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Sample records for basement membrane glomerulonephritis

  1. Antiglomerular basement membrane antibody-mediated glomerulonephritis after intranasal cocaine use.

    Science.gov (United States)

    Peces, R; Navascués, R A; Baltar, J; Seco, M; Alvarez, J

    1999-01-01

    We report a case of rapidly progressive glomerulonephritis due to antiglomerular basement membrane (anti-GBM) antibodies that progressed to end-stage renal disease in a 35-year-old man who used intranasal cocaine on an occasional basis. In contrast to many prior reports of acute renal failure occurring with cocaine-associated rhabdomyolysis, this patient did not have any evidence of acute muscle damage and myoglobin release. Circulating anti-GBM antibodies and renal biopsy with linear IgG and C3 deposits confirmed the diagnosis of anti-GBM disease. The possibility of anti-GBM must be considered in the differential diagnosis of acute renal failure in cocaine addicts. This unusual combination raises complex questions regarding the pathogenesis of this type of renal injury.

  2. Sequential development of pulmonary hemorrhage with MPO-ANCA complicating anti-glomerular basement membrane antibody-mediated glomerulonephritis.

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    Peces, R; Rodríguez, M; Pobes, A; Seco, M

    2000-05-01

    We report a case of rapidly progressive glomerulonephritis caused by anti-glomerular basement membrane (anti-GBM) antibodies that progressed to end-stage renal disease in a 67-year-old woman with diabetes. Intensive combined immunosuppressive therapy with methylprednisolone bolus, oral prednisone, and cyclophosphamide led to negativity of anti-GBM antibodies but was not able to restore renal function. After 28 months of hemodialysis, the patient suddenly presented with pulmonary hemorrhage. In this setting, high levels of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA) and negative anti-GBM antibodies were found. Therapy with oral prednisone and cyclophosphamide led to resolution of pulmonary hemorrhage and negativity of MPO-ANCA.

  3. PF-1355, a mechanism-based myeloperoxidase inhibitor, prevents immune complex vasculitis and anti-glomerular basement membrane glomerulonephritis.

    Science.gov (United States)

    Zheng, Wei; Warner, Roscoe; Ruggeri, Roger; Su, Chunyan; Cortes, Christian; Skoura, Athanasia; Ward, Jessica; Ahn, Kay; Kalgutkar, Amit; Sun, Dexue; Maurer, Tristan S; Bonin, Paul D; Okerberg, Carlin; Bobrowski, Walter; Kawabe, Thomas; Zhang, Yanwei; Coskran, Timothy; Bell, Sammy; Kapoor, Bhupesh; Johnson, Kent; Buckbinder, Leonard

    2015-05-01

    Small vessel vasculitis is a life-threatening condition and patients typically present with renal and pulmonary injury. Disease pathogenesis is associated with neutrophil accumulation, activation, and oxidative damage, the latter being driven in large part by myeloperoxidase (MPO), which generates hypochlorous acid among other oxidants. MPO has been associated with vasculitis, disseminated vascular inflammation typically involving pulmonary and renal microvasculature and often resulting in critical consequences. MPO contributes to vascular injury by 1) catabolizing nitric oxide, impairing vasomotor function; 2) causing oxidative damage to lipoproteins and endothelial cells, leading to atherosclerosis; and 3) stimulating formation of neutrophil extracellular traps, resulting in vessel occlusion and thrombosis. Here we report a selective 2-thiouracil mechanism-based MPO inhibitor (PF-1355 [2-(6-(2,5-dimethoxyphenyl)-4-oxo-2-thioxo-3,4-dihydropyrimidin-1(2H)-yl)acetamide) and demonstrate that MPO is a critical mediator of vasculitis in mouse disease models. A pharmacokinetic/pharmacodynamic response model of PF-1355 exposure in relation with MPO activity was derived from mouse peritonitis. The contribution of MPO activity to vasculitis was then examined in an immune complex model of pulmonary disease. Oral administration of PF-1355 reduced plasma MPO activity, vascular edema, neutrophil recruitment, and elevated circulating cytokines. In a model of anti-glomerular basement membrane disease, formerly known as Goodpasture disease, albuminuria and chronic renal dysfunction were completely suppressed by PF-1355 treatment. This study shows that MPO activity is critical in driving immune complex vasculitis and provides confidence in testing the hypothesis that MPO inhibition will provide benefit in treating human vasculitic diseases. Copyright © 2015 by The American Society for Pharmacology and Experimental Therapeutics.

  4. Cell invasion through basement membrane

    OpenAIRE

    Morrissey, Meghan A; Hagedorn, Elliott J; Sherwood, David R

    2013-01-01

    Cell invasion through basement membrane is an essential part of normal development and physiology, and occurs during the pathological progression of human inflammatory diseases and cancer. F-actin-rich membrane protrusions, called invadopodia, have been hypothesized to be the “drill bits” of invasive cells, mediating invasion through the dense, highly cross-linked basement membrane matrix. Though studied in vitro for over 30 y, invadopodia function in vivo has remained elusive. We have recent...

  5. Cell invasion through basement membrane

    Science.gov (United States)

    Morrissey, Meghan A; Hagedorn, Elliott J; Sherwood, David R

    2013-01-01

    Cell invasion through basement membrane is an essential part of normal development and physiology, and occurs during the pathological progression of human inflammatory diseases and cancer. F-actin-rich membrane protrusions, called invadopodia, have been hypothesized to be the “drill bits” of invasive cells, mediating invasion through the dense, highly cross-linked basement membrane matrix. Though studied in vitro for over 30 y, invadopodia function in vivo has remained elusive. We have recently discovered that invadopodia breach basement membrane during anchor cell invasion in C. elegans, a genetically and visually tractable in vivo invasion event. Further, we found that the netrin receptor DCC localizes to the initial site of basement membrane breach and directs invasion through a single gap in the matrix. In this commentary, we examine how the dynamics and structure of AC-invadopodia compare with in vitro invadopodia and how the netrin receptor guides invasion through a single basement membrane breach. We end with a discussion of our surprising result that the anchor cell pushes the basement membrane aside, instead of completely dissolving it through proteolysis, and provide some ideas for how proteases and physical displacement may work together to ensure efficient and robust invasion. PMID:24778942

  6. Basement membrane proteoglycans and development

    DEFF Research Database (Denmark)

    Couchman, J R; Abrahamson, D R; McCarthy, K J

    1993-01-01

    -CSPG was only strongly expressed in the vasculature invading late comma stage glomeruli, and later in presumptive and mature Bowman's capsule. Over the first six to eight weeks, the capillary basement membranes contained BM-CSPG, but in gradually decreasing amounts until it became completely undetectable...

  7. Glomerular basement membrane lipidosis in Alagille syndrome.

    Science.gov (United States)

    Davis, Jessica; Griffiths, Ryan; Larkin, Kay; Rozansky, David; Troxell, Megan

    2010-06-01

    Alagille syndrome is characterized by a paucity of interlobular bile ducts with chronic cholestasis, cardiac, skeletal, and eye abnormalities and is associated predominantly with JAG1 mutations. Various renal abnormalities have been sporadically described. The classic renal histopathology described in Alagille syndrome is mesangiolipidosis, with lipid deposits predominately confined to the mesangium and minimal deposition within the glomerular basement membrane (GBM). We report a 5-year-old girl with Alagille syndrome who presented with persistent subnephrotic proteinuria and renal tubular acidosis. A renal biopsy showed GBM irregularities (mimicking membranous glomerulonephritis), mesangial sclerosis, and focal segmental glomerulosclerosis (FSGS) on light microscopy. Electron microscopy revealed few lipid inclusions within the mesangium but extensive inclusions along the GBM. These findings are mostly consistent with those reported previously in Alagille syndrome. However, the histologic distribution of lipid vacuoles is seemingly reversed in this patient and is uniquely accompanied by FSGS, emphasizing the spectrum of renal histopathology seen in Alagille syndrome. The proteinuria observed in this patient is likely attributed to significant GBM lipid deposition, which over time may contribute to the development of FSGS.

  8. Anti-glomerular basement membrane disease superimposed on membranous nephropathy: a case report and review of the literature

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    Nivera Noel

    2010-08-01

    Full Text Available Abstract Introduction Anti-glomerular basement membrane disease is a rare autoimmune disorder characterized by pulmonary hemorrhage, crescentic glomerulonephritis and the presence of circulating anti-glomerular basement membrane antibodies. The simultaneous occurrence of both anti-glomerular basement membrane disease and membranous nephropathy is rare. Case presentation A 59-year-old Hispanic man presented with acute onset of nausea and vomiting and was found to have renal insufficiency. Work-up included a kidney biopsy, which revealed anti-glomerular basement membrane disease with underlying membranous nephropathy. He was treated with emergent hemodialysis, intravenous corticosteroids, plasmapheresis, and cyclophosphamide without improvement in his renal function. Conclusion Simultaneous anti-glomerular basement membrane disease and membranous nephropathy is very rare. There have been 16 previous case reports in the English language literature that have been associated with a high mortality and morbidity, and a very high rate of renal failure resulting in hemodialysis. Co-existence of membranous nephropathy and anti-glomerular basement membrane disease may be immune-mediated, although the exact mechanism is not clear.

  9. Membranous glomerulonephritis and Landry-Guillain-Barre syndrome.

    Science.gov (United States)

    Murphy, B F; Gonzales, M F; Ebeling, P; Fairley, K F; Kincaid-Smith, P

    1986-10-01

    Two cases of idiopathic membranous glomerulonephritis associated with acute inflammatory demyelinating polyradiculoneuropathy (Landry-Guillian-Barre syndrome) are described. In both of the patients, the onset of the nephrotic syndrome coincided with the development of severe ascending sensorimotor neuropathy. Although this association has previously been reported in four other isolated cases, it is not generally recognized by nephrologists and may be of significance in the future understanding of the immunopathogenesis of both diseases.

  10. ROCK1-directed basement membrane positioning coordinates epithelial tissue polarity.

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    Daley, William P; Gervais, Elise M; Centanni, Samuel W; Gulfo, Kathryn M; Nelson, Deirdre A; Larsen, Melinda

    2012-01-01

    The basement membrane is crucial for epithelial tissue organization and function. However, the mechanisms by which basement membrane is restricted to the basal periphery of epithelial tissues and the basement membrane-mediated signals that regulate coordinated tissue organization are not well defined. Here, we report that Rho kinase (ROCK) controls coordinated tissue organization by restricting basement membrane to the epithelial basal periphery in developing mouse submandibular salivary glands, and that ROCK inhibition results in accumulation of ectopic basement membrane throughout the epithelial compartment. ROCK-regulated restriction of PAR-1b (MARK2) localization in the outer basal epithelial cell layer is required for basement membrane positioning at the tissue periphery. PAR-1b is specifically required for basement membrane deposition, as inhibition of PAR-1b kinase activity prevents basement membrane deposition and disrupts overall tissue organization, and suppression of PAR-1b together with ROCK inhibition prevents interior accumulations of basement membrane. Conversely, ectopic overexpression of wild-type PAR-1b results in ectopic interior basement membrane deposition. Significantly, culture of salivary epithelial cells on exogenous basement membrane rescues epithelial organization in the presence of ROCK1 or PAR-1b inhibition, and this basement membrane-mediated rescue requires functional integrin β1 to maintain epithelial cell-cell adhesions. Taken together, these studies indicate that ROCK1/PAR-1b-dependent regulation of basement membrane placement is required for the coordination of tissue polarity and the elaboration of tissue structure in the developing submandibular salivary gland.

  11. Basement membrane proteoglycans are of epithelial origin in rodent skin

    DEFF Research Database (Denmark)

    Yamane, Y; Yaoita, H; Couchman, J R

    1996-01-01

    . For in vivo experiments, pieces of newborn rat epidermis obtained by dispase treatment were grafted onto athymic nude mice. Three and six weeks after grafting, immunofluorescence analysis of the grafted skin was carried out, using monoclonal antibodies specific for rat basement membrane chondroitin sulfate...... on mice demonstrated the presence of rat basement membrane chondroitin sulfate proteoglycan and rat perlecan on interfollicular and follicular basement membranes including that separating dermal papillae from adjacent hair follicle epithelium. In contrast, the basement membranes of all dermal capillaries......-epidermal junction and hair follicle epithelium are of epidermal (epithelial) origin in vivo. Stratified rat keratinocytes cultured on a collagen matrix at the air-liquid interface showed the synthesis of perlecan, laminin 1, and type IV collagen in basement membranes, but not clearly detectable basement membrane...

  12. Basement membrane proteoglycans are of epithelial origin in rodent skin

    DEFF Research Database (Denmark)

    Yamane, Y; Yaoita, H; Couchman, J R

    1996-01-01

    proteoglycan and rat and mouse perlecan. While the isolated rat epidermis was shown to completely lack rat basement membrane chondroitin sulfate proteoglycan and rat basement membrane heparan sulfate proteoglycans, including perlecan, immunofluorescence staining of tissue sections from the grafted sites......-epidermal junction and hair follicle epithelium are of epidermal (epithelial) origin in vivo. Stratified rat keratinocytes cultured on a collagen matrix at the air-liquid interface showed the synthesis of perlecan, laminin 1, and type IV collagen in basement membranes, but not clearly detectable basement membrane...

  13. Basement Membrane Defects in Genetic Kidney Diseases

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    Christine Chew

    2018-01-01

    Full Text Available The glomerular basement membrane (GBM is a specialized structure with a significant role in maintaining the glomerular filtration barrier. This GBM is formed from the fusion of two basement membranes during development and its function in the filtration barrier is achieved by key extracellular matrix components including type IV collagen, laminins, nidogens, and heparan sulfate proteoglycans. The characteristics of specific matrix isoforms such as laminin-521 (α5β2γ1 and the α3α4α5 chain of type IV collagen are essential for the formation of a mature GBM and the restricted tissue distribution of these isoforms makes the GBM a unique structure. Detailed investigation of the GBM has been driven by the identification of inherited abnormalities in matrix proteins and the need to understand pathogenic mechanisms causing severe glomerular disease. A well-described hereditary GBM disease is Alport syndrome, associated with a progressive glomerular disease, hearing loss, and lens defects due to mutations in the genes COL4A3, COL4A4, or COL4A5. Other proteins associated with inherited diseases of the GBM include laminin β2 in Pierson syndrome and LMX1B in nail patella syndrome. The knowledge of these genetic mutations associated with GBM defects has enhanced our understanding of cell–matrix signaling pathways affected in glomerular disease. This review will address current knowledge of GBM-associated abnormalities and related signaling pathways, as well as discussing the advances toward disease-targeted therapies for patients with glomerular disease.

  14. The major basement membrane components localize to the chondrocyte pericellular matrix--a cartilage basement membrane equivalent?

    DEFF Research Database (Denmark)

    Kvist, Alexander J.; Nyström, Alexander; Hultenby, Kjell

    2007-01-01

    In this study, we demonstrate that articular cartilage chondrocytes are surrounded by the defining basement membrane proteins laminin, collagen type IV, nidogen and perlecan, and suggest that these form the functional equivalent of a basement membrane. We found by real-time PCR that mouse...... to the progression of degenerative joint disorders....

  15. Basement membrane chondroitin sulfate proteoglycans: localization in adult rat tissues

    DEFF Research Database (Denmark)

    McCarthy, K J; Couchman, J R

    1990-01-01

    Heparan sulfate proteoglycans have been described as the major proteoglycan component of basement membranes. However, previous investigators have also provided evidence for the presence of chondroitin sulfate glycosaminoglycan in these structures. Recently we described the production and characte...

  16. Clinical outcome of patients with coexistent antineutrophil cytoplasmic antibodies and antibodies against glomerular basement membrane.

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    Lindic, Jelka; Vizjak, Alenka; Ferluga, Dusan; Kovac, Damjan; Ales, Andreja; Kveder, Radoslav; Ponikvar, Rafael; Bren, Andrej

    2009-08-01

    Antineutrophil cytoplasmic antibodies (ANCA) and antibodies against glomerular basement membrane (anti-GBM) rarely coexist. Both antibodies may be associated with rapidly progressive glomerulonephritis and pulmonary hemorrhage. We describe the clinical, serological and histological features of our patients with dual antibodies. From 1977 to 2008, 48 patients with anti-GBM antibody-associated renal disease were observed. Eight out of the 30 tested patients (26.7%), all females, had positive myeloperoxidase (MPO)-ANCA coexistent with anti-GBM antibodies. The patients' mean age was 63.4 +/- 7.8 years. Five presented with pulmonary-renal syndrome, all but one were dialysis-dependent on admission. They had constitutional symptoms and different organ involvement. The kidney biopsies revealed intense linear staining for immunoglobulin G and C3 along the glomerular and distal tubular basement membrane associated with irregular diffuse or focal extracapillary crescentic glomerulonephritis with necrosis of varying extent. Lesions of varying ages were characteristically expressed. Seven patients were treated with methylprednisolone and plasma exchange, four with cyclophosphamide, and one with intravenous immunoglobulin. After 28-74 months, there were three dialysis-dependent survivors and one patient with stable chronic renal disease. Two clinical relapses with pulmonary involvement and MPO-ANCA positivity without anti-GBM antibodies occurred in two dialysis-dependent patients. In summary, screening for ANCA and anti-GBM antibodies should be undertaken in patients with clinical signs of systemic vasculitis. In dialysis-dependent patients, the goal of treatment is to limit the damage of other involved organs and not to preserve renal function. Careful follow-up is necessary due to the relapsing nature of the ANCA component of the disease.

  17. Glomerular basement membrane composition and the filtration barrier.

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    Miner, Jeffrey H

    2011-09-01

    The glomerular basement membrane (GBM) is an especially thick basement membrane that contributes importantly to the kidney's filtration barrier. The GBM derives from the fusion of separate podocyte and endothelial cell basement membranes during glomerulogenesis and consists primarily of laminin-521 (α5β2γ1), collagen α3α4α5(IV), nidogens-1 and -2, and agrin. Of these nine proteins, mutations in the genes encoding four of them (LAMB2, COL4A3, COL4A4, and COL4A5) cause glomerular disease in humans as well as in mice. Furthermore, mutation of a fifth (Lama5) gene in podocytes in mice causes proteinuria, nephrotic syndrome, and progression to renal failure. These results highlight the importance of the GBM for establishing and maintaining a properly functioning glomerular filtration barrier.

  18. Mouse endometrial stromal cells produce basement-membrane components

    DEFF Research Database (Denmark)

    Wewer, U M; Damjanov, A; Weiss, J

    1986-01-01

    . Mouse decidual cells isolated from 6- to 7-day pregnant uteri explanted in vitro continue to synthesize basement-membrane-like extracellular matrix. Using immunohistochemistry and metabolic labeling followed by immunoprecipitation, SDS-PAGE, and fluorography, it was shown that the decidual cells...... to undergo pseudodecidualization. We thus showed that stromal cells from pregnant and nonpregnant mouse uteri synthesize significant amounts of basement-membrane components in vitro, and hence could serve as a good model for the study of normal basement-membrane components.......During mouse pregnancy, uterine stromal cells transform into morphologically distinct decidual cells under the influence of the implanting embryo and a proper hormonal environment. Mechanical stimulation of hormonally primed uterine stromal cells leads to the same morphologic alterations...

  19. A “Mini-Epidemic” of anti-glomerular basement membrane disease: Clinical and epidemiological study

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    Umesh Lingaraj

    2017-01-01

    Full Text Available Acute glomerulonephritis due to anti-glomerular basement membrane (anti-GBM antibody disease is rare, estimated to occur in fewer than one case per million population and accounts for less than 20% of rapidly progressive glomerulonephritis. The prevalence among patients evaluated for potential glomerular disease is lower. It accounts for fewer than 3% of all kidney biopsies done with crescentic glomerulonephritis. Cases of anti-GBM disease occurring in a cluster have rarely been reported. All biopsy proven anti-GBM disease cases were collected from January 2015 to March 2015 at our Institute. All cases were analyzed for demographic and clinical profile, pathological findings, treatment received and for any common environmental antigenic source. A total of 11 new biopsy proven anti-GBM cases were seen within a span of three months. Age group varied from 17–80 years. Seven were males and four were females. All were dialysis dependent at presentation. Seven had active cellular crescents, and four had fibrocellular. Only one patient was a smoker and none had a history of exposure to any forms of hydrocarbons. The peak seen from January 2015 to March 2015 does not correlate with any of seasonal occurrence of infections in southern India. Although there was clustering of cases to southern territories of Karnataka state, no common etiological agents could be identified. No patient had any previous urological surgeries. All patients received methylprednisolone with plasmapheresis 5–7 sessions and cyclophosphamide. All 11 patients were dialysis dependent at the end of three months. We conclude anti-GBM disease cannot be regarded as a rare cause of renal failure and lung hemorrhage. The occurrence of such epidemic within a short period suggests a possible unidentified environmental factor like infection or occupational agents as inciting agents. Identification of such inciting agents could help us in instituting appropriate preventing measures.

  20. Mouse endometrial stromal cells produce basement-membrane components

    DEFF Research Database (Denmark)

    Wewer, U M; Damjanov, A; Weiss, J

    1986-01-01

    . Mouse decidual cells isolated from 6- to 7-day pregnant uteri explanted in vitro continue to synthesize basement-membrane-like extracellular matrix. Using immunohistochemistry and metabolic labeling followed by immunoprecipitation, SDS-PAGE, and fluorography, it was shown that the decidual cells...

  1. Coarctation induces alterations in basement membranes in the cardiovascular system

    DEFF Research Database (Denmark)

    Lipke, D W; McCarthy, K J; Elton, T S

    1993-01-01

    A coarctation hypertensive rat model was used to examine the effects of elevated blood pressure on basement membrane component synthesis by cardiac myocytes and aorta using immunohistochemistry and Northern blot analysis. Carotid arterial pressure increased immediately on coarctation, and left ve...

