 years). The pattern of Stx-1 binding was identical between adults and children, with glomerular endothelial cells and cortical tubules being strongly labeled. Stx-1 binding affinity was similar between pediatric and adult kidneys. Antibodies to Gb3 showed a similar pattern and degree of staining regardless of donor age. In addition, Gb3 levels in glomeruli and tubules isolated from fresh kidney tissue were comparable between different aged donors. These data demonstrate that intrinsic renal binding of Stx-1 does not vary with age. It is suggested that factors other than basal renal Gb3 expression account for the age-related incidence of acute renal failure in D+HUS.

Adolescent↗

Transforming growth factor-beta-dependent and -independent pathways of induction of tubulointerstitial fibrosis in beta6(-/-) mice.

Transforming growth factor-beta1 (TGF-beta1) and the renin-angiotensin-aldosterone system are key mediators in kidney fibrosis. Integrin alphavbeta6, a heterodimeric matrix receptor expressed in epithelia, binds and activates latent TGF-beta1. We used beta6 integrin-null mice (beta6(-/-)) to determine the role of local TGF-beta1 activation in renal fibrosis in the unilateral ureteral obstruction (UUO) model. Obstructed kidneys from beta6(-/-) mice showed less injury than obstructed kidneys from wild-type (WT) mice, associated with lower collagen I, collagen III, plasminogen activator inhibitor (PAI-1), and TGF-beta1 mRNA levels and lower collagen content. Infusion with either angiotensin II (Ang II) or aldosterone (Aldo) or combination in beta6(-/-) UUO mice significantly increased collagen contents to levels comparable to those in identically treated WT. Active TGF-beta protein expression in beta6(-/-) mice was less in UUO kidneys with or without Ang II infusion compared to matched WT mice. Activated Smad 2 levels in beta6(-/-) obstructed kidneys were lower than in WT UUO mice, and did not increase when fibrosis was induced in beta6(-/-) UUO mice by Ang II infusion. Anti-TGF-beta antibody only partially decreased this Ang II-stimulated fibrosis in beta6(-/-) UUO kidneys. In situ hybridization and immunostaining showed low expression of PAI-1 mRNA and protein in tubular epithelium in beta6(-/-) UUO kidneys, with increased PAI-1 expression in response to Ang II, Aldo, or both. Our results indicate that interruption of alphavbeta6-mediated activation of TGF-beta1 can protect against tubulointerstitial fibrosis. Further, the robust induction of tubulointerstitial fibrosis without increase in activated Smad 2 levels in obstructed beta6(-/-) mice by Ang II suggests the existence of a TGF-beta1-independent pathway of induction of fibrosis through angiotensin.

Aldosterone↗

Monoclonal gammopathy: significance and possible causality in renal disease.

BACKGROUND: Patients with monoclonal gammopathy can develop a variety of related renal lesions or possibly have kidney disease unrelated to their monoclonal gammopathy. We characterized the spectrum of renal diseases associated with monoclonal gammopathy and renal diseases. METHODS: Patients who underwent renal biopsy and had monoclonal gammopathy on serum and/or urine electrophoresis and/or had a renal biopsy diagnosis related to paraprotein (cryoglobulinemic glomerulonephritis [CG], monoclonal immunoglobulin deposition disease [MIDD], light chain cast nephropathy [CN], or light chain amyloidosis [AL]) were identified. RESULTS: One hundred twenty-one patients met the inclusion criteria and were classified as having renal disease related or unrelated to monoclonal gammopathy. Among 66 cases of renal disease related to monoclonal gammopathy, diagnoses were CG (30.3%), MIDD (28.8%), CN (19.7%), AL (19.7%), and CN plus MIDD (1.5%). Among patients with monoclonal gammopathy in serum and/or urine (n = 87), 32 patients (36.8%, included in listing above) had related renal disease. Among 55 patients with monoclonal gammopathy and unrelated renal disease (63.2% of all patients with monoclonal gammopathy), various lesions were found, including diabetic nephropathy (18.1%), focal segmental glomerulosclerosis (18.1%), arterionephrosclerosis (12.7%), membranous glomerulonephritis (9.0%), minimal change disease (7.3%), various immune complex diseases, interstitial nephritis, or nonspecific changes. CONCLUSION: The majority of patients with serum and/or urine monoclonal gammopathy who undergo renal biopsy have disease unrelated to monoclonal gammopathy deposition. This likely reflects the high frequency of monoclonal gammopathy of undetermined significance in older patients and the frequent use of serum and/or urine protein electrophoresis as screening tools in adult patients with renal disease.

Adolescent↗

Model of robust induction of glomerulosclerosis in mice: importance of genetic background.