  2. Coarctation induces alterations in basement membranes in the cardiovascular system

    DEFF Research Database (Denmark)

    Lipke, D W; McCarthy, K J; Elton, T S

    1993-01-01

    A coarctation hypertensive rat model was used to examine the effects of elevated blood pressure on basement membrane component synthesis by cardiac myocytes and aorta using immunohistochemistry and Northern blot analysis. Carotid arterial pressure increased immediately on coarctation, and left...... with regard to protein and tissue type affected as well as intensity of the changes. However, changes in mRNA levels (but not protein deposition) for perlecan and type IV collagen were also observed in aortas from hypertensive rats compared with controls. Increases in steady-state mRNA levels for all basement...

  3. Basement membrane proteoglycans in glomerular morphogenesis: chondroitin sulfate proteoglycan is temporally and spatially restricted during development

    DEFF Research Database (Denmark)

    McCarthy, K J; Bynum, K; St John, P L

    1993-01-01

    basement membrane (GBM) but present in other basement membranes of the nephron, including collecting ducts, tubules, Bowman's capsule, and the glomerular mesangium. In light of this unique pattern of distribution and of the complex histoarchitectural reorganization occurring during nephrogenesis...

  4. Immunological characterization of a basement membrane-specific chondroitin sulfate proteoglycan

    DEFF Research Database (Denmark)

    McCarthy, K J; Accavitti, M A; Couchman, J R

    1989-01-01

    Reichert's membrane, an extraembryonic membrane present in developing rodents, has been proposed as an in vivo model for the study of basement membranes. We have used this membrane as a source for isolation of basement membrane proteoglycans. Reichert's membranes were extracted in a guanidine/3-[...

  5. Collective cell behavior on basement membranes floating in space

    Science.gov (United States)

    Ellison, Sarah; Bhattacharjee, Tapomoy; Morley, Cameron; Sawyer, W.; Angelini, Thomas

    The basement membrane is an essential part of the polarity of endothelial and epithelial tissues. In tissue culture and organ-on-chip devices, monolayer polarity can be established by coating flat surfaces with extracellular matrix proteins and tuning the trans-substrate permeability. In epithelial 3D culture, spheroids spontaneously establish inside-out polarity, morphing into hollow shell-like structures called acini, generating their own basement membrane on the inner radius of the shell. However, 3D culture approaches generally lack the high degree of control provided by the 2D culture plate or organ-on-chip devices, making it difficult to create more faithful in vitro tissue models with complex surface curvature and morphology. Here we present a method for 3D printing complex basement membranes covered in cells. We 3D print collagen-I and Matrigel into a 3D growth medium made from jammed microgels. This soft, yielding material allows extracellular matrix to be formed as complex surfaces and shapes, floating in space. We then distribute MCF10A epithelial cells across the polymerized surface. We envision employing this strategy to study 3D collective cell behavior in numerous model tissue layers, beyond this simple epithelial model.

  6. Effects of radiation on the permeability of human basement membranes

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    Fan, B.-T.; Achour, S.; Simmonet, F.; Guerin, D.

    1999-02-01

    The influence of radiation on the permeability properties of human basement membrane was investigated by measuring the diffusion rate of several organic compounds (glycine, proline, glucose, urea and insulin) through human anterior lens capsules. The basement membranes borne an γ-irradiation treatment change significantly their permeability vis-a-vis studied organic substances. This modification in physico-chemical properties is probably due to the radiation, which alters or degrades the complex structure (or architecture) of basement membranes. Moreover the change in permeability is dependent upon the diffusing compounds. An increase in diffusion has been observed for glucose, glycine and urea. However for insulin and proline, a decrease in diffusion rate was observed. L'influence de radiation sur la perméabilité de la membrane basale a été étudiée par la mesure de la vitesse de diffusion de plusieurs composés organiques d'intérêt biologique (glycine, proline, glucose, urée et insuline) à travers la lame basale antérieure du cristallin de l'oil humain. Les membranes basales qui sont traitées avec l'irradiation γ changent significativement leur perméabilité vis-à-vis des substances organiques. Ce changement de propriétés physico-chimiques est probablement dû à l'altération ou la dégradation de la structure (ou de l'architecture) de la membrane basale entraînée par l'irradiation. De plus, la modification de la perméabilité de la membrane basale est dépendante des composés diffusants. Une augmentation de la vitesse de diffusion a été observée pour le glucose, le glycine et l'urée. Par contre, dans les cas de l'insuline et de la proline, on a observé une diminution de la vitesse de diffusion.

  7. Immunohistochemical localization of basement membrane components during hair follicle morphogenesis

    DEFF Research Database (Denmark)

    Westgate, G E; Shaw, D A; Harrap, G J

    1984-01-01

    Specific antisera were used to investigate the distributions of several basement membrane zone (BMZ) components, namely, bullous pemphigoid antigen (BPA), heparan sulfate proteoglycan (HSPG), laminin, and type IV collagen, during the development of hair follicles in late embryo rats. BPA was not ......Specific antisera were used to investigate the distributions of several basement membrane zone (BMZ) components, namely, bullous pemphigoid antigen (BPA), heparan sulfate proteoglycan (HSPG), laminin, and type IV collagen, during the development of hair follicles in late embryo rats. BPA...... of the elongating follicle. HSPG was associated with the basal cell layer prior to the appearance of hair follicle primordia and became BMZ-associated before birth but after follicle buds were first observed. HSPG was also found to be associated with the basal cell surfaces in the epidermis, but not in the hair...... follicle. Laminin and type IV collagen were continually present in epidermal and follicular BMZ both before and during development of hair follicles and were later present in the dermal papilla matrix. From these observations we conclude that (1) laminin and type IV collagen are functionally important...

  8. Basement membrane abnormalities in human eyes with diabetic retinopathy

    DEFF Research Database (Denmark)

    Ljubimov, A V; Burgeson, R E; Butkowski, R J

    1996-01-01

    Vascular and parenchymal basement membranes (BMs) are thickened in diabetes, but alterations in individual BM components in diabetic eyes, especially in diabetic retinopathy (DR), are obscure. To identify abnormalities in the distribution of specific constituents, we analyzed cryostat sections...... of human eyes obtained at autopsy (seven normal, five diabetic without DR, and 13 diabetic with DR) by immunofluorescence with antibodies to 30 BM and extracellular matrix components. In non-DR eyes, no qualitative changes of ocular BM components were seen. In some DR corneas, epithelial BM was stained...... discontinuously for laminin-1, entactin/nidogen, and alpha3-alpha4 Type IV collagen, in contrast to non-DR corneas. Major BM alterations were found in DR retinas compared to normals and non-DR diabetics. The inner limiting membrane (retinal BM) of DR eyes had accumulations of fibronectin (including cellular...

  9. Basement membrane abnormalities in human eyes with diabetic retinopathy

    DEFF Research Database (Denmark)

    Ljubimov, A V; Burgeson, R E; Butkowski, R J

    1996-01-01

    discontinuously for laminin-1, entactin/nidogen, and alpha3-alpha4 Type IV collagen, in contrast to non-DR corneas. Major BM alterations were found in DR retinas compared to normals and non-DR diabetics. The inner limiting membrane (retinal BM) of DR eyes had accumulations of fibronectin (including cellular......Vascular and parenchymal basement membranes (BMs) are thickened in diabetes, but alterations in individual BM components in diabetic eyes, especially in diabetic retinopathy (DR), are obscure. To identify abnormalities in the distribution of specific constituents, we analyzed cryostat sections...... of human eyes obtained at autopsy (seven normal, five diabetic without DR, and 13 diabetic with DR) by immunofluorescence with antibodies to 30 BM and extracellular matrix components. In non-DR eyes, no qualitative changes of ocular BM components were seen. In some DR corneas, epithelial BM was stained...

  10. The Acinar Cage: Basement Membranes Determine Molecule Exchange and Mechanical Stability of Human Breast Cell Acini.

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    Aljona Gaiko-Shcherbak

    Full Text Available The biophysical properties of the basement membrane that surrounds human breast glands are poorly understood, but are thought to be decisive for normal organ function and malignancy. Here, we characterize the breast gland basement membrane with a focus on molecule permeation and mechanical stability, both crucial for organ function. We used well-established and nature-mimicking MCF10A acini as 3D cell model for human breast glands, with ether low- or highly-developed basement membrane scaffolds. Semi-quantitative dextran tracer (3 to 40 kDa experiments allowed us to investigate the basement membrane scaffold as a molecule diffusion barrier in human breast acini in vitro. We demonstrated that molecule permeation correlated positively with macromolecule size and intriguingly also with basement membrane development state, revealing a pore size of at least 9 nm. Notably, an intact collagen IV mesh proved to be essential for this permeation function. Furthermore, we performed ultra-sensitive atomic force microscopy to quantify the response of native breast acini and of decellularized basement membrane shells against mechanical indentation. We found a clear correlation between increasing acinar force resistance and basement membrane formation stage. Most important native acini with highly-developed basement membranes as well as cell-free basement membrane shells could both withstand physiologically relevant loads (≤ 20 nN without loss of structural integrity. In contrast, low-developed basement membranes were significantly softer and more fragile. In conclusion, our study emphasizes the key role of the basement membrane as conductor of acinar molecule influx and mechanical stability of human breast glands, which are fundamental for normal organ function.

  11. Pauci-Immune Necrotizing and Crescentic Glomerulonephritis with Membranous Lupus Nephritis, Fifteen Years after Initial Diagnosis of Secondary Membranous Nephropathy

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    Ryan Burkhart

    2015-01-01

    Full Text Available Renal involvement in systemic lupus erythematosus (SLE is usually immune complex mediated and may have multiple different presentations. Pauci-immune necrotizing and crescentic glomerulonephritis (NCGN refers to extensive glomerular inflammation with few or no immune deposits that may result in rapid decline in renal function. We report a case of a 79-year-old Hispanic male with a history of secondary membranous nephropathy (diagnosed by renal biopsy 15 years previously who was admitted with acute kidney injury and active urinary sediment. P-ANCA titers and anti-myeloperoxidase antibodies were positive. The renal biopsy was diagnostic for NCGN superimposed on a secondary membranous nephropathy. A previous diagnosis of SLE based on American College of Rheumatology criteria was discovered via Veteran’s Administration records review after the completion of treatment for pauci-immune NCGN. ANCAs are detected in 20–31% of patients with SLE. There may be an association between SLE and ANCA seropositivity. In patients with lupus nephritis and biopsy findings of necrotizing and crescentic glomerulonephritis, without significant immune complex deposition, ANCA testing should be performed. In patients with secondary membranous nephropathy SLE should be excluded.

  12. Long-term outcome of anti-glomerular basement membrane antibody disease treated with immunoadsorption.

    Directory of Open Access Journals (Sweden)

    Peter Biesenbach

    Full Text Available Anti-glomerular basement membrane (GBM antibody disease may lead to acute crescentic glomerulonephritis with poor renal prognosis. Current therapy favours plasma exchange (PE for removal of pathogenic antibodies. Immunoadsorption (IAS is superior to PE regarding efficiency of antibody-removal and safety. Apart from anecdotal data, there is no systemic analysis of the long-term effects of IAS on anti-GBM-disease and antibody kinetics.To examine the long-term effect of high-frequency IAS combined with standard immunosuppression on patient and renal survival in patients with anti-GBM-disease and to quantify antibody removal and kinetics through IAS.Retrospective review of patients treated with IAS for anti-GBM-antibody disease confirmed by biopsy and/or anti-GBM-antibodies.University Hospital of Vienna, Austria.10 patients with anti-GBM-disease treated with IAS.Patient and renal survival, renal histology, anti-GBM-antibodies.Anti-GBM-antibodies were reduced by the first 9 IAS treatments (mean number of 23 to negative levels in all patients. Renal survival was 40% at diagnosis, 70% after the end of IAS, 63% after one year and 50% at the end of observation (mean 84 months, range 9 to 186. Dialysis dependency was successfully reversed in three of six patients. Patient survival was 90% at the end of observation.IAS efficiently eliminates anti-GBM-antibodies suggesting non-inferiority to PE with regard to renal and patient survival. Hence IAS should be considered as a valuable treatment option for anti-GBM-disease, especially in patients presenting with a high percentage of crescents and dialysis dependency due to an unusual high proportion of responders.

  13. Supportive properties of basement membrane layer of human amniotic membrane enable development of tissue engineering applications.

    Science.gov (United States)

    Iranpour, Sonia; Mahdavi-Shahri, Nasser; Miri, Raheleh; Hasanzadeh, Halimeh; Bidkhori, Hamid Reza; Naderi-Meshkin, Hojjat; Zahabi, Ehsan; Matin, Maryam M

    2018-01-08

    Human amniotic membrane (HAM) has been widely used as a natural scaffold in tissue engineering due to many of its unique biological properties such as providing growth factors, cytokines and tissue inhibitors of metalloproteinases. This study aimed at finding the most suitable and supportive layer of HAM as a delivery system for autologous or allogeneic cell transplantation. Three different layers of HAM were examined including basement membrane, epithelial and stromal layers. In order to prepare the basement membrane, de-epithelialization was performed using 0.5 M NaOH and its efficiency was investigated by histological stainings, DNA quantification, biomechanical testing and electron microscopy. Adipose-derived stromal cells (ASCs) and a human immortalized keratinocyte cell line (HaCaT) were seeded on the three different layers of HAM and cultured for 3 weeks. The potential of the three different layers of HAM to support the attachment and viability of cells were then monitored by histology, electron microscopy and (3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) assay. Moreover, mechanical strengths of the basement membrane were assessed before and after cell culture. The results indicated that the integrity of extra cellular matrix (ECM) components was preserved after de-epithelialization and resulted in producing an intact basement amniotic membrane (BAM). Moreover, all three layers of HAM could support the attachment and proliferation of cells with no visible cytotoxic effects. However, the growth and viability of both cell types on the BAM were significantly higher than the other two layers. We conclude that growth stimulating effectors of BAM and its increased mechanical strength after culturing of ASCs, besides lack of immunogenicity make it an ideal model for delivering allogeneic cells and tissue engineering applications.

  14. The basement membrane and tumor progression in the uterine cervix.

    Science.gov (United States)

    Nair, S A; Nair, M B; Jayaprakash, P G; Rajalekshmy, T N; Nair, M K; Pillai, M R

    1997-06-01

    Immunocytochemical localization of the basement membrane (BM) proteins laminin, type-IV collagen and fibronectin were analyzed in normal cervical epithelium, low grade squamous intraepithelial lesions (SILs), high grade SILs and invasive squamous cell carcinoma (SCC) of the uterine cervix. A regular, thick and continuous BM was present in normal cervical epithelium and low grade SIL. Interruptions and discontinuity of the BM were more evident in high grade SILs. There was a good correlation between increasing severity of the lesion and increasing number of breaks. In SCC, the distribution of laminin, collagen IV and fibronectin was related to the degree of cellular differentiation, with decreased immunoreactivity being evident in moderately and poorly differentiated tumors. As the invasive potential of the tumor increased, the fragmentation and loss of BM was more evident. Fibronectin showed only moderate to mild immunoreactivity in normal cervical epithelium and low grade SILs. However, the intensity of expression increased in high grade SILs especially in the peritumoral stroma. It may therefore be concluded from these results that snythesis and reabsorption of BM proteins may be related to shifts in cellular metabolism during tumorigenesis.

  15. Lack of endothelial nitric oxide synthase aggravates murine accelerated anti-glomerular basement membrane glomerulonephritis

    NARCIS (Netherlands)

    Heeringa, P; van Goor, H; Itoh-Lindstrom, Y; Maeda, N; Falk, RJ; Assmann, KJM; Kallenberg, CGM; Jennette, JC

    Nitric oxide (NO) radicals generated by endothelial nitric oxide synthase (eNOS) are involved in the regulation of vascular tone. In addition, NO radicals derived from eNOS inhibit platelet aggregation and leukocyte adhesion to the endothelium and, thus, may have anti-inflammatory effects. To study

  16. Co-deposition of basement membrane components during the induction of murine splenic AA amyloid

    DEFF Research Database (Denmark)

    Lyon, A W; Narindrasorasak, S; Young, I D

    1991-01-01

    Past studies have demonstrated that during murine AA amyloid induction there is co-deposition of the AA amyloid peptide and the basement membrane form of heparan sulfate proteoglycan. The synthesis and accumulation of heparan sulfate proteoglycan does not usually occur in the absence of other...... basement membrane components, such as type IV collagen, laminin, and fibronectin. Using immunohistochemical techniques, the present experiments have demonstrated that in addition to the heparan sulfate proteoglycan, there are other basement membrane components present in splenic AA amyloid deposits...... and these are present as soon as AA amyloid deposits are detectable. The results indicate that within the time constraints imposed by the experiments, the basement membrane components, fibronectin, laminin, type IV collagen, and heparan sulfate proteoglycan are co-deposited 36 to 48 hours after the AgNO3 and amyloid...

  17. Peroxynitrous acid induces structural and functional modifications to basement membranes and its key component, laminin

    DEFF Research Database (Denmark)

    Degendorfer, Georg; Chuang, Christine Y.; Hammer, Astrid

    2015-01-01

    Basement membranes (BM) are specialized extracellular matrices underlying endothelial cells in the artery wall. Laminin, the most abundant BM glycoprotein, is a structural and biologically active component. Peroxynitrous acid (ONOOH), a potent oxidizing and nitrating agent, is formed in vivo...

  18. Expression of basement membrane components through morphological changes in the hair growth cycle

    DEFF Research Database (Denmark)

    Couchman, J R; Gibson, W T

    1985-01-01

    The amount and distribution of fibronectin associated with hair follicles was found to vary during the hair growth cycle in the rat. Immunocytochemical staining of follicles in mid-late anagen (the growth stage) revealed the presence of fibronectin in the dermal papilla matrix, in the basement...... membrane separating this from the epithelial cells of the hair bulb, and in the basement membrane and connective tissue sheath which underly the cells of the outer root sheath. Early in catagen, the transitional stage, staining of the dermal papilla matrix disappeared. Fibronectin persisted in the basement...

  19. Histones have high affinity for the glomerular basement membrane. Relevance for immune complex formation in lupus nephritis

    International Nuclear Information System (INIS)

    Schmiedeke, T.M.; Stoeckl, F.W.W.; Weber, R.; Sugisaki, Y.; Batsford, S.R.; Vogt, A.

    1989-01-01

    An effort has been made to integrate insights on charge-based interactions in immune complex glomerulonephritis with nuclear antigen involvement in lupus nephritis. Attention was focussed on the histones, a group of highly cationic nuclear constituents, which could be expected to bind to fixed anionic sites present in the glomerular basement membrane (GBM). We demonstrated that all histone subfractions, prepared according to Johns, have a high affinity for GBM and the basement membrane of peritubular capillaries. Tissue uptake of 125 I-labeled histones was measured by injecting 200 micrograms of each fraction into the left kidney via the aorta and measuring organ uptake after 15 min. In glomeruli isolated from the left kidneys, the following quantities of histones were found: f1, 13 micrograms; f2a (f2al + f2a2), 17 micrograms; f2b, 17 micrograms; and f3, 32 micrograms. Kinetic studies of glomerular binding showed that f1 disappeared much more rapidly than f2a. The high affinity of histones (pI between 10.5 and 11.0; mol wt 10,000-22,000) for the GBM correlates well with their ability to form aggregates (mol wt greater than 100,000) for comparison lysozyme (pI 11, mol wt 14,000), which does not aggregate spontaneously bound poorly (0.4 micrograms in isolated glomeruli). The quantity of histones and lysozyme found in the isolated glomeruli paralleled their in vitro affinity for a Heparin-Sepharose column (gradient elution studies). This gel matrix contains the sulfated, highly anionic polysaccharide heparin, which is similar to the negatively charged heparan sulfate present in the GBM. Lysozyme eluted with 0.15 M NaCl, f1 with 1 M NaCl, and f2a, f2b, and f3 could not be fully desorbed even with 2 M NaCl; 6 M guanidine-HCl was necessary

  20. Histones have high affinity for the glomerular basement membrane. Relevance for immune complex formation in lupus nephritis

    Energy Technology Data Exchange (ETDEWEB)

    Schmiedeke, T.M.; Stoeckl, F.W.W.; Weber, R.; Sugisaki, Y.; Batsford, S.R.; Vogt, A.

    1989-06-01

    An effort has been made to integrate insights on charge-based interactions in immune complex glomerulonephritis with nuclear antigen involvement in lupus nephritis. Attention was focussed on the histones, a group of highly cationic nuclear constituents, which could be expected to bind to fixed anionic sites present in the glomerular basement membrane (GBM). We demonstrated that all histone subfractions, prepared according to Johns, have a high affinity for GBM and the basement membrane of peritubular capillaries. Tissue uptake of /sup 125/I-labeled histones was measured by injecting 200 micrograms of each fraction into the left kidney via the aorta and measuring organ uptake after 15 min. In glomeruli isolated from the left kidneys, the following quantities of histones were found: f1, 13 micrograms; f2a (f2al + f2a2), 17 micrograms; f2b, 17 micrograms; and f3, 32 micrograms. Kinetic studies of glomerular binding showed that f1 disappeared much more rapidly than f2a. The high affinity of histones (pI between 10.5 and 11.0; mol wt 10,000-22,000) for the GBM correlates well with their ability to form aggregates (mol wt greater than 100,000) for comparison lysozyme (pI 11, mol wt 14,000), which does not aggregate spontaneously bound poorly (0.4 micrograms in isolated glomeruli). The quantity of histones and lysozyme found in the isolated glomeruli paralleled their in vitro affinity for a Heparin-Sepharose column (gradient elution studies). This gel matrix contains the sulfated, highly anionic polysaccharide heparin, which is similar to the negatively charged heparan sulfate present in the GBM. Lysozyme eluted with 0.15 M NaCl, f1 with 1 M NaCl, and f2a, f2b, and f3 could not be fully desorbed even with 2 M NaCl; 6 M guanidine-HCl was necessary.

  1. Membranous glomerulonephritis in a patient with anti-u1 ribonucleoprotein (RNP antibody-positive mixed connective tissue disease: A case report

    Directory of Open Access Journals (Sweden)

    Naoya Toriu

    2018-03-01

    Full Text Available We report a 33-year-old Japanese man diagnosed with mixed connective tissue disease (MCTD who developed nephrotic proteinuria. Both speckled antinuclear antibody (ANA and anti-U1 ribonucleoprotein (RNP antibody were positive, but anti-double-stranded DNA (dsDNA antibody and anti-Smith (Sm antibody were negative, while complement levels were normal. Renal biopsy revealed membranous glomerulonephritis (MGN with diffuse thickening of the glomerular basement membrane (GBM plus spike and bubble formation. Immunofluorescence demonstrated granular deposits of IgG and C3 along the GBM. Analysis of IgG subclasses showed predominant deposition of IgG1 and IgG4, unlike typical lupus nephritis in which there is predominant deposition of IgG1, IgG2, IgG3, and C1q. Electron microscopy identified numerous large electron-dense deposits (EDD of various types in the subepithelial region of the GBM, but there were no EDD localized in the mesangium or subendothelium. Based on these findings, MGN was considered to be closely related to MCTD in this patient.

  2. Ultrastructure of Reichert's membrane, a multilayered basement membrane in the parietal wall of the rat yolk sac

    OpenAIRE

    1983-01-01

    The ultrastructure of Reichert's membrane, a thick basement membrane in the parietal wall of the yolk sac, has been examined in 13-14-d pregnant rats. This membrane is composed of more or less distinct parallel layers, each one of which resembles a common basement membrane. After routine fixation in glutaraldehyde followed by osmium tetroxide, the layers appear to be mainly composed of 3-8-nm thick cords arranged in a three-dimensional network. Loosely scattered among the cords are unbranched...

  3. Anti-glomerular basement membrane disease: Case series from a tertiary center in North India

    Directory of Open Access Journals (Sweden)

    D Prabhakar

    2017-01-01

    Full Text Available Anti-glomerular basement (anti-GBM disease is an uncommon disorder with a bimodal age of presentation. Patients presenting with dialysis-dependent renal failure have poor renal outcomes. There is limited data regarding the clinical presentation and outcomes of anti-GBM disease from India. We conducted this prospective study to analyze the clinical presentation and outcomes of anti-GBM disease at a large tertiary care hospital in North India over 1½ years. Subjects with a biopsy proven anti-GBM disease (light microscopic examination showing crescents and immunofluorescence examination showing linear deposition of IgG with or without positive anti-GBM antibodies in serum were included in the study and followed-up for at least 12 months. All the patients were treated with steroids, cyclophosphamide, and plasma exchange. A total of 17 patients (nine males were included. The mean age at presentation was 39.11 ± 16.58 (range 11–72 years. Twelve patients (70% presented with rapidly progressive glomerulonephritis (RPGN, 4 (23.5% presented with Goodpasture syndrome, while 1 (5.8% had nephritic syndrome, 7 (41% were hypertensive, and 14 (82.3% required dialysis at the time of presentation. Four patients (23.5% had associated anti-neutrophil cytoplasmic antibody positivity (anti-myeloperoxidase antibodies in all. Fourteen (87.5% patients had crescentic glomerulonephritis, while 5 (31.25% showed necrotizing (n = 4 or granulomatous (n = 1 in the vasculitis. Of 16 patients who received treatment, four (23.25% achieved complete remission. In this single-center study, the majority of anti-GBM disease patients presented with RPGN and had crescentic glomerulonephritis on biopsy with poor treatment outcome.

  4. Accelerating repaired basement membrane after bevacizumab treatment on alkali-burned mouse cornea

    Science.gov (United States)

    Lee, Koon-Ja; Lee, Ji-Young; Lee, Sung Ho; Choi, Tae Hoon

    2013-01-01

    To understand the corneal regeneration induced by bevacizumab, we investigated the structure changes of stroma and basement membrane regeneration. A Stick soaked in 0.5 N NaOH onto the mouse cornea and 2.5 mg/ml of bevacizumab was delivered into an alkali-burned cornea (2 μl) by subconjunctival injections at 1 hour and 4 days after injury. At 7 days after injury, basement membrane regeneration was observed by transmission electron microscope. Uneven and thin epithelial basement membrane, light density of hemidesmosomes, and edematous collagen fibril bundles are shown in the alkali-burned cornea. Injured epithelial basement membrane and hemidesmosomes and edematous collagen fibril bundles resulting from alkali-burned mouse cornea was repaired by bevacizumab treatment. This study demonstrates that bevacizumab can play an important role in wound healing in the cornea by accelerating the reestablishment of basement membrane integrity that leads to barriers for scar formation. [BMB Reports 2013; 46(4): 195-200] PMID:23615260

  5. EBV-positive diffuse large B-cell lymphoma in a patient with primary Sjögren’s syndrome and membranous glomerulonephritis

    Directory of Open Access Journals (Sweden)

    Kim Chang

    2012-11-01

    Full Text Available Abstract Background Sjögren’s syndrome is a systemic autoimmune disease in which lymphatic cells destroy the salivary and lacrimal glands. Glomerulonephritis is thought to be a rare occurrence in primary Sjögren’s syndrome. Furthermore, concurrent glomerular involvement and lymphoma in patients with Sjögren’s syndrome has seldom been reported. Case presentation A 52-year-old woman with primary Sjögren’s syndrome developed membranous glomerulonephritis and Epstein-Barr virus-positive diffuse large B-cell lymphoma (DLBCL. She was diagnosed with Sjögren’s syndrome based on the dry eyes, dry mouth, positive anti-nuclear antibody test, anti-Ro (SS-A antibody, salivary gland biopsy, and salivary scintigraphy. Moreover, renal biopsy confirmed the diagnosis of membranous glomerulonephritis. Three months later, her small bowel was perforated with pneumoperitoneum, and the biopsy revealed Epstein-Barr virus-positive DLBCL. Conclusions We observed the first case of primary Sjögren’s syndrome associated with Epstein-Barr Virus-positive DLBCL and membranous glomerulonephritis. Because of the possibility of malignancy-associated membranous glomerulonephritis in patients with primary Sjögren’s syndrome, we should be careful and examine such patients for hidden malignancy.

  6. Rat hair follicle dermal papillae have an extracellular matrix containing basement membrane components

    DEFF Research Database (Denmark)

    Couchman, J R

    1986-01-01

    Dermal papillae are small mesenchymally derived zones at the bases of hair follicles which have an important role in hair morphogenesis in the embryo and control of the hair growth cycle in postnatal mammals. The cells of the papilla are enmeshed in a dense extracellular matrix which undergoes...... extensive changes in concert with the hair cycle. Here it is shown that this matrix in anagen pelage follicles of postnatal rats contains an abundance of basement membrane components rather than dermal components such as interstitial collagens. In particular, type IV collagen, laminin, and basement membrane...

  7. Heparan sulfate proteoglycans made by different basement-membrane-producing tumors have immunological and structural similarities

    DEFF Research Database (Denmark)

    Wewer, U M; Albrechtsen, R; Hassell, J R

    1985-01-01

    Using immunological assays, we determined the relationship between the heparan sulfate proteoglycans produced by two different murine basement-membrane-producing tumors, i.e., the mouse Engelbreth-Holm-Swarm (EHS) tumor and the L2 rat yolk-sac tumor. Antibodies prepared against the heparan sulfate...... mainly heparan sulfate (75%) along with smaller amounts of chondroitin sulfate (19%), whereas the L2 rat yolk-sac tumor produced mainly chondroitin sulfate (76%) with smaller amounts of heparan sulfate (21%). We conclude that these two murine basement-membrane-producing tumors elaborate...

  8. Expression of basement membrane components through morphological changes in the hair growth cycle

    DEFF Research Database (Denmark)

    Couchman, J R; Gibson, W T

    1985-01-01

    The amount and distribution of fibronectin associated with hair follicles was found to vary during the hair growth cycle in the rat. Immunocytochemical staining of follicles in mid-late anagen (the growth stage) revealed the presence of fibronectin in the dermal papilla matrix, in the basement...... membrane separating this from the epithelial cells of the hair bulb, and in the basement membrane and connective tissue sheath which underly the cells of the outer root sheath. Early in catagen, the transitional stage, staining of the dermal papilla matrix disappeared. Fibronectin persisted in the basement...... of anagen, involving cell division and follicle elongation, was associated with a great increase in the amount of fibronectin in this zone and in and around the dermal papilla. Analysis of entry into anagen by [3H]thymidine incorporation and autoradiography revealed that growth could be detected before...

  9. Evidence for the existence of multiple heparan sulfate proteoglycans in the human glomerular basement membrane and mesangial matrix

    NARCIS (Netherlands)

    Groffen, Alexander J A; Hop, Frank W H; Tryggvason, Karl; Dijkman, Henri; Assmann, Karel J M; Veerkamp, Jacques H.; Monnens, Leo A H; Van Den Heuvel, Lambert P W J

    1997-01-01

    Heparan sulfate proteoglycans (HSPGs) are essential components of the glomerular basement membrane (GBM) carrying a strong anionic charge. A well- characterized extracellular HSPG is perlecan, ubiquitously expressed in basement membranes. A cDNA construct encoding domains I and II of human perlecan

  10. Antibody response against the glomerular basement membrane protein agrin in patients with transplant glomerulopathy.

    NARCIS (Netherlands)

    Joosten, S.A.; Sijpkens, Y.W.; Ham, V. van; Trouw, L.A.; Vlag, J. van der; Heuvel, L.P.W.J. van den; Kooten, C. van; Paul, L.C.

    2005-01-01

    Chronic allograft nephropathy (CAN) of renal allografts is still the most important cause of graft loss. A subset of these patients have transplant glomerulopathy (TGP), characterized by glomerular basement membrane (GBM) duplications, but of unknown etiology. Recently, a role for the immune system

  11. Basement membrane chondroitin sulfate proteoglycan alterations in a rat model of polycystic kidney disease

    DEFF Research Database (Denmark)

    Ehara, T; Carone, F A; McCarthy, K J

    1994-01-01

    Alterations in basement membrane components, notably proteoglycans, in a rat model of polycystic kidney disease have been investigated. Rats were fed phenol II (2-amino-4-hydroxyphenyl-5-phenyl thiazole) for 4 days and then changed to normal diet for a 7-day recovery period. Marked dilation of di...

  12. Macrophage Chemotaxis in Anti-tubular Basement Membrane-Induced Interstitial Nephritis in Guinea Pigs

    NARCIS (Netherlands)

    Kennedy, Thomas L.; Merrow, Martha; Phillips, S. Michael; Norman, Michael; Neilson, Eric G.

    1985-01-01

    Interstitial renal lesions containing T cells and macrophages develop after 14 days in guinea pigs immunized to produce anti-tubular basement membrane-induced interstitial nephritis. We serially examined the renal venous and systemic arterial sera from such animals to determine if chemotactic

  13. Spumiform capillary basement membrane swelling : A new type of microvascular degeneration in senescent hamster

    NARCIS (Netherlands)

    Gerrits, P.O.; Kortekaas, R.; de Weerd, H.; Veenstra-Algra, A.; Luiten, P.G.M.; van der Want, J.J.L.; Veening, Jan

    Brain microvasculature plays a critical role in the regulation of homeostasis of neural tissues. The present study focuses on characteristic microvascular basement membrane (bm) aberrations in the midbrain periaqueductal gray matter (PAG) and their relation to aging. The PAG can be considered a

  14. Spumiform capillary basement membrane swelling: a new type of microvascular degeneration in senescent hamster.

    NARCIS (Netherlands)

    Gerrits, P.O.; Kortekaas, R.; Weerd, H. de; Veenstra-Algra, A.; Luiten, P.G.M.; Want, J.J. van der; Veening, J.G.

    2013-01-01

    Brain microvasculature plays a critical role in the regulation of homeostasis of neural tissues. The present study focuses on characteristic microvascular basement membrane (bm) aberrations in the midbrain periaqueductal gray matter (PAG) and their relation to aging. The PAG can be considered a

  15. Deposition of nucleosomal antigens (histones and DNA) in the epidermal basement membrane in human lupus nephritis.

    NARCIS (Netherlands)

    Grootscholten, C.; Bruggen, M.C.J. van; Pijl, J.W. van der; Jong, E.M.G.J. de; Ligtenberg, G.; Derksen, R.H.W.M.; Berden, J.H.M.

    2003-01-01

    OBJECTIVE: Antinuclear autoantibodies complexed to nucleosomes can bind to heparan sulfate (HS) in the glomerular basement membrane. This binding is due to the binding of the positively charged histones to the strongly anionic HS. Nucleosomes and histones have been identified in glomerular deposits

  16. Involvement of MIF in basement membrane damage in chronically UVB-exposed skin in mice.

    Directory of Open Access Journals (Sweden)

    Yoko Yoshihisa

    Full Text Available Solar ultraviolet (UV B radiation is known to induce matrix metalloproteinases (MMPs that degrade collagen in the basement membrane. Macrophage migration inhibitory factor (MIF is a pluripotent cytokine that plays an essential role in the pathophysiology of skin inflammation induced by UV irradiation. This study examined the effects of MIF on basement membrane damage following chronic UVB irradiation in mice. The back skin of MIF transgenic (Tg and wild-type (WT mice was exposed to UVB three times a week for 10 weeks. There was a decrease in intact protein levels of type IV collagen and increased basement membrane damage in the exposed skin of the MIF Tg mice compared to that observed in the WT mice. Moreover, the skin of the MIF Tg mice exhibited higher MIF, MMP-2 and MMP-9 expression and protein levels than those observed in the WT mice. We also found that chronic UVB exposure in MIF Tg mice resulted in higher levels of neutrophil infiltration in the dermis compared with that observed in the WT mice. In vitro experiments revealed that MIF induced increases in the MMPs expression, including that of MMP-9 in keratinocytes and MMP-2 in fibroblasts. Cultured neutrophils also secreted MMP-9 stimulated by MIF. Therefore, MIF-mediated basement membrane damage occurs primarily through MMPs activation and neutrophil influx in murine skin following chronic UVB irradiation.

  17. Co-deposition of basement membrane components during the induction of murine splenic AA amyloid

    DEFF Research Database (Denmark)

    Lyon, A W; Narindrasorasak, S; Young, I D

    1991-01-01

    Past studies have demonstrated that during murine AA amyloid induction there is co-deposition of the AA amyloid peptide and the basement membrane form of heparan sulfate proteoglycan. The synthesis and accumulation of heparan sulfate proteoglycan does not usually occur in the absence of other bas...

  18. Goodpasture's syndrom. Antiglomerulaer basalmembranantisofmedieret glomerulonephritis

    DEFF Research Database (Denmark)

    Pagsberg, K; Pedersen, G; Hansen, F M

    1989-01-01

    A review is presented of antiglomerular basal membrane antibody-mediated glomerulonephritis (anti-GBM-Ab-nephritis) which constitutes 2-5% of all cases of acute glomerulonephritis. The disease frequently commences in the age group 20-30 years but may be encountered in all age groups, in women par...

  19. Isotropic Versus Bipolar Functionalized Biomimetic Artificial Basement Membranes and Their Evaluation in Long-Term Human Cell Co-Culture.

    Science.gov (United States)

    Rossi, Angela; Wistlich, Laura; Heffels, Karl-Heinz; Walles, Heike; Groll, Jürgen

    2016-08-01

    In addition to dividing tissues into compartments, basement membranes are crucial as cell substrates and to regulate cellular behavior. The development of artificial basement membranes is indispensable for the ultimate formation of functional engineered tissues; however, pose a challenge due to their complex structure. Herein, biodegradable electrospun polyester meshes are presented, exhibiting isotropic or bipolar bioactivation as a biomimetic and biofunctional model of the natural basement membrane. In a one-step preparation process, reactive star-shaped prepolymer additives, which generate a hydrophilic fiber surface, are electrospun with cell-adhesion-mediating peptides, derived from major components of the basement membrane. Human skin cells adhere to the functionalized meshes, and long-term co-culture experiments confirm that the artificial basement membranes recapitulate and preserve tissue specific functions. Several layers of immortalized human keratinocytes grow on the membranes, differentiating toward the surface and expressing typical epithelial markers. Fibroblasts migrate into the reticular lamina mimicking part of the mesh. Both cells types begin to produce extracellular matrix proteins and to remodel the initial membrane. It is shown at the example of skin that the artificial basement membrane design provokes biomimetic responses of different cell types and can thus be used as basis for the future development of basement membrane containing tissues. © 2016 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  20. Accelerating repaired basement membrane after bevacizumab treatment on alkali-burned mouse cornea

    Directory of Open Access Journals (Sweden)

    Koon-Ja Lee

    2013-04-01

    Full Text Available To understand the corneal regeneration induced by bevacizumab,we investigated the structure changes of stroma andbasement membrane regeneration. A Stick soaked in 0.5 NNaOH onto the mouse cornea and 2.5 mg/ml of bevacizumabwas delivered into an alkali-burned cornea (2 μl by subconjunctivalinjections at 1 hour and 4 days after injury. At 7 daysafter injury, basement membrane regeneration was observedby transmission electron microscope. Uneven and thin epithelialbasement membrane, light density of hemidesmosomes,and edematous collagen fibril bundles are shown in thealkali-burned cornea. Injured epithelial basement membraneand hemidesmosomes and edematous collagen fibril bundlesresulting from alkali-burned mouse cornea was repaired bybevacizumab treatment. This study demonstrates that bevacizumabcan play an important role in wound healing in thecornea by accelerating the reestablishment of basementmembrane integrity that leads to barriers for scar formation.[BMB Reports 2013; 46(4: 195-200

  1. Alterations of epithelial adhesion molecules and basement membrane components in lattice corneal dystrophy (LCD).

    Science.gov (United States)

    Resch, Miklós D; Schlötzer-Schrehardt, Ursula; Hofmann-Rummelt, Carmen; Kruse, Friedrich E; Seitz, Berthold

    2009-08-01

    The aim of the study was to investigate the histopathological and ultrastructural correlate of delayed epithelial healing in eyes with lattice corneal dystrophy (LCD). Corneal buttons from 4 patients with LCD (two with subepithelial, two with stromal amyloid deposits) and 2 control corneas were examined. Cell-matrix adhesion molecules and basement membrane components of the corneal epithelium were analyzed by immunohistochemistry and hemidesmosomes between epithelium and stroma were quantified by transmission electron microscopy (TEM). By TEM well-developed hemidesmosomes anchored the basal epithelial cells to the underlying basement membrane in all normal and LCD corneas. Hemidesmosome density was not significantly different in subepithelial (224.7 +/- 34.1/100 microm) and stromal (234.3 +/- 36.3/100 microm) LCD compared to controls (241.3 +/- 26.8/100 microm). The basement membrane was interrupted in subepithelial, but continuous in stromal LCD. Integrin alpha6 and beta4 staining formed a continuous line along the basal surface of the corneal epithelium in control corneas, whereas it appeared discontinuous and patchy both in subepithelial and stromal forms of LCD. Staining for alphaV integrin showed irregular staining patterns, i.e. enhanced labelling intensity in subepithelial and interrupted pattern in stromal LCD, respectively. Integrins alpha3, beta1, beta2, and beta5, dystroglycan, and plectin were not markedly different in dystrophic corneas. Type VII collagen showed a discontinuous staining in subepithelial forms of LCD. In stromal forms of LCD, type VII collagen staining occurred in additional patches underneath the epithelial basement membrane zone. Type XVII collagen staining was reduced in subepithelial LCD. Laminin-1, laminin-5 and laminin gamma2 showed variable irregular staining patterns in dystrophic corneas with focal interruptions, focal thickenings, and reduplications of basement membrane. Some irregularities in corneas with subepithelial

  2. Immunochemical and autoantigenic properties of the globular domain of basement membrane collagen (type IV).

    Science.gov (United States)

    von der Mark, H; Oberbäumer, I; Timpl, R; Kemler, R; Wick, G

    1985-02-01

    Polyclonal rabbit antibodies raised against the globular domain NC1 of collagen IV from human placenta and a mouse tumor react with conformational antigenic determinants present on the NC1 hexamers and also with the three major subunits obtained after dissociation. The antibodies recognized unique structures within basement membranes and showed a broad tissue reactivity but only limited species cross-reactivity. Using these antibodies, it was possible to detect small amounts of collagen IV antigens from cell cultures and in serum. Monoclonal rat antibodies against mouse NC1 revealed a similar reaction potential. Autoantibodies could be produced in mice against mouse NC1 which react with kidney and lung basement membranes in a pathological manner, mimicking Goodpasture syndrome.

  3. The basement membrane constituents in the mouse embryo's tooth. An autoradiographic study

    International Nuclear Information System (INIS)

    Osman, M.

    1987-01-01

    Enamel organs isolated from the lower first teeth of 18-days old white mouse embryo by trypsin treatment were used in this study. The organs were cultured during periods of increasing time on a semi-solid medium containing cock serum. In another chase experiments, the organs were cultured on a liquid medium containing proline- 3 H, leucine- 3 H, and glucosamine- 3 H, were studied by autoradiography using both light and electron microscopes. It has been shown that the nature of the culture medium does not apparently interfere with the ability of the enamel to reconstitute the basement membrane. On the other hand, it have been found obvious differences concerning the kinetic of the used isotopes. The results indicate that the turn-over of the basement membrane constituents represents a continuous and homogenous process which continues to take place during, before and after reconstitution. 42 refs. (author)

  4. [Effect of collagenase on the permeability of the glomerular basement membrane in the rat kidney].

    Science.gov (United States)

    Laloi, C; Geloso-Meyer, A; Cheignon, M; Schaeverbeke, J

    1981-03-16

    Recently, several authors have emphasized the role of negative sites located in th laminae rarae of the glomerular basement membrane (GBM), in restricting glomerular permeability to anionic macromolecules. In this work, we point out that ultrafiltration properties involve integrity of the GBM. Indeed after intravenous perfusion of bacterian collagenase, anionic ferritin permeates the GBM though negative site distribution (as shown by fixation of colloidal iron) is unaffected.