BACKGROUND: Increasing evidence suggests that genetic background plays an important role in the development of progressive glomerulosclerosis. The remnant kidney model (RKM) of progressive renal disease has been used extensively in rats. However, C57BL/6 mice are resistant to glomerulosclerosis with RKM induced by either pole amputation or renal artery ligation. A pole resection protocol, applied in 129/Sv mice, induced only mild glomerulosclerosis. We present here a highly reproducible, modified RKM approach to successfully establish a glomerulosclerosis model in mice. METHODS: Male C57BL/6 (N = 17), 129/Sv (N = 20) and Swiss-Webster (N = 3) mice underwent RKM as follows: the lower branch of the left renal artery was ligated to produce about one third infarct; the upper pole of the left kidney (about one third kidney size) was removed by cautery and the right kidney was nephrectomized to induce a total 5/6 nephrectomy (Nx). In some C57BL/6 mice, 7/8 nephrectomy was induced by removing additional renal mass from the upper pole of the left kidney by cautery. Systolic blood pressure (BP) was measured in conscious mice using a tail-cuff blood pressure monitor and animals were sacrificed at 9, 12, 18, and 24 weeks after nephrectomy. Kidneys were harvested for morphologic analysis. RESULTS: BP in C57BL/6 mice increased slightly after 5/6 nephrectomy over time without significant difference compared to baseline blood pressure except at 8 weeks (blood pressure at week 0, 98 +/- 1 mm Hg; week 4, 105 +/- 2 mm Hg; week 8, 113 +/- 4 mm Hg; and week 12, 110 +/- 3 mm Hg). Blood presssure remained normal in C57BL/6 mice at 18 weeks after 7/8 nephrectomy (103 +/- 2 mm Hg). Blood pressure in 129/Sv mice increased significantly after 5/6 nephrectomy from 4 to 12 weeks (week 0, 112 +/- 3 mm Hg; week 4, 161 +/- 9 mm Hg; week 8, 166 +/- 5 mm Hg; and week 12, 176 +/- 5 mm Hg; P < 0.01 weeks 4, 8, and 12 vs. week 0 blood pressure). Urine protein excretion in C57BL/6 mice increased only at 4 weeks after 5/6 nephrectomy, and was back to normal at 8 and 12 weeks (week 0, 13.2 +/- 1.4 mg/24 hours; week 4, 20.5 +/- 1.8 mg/24 hours; week 8, 18.8 +/- 1.6 mg/24 hours; and week 12, 17.2 +/- 1.2 mg/24 hours, P < 0.05 week 4 vs. week 0). 129/Sv mice developed significant proteinuria 12 weeks after 5/6 nephrectomy compared to their baseline and to levels achieved in C57BL/6 mice (week 0, 17.2 +/- 1 mg/24 hours; week 4, 14.9 +/- 1.8 mg/24 hours; week 8, 23.8 +/- 6.7 mg/24 hours; and week 12, 36.3 +/- 6.6 mg/24 hours, P < 0.01 week 12 vs. week 0; P < 0.01 129/Sv vs. C57BL/6 at week 12). Mortality varied in response to nephrectomy injury in the different strains. Ten percent of C57BL/6 and 43% of 129/Sv died within 12 weeks after 5/6 nephrectomy. Although 50% of C57BL/6 mice died by 12 weeks after 7/8 nephrectomy, there was only mild glomerulosclerosis (<5%) in C57BL/6 mice even at 24 weeks after 5/6 nephrectomy or 18 weeks after 7/8 nephrectomy. In contrast, glomerulosclerosis was marked in both 129/Sv mice and Swiss-Webster mice as early as 9 weeks after 5/6 nephrectomy: 42% of glomeruli showed sclerosis in 129/Sv mice [average sclerosis index (SI), 0 to 4+ scale, 1.08] vs. 24% in Swiss-Webster mice (average SI, 0.57). Tubulointerstitial fibrosis developed in parallel with glomerulosclerosis in both 129/Sv and Swiss-Webster mice. CONCLUSION: We conclude that genetic background is one of the important factors determining the susceptibility to the development of glomerulosclerosis in mice. We speculate that the superior effects of renal artery ligation plus cautery to produce glomerulosclerosis may result from higher blood pressure responses due to local ischemia activating the renin-angiotensin system.

Animals↗

Hypertensive risk factors in kidney disease in African Americans.

African Americans with hypertension more commonly develop renal insufficiency compared to Caucasians. The African American Study of Kidney Disease (AASK) included a renal biopsy pilot study that demonstrated that the clinical diagnosis of so-called hypertensive nephrosclerosis in these African American patients indeed was accurate. This biopsy study demonstrated extensive global glomerulosclerosis, far exceeding that expected for patients' age. We further compared our clinically indicated renal biopsies to determine if any phenotypic differences were present in hypertensive nephrosclerosis in African Americans versus Caucasians. These studies point to an excess of the solidified type of global glomerulosclerosis (GS) (also called "decompensated benign nephrosclerosis") in African Americans compared to more obsolescent type GS in Caucasians. We speculate that these phenotypic differences might reflect differing mechanisms of tissue injury in hypertensive African Americans versus Caucasians, and discuss possible contributors to this injury.

Black or African American↗

Animal models of FSGS: lessons for pathogenesis and treatment.

Glomerulosclerosis in a heterogeneous pattern, ie, focal and segmental glomerulosclerosis (FSGS), is a common endpoint in a variety of settings, including idiopathic FSGS, and scarring secondary to other renal or systemic diseases. These different causes contribute to the diverse clinical outcomes of histological focal sclerosis, and the varying histologic manifestations of sclerosis. Numerous models have been established in the rat that aim to mirror the various elements of human glomerulosclerosis. With the availability of knockout gene technology, many, but not all of these models have been translated to mouse species. This review will focus on the remnant kidney model, the podocyte injury models of puromycin aminonucleoside or adriamycin injection, and examples of newly developed genetic models, such as knockout of CD2 associated protein (CD2AP).

Adaptor Proteins, Signal Transducing↗