  5. The timeline of lamellar basement membrane changes during equine laminitis development.

    Science.gov (United States)

    Visser, M B; Pollitt, C C

    2011-07-01

    The timing of lamellar basement membrane (BM) changes occurring during laminitis development is incompletely understood. To determine the temporal progression of lamellar BM changes and whether laminin-332 (Ln-332) γ2 cleavage products are generated during laminitis development. Eight clinically normal Standardbred horses were allocated into treatment (n = 5) or sham (n = 3) groups. The treatment group received, via nasogastric intubation, an oligofructose (OF) bolus (10 g/kg bwt) while the sham group was given water. Laminitis induction proceeded for 48 h followed by euthanasia. Lamellar biopsies were obtained prior to dosing and at intervals during the treatment period for analysis (at 12, 18, 24, 30 and 36 h and at 48 h following euthanasia). Changes in lamellar collagen type IV and Ln-332 were first observed at 12 h post dosing. A unique pattern of reactivity for the Ln-332 γ2 antibody D4B5 occurred, in which reactivity was observed only in lamellar tissue affected by laminitis. No bioactive Ln-332 γ2 proteolytic fragments were detected in lamellar samples. Basement membrane changes occurred early during the laminitis process. Direct Ln-332 γ2 cleavage to release biologically active products did not appear to occur. Thus loss of stability or protein interaction of the BM is probably responsible for the γ2 specific reactivity observed. Basement membrane changes may a first step in lamellar failure occurring prior to detection with conventional methods. Thus, more sensitive detection methods of BM changes are required to study laminitis development. © 2011 EVJ Ltd.

  6. Normal mammary epithelial cells promote carcinoma basement membrane invasion by inducing microtubule-rich protrusions.

    Science.gov (United States)

    Lee, Meng-Horng; Wu, Pei-Hsun; Gilkes, Daniele; Aifuwa, Ivie; Wirtz, Denis

    2015-10-20

    Recent work suggests that the dissemination of tumor cells may occur in parallel with, and even preceed, tumor growth. The mechanism for this early invasion is largely unknown. Here, we find that mammary epithelial cells (MECs) induce neighboring breast carcinoma cells (BCCs) to cross the basement membrane by secreting soluble laminin. Laminin continuously produced by MECs induce long membrane cellular protrusions in BCCs that promote their contractility and invasion into the surrounding matrix. These protrusions depend on microtubule bundles assembled de novo through laminin-integrin β1 signaling. These results describe how non-cancerous MECs can actively participate in the invasive process of BCCs.

  7. Regeneration of the epidermis and basement membrane of the planarian Dugesia japonica after total-body x irradiation

    International Nuclear Information System (INIS)

    Hori, I.

    1979-01-01

    Fresh-water planarians were studied to examine effects of x rays on regeneration of the epidermis and basement membrane. During early stages of regeneration, free rhabdite-forming cells were associated with the wound epidermis and recruited it. In later stages, however, a gradual degeneration occurred in the epidermis and cells undergoing epithelization decreased in number. Eventually epidermal cells on the wound surface appeared necrotic as evidenced by pyknotic nuclei and vacuolized dense cytoplasm. The entire basement membrane could not be reconstituted in any stage after wounding though its precursor-like material was secreted in the interspace between epidermis and parenchyma. Morphological changes in extracellular products and in the cells surrounding the products suggest that epidermal cells which have covered the wound surface synthesize precursors of the basement membrane. Possible factors of a characteristic perturbation in epithelization and basement membrane formation after total-body irradiation are discussed

  8. Regeneration of the epidermis and basement membrane of the planarian Dugesia japonica after total-body x irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Hori, I.

    1979-03-01

    Fresh-water planarians were studied to examine effects of x rays on regeneration of the epidermis and basement membrane. During early stages of regeneration, free rhabdite-forming cells were associated with the wound epidermis and recruited it. In later stages, however, a gradual degeneration occurred in the epidermis and cells undergoing epithelization decreased in number. Eventually epidermal cells on the wound surface appeared necrotic as evidenced by pyknotic nuclei and vacuolized dense cytoplasm. The entire basement membrane could not be reconstituted in any stage after wounding though its precursor-like material was secreted in the interspace between epidermis and parenchyma. Morphological changes in extracellular products and in the cells surrounding the products suggest that epidermal cells which have covered the wound surface synthesize precursors of the basement membrane. Possible factors of a characteristic perturbation in epithelization and basement membrane formation after total-body irradiation are discussed.

  9. Anti-glomerular basement membrane: A rare cause of renal failure in children

    Directory of Open Access Journals (Sweden)

    Indira Agarwal

    2017-01-01

    Full Text Available Anti-glomerular basement membrane (GBM disease is a rare cause of acute renal failure and known to have bad prognosis regarding renal functions recovery and patient survival specially when diagnosed late and presents with severe renal failure that requires dialysis. We report a case of 11-year-old child with acute renal failure secondary to anti-GBM disease and associated with antineutrophil cytoplasmic antibody-positive vasculitis. He was treated with plasmapheresis, steroids, and cyclophosphamide with recovery of his kidney functions.

  10. Basement membrane heparan sulfate proteoglycan from the L2 rat yolk sac carcinoma

    DEFF Research Database (Denmark)

    Fenger, M; Wewer, U; Albrechtsen, R

    1984-01-01

    Heparan sulfate proteoglycan from the L2 rat yolk sac carcinoma has been purified and partially characterized. The proteoglycan has an apparent Mr of 750 000, 35% of which represents the core protein. The core protein seems to be homogeneous, whereas the heparan sulfate chains are heterogeneous...... with an Mr of about 50 000-70 000, with 30% of the glucosamine being N-sulfated. Antibodies raised against the core protein of the heparan sulfate proteoglycan reacted with basement membranes of various rat and human tissue....

  11. Immunochemical and ultrastructural assessment of the nature of the pericellular basement membrane of human decidual cells

    DEFF Research Database (Denmark)

    Wewer, U M; Faber, M; Liotta, L A

    1985-01-01

    Human decidual cells of early and late pregnancy were studied immunochemically and ultrastructurally with respect to the presence and nature of pericellular basement membrane material. The most prominent cell type in decidual tissue of both early and late pregnancy were large, mature epithelioid...... of stromal cells into decidual cells of the pregnant endometrium. Predecidualization of the human endometrium, which is seen in the late secretory phase of the normal menstrual cycle and in some states of hyperplasia, was also shown to be accompanied by the presence of deposits of laminin-positive material...

  12. In vivo turnover of the basement membrane and other heparan sulfate proteoglycans of rat glomerulus

    DEFF Research Database (Denmark)

    Beavan, L A; Davies, M; Couchman, J R

    1989-01-01

    The metabolic turnover of rat glomerular proteoglycans in vivo was investigated. Newly synthesized proteoglycans were labeled during a 7-h period after injecting sodium [35S]sulfate intraperitoneally. At the end of the labeling period a chase dose of sodium sulfate was given. Subsequently......-propanesulfonate-4 M guanidine hydrochloride, a procedure which solubilized greater than 95% of the 35S-labeled macromolecules. Of these 11-13% was immunoprecipitated by an antiserum against heparan sulfate proteoglycan which, in immunolocalization experiments, showed specificity for staining the basement membrane...

  13. Increasingly transformed MCF-10A cells have a progressively tumor-like phenotype in three-dimensional basement membrane culture.

    Science.gov (United States)

    Imbalzano, Karen M; Tatarkova, Iva; Imbalzano, Anthony N; Nickerson, Jeffrey A

    2009-03-16

    MCF-10A cells are near diploid and normal human mammary epithelial cells. In three-dimensional reconstituted basement membrane culture, they undergo a well-defined program of proliferation, differentiation, and growth arrest, forming acinar structures that recapitulate many aspects of mammary architecture in vivo. The pre-malignant MCF-10AT cells and malignant MCF-10CA1a lines were sequentially derived from the MCF-10A parental cell line first by expression of a constitutively active T24 H-Ras generating the MCF-10AT cell line. This was followed by repeated selection for increasingly aggressive tumor formation from cells recovered from xenograft tumors in immuno-compromised mice, generating the MCF-10CA1a cell line. When inoculated subcutaneously into the flanks of immuno-compromised mice, MCF-10AT cells occasionally form tumors, whereas MCF-10CA1a cells invariably form tumors with a shorter latency than MCF-10AT derived tumors. MCF-10AT cells grown in three-dimensional basement membrane culture form complex multi-acinar structures that produce a basement membrane but undergo delayed cell cycle arrest and have incomplete luminal development. MCF-10CA1a cells grown in three-dimensional basement membrane culture form large, hyper-proliferative masses, that retain few characteristics of MCF10A acini and more closely resemble tumors. Here we report on the growth and differentiation properties of these three matched cell lines in three-dimensional basement membrane culture. Features of tissue morphogenesis were assessed, including proliferation, basement membrane formation, polarization of alpha-6 beta-4 integrin to the basement membrane, formation of cell:cell junctions, and apoptosis for luminal clearance. The matched series of normal MCF-10A, pre-malignant MCF-10AT, and malignant MCF-10CA1a cells offers a unique opportunity to study the mechanisms of malignant progression both in a three-dimensional microenvironment and in the same cell background.

  14. Organogenesis of the kidney glomerulus: focus on the glomerular basement membrane.

    Science.gov (United States)

    Miner, Jeffrey H

    2011-01-01

    The glomerular basement membrane (GBM) is a crucial component of the kidney's filtration barrier that separates the vasculature from the urinary space. During glomerulogenesis, the GBM is formed from fusion of two distinct basement membranes, one synthesized by the glomerular epithelial cell (podocyte) and the other by the glomerular endothelial cell. The main components of the GBM are laminin-521 (α5β2γ1), collagen α3α4α5(IV), nidogen and the heparan sulfate proteoglycan, agrin. By studying mice lacking specific GBM components, we have shown that during glomerulogenesis, laminin is the only one that is required for GBM integrity and in turn, the GBM is required for completion of glomerulogenesis and glomerular vascularization. In addition, our results from laminin β2-null mice suggest that laminin-521, and thus the GBM, contribute to the establishment and maintenance of the glomerular filtration barrier to plasma albumin. In contrast, mutations that affect GBM collagen IV or agrin do not impair glomerular development or cause immediate leakage of plasma proteins. However, collagen IV mutation, which causes Alport syndrome and ESRD in humans, leads to gradual damage to the GBM that eventually leads to albuminuria and renal failure. These results highlight the importance of the GBM for establishing and maintaining a perfectly functioning, highly selective glomerular filter.

  15. Morphological diagnosis of Alport syndrome and thin basement membrane nephropathy by low vacuum scanning electron microscopy.

    Science.gov (United States)

    Okada, Shinichi; Inaga, Sumire; Kitamoto, Koichi; Kawaba, Yasuo; Nakane, Hironobu; Naguro, Tomonori; Kaidoh, Toshiyuki; Kanzaki, Susumu

    2014-01-01

    Alport syndrome (AS) and thin basement membrane nephropathy (TBMN) are genetic disorders caused by mutations of the type IV collagen genes COL4A3, COL4A4, and/or COL4A5. We here aimed to investigate the three-dimensional ultrastructure of the glomerular basement membrane (GBM) in order to introduce a novel method of diagnosing AS and TBMN. The subjects were 4 patients with AS and 6 patients with TBMN. Conventional renal biopsy paraffin sections from AS and TBMN patients were stained with periodic acid methenamine silver (PAM) and observed directly under low vacuum scanning electron microscopy (LVSEM). The PAM-positive GBMs were clearly visible under LVSEM through the overlying cellular components. The GBMs showed characteristic coarse meshwork appearances in AS, and thin and sheet-like appearances in TBMN. At the cut side view of the capillary wall, the GBMs in AS appeared as fibrous inclusions between a podocyte and an endothelial cell, while the GBMs in TBMN showed thin linear appearances. These different findings of GBMs between AS and TBMN were easily observed under LVSEM. Thus, we conclude that three-dimensional morphological evaluation by LVSEM using conventional renal biopsy paraffin sections will likely be useful for the diagnosis of AS and TBMN, including for retrospective investigations.

  16. The chest X-ray in antiglomerular basement membrane antibody disease (Goodpasture's syndrome)

    International Nuclear Information System (INIS)

    Bowley, N.B.; Steiner, R.E.; Chin, W.S.

    1979-01-01

    The chest radiographs of 25 patients with proven antiglomerular basement membrane antibody disease (Goodpasture's syndrome) were analysed. All except two of the patients had pulmonary haemorrhage at some stage of their disease. Altogether there were 39 episodes of pulmonary haemorrhage, 25 being relapses. During seven episodes the chest radiograph was normal. Relapses of pulmonary haemorrhage never occurred in isolation but were usually associated with infection (not necessarily a chest infection) or occasionally fluid overload. Conversely fluid overload or infection were always associated with pulmonary haemorrhage provided there were high or rising titres of circulating antibodies at the time. Therefore in a patient with antiglomerular basement membrane antibody disease, the presence of shadowing in the lung fields on the chest radiograph almost invariably means the patient has pulmonary haemorrhage whether or not pulmonary oedema or a chest infection are present. Limitation of shadowing by a fissure, loss of major portions of the diaphragmatic or cardiac silhouette, involvement of the lung apex or costophrenic angles suggest an underlying chest infection. Septal lines suggest fluid overload. Pleural effusions are seen with chest infections and fluid overload. The carbon monoxide uptake (KCO) was invariably high in the presence of pulmonary haemorrhage even if the chest radiograph was normal. A combined use of KCO and chest radiographs is the best method of monitoring lung disease in these patients. (author)

  17. Effect of diabetes on in vivo metabolism of [35S]-labeled glomerular basement membrane

    International Nuclear Information System (INIS)

    Cohen, M.P.; Surma, M.L.

    1984-01-01

    Glomerular basement membrane (GBM) was labeled in vivo by the injection of tracer amounts of [ 35 S]-sulfate into normal and streptozotocin-diabetic rats. The biosynthesis and turnover of sulfated glycosaminoglycans in the GBM was determined from the specific activity of [ 35 S] after pronase digestion of basement membranes purified from glomeruli isolated 1-7 days after injection. Peak radiolabeling of both normal and diabetic GBM occurred 24 h after injection and, when corrected for differences in serum sulfate specific activities, was less in diabetic than in normal samples. The specific activity of GBM sulfate, expressed as cpm/microgram uronic acid, progressively diminished over the ensuing period of study in both normal and diabetic samples. The rate of decrease in specific activity of [ 35 S]-labeled GBM was not significantly different in diabetic preparations compared with that in normal controls. The findings are compatible with diminished sulfation and/or production but normal turnover of glycosaminoglycans in the renal GBM in experimental diabetes

  18. Specific fixation of bovine brain and retinal acidic and basic fibroblast growth factors to mouse embryonic eye basement membranes

    International Nuclear Information System (INIS)

    Jeanny, J.C.; Fayein, N.; Courtois, Y.; Moenner, M.; Chevallier, B.; Barritault, D.

    1987-01-01

    The labeling pattern of mouse embryonic eye frozen sections incubated with radioiodinated brain acidic and basic fibroblasts growth factors (aFGF and bFGF) was investigated by autoradiography. Both growth factors bind to basement membranes in a dose-dependent way, with a higher affinity for bFGF. Similar data were obtained with eye-derived growth factors (EDGF), the retinal forms of FGF. There was a heterogeneity in the affinity of the various basement membranes toward these growth factors. The specificity of the growth factor-basement membrane interaction was demonstrated by the following experiments: (i) an excess of unlabeled growth factor displaced the labeling; (ii) unrelated proteins with different isoelectric points did not modify the labeling; and (iii) iodinated EGF or PDGF did not label basement membrane. In order to get a better understanding of the nature of this binding, the authors performed the incubation of the frozen sections with iodinated FGFs preincubated with various compounds. These results demonstrate that FGFs bind specifically to basement membranes, probably on the polysaccharidic part of the proteoheparan sulfate, and suggest that this type of interaction may be a general feature of the mechanism of action of these growth factors

  19. Laminin, a noncollagenous component of epithelial basement membranes synthesized by a rat yolk sac tumor

    DEFF Research Database (Denmark)

    Wewer, U; Albrechtsen, R; Ruoslahti, E

    1981-01-01

    Laminin, a glycoprotein antigenically similar or identical to a component of epithelial basement membranes, was identified as a major component of the abundant extracellular matrix synthesized by an experimentally induced rat yolk sac tumor. Immunocytochemical staining revealed laminin in cultured...... polypeptides with molecular weights of approximately 200,000 and 400,000. These comigrated with the polypeptides of mouse laminin isolated previously. The yolk sac tumor tissue grown in vivo contained laminin in the tumor cells and in the extracellular material as evidenced by immunofluorescence...... membranes in rat tissues in a manner indistinguishable from antilaminin. The presence of laminin in rat yolk sac cells, the presumed origin of our yolk sac tumor, was studied in some detail. Laminin was found to be present in normal cells of the visceral as well as the parietal yolk sac layer...

  20. Human skin basement membrane-associated heparan sulphate proteoglycan: distinctive differences in ultrastructural localization as a function of developmental age

    DEFF Research Database (Denmark)

    Horiguchi, Y; Fine, J D; Couchman, J R

    1991-01-01

    Recent studies have demonstrated that skin basement membrane components are expressed within the dermo-epidermal junction in an orderly sequence during human foetal development. We have investigated the ultrastructural localization of basement membrane-related antigens in human foetal skin...... at different developmental ages using two monoclonal antibodies to a well-characterized basement membrane-associated heparan sulphate proteoglycan. A series of foetal skin specimens (range, 54-142 gestational days) were examined using an immunoperoxidase immunoelectron microscopic technique. In specimens...... representing very early developmental ages, very diffuse immunoreaction products were detected. However, by approximately 76 gestational days, some accentuation of heparan sulphate proteoglycan was noted along the lamina densa, and by 142 gestational days, the distribution of heparan sulphate proteoglycan...

  1. Nephritogenic antigen determinants in epidermal and renal basement membranes of kindreds with Alport-type familial nephritis.

    Science.gov (United States)

    Kashtan, C; Fish, A J; Kleppel, M; Yoshioka, K; Michael, A F

    1986-10-01

    We probed epidermal basement membranes (EBM) of acid-urea denatured skin from members of kindreds with Alport-type familial nephritis (FN) for the presence of antigens reactive with Goodpasture sera (GPS) and serum (FNS) from an Alport patient who developed anti-glomerular basement membrane (GBM) nephritis in a renal allograft. By immunoblotting, GPS reacted primarily with the 28,000 molecular weight (mol wt) monomer but also the 24,000 mol wt and 26,000 mol wt monomers of the noncollagenous globular domain (NC1) of type IV collagen from normal human GBM, while FNS identified only the 26,000-mol wt monomer. FNS reacted with EBM of 12 controls and nine unaffected male kindred members but not EBM of eight affected males. Five affected females exhibited interrupted reactivity of FNS with EBM. GPS showed variable reactivity with EBM and was not discriminating with respect to Alport-type FN. FNS did not stain renal basement members of five affected males. However, the EBM, tubular basement membrane, and Bowman's capsules of affected males contained antigens reactive with GPS. These immunochemical studies suggest that the FNS antigen is distinct from Goodpasture antigen(s). The expression of FNS antigen located on the NC1 domain of type IV collagen is altered in basement membranes of patients with Alport-type FN, and the distribution of this antigenic anomaly within kindreds suggests X-linked dominant transmission of a defective gene.

  2. Distribution of individual components of basement membrane in human colon polyps and adenocarcinomas as revealed by monoclonal antibodies

    DEFF Research Database (Denmark)

    Ljubimov, A V; Bartek, J; Couchman, J R

    1992-01-01

    -membrane components (laminin, entactin/nidogen, collagen type IV and large heparan sulfate proteoglycan), as well as to keratin 8. In all adenocarcinomas, including mucinous, basement membranes were altered more at the invasive front than in the parenchyma. The degree of this alteration was inversely correlated......-membrane components and to a specific keratin may constitute an adequate immunohistochemical test for the presence of invasion, and may be useful in the histologic analysis of polyps, especially in dubious cases....

  3. Nephritogenic antigen determinants in epidermal and renal basement membranes of kindreds with Alport-type familial nephritis.

    OpenAIRE

    Kashtan, C; Fish, A J; Kleppel, M; Yoshioka, K; Michael, A F

    1986-01-01

    We probed epidermal basement membranes (EBM) of acid-urea denatured skin from members of kindreds with Alport-type familial nephritis (FN) for the presence of antigens reactive with Goodpasture sera (GPS) and serum (FNS) from an Alport patient who developed anti-glomerular basement membrane (GBM) nephritis in a renal allograft. By immunoblotting, GPS reacted primarily with the 28,000 molecular weight (mol wt) monomer but also the 24,000 mol wt and 26,000 mol wt monomers of the noncollagenous ...

  4. [Relationship between the changes in ischemia/reperfusion cerebro-microvessel basement membrane injury and gelatinase system in senile rat].

    Science.gov (United States)

    Li, Jian-sheng; Liu, Ke; Liu, Jing-xia; Wang, Ming-hang; Zhao, Yue-wu; Liu, Zheng-guo

    2008-11-01

    To study the relationship of cerebro-microvessel basement membrane injury and gelatinase system after cerebral ischemia/reperfusion (I/R) in aged rats. Cerebral I/R injury model was reproduced by intraluminal silk ligature thrombosis of the middle cerebral artery occlusion (MCAO). Rats were divided randomly into sham control and I/R groups in young rats [ischemia 3 hours (I 3 h) and reperfusion 6 hours (I/R 6 h), 12 hours (I/R 12 h), 24 hours (I/R 24 h), 3 days (I/R 3 d), 6 days (I/R 6 d)], and sham control group and I/R group in aged rats (I 3 h and I/R 6 h, I/R 12 h, I/R 24 h , I/R 3 d, I/R 6 d). The change in cerebro-cortex microvessel basement membrane structure, basement membrane type IV collagen (Col IV) and laminin (LN) contents, matrix metalloproteinases (MMPs) and tissue inhibitor of metalloproteinases (TIMPs) expression in every group were determined with immunohistochemical method and zymogram analysis. With the increase in age, Col IV and LN contents of the microvessel basement membrane were increased, and MMP-2 and MMP-9 expressions were stronger. With prolongation of I/R, the degradation of microvessel basement membrane components (Col IV and LN) was positively correlated with the duration of cerebral I/R. MMP-2 expression was increased gradually, and MMP-9 and TIMP-1 expression increased at the beginning and decreased subsequently. Col IV(I 3 h, I/R 6 h , I/R 12 h), LN (I 3 h, I/R 6-24 h), MMP-2 (I 3 h, I/R 6 h-6 d) and MMP-9 (I 3 h, I/R 6-24 h) expression level in aged rats with I/R injury were higher, and TIMP-1 (I/R 24 h) expression was lower than those in young rats (Pcerebro-microvessel basement membrane in rats is related with MMPs and TIMP. Cerebro-microvessel basement membrane injury is more serious in aged rats than that of young rats. Changes in cerebro-microvessel basement membrane injury in aged rats is related with gelatinase system change.

  5. Ultrastructure of Reichert's membrane, a multilayered basement membrane in the parietal wall of the rat yolk sac.

    Science.gov (United States)

    Inoué, S; Leblond, C P; Laurie, G W

    1983-11-01

    The ultrastructure of Reichert's membrane, a thick basement membrane in the parietal wall of the yolk sac, has been examined in 13-14-d pregnant rats. This membrane is composed of more or less distinct parallel layers, each one of which resembles a common basement membrane. After routine fixation in glutaraldehyde followed by osmium tetroxide, the layers appear to be mainly composed of 3-8-nm thick cords arranged in a three-dimensional network. Loosely scattered among the cords are unbranched, straight tubular structures with a diameter of 7-10 nm, which mainly run parallel to the surface and to one another; they are referred to as basotubules. Permanganate fixation emphasizes the presence of a thick feltwork of irregular material around basotubules. Finally, minute dot-like structures measuring 3.5 nm and referred to as double pegs are present within the meshes of the cord network. Reichert's membranes have been treated for 2-48 h at 25 degrees C with plasmin, a proteolytic enzyme known to rapidly digest laminin and fibronectin. After a 2-h treatment, most of the substance of the cords is digested away leaving a three-dimensional network of 1.5-2.0-nm thick filaments. The interpretation is that the cords are formed of a plasmin-resistant core filament and a plasmin-extractable sheath. When plasmin treatment is prolonged for 15 h or longer, the filaments are dissociated and disappear, while basotubules are maintained. Plasmin digestion also reveals that basotubules are composed of two parts: a ribbon-like helical wrapping and tubule proper. Further changes in the tubule under plasmin influence are interpreted as a dissociation into pentagonal units suggestive of the presence of the amyloid P component. After 48 h of plasmin treatment, basotubules are further disaggregated and dispersed, leaving only linearly arranged double pegs. Reichert's membranes with or without a 2-hr plasmin treatment have been immunostained by exposure to antibodies against either laminin or

  6. Cell Receptor-Basement Membrane Interactions in Health and Disease: a Kidney-Centric View

    Science.gov (United States)

    Borza, Corina M.; Chen, Xiwu; Zent, Roy; Pozzi, Ambra

    2016-01-01

    Cell-extracellular matrix (ECM) interactions are essential for tissue development, homeostasis, and response to injury. Basement membranes (BMs) are specialized ECMs that separate epithelial or endothelial cells from stromal components and interact with cells via cellular receptors, including integrins and discoidin domain receptors. Disruption of cell-BM interactions due to either injury or genetic defects in either the ECM components or cellular receptors often lead to irreversible tissue injury and loss of organ function. Animal models that lack specific BM components or receptors either globally or in selective tissues have been used to help with our understanding of the molecular mechanisms whereby cell-BM interactions regulate organ function in physiological and pathological conditions. We review recently published work on animal models that explore how cell-BM interactions regulate kidney homeostasis in both health and disease. PMID:26610916

  7. Heterogeneous distribution of a basement membrane heparan sulfate proteoglycan in rat tissues

    DEFF Research Database (Denmark)

    Couchman, J R

    1987-01-01

    A heparan sulfate proteoglycan (HSPG) synthesized by murine parietal yolk sac (PYS-2) cells has been characterized and purified from culture supernatants. A monospecific polyclonal antiserum was raised against it which showed activity against the HSPG core protein and basement membrane specificity...... for multiple HSPGs was derived from the finding that skeletal neuromuscular junction and liver epithelia also did not contain this type of HSPG, though previous reports have indicated the presence of HSPGs at these sites. The PYS-2 HSPG was shown to be antigenically related to the large, low buoyant density...... HSPG from the murine Engelbreth-Holm swarm tumor. It was, however, confirmed that only a single population of antibodies was present in the serum. Despite the presence of similar epitopes on these two proteoglycans of different hydrodynamic properties, it was apparent that the PYS-2 HSPG represents...

  8. Investigation of basement membrane proteins in a case of granular cell ameloblastoma

    Science.gov (United States)

    Lapthanasupkul, Puangwan; Poomsawat, Sopee; Chindasombatjaroen, Jira

    2012-01-01

    Granular cell ameloblastoma is a rare, benign neoplasm of the odontogenic epithelium. A case of massive granular cell ameloblastoma in a 44-year-old Thai female is reported. Histopathological features displayed a follicular type of ameloblastoma with an accumulation of granular cells residing within the tumor follicles. After treatment by partial mandibulectomy, the patient showed a good prognosis without recurrence in a 2-year follow-up. To characterize the granular cells in ameloblastoma, we examined the expression of basement membrane (BM) proteins, including collagen type IV, laminins 1 and 5 and fibronectin using immunohistochemistry. Except for the granular cells, the tumor cells demonstrated a similar expression of BM proteins compared to follicular and plexiform ameloblastomas in our previous study, whereas the granular cells showed strong positivity to laminins 1 and 5 and fibronectin. The increased fibronectin expression in granular cells suggests a possibility of age-related transformation of granular cells in ameloblastoma. PMID:22361945

  9. Therapeutic Mechanism of Glucocorticoids on Cellular Crescent Formation in Patients With Antiglomerular Basement Membrane Disease.

    Science.gov (United States)

    Wu, Xiaomei; Zhang, Mingchao; Huang, Xiao; Zhang, Lihua; Zeng, Caihong; Zhang, Jiong; Liu, Zhihong; Tang, Zheng

    2017-08-01

    This study aimed to explore the therapeutic mechanism of glucocorticoids (GCs) in antiglomerular basement membrane disease. Thirty-four patients with biopsy-proven antiglomerular basement membrane nephritis were divided into the following 2 groups: group 1 (patients treated with GCs, n = 22) and group 2 (patients who were not treated with GCs, n = 12). The expression of parietal epithelial cells (PECs), activated PECs and glucocorticoid receptors (GRs) was examined quantitatively and compared between the 2 groups. Correlations between GR expression in glomeruli and patients' clinicopathological indices were also analyzed. Compared with patients in group 2, patients in group 1 showed lower levels of serum creatinine (SCr) (P = 0.03), average cellular crescent percentage (P = 0.005) and macrophages infiltrating in renal interstitium (P = 0.03). PECs (P = 0.007) and activated PECs (P = 0.03) were strongly detected in the cellular components of classic crescents, and both were significantly reduced in group 1 compared to group 2. GR expression either in glomeruli (P = 0.01) or interstitium (P = 0.009) was lower in group 1 after GCs treatment than in group 2. Additionally, GR expression in glomeruli was strongly correlated with renal function (SCr: r = 0.45, P = 0.009; eGFR: r = -0.35, P = 0.046), the proportion of cellular crescents (r = 0.67, P < 0.001), PECs (r = 0.64, P < 0.001) and activated PECs (r = 0.72, P < 0.001), and the degree of interstitial (r = 0.50, P = 0.004) and glomerular (r = 0.49, P = 0.007) macrophage infiltration. GCs might exert their therapeutic effects via inhibiting the activation and proliferation of PECs, as well as macrophage infiltration, which could contribute to crescent formation and determine renal survival. GRs are involved in this process as well. Copyright © 2017 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

  10. Cloning and chromosomal mapping of mouse ladinin, a novel basement membrane zone component

    Energy Technology Data Exchange (ETDEWEB)

    Motoki, K.; Megahed, M.; LaForgia, S.; Uitto, J. [Thomas Jefferson Univ., Philadelphia, PA (United States)

    1997-02-01

    Linear IgA disease is characterized by circulating IgA autoantibodies recognizing basement membrane zone components, including an anchoring filament protein, ladinin. In this study, we have cloned the mouse ladinin cDNA, elucidated the intron-exon organization of the corresponding gene (Lad1), and determined its chromosomal assignment. We have also characterized the promoter region of Lad1 and examined its tissue-specific expression. The mouse Lad1 gene consists of 10 exons spanning {approximately}13.4 kb of the mouse genome on chromosome 1, and Southern analysis suggested that Lad1 is a single-copy gene. The coding region comprises 1584 nucleotides and encodes a 528-amino-acid polypeptide with a calculated molecular mass of 59 kDa. The deduced polypeptide contained two putative N-glycosylation and two O-glycosylation sites, and sequence analysis predicted a 15-amino-acid signal peptide. The 5{prime} upstream region demonstrated the presence of consensus cis-elements for AP2 and SP1 and was GC rich, consistent with eukaryotic promoter. Northern analysis revealed expression in cultured keratinocytes, but not in fibroblasts, with the mRNA transcript being {approximately}2.5 kb in size. A significant level of expression was also noted in the kidney and lung, and to a lesser degree in the liver, spleen, and brain. Ladinin is a novel component of the basement membranes and may function in contributing to the stability of the association of the epithelial layers with the underlying mesenchyme. 39 refs., 4 figs.

  11. [3H]glucosamine and [3H]proline radioautography of embryonic mouse dental basement membrane

    International Nuclear Information System (INIS)

    Osman, M.; Ruch, J.V.

    1981-01-01

    [ 3 H]proline and [ 3 H]glucosamine radioautography was performed to analyze the labeling pattern of mouse embryonic dental basement membrane before and during odontoblast terminal differentiation. Sixteen- and eighteen-day-old first lower molars and trypsin-isolated enamel organs, as well as EDTA-isolated dental papillae, were used. Continuous labeling for 12 to 24 hr was required with [ 3 H]proline to obtain a clear labeling of epithelial-mesenchymal junction in intact tooth germs or accumulation of surface label in trypsin-isolated enamel organs. With [ 3 H]glucosamine, after 6-hr labeling, the epithelial-mesenchymal junction was heavily labeled and the trypsin-isolated enamel organs accumulated substantial amounts of surface label, corresponding to the redeposited basement membrane. At Day 16 stage, these labels always had a uniform distribution and decreased during chase without any redistribution. At Day 18 stage, when the terminal differentiation of odontoblasts occurred the label accumulated in a unique pattern: much more label was at the epithelial surface corresponding to the top of the cusps than in the apical parts. During chase and only in intact tooth germs epithelial surfaces which had labeled poorly during pulse became labeled, but those labeling heavily during pulse lost label. This pattern existed only in the presence of mesenchyme. EDTA treatment of [ 3 H]glucosamine-labeled teeth enabled us to obtain isolated dental papillae with surface label. Distribution of this label was exactly the same as that for the epithelial-mesenchymal junction of intact teeth. During chase, these dental papillae completely lost the surface label. The mesenchyme seen to control the synthesis and/or the degradation of epithelially derived [ 3 H]glucosamine-labeled material

  12. In vivo turnover of the basement membrane and other heparan sulfate proteoglycans of rat glomerulus

    International Nuclear Information System (INIS)

    Beavan, L.A.; Davies, M.; Couchman, J.R.; Williams, M.A.; Mason, R.M.

    1989-01-01

    The metabolic turnover of rat glomerular proteoglycans in vivo was investigated. Newly synthesized proteoglycans were labeled during a 7-h period after injecting sodium [35S]sulfate intraperitoneally. At the end of the labeling period a chase dose of sodium sulfate was given. Subsequently at defined times (0-163 h) the kidneys were perfused in situ with 0.01% cetylpyridinium chloride in phosphate-buffered saline to maximize the recovery of 35S-proteoglycans. Glomeruli were isolated from the renal cortex and analyzed for 35S-proteoglycans by autoradiographic, biochemical, and immunochemical methods. Grain counting of autoradiographs revealed a complex turnover pattern of 35S-labeled macromolecules, commencing with a rapid phase followed by a slower phase. Biochemical analysis confirmed the biphasic pattern and showed that the total population of [35S]heparan sulfate proteoglycans had a metabolic half-life (t1/2) of 20 and 60 h in the early and late phases, respectively. Heparan sulfate proteoglycans accounted for 80% of total 35S-proteoglycans, the remainder being chondroitin/dermatan sulfate proteoglycans. Whole glomeruli were extracted with 4% 3-[(cholamidopropyl)dimethy-lammonio]-1-propanesulfonate-4 M guanidine hydrochloride, a procedure which solubilized greater than 95% of the 35S-labeled macromolecules. Of these 11-13% was immunoprecipitated by an antiserum against heparan sulfate proteoglycan which, in immunolocalization experiments, showed specificity for staining the basement membrane of rat glomeruli. Autoradiographic analysis showed that 18% of total radioactivity present at the end of the labeling period was associated with the glomerular basement membrane

  13. The Peri-islet Basement Membrane, a Barrier to Infiltrating Leukocytes in Type 1 Diabetes in Mouse and Human

    DEFF Research Database (Denmark)

    Korpos, Eva; Kadri, Nadir; Kappelhoff, Reinhild

    2013-01-01

    We provide the first comprehensive analysis of the extracellular matrix (ECM) composition of peri-islet capsules, composed of the peri-islet basement membrane (BM) and subjacent interstitial matrix (IM), in development of type 1 diabetes in NOD mice and in human type 1 diabetes. Our data demonstr...

  14. Ultrastructural localization of the core protein of a basement membrane-specific chondroitin sulfate proteoglycan in adult rat skin

    DEFF Research Database (Denmark)

    McCarthy, K J; Horiguchi, Y; Couchman, J R

    1990-01-01

    Basement membranes are complex extracellular matrices present at epithelial/mesenchymal interfaces of tissues. The dermal-epidermal junction has been shown to contain numerous components, some of the most well known being laminin, types IV and VII collagens, heparan sulfate proteoglycan, fibronec...

  15. Distribution of two basement membrane proteoglycans through hair follicle development and the hair growth cycle in the rat

    DEFF Research Database (Denmark)

    Couchman, J R; King, J L; McCarthy, K J

    1990-01-01

    The distribution of two distinct populations of basement membrane proteoglycans has been monitored through hair growth development in the rat embryo and subsequent hair growth cycle. An antiserum against a small heparan sulfate proteoglycan uniformly stained the dermal-epidermal junction of embry...

  16. Direct Observation of Early-Stage High-Dose Radiotherapy-Induced Vascular Injury via Basement Membrane-Targeting Nanoparticles.

    Science.gov (United States)

    Au, Kin Man; Hyder, Sayed Nabeel; Wagner, Kyle; Shi, Caihong; Kim, Young Seok; Caster, Joseph M; Tian, Xi; Min, Yuanzeng; Wang, Andrew Z

    2015-12-22

    Collagen IV-targeting peptide-conjugated basement membrane-targeting nanoparticles are successfully engineered to identify early-stage blood vessel injury induced by high-dose radiotherapy. © 2015 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  17. Electron microscopic study of the myelinated nerve fibres and the perineurial cell basement membrane in the diabetic human peripheral nerves

    International Nuclear Information System (INIS)

    ElBarrany, Wagih G.; Hamdy, Raid M.; AlHayani, Abdulmonem A.; Jalalah, Sawsan M.

    2009-01-01

    To study the quantitative and ultrastructural changes in myelinated nerve fibers and the basement membranes of the perineurial cells in diabetic nerves. The study was performed at the Department of Anatomy, Faculty of Medicine, King Abdul-Aziz University, Jeddah, Saudi Arabia from 2003 to 2005. Human sural nerves were obtained from 15 lower limbs and 5 diabetic nerve biopsies. The total mean and density of myelinated nerve fibers per fascicle were calculated, with density of microtubules and mitochondria in the axoplasm. The number of the perineurial cell basement membrane layers was counted, and thickness of the basement membrane was measured. Among the 15 diabetic and 5 normal human sural nerves, the average diameters, number and surface area of myelinated nerve fibers and axonal microtubules density were found to be less in diabetic nerves. Mitochondrial density was higher in diabetic axons. Thickness of the perineurial cell basement membrane had a greater mean, but the number of perineurial cell layers was less than that of the diabetic group. The inner cellular layer of the perineurium of the diabetic nerves contained large vacuoles containing electron-dense degenerated myelin. A few specimens showed degenerated myelinated nerve fibers, while others showed recovering ones. Retracted axoplasms were encountered with albumin extravasation. Diabetes caused an increase in perineurial permeability. The diabetic sural nerve showed marked decrease in the myelinated nerve fibres, increase degenerated mitochondria, and decreased microtubules. (author)

  18. Role of 17 beta-estradiol on type IV collagen fibers volumetric density in the basement membrane of bladder wall.

    Science.gov (United States)

    de Fraga, Rogerio; Dambros, Miriam; Miyaoka, Ricardo; Riccetto, Cássio Luís Zanettini; Palma, Paulo César Rodrigues

    2007-10-01

    The authors quantified the type IV collagen fibers volumetric density in the basement membrane of bladder wall of ovariectomized rats with and without estradiol replacement. This study was conducted on 40 Wistar rats (3 months old) randomly divided in 4 groups: group 1, remained intact (control); group 2, submitted to bilateral oophorectomy and daily replacement 4 weeks later of 17 beta-estradiol for 12 weeks; group 3, sham operated and daily replacement 4 weeks later of sesame oil for 12 weeks; and group 4, submitted to bilateral oophorectomy and killed after 12 weeks. It was used in immunohistochemistry evaluation using type IV collagen polyclonal antibody to stain the fibers on paraffin rat bladder sections. The M-42 stereological grid system was used to analyze the fibers. Ovariectomy had an increase effect on the volumetric density of the type IV collagen fibers in the basement membrane of rat bladder wall. Estradiol replacement in castrated animals demonstrated a significative difference in the stereological parameters when compared to the castrated group without hormonal replacement. Surgical castration performed on rats induced an increasing volumetric density of type IV collagen fibers in the basement membrane of rats bladder wall and the estradiol treatment had a significant effect in keeping a low volumetric density of type IV collagen fibers in the basement membrane of rats bladder wall.

  19. Spumiform basement membrane aberrations in the microvasculature of the midbrain periaqueductal gray region in hamster : Rostro-caudal pathogenesis?

    NARCIS (Netherlands)

    Gerrits, P.O.; Kortekaas, R.; de Weerd, Heleen; Luiten, P.G.M.; van der Want, J.J.L.; Veening, Jan

    2013-01-01

    Spumiform basement membrane degeneration (sbmd) is a specific kind of aberration present in the capillaries of the midbrain periaqueductal gray (PAG) region of the senescent hamster. These capillaries, separated by the ependymal cell layer, are bordering the Sylvian cerebral aqueduct. The aqueduct,

  20. Spumiform basement membrane aberrations in the microvasculature of the midbrain periaqueductal gray region in hamster: rostro-caudal pathogenesis?

    NARCIS (Netherlands)

    Gerrits, P.O.; Kortekaas, R.; Weerd, H. de; Luiten, P.G.M.; Want, J.J. van der; Veening, J.G.

    2013-01-01

    Spumiform basement membrane degeneration (sbmd) is a specific kind of aberration present in the capillaries of the midbrain periaqueductal gray (PAG) region of the senescent hamster. These capillaries, separated by the ependymal cell layer, are bordering the Sylvian cerebral aqueduct. The aqueduct,

  1. Ultrastructural localization of the core protein of a basement membrane-specific chondroitin sulfate proteoglycan in adult rat skin

    DEFF Research Database (Denmark)

    McCarthy, K J; Horiguchi, Y; Couchman, J R

    1990-01-01

    , fibronectin, and entactin/nidogen. IN this paper we show, using core protein-specific antibodies, the presence of a newly described basement membrane-specific chondroitin sulfate proteoglycan at the epithelial/mesenchymal interface of adult rat skin. Ultrastructurally, this antigen was proven to reside...

  2. Increasingly transformed MCF-10A cells have a progressively tumor-like phenotype in three-dimensional basement membrane culture

    Directory of Open Access Journals (Sweden)

    Imbalzano Anthony N

    2009-03-01

    Full Text Available Abstract Background MCF-10A cells are near diploid and normal human mammary epithelial cells. In three-dimensional reconstituted basement membrane culture, they undergo a well-defined program of proliferation, differentiation, and growth arrest, forming acinar structures that recapitulate many aspects of mammary architecture in vivo. The pre-malignant MCF-10AT cells and malignant MCF-10CA1a lines were sequentially derived from the MCF-10A parental cell line first by expression of a constitutively active T24 H-Ras generating the MCF-10AT cell line. This was followed by repeated selection for increasingly aggressive tumor formation from cells recovered from xenograft tumors in immuno-compromised mice, generating the MCF-10CA1a cell line. When inoculated subcutaneously into the flanks of immuno-compromised mice, MCF-10AT cells occasionally form tumors, whereas MCF-10CA1a cells invariably form tumors with a shorter latency than MCF-10AT derived tumors. Results MCF-10AT cells grown in three-dimensional basement membrane culture form complex multi-acinar structures that produce a basement membrane but undergo delayed cell cycle arrest and have incomplete luminal development. MCF-10CA1a cells grown in three-dimensional basement membrane culture form large, hyper-proliferative masses, that retain few characteristics of MCF10A acini and more closely resemble tumors. Conclusion Here we report on the growth and differentiation properties of these three matched cell lines in three-dimensional basement membrane culture. Features of tissue morphogenesis were assessed, including proliferation, basement membrane formation, polarization of alpha-6 beta-4 integrin to the basement membrane, formation of cell:cell junctions, and apoptosis for luminal clearance. The matched series of normal MCF-10A, pre-malignant MCF-10AT, and malignant MCF-10CA1a cells offers a unique opportunity to study the mechanisms of malignant progression both in a three

  3. Mesangioproliferative glomerulonephritis: An important glomerulonephritis in nephrotic syndrome of young adult

    Directory of Open Access Journals (Sweden)

    Usha

    2008-07-01

    Full Text Available Mesangioproliferative glomerulonephritis (MesPGN consists 10% of the total renal biopsy of glomerulonephritis. Aim of the present study was to find out clinicopathological changes in MesPGN and differences between diffuse and focal variety. MesPGN was seen mostly in young adults with mean age of 28.63 years for males and 26.3 years for females. Male predominance was noted (M:F ratio - 1.4:1. About 70.83% patient presented with edema feet, followed by hypertension (29.19%, fever (16.66%, oliguria, nausea and vomiting (10.41%. Urine analysis in 50 patients revealed that 70% patients presented with nephrotic-range proteinuria, 36% patients with microscopic hematuria and 56% patients with leukocyturia. Statistically, no significant difference was found in clinical features of diffuse and focal MesPGN. Microscopic comparison between diffuse and focal variety showed that significant increase of focal glomerular basement membrane thickening, focal endothelial cell proliferation, focal smooth muscle hyperplasia, hyaline sclerosis and vasculitis was more common in diffuse variety. In focal variety, Capillary loop congestion, periglomerulitis, cloudy swelling and vacuolar degeneration in tubules were significantly more as compared to diffuse variety. Details of the clinical features, special laboratory tests and histological details revealed that diffuse variety had systemic diseases, which included Wegner′s granulomatosis, microscopic polyangitis, Henoch′s schonlein purpura, systemic lupus erythematosus (two cases and one case each of Kimura′s disease, pyelonephritis and tuberculosis. Only one case of focal MesPGN showed tuberculosis. Thus, our study concludes that MesPGN is an important cause of nephrotic syndrome among young adults. Secondly, search for some other diseases should be made and thirdly, if biopsy shows focal mesangial cell proliferations in minimal change glomerulonephritis (MCGN, it should be diagnosed as focal MesPGN rather than

  4. Quantitative image analysis of laminin immunoreactivity in skin basement membrane irradiated with 1 GeV/nucleon iron particles.

    Science.gov (United States)

    Costes, S; Streuli, C H; Barcellos-Hoff, M H

    2000-10-01

    We previously reported that laminin immunoreactivity in mouse mammary epithelium is altered shortly after whole-body irradiation with 0.8 Gy from 600 MeV/nucleon iron ions but is unaffected after exposure to sparsely ionizing radiation. This observation led us to propose that the effect could be due to protein damage from the high ionization density of the ion tracks. If so, we predicted that it would be evident soon after radiation exposure in basement membranes of other tissues and would depend on ion fluence. To test this hypothesis, we used immunofluorescence, confocal laser scanning microscopy, and image segmentation techniques to quantify changes in the basement membrane of mouse skin epidermis. At 1 h after exposure to 1 GeV/nucleon iron ions with doses from 0.03 to 1.6 Gy, neither the visual appearance nor the mean pixel intensity of laminin in the basement membrane of mouse dorsal skin epidermis was altered compared to sham-irradiated tissue. This result does not support the hypothesis that particle traversal directly affects laminin protein integrity. However, the mean pixel intensity of laminin immunoreactivity was significantly decreased in epidermal basement membrane at 48 and 96 h after exposure to 0.8 Gy 1 GeV/nucleon iron ions. We confirmed this effect with two additional antibodies raised against affinity-purified laminin 1 and the E3 fragment of the long-arm of laminin 1. In contrast, collagen type IV, another component of the basement membrane, was unaffected. Our studies demonstrate quantitatively that densely ionizing radiation elicits changes in skin microenvironments distinct from those induced by sparsely ionizing radiation. Such effects may might contribute to the carcinogenic potential of densely ionizing radiation by altering cellular signaling cascades mediated by cell-extracellular matrix interactions.

  5. MT1-MMP-mediated basement membrane remodeling modulates renal development

    Energy Technology Data Exchange (ETDEWEB)

    Riggins, Karen S.; Mernaugh, Glenda [Department of Cancer Biology, Vanderbilt University Medical Center, Nashville, TN 37232 (United States); Su, Yan; Quaranta, Vito [Division of Nephrology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN 37232 (United States); Koshikawa, Naohiko; Seiki, Motoharu [Division of Cancer Cell Research, Institute of Medical Science, The University of Tokyo, Shirokanedai, Minato-ku, Tokyo 108-8639 (Japan); Pozzi, Ambra [Division of Nephrology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN 37232 (United States); Department of Cancer Biology, Vanderbilt University Medical Center, Nashville, TN 37232 (United States); Department of Research Medicine, Veterans Affairs Hospital, Nashville, TN 37232 (United States); Zent, Roy, E-mail: roy.zent@vanderbilt.edu [Division of Nephrology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN 37232 (United States); Department of Cancer Biology, Vanderbilt University Medical Center, Nashville, TN 37232 (United States); Department of Cell and Developmental Biology, Vanderbilt University Medical Center, Nashville, TN 37232 (United States); Department of Research Medicine, Veterans Affairs Hospital, Nashville, TN 37232 (United States)

    2010-10-15

    Extracellular matrix (ECM) remodeling regulates multiple cellular functions required for normal development and tissue repair. Matrix metalloproteinases (MMPs) are key mediators of this process and membrane targeted MMPs (MT-MMPs) in particular have been shown to be important in normal development of specific organs. In this study we investigated the role of MT1-MMP in kidney development. We demonstrate that loss of MT1-MMP leads to a renal phenotype characterized by a moderate decrease in ureteric bud branching morphogenesis and a severe proliferation defect. The kidneys of MT1-MMP-null mice have increased deposition of collagen IV, laminins, perlecan, and nidogen and the phenotype is independent of the MT-1MMP target, MMP-2. Utilizing in vitro systems we demonstrated that MTI-MMP proteolytic activity is required for renal tubule cells to proliferate in three dimensional matrices and to migrate on collagen IV and laminins. Together these data suggest an important role for MT1-MMP in kidney development, which is mediated by its ability to regulate cell proliferation and migration by proteolytically cleaving kidney basement membrane components.

  6. MT1-MMP-mediated basement membrane remodeling modulates renal development

    International Nuclear Information System (INIS)

    Riggins, Karen S.; Mernaugh, Glenda; Su, Yan; Quaranta, Vito; Koshikawa, Naohiko; Seiki, Motoharu; Pozzi, Ambra; Zent, Roy

    2010-01-01

    Extracellular matrix (ECM) remodeling regulates multiple cellular functions required for normal development and tissue repair. Matrix metalloproteinases (MMPs) are key mediators of this process and membrane targeted MMPs (MT-MMPs) in particular have been shown to be important in normal development of specific organs. In this study we investigated the role of MT1-MMP in kidney development. We demonstrate that loss of MT1-MMP leads to a renal phenotype characterized by a moderate decrease in ureteric bud branching morphogenesis and a severe proliferation defect. The kidneys of MT1-MMP-null mice have increased deposition of collagen IV, laminins, perlecan, and nidogen and the phenotype is independent of the MT-1MMP target, MMP-2. Utilizing in vitro systems we demonstrated that MTI-MMP proteolytic activity is required for renal tubule cells to proliferate in three dimensional matrices and to migrate on collagen IV and laminins. Together these data suggest an important role for MT1-MMP in kidney development, which is mediated by its ability to regulate cell proliferation and migration by proteolytically cleaving kidney basement membrane components.

  7. Lack of collagen XVIII/endostatin exacerbates immune-mediated glomerulonephritis.

    Science.gov (United States)

    Hamano, Yuki; Okude, Takashi; Shirai, Ryota; Sato, Ikumi; Kimura, Ryota; Ogawa, Makoto; Ueda, Yoshihiko; Yokosuka, Osamu; Kalluri, Raghu; Ueda, Shiro

    2010-09-01

    Collagen XVIII is a component of the highly specialized extracellular matrix associated with basement membranes of epithelia and endothelia. In the normal kidney, collagen XVIII is distributed throughout glomerular and tubular basement membranes, mesangial matrix, and Bowman's capsule. Proteolytic cleavage within its C-terminal domain releases the fragment endostatin, which has antiangiogenic properties. Because damage to the glomerular basement membrane (GBM) accompanies immune-mediated renal injury, we investigated the role of collagen XVIII/endostatin in this disorder. We induced anti-GBM glomerulonephritis in collagen XVIII alpha1-null and wild-type mice and compared the resulting matrix accumulation, inflammation, and capillary rarefaction. Anti-GBM disease upregulated collagen XVIII/endostatin expression within the GBM and Bowman's capsule of wild-type mice. Collagen XVIII/endostatin-deficient mice developed more severe glomerular and tubulointerstitial injury than wild-type mice. Collagen XVIII/endostatin deficiency altered matrix remodeling, enhanced the inflammatory response, and promoted capillary rarefaction and vascular endothelial cell damage, but did not affect endothelial proliferation. Supplementing collagen XVIII-deficient mice with exogenous endostatin did not affect the progression of anti-GBM disease. Taken together, these results suggest that collagen XVIII/endostatin preserves the integrity of the extracellular matrix and capillaries in the kidney, protecting against progressive glomerulonephritis.

  8. Autoantibodies to myeloperoxidase aggravate mild anti-glomerular-basement-membrane-mediated glomerular injury in the rat

    NARCIS (Netherlands)

    Heeringa, P.; Brouwer, E.; Klok, P. A.; Huitema, M. G.; van den Born, J.; Weening, J. J.; Kallenberg, C. G.

    1996-01-01

    Autoantibodies to myeloperoxidase (MPO) are present in sera from patients with various forms of vasculitis-associated glomerulonephritis. Evidence for a pathogenic role of anti-MPO antibodies has been provided mainly by in vitro studies. We studied the pathogenic role of autoantibodies to MPO in a

  9. Functional differentiation and alveolar morphogenesis of primary mammary cultures on reconstituted basement membrane

    Energy Technology Data Exchange (ETDEWEB)

    BARCELLOS-HOFF, M. H; AGGELER, J.; RAM, T. G; BISSELL, M. J

    1989-02-01

    An essential feature of mammary gland differentiation during pregnancy is the formation of alveoli composed of polarized epithelial cells, which, under the influence of lactogenic hormones, secrete vectorially and sequester milk proteins. Previous culture studies have described either organization of cells polarized towards lumina containing little or no demonstrable tissue-specific protein, or establishment of functional secretory cells exhibiting little or no glandular architecture. In this paper, we report that tissue-specific vectorial secretion coincides with the formation of functional alveoli-like structures by primary mammary epithelial cells cultured on a reconstituted basement membrane matrix (derived from Engelbreth-Holm-Swarm murine tumour). Morphogenesis of these unique three-dimensional structures was initiated by cell-directed remodelling of the exogenous matrix leading to reorganization of cells into matrixensheathed aggregates by 24 h after plating. The aggregates subsequently cavitated, so that by day 6 the cells were organized into hollow spheres in which apical cell surfaces faced lumina sealed by tight junctions and basal surfaces were surrounded by a distinct basal lamina. The profiles of proteins secreted into the apical (luminal) and basal (medium) compartments indicated that these alveoli-like structures were capable of an appreciable amount of vectorial secretion. Immunoprecipitation with a broad spectrum milk antiserum showed that more than 80% of caseins were secreted into the lumina, whereas iron-binding proteins (both lactoferrin and transferrin) were present in comparable amounts in each compartment. Thus, these mammary cells established protein targeting pathways directing milk-specific proteins to the luminal compartment. A time course monitoring secretory activity demonstrated that establishment of tissue-specific vectorial secretion and increased total and milk protein secretion coincided with functional alveolar

  10. Crosslinked basement membrane-based coatings enhance glucose sensor function and continuous glucose monitoring in vivo.

    Science.gov (United States)

    Klueh, Ulrike; Ludzinska, Izabela; Czajkowski, Caroline; Qiao, Yi; Kreutzer, Donald L

    2018-01-01

    Overcoming sensor-induced tissue reactions is an essential element of achieving successful continuous glucose monitoring (CGM) in the management of diabetes, particularly when used in closed loop technology. Recently, we demonstrated that basement membrane (BM)-based glucose sensor coatings significantly reduced tissue reactions at sites of device implantation. However, the biocompatible BM-based biohydrogel sensor coating rapidly degraded over a less than a 3-week period, which effectively eliminated the protective sensor coating. In an effort to increase the stability and effectiveness of the BM coating, we evaluated the impact of crosslinking BM utilizing glutaraldehyde as a crosslinking agent, designated as X-Cultrex. Sensor performance (nonrecalibrated) was evaluated for the impact of these X-Cultrex coatings in vitro and in vivo. Sensor performance was assessed over a 28-day time period in a murine CGM model and expressed as mean absolute relative difference (MARD) values. Tissue reactivity of Cultrex-coated, X-Cultrex-coated, and uncoated glucose sensors was evaluated over a 28-day time period in vivo using standard histological techniques. These studies demonstrated that X-Cultrex-based sensor coatings had no effect on glucose sensor function in vitro. In vivo, glucose sensor performance was significantly enhanced following X-Cultrex coating throughout the 28-day study. Histological evaluations of X-Cultrex-treated sensors demonstrated significantly less tissue reactivity when compared to uncoated sensors. © 2017 Wiley Periodicals, Inc. J Biomed Mater Res Part A: 106A: 7-16, 2018. © 2017 Wiley Periodicals, Inc.

  11. Immunohistochemical expression of basement membrane proteins of verrucous carcinoma of the oral mucosa.

    Science.gov (United States)

    Arduino, Paolo G; Carrozzo, Marco; Pagano, Marco; Broccoletti, Roberto; Scully, Crispian; Gandolfo, Sergio

    2010-06-01

    Squamous cell carcinoma (SCC) of the oral cavity is an extremely invasive tumour of stratified squamous epithelium that spreads throughout degradation of the basement membrane (BM) and extra-cellular matrix. Oral verrucous carcinoma (VC) is a rare low-grade variant of oral SCC that penetrates into the subepithelial connective tissue. It also has a different clinical behaviour from classical oral SCC. We investigated the immunohistochemical expression of laminin, laminin-5, collagen IV and fibronectin in VC, severe epithelial dysplasia (SED) and SCC in order to analyse if the pattern of these molecules expression contributes to the differences in the biological behaviour of these diseases. The staining pattern of laminin was less intensive in SCC compared with SED and VC, and collagen IV expression was increased in VC compared with SED. Discontinuities of laminin, collagen IV and fibronectin were more evident in SED than in VC. This study indicates that VC has a biological behaviour different from SED or SCC, observable by immunohistochemistry in the BM zone.

  12. Retinoid inhibition of in vitro invasion of human amnion basement membrane by human tumor cells

    International Nuclear Information System (INIS)

    Fazely, F.

    1988-01-01

    The effects measured were the inhibition of tumor cell migration through the basement membrane (BM) and tumor cell degradative enzyme activity on 3 H-proline labeled collagenous and non collagenous components of the BM. The human lung carcinoma A549 or the human Ewing's sarcoma TC-106 cell lines treated with retinoids for two days were incubated on the BM in the absence of retinoids. A dose-dependent inhibition of cell invasion was produced by retinoids. Among the retinoids tested the most powerful was retinol acetate which inhibited invasion by 50% of A549 cells at a concentration of 0.09 μg/ml, and TC-106 cells at 0.08 μg/ml. Retinol acetate inhibited A549 and TC-106 cell growth by approximately 50% at levels almost 100-fold higher than those needed for antiinvasive activity. Retinol acetate was about 20 times more potent than retinoic acid and 30 times more than retinol palmitate. Furthermore, A549 cells treated with retinol acetate, under conditions whereby an anti-invasive state was induced,showed an increase in the number of cellular retinoic acid binding proteins (CRABP), a decrease in the activity of type IV collagenase and ectosialyltransferase, and no change in the activity of transglutaminase

  13. AMACO is a novel component of the basement membrane associated Fraser complex

    Science.gov (United States)

    Richardson, Rebecca J.; Gebauer, Jan M.; Zhang, Jin-Li; Kobbe, Birgit; Keene, Douglas R.; Karlsen, Kristina Røkenes; Richetti, Stefânia; Wohl, Alexander P.; Sengle, Gerhard; Neiss, Wolfram F.; Paulsson, Mats; Hammerschmidt, Matthias; Wagener, Raimund

    2015-01-01

    Fraser syndrome (FS) is a phenotypically variable, autosomal recessive disorder characterized by cryptophthalmus, cutaneous syndactyly and other malformations resulting from mutations in FRAS1, FREM2 and GRIP1. Transient embryonic epidermal blistering causes the characteristic defects of the disorder. Fras1, Frem1 and Frem2 form the extracellular Fraser complex, which is believed to stabilize the basement membrane (BM). However, several cases of FS could not be attributed to mutations in FRAS1, FREM2 or GRIP1, while Fraser syndrome displays high clinical variability, suggesting there is an additional genetic, possibly modifying contribution to this disorder. AMACO, encoded by the VWA2 gene, has a very similar tissue distribution to the Fraser complex proteins in both mouse and zebrafish. Here, we show that AMACO deposition is lost in Fras1 deficient zebrafish and mice and that Fras1 and AMACO interact directly via their CSPG and P2 domains. Knockdown of vwa2, which alone causes no phenotype, enhances the phenotype of hypomorphic Fras1 mutant zebrafish. Together, our data suggest that AMACO represents a novel member of the Fraser complex. PMID:24232570

  14. AMACO is a component of the basement membrane-associated Fraser complex.

    Science.gov (United States)

    Richardson, Rebecca J; Gebauer, Jan M; Zhang, Jin-Li; Kobbe, Birgit; Keene, Douglas R; Karlsen, Kristina Røkenes; Richetti, Stefânia; Wohl, Alexander P; Sengle, Gerhard; Neiss, Wolfram F; Paulsson, Mats; Hammerschmidt, Matthias; Wagener, Raimund

    2014-05-01

    Fraser syndrome (FS) is a phenotypically variable, autosomal recessive disorder characterized by cryptophthalmus, cutaneous syndactyly, and other malformations resulting from mutations in FRAS1, FREM2, and GRIP1. Transient embryonic epidermal blistering causes the characteristic defects of the disorder. Fras1, Frem1, and Frem2 form the extracellular Fraser complex, which is believed to stabilize the basement membrane. However, several cases of FS could not be attributed to mutations in FRAS1, FREM2, or GRIP1, and FS displays high clinical variability, suggesting that there is an additional genetic, possibly modifying contribution to this disorder. An extracellular matrix protein containing VWA-like domains related to those in matrilins and collagens (AMACO), encoded by the VWA2 gene, has a very similar tissue distribution to the Fraser complex proteins in both mouse and zebrafish. Here, we show that AMACO deposition is lost in Fras1-deficient zebrafish and mice and that Fras1 and AMACO interact directly via their chondroitin sulfate proteoglycan (CSPG) and P2 domains. Knockdown of vwa2, which alone causes no phenotype, enhances the phenotype of hypomorphic Fras1 mutant zebrafish. Together, our data suggest that AMACO represents a member of the Fraser complex.

  15. Collagen IV and basement membrane at the evolutionary dawn of metazoan tissues.

    Science.gov (United States)

    Fidler, Aaron L; Darris, Carl E; Chetyrkin, Sergei V; Pedchenko, Vadim K; Boudko, Sergei P; Brown, Kyle L; Gray Jerome, W; Hudson, Julie K; Rokas, Antonis; Hudson, Billy G

    2017-04-18

    The role of the cellular microenvironment in enabling metazoan tissue genesis remains obscure. Ctenophora has recently emerged as one of the earliest-branching extant animal phyla, providing a unique opportunity to explore the evolutionary role of the cellular microenvironment in tissue genesis. Here, we characterized the extracellular matrix (ECM), with a focus on collagen IV and its variant, spongin short-chain collagens, of non-bilaterian animal phyla. We identified basement membrane (BM) and collagen IV in Ctenophora, and show that the structural and genomic features of collagen IV are homologous to those of non-bilaterian animal phyla and Bilateria. Yet, ctenophore features are more diverse and distinct, expressing up to twenty genes compared to six in vertebrates. Moreover, collagen IV is absent in unicellular sister-groups. Collectively, we conclude that collagen IV and its variant, spongin, are primordial components of the extracellular microenvironment, and as a component of BM, collagen IV enabled the assembly of a fundamental architectural unit for multicellular tissue genesis.

  16. Chitosan facilitates structure formation of the salivary gland by regulating the basement membrane components.

    Science.gov (United States)

    Yang, Tsung-Lin; Hsiao, Ya-Chuan

    2015-10-01

    Tissue structure is important for inherent physiological function and should be recapitulated during tissue engineering for regenerative purposes. The salivary gland is a branched organ that is responsible for saliva secretion and regulation. The salivary glands develop from epithelial-mesenchymal interactions, and depend on the support of the basement membrane (BM). Chitosan-based biomaterials have been demonstrated to be competent in facilitating the formation of salivary gland tissue structure. However, the underlying mechanisms have remained elusive. In the developing submandibular gland (SMG), the chitosan effect was found to diminish when collagen and laminin were removed from cultured SMG explants. Chitosan increased the expression of BM components including collagen, laminin, and heparan sulfate proteoglycan, and also facilitated BM components and the corresponding receptors to be expressed in tissue-specific patterns beneficial for SMG branching. The chitosan effect decreased when either laminin components or receptors were inhibited, as well when the downstream signaling was blocked. Our results revealed that chitosan promotes salivary glands branching through the BM. By regulating BM components and receptors, chitosan efficiently stimulated downstream signaling to facilitate salivary gland branching. The present study revealed the underlying mechanism of the chitosan effect in engineering SMG structure formation. Copyright © 2015 Elsevier Ltd. All rights reserved.

  17. Epithelial basement membrane injury and regeneration modulates corneal fibrosis after pseudomonas corneal ulcers in rabbits.

    Science.gov (United States)

    Marino, Gustavo K; Santhiago, Marcony R; Santhanam, Abirami; Lassance, Luciana; Thangavadivel, Shanmugapriya; Medeiros, Carla S; Bose, Karthikeyan; Tam, Kwai Ping; Wilson, Steven E

    2017-08-01

    The purpose of this study was to investigate whether myofibroblast-related fibrosis (scarring) after microbial keratitis was modulated by the epithelial basement membrane (EBM) injury and regeneration. Rabbits were infected with Pseudomonas aeruginosa after epithelial scrape injury and the resultant severe keratitis was treated with topical tobramycin. Corneas were analyzed from one to four months after keratitis with slit lamp photos, immunohistochemistry for alpha-smooth muscle actin (α-SMA) and monocyte lineage marker CD11b, and transmission electron microscopy. At one month after keratitis, corneas had no detectible EBM lamina lucida or lamina densa, and the central stroma was packed with myofibroblasts that in some eyes extended to the posterior corneal surface with damage to Descemet's membrane and the endothelium. At one month, a nest of stromal cells in the midst of the SMA + myofibroblasts in the stroma that were CD11b+ may be fibrocyte precursors to myofibroblasts. At two to four months after keratitis, the EBM fully-regenerated and myofibroblasts disappeared from the anterior 60-90% of the stroma of all corneas, except for one four-month post-keratitis cornea where anterior myofibroblasts were still present in one localized pocket in the cornea. The organization of the stromal extracellular matrix also became less disorganized from two to four months after keratitis but remained abnormal compared to controls at the last time point. Myofibroblasts persisted in the posterior 10%-20% of posterior stroma even at four months after keratitis in the central cornea where Descemet's membrane and the endothelium were damaged. This study suggests that the EBM has a critical role in modulating myofibroblast development and fibrosis after keratitis-similar to the role of EBM in fibrosis after photorefractive keratectomy. Damage to EBM likely allows epithelium-derived transforming growth factor beta (TGFβ) to penetrate the stroma and drive development and

  18. Distribution of two basement membrane proteoglycans through hair follicle development and the hair growth cycle in the rat

    DEFF Research Database (Denmark)

    Couchman, J R; King, J L; McCarthy, K J

    1990-01-01

    The distribution of two distinct populations of basement membrane proteoglycans has been monitored through hair growth development in the rat embryo and subsequent hair growth cycle. An antiserum against a small heparan sulfate proteoglycan uniformly stained the dermal-epidermal junction...... as they developed. Through the hair growth cycle, it was found that the heparan sulfate proteoglycan persisted around the follicles, while the chondroitin sulfate proteoglycan decreased in amount through catagen until it was undetectable at the base and dermal papilla of the telogen follicle. As anagen commenced...... of embryonic rats throughout the period of hair follicle formation. On the other hand, monoclonal antibodies recognizing a basement membrane-specific chondroitin sulfate proteoglycan only weakly stained 16-d embryo dermal-epidermal junction, but strong staining was associated with hair follicle buds...

  19. Human skin basement membrane-associated heparan sulphate proteoglycan: distinctive differences in ultrastructural localization as a function of developmental age

    DEFF Research Database (Denmark)

    Horiguchi, Y; Fine, J D; Couchman, J R

    1991-01-01

    at different developmental ages using two monoclonal antibodies to a well-characterized basement membrane-associated heparan sulphate proteoglycan. A series of foetal skin specimens (range, 54-142 gestational days) were examined using an immunoperoxidase immunoelectron microscopic technique. In specimens...... representing very early developmental ages, very diffuse immunoreaction products were detected. However, by approximately 76 gestational days, some accentuation of heparan sulphate proteoglycan was noted along the lamina densa, and by 142 gestational days, the distribution of heparan sulphate proteoglycan...... was identical to that observed in neonatal and adult human skin. These findings demonstrate that active remodelling of the dermo-epidermal junction occurs during at least the first two trimesters, and affects not only basement membrane-associated structures but also specific antigens....

  20. Aluminum-containing dense deposits of the glomerular basement membrane: identification by energy dispersive X-ray analysis

    International Nuclear Information System (INIS)

    Smith, D.M. Jr.; Pitcock, J.A.; Murphy, W.M.

    1982-01-01

    Heavy metals, including gold, mercury, lead, bismuth, and cadmium, have the potential to cause renal disease. With the development of X-ray microanalysis, these heavy metals can now be identified in tissue deposits. This report describes a case of renal failure, probably related to dysproteinemia, in which granular, electron-opaque dense deposits were present in the glomerular basement membranes. Energy dispersive X-ray analysis demonstrated that these dense deposits contained aluminum. An analysis of this patient's history in relation to the current knowledge of aluminum metabolism suggests that the aluminum deposition occurred secondary to previous glomerular injury. This case emphasizes the need to utilize heavy metal identification technology whenever granular, electron-opaque dense deposits are identified and represents, to our knowledge, the first study to document aluminum deposits within the glomerular basement membrane of humans

  1. CCN2/connective tissue growth factor is essential for pericyte adhesion and endothelial basement membrane formation during angiogenesis.

    Directory of Open Access Journals (Sweden)

    Faith Hall-Glenn

    Full Text Available CCN2/Connective Tissue Growth Factor (CTGF is a matricellular protein that regulates cell adhesion, migration, and survival. CCN2 is best known for its ability to promote fibrosis by mediating the ability of transforming growth factor β (TGFβ to induce excess extracellular matrix production. In addition to its role in pathological processes, CCN2 is required for chondrogenesis. CCN2 is also highly expressed during development in endothelial cells, suggesting a role in angiogenesis. The potential role of CCN2 in angiogenesis is unclear, however, as both pro- and anti-angiogenic effects have been reported. Here, through analysis of Ccn2-deficient mice, we show that CCN2 is required for stable association and retention of pericytes by endothelial cells. PDGF signaling and the establishment of the endothelial basement membrane are required for pericytes recruitment and retention. CCN2 induced PDGF-B expression in endothelial cells, and potentiated PDGF-B-mediated Akt signaling in mural (vascular smooth muscle/pericyte cells. In addition, CCN2 induced the production of endothelial basement membrane components in vitro, and was required for their expression in vivo. Overall, these results highlight CCN2 as an essential mediator of vascular remodeling by regulating endothelial-pericyte interactions. Although most studies of CCN2 function have focused on effects of CCN2 overexpression on the interstitial extracellular matrix, the results presented here show that CCN2 is required for the normal production of vascular basement membranes.

  2. Basement membrane heterogeneity during chick development as shown by tomato (Lycopersicon esculentum) lectin binding.

    Science.gov (United States)

    Ojeda, J L; Icardo, J M

    2006-03-01

    Basement membranes (BMs) constitute a distinct compartment of the extracellular matrix (ECM). All BMs show a similar structural appearance but differ in molecular composition. These variations have critical functional implications. The aim of this study is to establish the pattern of the tomato lectin (Lycopersicon esculentum agglutinin--LEA) binding sites in the BMs of the developing chick embryo (stages 4-21, Hamburger and Hamilton, 1951) in order to achieve a better understanding of the molecular heterogeneity of BMs. The study was performed with transmission electron microscopy (TEM) histochemistry, and confocal laser microscopy. TEM showed that LEA bound to the lamina densa and to the lamina fibroreticularis of the BMs. Through the period studied, most of the LEA binding appeared in the ectodermal BM and its derivatives. In the limb bud, LEA binding to the ectoderm BM was more intense in the ventral half than in the dorsal half. Furthermore, LEA allowed the early (HH16) detection of the transverse fibrillar tracts. In the lens and in the inner ear primordium, the BMs were LEA positive through the placode and cup stages. The binding was progressively reduced through the vesicle stage. The BMs of the olfactory primordium, and of the Rathke's pouch were positive. In contrast, the BMs of the developing central nervous system were negative. The BMs of both the paraxial and the lateral plates of the mesoderm were negative, whereas the notochord and the BM of the Wolffian duct were positive. The endodermal BM and its derivatives were negative. The ECM located between the fusing endocardial tubes, and the BM of the fusion zone of the paired aortae, were positive. This suggested an active role of the LEA-positive glycoproteins in the fusion of endothelia. Our results show the heterogeneity of the chick embryo BMs during development. In addition, LEA constitutes an excellent marker for the primordial germ cells.

  3. Ultrastructural and Temporal Changes of the Microvascular Basement Membrane and Astrocyte Interface Following Focal Cerebral Ischemia

    Science.gov (United States)

    Kwon, Il; Kim, Eun Hee; del Zoppo, Gregory J.; Heo, Ji Hoe

    2009-01-01

    Microvascular integrity is lost during cerebral ischemia. Detachment of the microvascular basement membrane (BM) from the astrocyte, as well as degradation of the BM, is responsible for the loss of microvascular integrity. However, their ultrastructural and temporal changes during cerebral ischemia are not well known. Male Sprague-Dawley rats were subjected to permanent middle cerebral artery occlusion (MCAO) for 1, 4, 8, 12, 16, 20, and 48 hr. By using transmission electron microscopy, the proportion of intact BM–astrocyte contacts and electron densities of the BM were measured from five randomly selected microvessels in the ischemic basal ganglia. Their temporal changes and associations with activities of the matrix metalloproteinases (MMPs) were investigated. The intact portion of the BM–astrocyte contacts was decreased significantly within 4 hr and was rarely observed at 48 hr after MCAO. Decreases in the electron density and degradation of the BM were significant 12 hr after MCAO. The intact BM–astrocyte contacts and the mean BM density showed a significant positive correlation (r = 0.784, P < 0.001). MMP-9 activity was correlated negatively with the intact BM–astrocyte contacts (r = −0.711, P < 0.001) and with the BM density (r = −0.538, P 5 0.0016). The increase in MMP-9 coincided temporally with the loss of the BM–astrocyte contacts and a decrease in the BM density. Ultrastructural alterations occurring in the microvascular BM and its contacts with astrocyte endfeet were temporally associated in cerebral ischemia. Time courses of their alterations should be considered in the treatment targeted to the microvascular BM and its contact with astrocytes. PMID:18831008

  4. Permeation of macromolecules into the renal glomerular basement membrane and capture by the tubules

    Science.gov (United States)

    Lawrence, Marlon G.; Altenburg, Michael K.; Sanford, Ryan; Willett, Julian D.; Bleasdale, Benjamin; Ballou, Byron; Wilder, Jennifer; Li, Feng; Miner, Jeffrey H.; Berg, Ulla B.; Smithies, Oliver

    2017-01-01

    How the kidney prevents urinary excretion of plasma proteins continues to be debated. Here, using unfixed whole-mount mouse kidneys, we show that fluorescent-tagged proteins and neutral dextrans permeate into the glomerular basement membrane (GBM), in general agreement with Ogston's 1958 equation describing how permeation into gels is related to molecular size. Electron-microscopic analyses of kidneys fixed seconds to hours after injecting gold-tagged albumin, negatively charged gold nanoparticles, and stable oligoclusters of gold nanoparticles show that permeation into the lamina densa of the GBM is size-sensitive. Nanoparticles comparable in size with IgG dimers do not permeate into it. IgG monomer-sized particles permeate to some extent. Albumin-sized particles permeate extensively into the lamina densa. Particles traversing the lamina densa tend to accumulate upstream of the podocyte glycocalyx that spans the slit, but none are observed upstream of the slit diaphragm. At low concentrations, ovalbumin-sized nanoparticles reach the primary filtrate, are captured by proximal tubule cells, and are endocytosed. At higher concentrations, tubular capture is saturated, and they reach the urine. In mouse models of Pierson’s or Alport’s proteinuric syndromes resulting from defects in GBM structural proteins (laminin β2 or collagen α3 IV), the GBM is irregularly swollen, the lamina densa is absent, and permeation is increased. Our observations indicate that size-dependent permeation into the lamina densa of the GBM and the podocyte glycocalyx, together with saturable tubular capture, determines which macromolecules reach the urine without the need to invoke direct size selection by the slit diaphragm. PMID:28246329

  5. Assessment of proteolytic degradation of the basement membrane: a fragment of type IV collagen as a biochemical marker for liver fibrosis

    DEFF Research Database (Denmark)

    Veidal, Sanne S.; Karsdal, Morten A.; Nawrocki, Arkadiusz

    2011-01-01

    Collagen deposition and an altered matrix metalloproteinase (MMP) expression profile are hallmarks of fibrosis. Type IV collagen is the most abundant structural basement membrane component of tissue, which increases 14-fold during fibrogenesis in the liver. Proteolytic degradation of collagens...

  6. Crescentic glomerulonephritis in a polar bear (Ursus maritimus).

    Science.gov (United States)

    Baba, Hiroshi; Kudo, Tomoo; Makino, Yoshinori; Mochizuki, Yasumasa; Takagi, Takayo; Une, Yumi

    2013-11-01

    Spontaneous crescentic glomerulonephritis (CrGN) in animals has only been reported in dog and sheep. We report the pathological features of CrGN in a 17-year-old male polar bear that died due to renal failure. Histologically, the lesions were characterized by fibrocellular crescents, adhesion between Bowman's capsule and the glomerular capillary tuft and an increase in the mesangial matrix in glomeruli. The proliferating cells in the crescent were partly immunopositive for cytokeratin and intensely positive for vimentin, WT-1 and α-smooth muscle actin, suggesting they originated from parietal epithelial cells. Ultrastructually, thickening of the glomerular basement membrane and loss of epithelial cell foot processes were observed with electron-dense deposits.

  7. Fibrillary glomerulonephritis: A diagnosis not to be missed′

    Directory of Open Access Journals (Sweden)

    Hala Kassouf Kfoury

    2012-01-01

    Full Text Available Fibrillary glomerulonephritis (Fib GN is among the newly recognized primary glomerular diseases. This rare cause of end-stage kidney disease has characteristic electron microscopic findings based upon the deposition of randomly distributed (18-22 nm microfibrills in the mesangium and less frequently in the capillary basement membrane. The main differential diagnosis at the pathological level is amyloidosis; however, the apple green birefringence Congo red positivity of amyloid deposition is not seen in Fib GN. Clinically, the patient usually presents with proteinuria of nephrotic range, and the sine qua non for the diagnosis of Fib GN is the availability of high-magnification electron micrographs. Here is a case report of Fib GN with special emphasis on electron microscopy study and its role in the diagnosis.

  8. Crescentic Glomerulonephritis in a Polar Bear (Ursus maritimus)

    Science.gov (United States)

    BABA, Hiroshi; KUDO, Tomoo; MAKINO, Yoshinori; MOCHIZUKI, Yasumasa; TAKAGI, Takayo; UNE, Yumi

    2013-01-01

    ABSTRACT Spontaneous crescentic glomerulonephritis (CrGN) in animals has only been reported in dog and sheep. We report the pathological features of CrGN in a 17-year-old male polar bear that died due to renal failure. Histologically, the lesions were characterized by fibrocellular crescents, adhesion between Bowman’s capsule and the glomerular capillary tuft and an increase in the mesangial matrix in glomeruli. The proliferating cells in the crescent were partly immunopositive for cytokeratin and intensely positive for vimentin, WT-1 and α-smooth muscle actin, suggesting they originated from parietal epithelial cells. Ultrastructually, thickening of the glomerular basement membrane and loss of epithelial cell foot processes were observed with electron-dense deposits. PMID:23856758

  9. Quantitative Proteome Analysis Reveals Increased Content of Basement Membrane Proteins in Arteries from Patients with Type 2 Diabetes and Lower Levels among Metformin Users

    DEFF Research Database (Denmark)

    Rørdam Preil, Simone; Kristensen, Lars P; Beck, Hans C

    2015-01-01

    analysis was done by iTRAQ-labelling and LC-MS/MS analysis on individual arterial samples. The amounts of the basement membrane (BM) components, alpha-1- and alpha-2- type IV collagen, gamma-1- and beta-2-laminin were significantly increased in patients with diabetes. Moreover, the expressions of basement...... of collagen-stainable material in tunica intima and media among patients with diabetes. CONCLUSIONS: -The distinct accumulation of arterial basement membrane proteins in type 2 diabetes discloses a similarity between the diabetic macro- and micro-angiopathy and suggests a molecular explanation behind...

  10. Pulmonary renal syndrome in a child with coexistence of anti-neutrophil cytoplasmic antibodies and anti-glomerular basement membrane disease: case report and literature review.

    Science.gov (United States)

    Bogdanović, Radovan; Minić, Predrag; Marković-Lipkovski, Jasmina; Stajić, Nataša; Savić, Nataša; Rodić, Milan

    2013-03-22

    Pulmonary renal syndrome (PRS), denoting the presence of diffuse alveolar hemorrhage and glomerulonephritis as manifestations of systemic autoimmune disease, is very rare in childhood. The coexistence of circulating anti-neutrophil cytoplasmic antibody (ANCA) and anti-glomerular basement membrane (GBM) disease in children affected by this syndrome is exceptional, with unfavorable outcome in five out of seven patients reported to date. We describe a child with PRS associated with both circulating anti-myeloperoxidase (anti-MPO) ANCA and anti-GBM disease on renal biopsy who was successfully treated with immunosuppressive therapy. A 10-year old girl presented with fever, fatigue, malaise, and pallor followed by hemoptysis and severe anemia. Diffuse alveolar hemorrhage was revealed on fiberoptic bronchoscopy. Renal findings consisted of microscopic hematuria, moderate proteinuria, and anti-GBM disease on renal biopsy. ANCA with anti-MPO specificity were present whereas anti-GBM antibodies were on borderline for positivity. Methyl-prednisolone pulses followed by prednisone led to cessation of hemoptysis, marked improvement of lung fuction, and normal finding on chest x-ray within 10 days. An immunosuppressive regimen was then given consisting of prednisone daily for 4 weeks with subsequent taper on alternate day, i.v. cyclophosphamide pulses monthly for 6 doses, followed by mycophenolate mofetil that resulted in normal lung function tests, hemoglobin concentration, and anti-MPO level within four subsequent weeks. During 10-months of follow-up she remained well, her blood pressure and renal function tests were normal, and proteinuria and hematuria gradually resolved. We report a child with an exceptionally rare coexistence of circulating ANCA and anti-GBM disease manifesting as PRS in whom renal disease was not the prominent part of clinical presentation, contrary to other reported pediatric patients. A review of literature on disease with double positive antibodies is

  11. Crescentic glomerulonephritis: A clinical and histomorphological analysis of 46 cases

    Directory of Open Access Journals (Sweden)

    Ruchika Gupta

    2011-01-01

    Full Text Available Background: Crescentic glomerulonephritis (CrGN, defined as crescents involving more than 50% of the glomeruli, includes pauci-immune, immune complex-mediated and anti-glomerular basement membrane disease. Objectives: The present study was aimed at evaluating the various clinical, biochemical and histological parameters in CrGN with respect to these categories and clinical outcome. Materials and Methods: Renal biopsies diagnosed as CrGN between Jan 2008 and Feb 2010 were included. Clinical and laboratory parameters were retrieved along with the therapeutic approach and clinical outcome, wherever available. Renal biopsy slides were evaluated for various glomerular, tubulo-interstitial and arteriolar features. Appropriate statistical tests were applied for significance. Results: A total of 46 cases of CrGN were included; majority (71.7% of cases were pauci-immune (PI while 28.3% were immune complex-mediated (IC. Among clinical features, gender ratio was significantly different between PI and IC groups (P = 0.006. The various histological parameters, including proportion of cellular crescents, tuft necrosis and Bowman′s capsule rupture, were similar in both the groups. Four unusual associations, including idiopathic membranoproliferative glomerulonephritis (MPGN, multibacillary leprosy, acute lymphoblastic leukemia and C1q nephropathy were detected. Adequate follow-up information was available in 21 (46% of the patients. Of these, 11 (52.4% were dialysis-dependent at the last follow-up. Adult patients required renal replacement therapy more frequently than pediatric cases (P = 0.05. Presence of arteriolar fibrinoid necrosis also showed association with poor clinical outcome (P = 0.05. Conclusions: Crescentic glomerulonephritis remains one of the main causes of acute renal failure with histological diagnosis. Immunohistologic examination is essential for accurate classification into one of the three categories. This condition should be considered

  12. De novo deposition of laminin-positive basement membrane in vitro by normal hepatocytes and during hepatocarcinogenesis

    DEFF Research Database (Denmark)

    Albrechtsen, R; Wewer, U M; Thorgeirsson, S S

    1988-01-01

    De novo formation of laminin-positive basement membranes was found to be a distinct morphologic feature of diethylnitrosamine/phenobarbital-induced hepatocellular carcinomas of the rat. The first appearance of extracellularly located laminin occurred in the preneoplastic liver lesions......, a location where no laminin was seen in normal rat liver. The amount of extractable laminin from hepatocellular carcinomas was significantly higher (approximately 100 ng per mg tissue) than that of normal liver tissue (less than 20 ng per mg). In vitro experiments demonstrated that normal and preneoplastic...

  13. Expression and deposition of basement membrane proteins by brain capillary endothelial cells in a primary murine model of the blood-brain barrier

    DEFF Research Database (Denmark)

    Thomsen, Maj Schneider; Birkelund, Svend; Larsen, Annette Burkhart

    2016-01-01

    of the present study was to create four different in vitro constructs of the murine BBB to characterise if the expression and secretion of basement membrane proteins by the murine brain capillary endothelial cells (mBCECs) was affected by co-culturing with pericytes, mixed glial cells, or both. Primary m...... membrane, and astrocyte endfeet. To study the interaction of the different cells of the BBB, construction of in vitro BBB models is valuable. However, the modulation and contribution of the cells of the BBB to the synthesis of basement membrane proteins in vitro is not fully elaborated. Thus, the aim......, and immunofluorescent labelling were used. The mBCECs were found to express major basement membrane proteins in vitro and increased expression of laminin α5 and collagen IV α1 was correlated to the addition of BBB inducing factors (hydrocortisone, Ro20-1724, pCPT-cAMP). Co-culturing of the mBCECs with pericytes, mixed...

  14. A Novel Function for the nm23-Hl Gene: Overexpression in Human Breast Carcinoma Cells Leads to the Formation of Basement Membrane and Growth Arrest

    Energy Technology Data Exchange (ETDEWEB)

    Howlett, Anthony R; Petersen, Ole W; Steeg, Patricia S; Bissell, Mina J

    1994-01-01

    We have developed a culture system using reconstituted basement membrane components in which normal human mammary epithelial cells exhibit several aspects of the development and differentiation process, including formation of acinar-like structures, production and basal deposition of basement membrane components, and production and apical secretion of sialomucins. Cell lines and cultures from human breast carcinomas failed to recapitulate this process. The data indicate the importance of cellular interactions with the basement membrane in the regulation of normal breast differentiation and, potentially, its loss in neoplasia. Our purpose was to use this assay to investigate the role of the putative metastasis suppressor gene nm23-H1 in mammary development and differentiation. The metastatic human breast carcinoma cell line MDA-MB-435, clones transfected with a control pCMVBamneo vector, and clones transfected with pCMVBamneo vector containing nm23-H1 complementary DNA (the latter of which exhibited a substantial reduction in spontaneous metastatic potential in vivo) were cultured within a reconstituted basement membrane. Clones were examined for formation of acinus-like spheres, deposition of basement membrane components, production of sialomucin, polarization, and growth arrest. In contrast to the parental cell line and control transfectants, MDA-MB-435 breast carcinoma cells overexpressing Nm23-H1 protein regained several aspects of the normal phenotype within reconstituted basement membrane. Nm23-H1 protein-positive cells formed organized acinus-like spheres, deposited the basement membrane components type IV collagen and, to some extent, laminin to the outside of the spheres, expressed sialomucin, and growth arrested. Growth arrest of Nm23-H1 protein-positive cells was preceded by and correlated with formation of a basement membrane, suggesting a causal relationship. The data indicate a previously unidentified cause-and-effect relationship between nm23-H1 gene

  15. Improved proliferation and differentiation capacity of human mesenchymal stromal cells cultured with basement-membrane extracellular matrix proteins.

    Science.gov (United States)

    Lindner, Ulrich; Kramer, Jan; Behrends, Jochen; Driller, Birgit; Wendler, Nils-Ole; Boehrnsen, Florian; Rohwedel, Jürgen; Schlenke, Peter

    2010-12-01

    In vitro cultured mesenchymal stromal cells (MSC) are characterized by a short proliferative lifespan, an increasing loss of proliferation capacity and progressive reduction of differentiation potential. Laminin-1, laminin-5, collagen IV and fibronectin are important constituents of the basement membrane extracellular matrix (ECM) that are involved in a variety of cellular activities, including cell attachment and motility. The in vitro proliferation capacity of MSC was significantly improved when the cells were incubated in the presence of basement membrane ECM proteins. For example, a mixture of proteins improved proliferation capacity 250-fold in comparison with standard conditions after five passages. Furthermore, in colony-forming unit-fibroblast (CFU-F) assays colony numbers and size were significantly extended. Blocking specific integrin cell-surface receptors, positive effects on the proliferation capacity of MSC were inhibited. Additionally, when MSC were co-cultivated with ECM proteins, cells maintained their multipotential differentiation capacity throughout many culture passages in comparison with cells cultivated on plastic. However, expansion of MSC on laminin-5 suppressed any subsequent chondrogenic differentiation. Our results suggest that expansion of bone marrow-derived MSC in the presence of ECM proteins is a powerful approach for generating large numbers of MSC, showing a prolonged capacity to differentiate into mesodermal cell lineages, with the exception of the lack of chondrogenesis by using laminin-5 coating.

  16. Deletion of the basement membrane heparan sulfate proteoglycan type XVIII collagen causes hypertriglyceridemia in mice and humans.

    Directory of Open Access Journals (Sweden)

    Joseph R Bishop

    2010-11-01

    Full Text Available Lipoprotein lipase (Lpl acts on triglyceride-rich lipoproteins in the peripheral circulation, liberating free fatty acids for energy metabolism or storage. This essential enzyme is synthesized in parenchymal cells of adipose tissue, heart, and skeletal muscle and migrates to the luminal side of the vascular endothelium where it acts upon circulating lipoproteins. Prior studies suggested that Lpl is immobilized by way of heparan sulfate proteoglycans on the endothelium, but genetically altering endothelial cell heparan sulfate had no effect on Lpl localization or lipolysis. The objective of this study was to determine if extracellular matrix proteoglycans affect Lpl distribution and triglyceride metabolism.We examined mutant mice defective in collagen XVIII (Col18, a heparan sulfate proteoglycan present in vascular basement membranes. Loss of Col18 reduces plasma levels of Lpl enzyme and activity, which results in mild fasting hypertriglyceridemia and diet-induced hyperchylomicronemia. Humans with Knobloch Syndrome caused by a null mutation in the vascular form of Col18 also present lower than normal plasma Lpl mass and activity and exhibit fasting hypertriglyceridemia.This is the first report demonstrating that Lpl presentation on the lumenal side of the endothelium depends on a basement membrane proteoglycan and demonstrates a previously unrecognized phenotype in patients lacking Col18.

  17. Zhen-wu-tang attenuates cationic bovine serum albumin-induced inflammatory response in membranous glomerulonephritis rat through inhibiting AGEs/RAGE/NF-κB pathway activation.

    Science.gov (United States)

    Wu, Junbiao; Liu, Bihao; Liang, Chunling; Ouyang, Hui; Lin, Jin; Zhong, Yanchun; He, Yu; Zhou, Jie; Zhou, Yuan; Zhou, Jiuyao

    2016-04-01

    Zhen-wu-tang (ZWT), a traditional Chinese compound formula recorded in the Treatise on Febrile Diseases, has significant inhibitory effects on inflammatory damage and oxidative lesions in rats, but its mechanism of action remains unclear. The aim of the present study was to explore whether the anti-inflammatory and anti-oxidative effects of ZWT were mediated by the AGEs/RAGE/NF-κB signaling pathway in rats with cationic bovine serum albumin (C-BSA)-induced membranous glomerulonephritis (MGN). We found that ZWT significantly reduced the production of malondialdehyde (MDA), but enhanced the superoxide dismutase (SOD) activity. The ELISA results showed that ZWT not only reduced the serum levels of AGEs but also decreased the release of inflammatory mediators (TNF-α, IL-1β, and IL-6). Meanwhile, HE staining showed that pathological kidney injury was alleviated by ZWT. In addition, ZWT suppressed the expression of RAGE1 and NF-κB p65, as well as the nuclear translocation of NF-κB p65. The accumulation of AGEs, oxidative lesions and inflammation damage were reduced by an AGE inhibitor. Thus, the present study demonstrates that AGEs play a role in the pathogenesis of MGN and that AGE inhibition could reduce the inflammatory reactions and oxidative lesions in MGN. In general, ZWT attenuated MGN, in part, by inhibiting the AGEs/RAGE/NF-κB pathway. Copyright © 2016 Elsevier B.V. All rights reserved.

  18. An in vitro model of the glomerular capillary wall using electrospun collagen nanofibres in a bioartificial composite basement membrane.

    Directory of Open Access Journals (Sweden)

    Sadie C Slater

    Full Text Available The filtering unit of the kidney, the glomerulus, contains capillaries whose walls function as a biological sieve, the glomerular filtration barrier. This comprises layers of two specialised cells, glomerular endothelial cells (GEnC and podocytes, separated by a basement membrane. Glomerular filtration barrier function, and dysfunction in disease, remains incompletely understood, partly due to difficulties in studying the relevant cell types in vitro. We have addressed this by generation of unique conditionally immortalised human GEnC and podocytes. However, because the glomerular filtration barrier functions as a whole, it is necessary to develop three dimensional co-culture models to maximise the benefit of the availability of these cells. Here we have developed the first two tri-layer models of the glomerular capillary wall. The first is based on tissue culture inserts and provides evidence of cell-cell interaction via soluble mediators. In the second model the synthetic support of the tissue culture insert is replaced with a novel composite bioartificial membrane. This consists of a nanofibre membrane containing collagen I, electrospun directly onto a micro-photoelectroformed fine nickel supporting mesh. GEnC and podocytes grew in monolayers on either side of the insert support or the novel membrane to form a tri-layer model recapitulating the human glomerular capillary in vitro. These models will advance the study of both the physiology of normal glomerular filtration and of its disruption in glomerular disease.

  19. A role for PDGF-C/PDGFRα signaling in the formation of the meningeal basement membranes surrounding the cerebral cortex

    Directory of Open Access Journals (Sweden)

    Johanna Andrae

    2016-04-01

    Full Text Available Platelet-derived growth factor-C (PDGF-C is one of three known ligands for the tyrosine kinase receptor PDGFRα. Analysis of Pdgfc null mice has demonstrated roles for PDGF-C in palate closure and the formation of cerebral ventricles, but redundancy with other PDGFRα ligands might obscure additional functions. In search of further developmental roles for PDGF-C, we generated mice that were double mutants for Pdgfc−/− and PdgfraGFP/+. These mice display a range of severe phenotypes including spina bifida, lung emphysema, abnormal meninges and neuronal over-migration in the cerebral cortex. We focused our analysis on the central nervous system (CNS, where PDGF-C was identified as a critical factor for the formation of meninges and assembly of the glia limitans basement membrane. We also present expression data on Pdgfa, Pdgfc and Pdgfra in the cerebral cortex and microarray data on cerebral meninges.

  20. Defective muscle basement membrane and lack of M-laminin in the dystrophic dy/dy mouse

    DEFF Research Database (Denmark)

    Xu, H; Christmas, P; Wu, X R

    1994-01-01

    -linked Duchenne and Becker muscular dystrophies. We have examined M-laminin expression in mice with autosomal recessive muscular dystrophy caused by the mutation dy. The heavy chain of M-laminin was undetectable in skeletal muscle, heart muscle, and peripheral nerve by immunofluorescence and immunoblotting......M-laminin is a major member of the laminin family of basement membrane proteins. It is prominently expressed in striated muscle and peripheral nerve. M-laminin is deficient in patients with the autosomal recessive Fukuyama congenital muscular dystrophy but is normal in patients with the sex...... tissue from dy/dy mice, suggesting that M-laminin heavy-chain mRNA may be produced at very low levels or is unstable. Information about the chromosomal localization of the M heavy-chain in human and mouse suggests that a mutation in the M-chain gene causes the muscular dystrophy in dy/dy mice. The dy...

  1. [Chronic glomerulonephritis syndrome (CGNS)].

    Science.gov (United States)

    Hayama, Naoaki

    2003-08-01

    Chronic glomerulonephritis syndrome is one of clinical classification in primary glomerulonephritis. Clinical classification of primary glomerulonephritis consists of five groups. (WHO 1995) They are acute nephritic syndrome, rapidly progressive nephritic syndrome, recurrent or persistent hematuria (proteinuria), chronic nephritic syndrome, and nephrotic syndrome. After therapy or renal biopsy, they have interaction with one another and many cases are followed up as chronic glomerulonephritis. In the long term clinical course, it is most important thing that not to deteriorate renal function by therapies. (blood pressure control, low protein diet, salt restriction) If there are not only glomerular change but also interstitial and vessel changes in renal biopsy the prediction of prognosis will be poor so it will be necessary to administer medication under strict observation.

  2. Immunohistochemical localization of chondroitin sulfate, chondroitin sulfate proteoglycan, heparan sulfate proteoglycan, entactin, and laminin in basement membranes of postnatal developing and adult rat lungs

    DEFF Research Database (Denmark)

    Sannes, P L; Burch, K K; Khosla, J

    1993-01-01

    Histologic preparations of lungs from 1-, 5-, 10-, 18-, and 25-day-old postnatal and adult rats were examined immunohistochemically with antibodies specific against chondroitin sulfate (CS), basement membrane chondroitin sulfate proteoglycan (BM-CSPG), heparan sulfate proteoglycan (HSPG), entacti...

  3. 19-DEJ-1, a hemidesmosome-anchoring filament complex-associated monoclonal antibody. Definition of a new skin basement membrane antigenic defect in junctional and dystrophic epidermolysis bullosa

    DEFF Research Database (Denmark)

    Fine, J D; Horiguchi, Y; Couchman, J R

    1989-01-01

    50% of patients with recessive dystrophic EB further suggests, for the first time, that an inherited defect in basement membrane is shared in patients with junctional and some recessive dystrophic EB. The difference in site of skin cleavage in the latter two disorders gives additional support...

  4. A bioartificial environment for kidney epithelial cells based on a supramolecular polymer basement membrane mimic and an organotypical culture system.

    Science.gov (United States)

    Mollet, Björne B; Bogaerts, Iven L J; van Almen, Geert C; Dankers, Patricia Y W

    2017-06-01

    Renal applications in healthcare, such as renal replacement therapies and nephrotoxicity tests, could potentially benefit from bioartificial kidney membranes with fully differentiated and functional human tubular epithelial cells. A replacement of the natural environment of these cells is required to maintain and study cell functionality cell differentiation in vitro. Our approach was based on synthetic supramolecular biomaterials to mimic the natural basement membrane (BM) on which these cells grow and a bioreactor to provide the desired organotypical culture parameters. The BM mimics were constructed from ureidopyrimidinone (UPy)-functionalized polymer and bioactive peptides by electrospinning. The resultant membranes were shown to have a hierarchical fibrous BM-like structure consisting of self-assembled nanofibres within the electrospun microfibres. Human kidney-2 (HK-2) epithelial cells were cultured on the BM mimics under organotypical conditions in a custom-built bioreactor. The bioreactor facilitated in situ monitoring and functionality testing of the cultures. Cell viability and the integrity of the epithelial cell barrier were demonstrated inside the bioreactor by microscopy and transmembrane leakage of fluorescently labelled inulin, respectively. Furthermore, HK-2 cells maintained a polarized cell layer and showed modulation of both gene expression of membrane transporter proteins and metabolic activity of brush border enzymes when subjected to a continuous flow of culture medium inside the new bioreactor for 21 days. These results demonstrated that both the culture and study of renal epithelial cells was facilitated by the bioartificial in vitro environment that is formed by synthetic supramolecular BM mimics in our custom-built bioreactor. Copyright © 2015 John Wiley & Sons, Ltd. Copyright © 2015 John Wiley & Sons, Ltd.

  5. Ric-8a, a guanine nucleotide exchange factor for heterotrimeric G proteins, regulates bergmann glia-basement membrane adhesion during cerebellar foliation.

    Science.gov (United States)

    Ma, Shang; Kwon, Hyo Jun; Huang, Zhen

    2012-10-24

    The cerebellum consists of an intricate array of lobules that arises during the process of foliation. Foliation not only increases surface area, but may also facilitate organization of cerebellar neural circuitry. Defects in cerebellar foliation are associated with a number of diseases. Yet, little is known about how foliation, a process involving large-scale and simultaneous movement of several different cell types, is coordinated by cell-cell signaling at the molecular level. Here we show that Ric-8a, a guanine nucleotide exchange factor in the G-protein-coupled receptor pathway, is specifically required in Bergmann glia during cerebellar foliation. We find that ric-8a mutation in mice results in disorganized Bergmann glial scaffolding, defective granule cell migration, and disrupted Purkinje cell positioning. These abnormalities result from primary defects in Bergmann glia since mutations in granule cells do not show similar effects. They first arise during late embryogenesis, at the onset of foliation, when ric-8a mutant Bergmann glia fail to maintain adhesion to the basement membrane specifically at emerging fissures. This suggests that Ric-8a is essential for the enhanced Bergmann glia-basement membrane adhesion required for fissure formation. Indeed, we find that ric-8a-deficient cerebellar glia show decreased affinity for basement membrane components. We also find that weakening Bergmann glia-basement membrane interaction by β1 integrin deletion results in a similar phenotype. These results thus reveal a novel role of Ric-8a in modulating Bergmann glia-basement membrane adhesion during foliation, and provide new insights into the signaling pathways that coordinate cellular movement during cerebellar morphogenesis.

  6. Synthesis and localization of two sulphated glycoproteins associated with basement membranes and the extracellular matrix

    DEFF Research Database (Denmark)

    Hogan, B L; Taylor, A; Kurkinen, M

    1982-01-01

    interactions and are not precursors or products of each other. They contain asparagine-linked oligosaccharides, but these are not the exclusive sites of sulphate labeling. Antiserum raised against the Mr 150,000 sgp C of Reichert's membranes has been used in an immunohistochemical analysis of rat skin...

  7. A direct contact between astrocyte and vitreous body is possible in the rabbit eye due to discontinuities in the basement membrane of the retinal inner limiting membrane

    Directory of Open Access Journals (Sweden)

    A. Haddad

    2003-02-01

    Full Text Available Different from most mammalian species, the optic nerve of the rabbit eye is initially formed inside the retina where myelination of the axons of the ganglion cells starts and vascularization occurs. Astrocytes are confined to these regions. The aforementioned nerve fibers known as medullated nerve fibers form two bundles that may be identified with the naked eye. The blood vessels run on the inner surface of these nerve fiber bundles (epivascularization and, accordingly, the accompanying astrocytes lie mostly facing the vitreous body from which they are separated only by the inner limiting membrane of the retina. The arrangement of the astrocytes around blood vessels leads to the formation of structures known as glial tufts. Fragments (N = 3 or whole pieces (N = 3 of the medullated nerve fiber region of three-month-old male rabbits (Orictolagus cuniculus were fixed in glutaraldehyde followed by osmium tetroxide, and their thin sections were examined with a transmission electron microscope. Randomly located discontinuities (up to a few micrometers long of the basement membrane of the inner limiting membrane of the retina were observed in the glial tufts. As a consequence, a direct contact between the astrocyte plasma membrane and vitreous elements was demonstrated, making possible functional interactions such as macromolecular exchanges between this glial cell type and the components of the vitreous body.

  8. Fibrosis is not just fibrosis - basement membrane modelling and collagen metabolism differs between hepatitis B- and C-induced injury

    DEFF Research Database (Denmark)

    Nielsen, M J; Karsdal, M A; Kazankov, K

    2016-01-01

    and fibrosis only in CHC. Basement membrane collagen fragments P4NP7S and C4M were significantly higher in matched activity and fibrosis cohorts within CHB vs CHC. CONCLUSION: The main parameters to determine extracellular matrix biomarker levels are inflammation, fibrosis, and type of viral insult. Compared...... fragments in plasma from 197 chronic hepatitis B (CHB) patients and 403 chronic hepatitis C (CHC) patients matched for inflammation grade and fibrosis stage. Markers of matrix metalloprotease degraded type I, III, IV and VI collagen (C1M, C3M, C4M, C6M) and type III and IV collagen formation (Pro-C3, P4NP7S......). RESULTS: P4NP7S, C3M, C4M and C6M were significantly elevated in CHB compared to CHC. In contrast, Pro-C3 was significantly elevated in CHC compared to CHB. Pro-C3, C3M and C4M were increased in parallel with inflammation and fibrosis in both cohorts. C6M and P4NP7S were associated with inflammation...

  9. An immunohistochemical study of basement membrane heparan sulfate proteoglycan (perlecan) in oral epithelial dysplasia and squamous cell carcinoma.

    Science.gov (United States)

    Mishra, Mithilesh; Chandavarkar, Vidyadevi; Naik, Veena V; Kale, Alka D

    2013-01-01

    Basement membrane heparan sulfate proteoglycan (perlecan) has been demonstrated in precancer lesions and carcinomas of oral cavity. It helps in malignant transformation of epithelial cells. The aim of our study was to understand the immuno-localization of perlecan in oral dysplastic epithelium and oral carcinomas. A total of 50 cases comprising 10 normal mucosa, 20 dysplastic mucosa, and 20 oral squamous cell carcinomas (OSCC) were included in the retrospective study. They were examined for the presence of perlecan protein core by immunohistochemistry using monoclonal antibody. Interpretation of the pattern of staining was done, and majority of the observations were taken for statistical analysis. In normal epithelium, perlecan was found to be present in basal layer at the cell border. In dysplastic epithelium, it was present in suprabasal layers also. With the increase in severity of dysplasia, its expression was more in suprabasal layers, and the immuno-localization was found to be at cell border and cytoplasm. In OSCC cases, perlecan was present in stroma and tumor islands. It was deduced from the above results that perlecan helps potentially in dysplastic changes of epithelial cells. It gets accumulated within the cell and intercellular spaces and serves as a reservoir for various growth factors. In OSCC, it breaks down and releases growth factors, which help in tumor progression, angiogenesis, and metastasis of the carcinoma.

  10. Skin Basement Membrane: The Foundation of Epidermal Integrity—BM Functions and Diverse Roles of Bridging Molecules Nidogen and Perlecan

    Directory of Open Access Journals (Sweden)

    Dirk Breitkreutz

    2013-01-01

    Full Text Available The epidermis functions in skin as first defense line or barrier against environmental impacts, resting on extracellular matrix (ECM of the dermis underneath. Both compartments are connected by the basement membrane (BM, composed of a set of distinct glycoproteins and proteoglycans. Herein we are reviewing molecular aspects of BM structure, composition, and function regarding not only (i the dermoepidermal interface but also (ii the resident microvasculature, primarily focusing on the per se nonscaffold forming components perlecan and nidogen-1 and nidogen-2. Depletion or functional deficiencies of any BM component are lethal at some stage of development or around birth, though BM defects vary between organs and tissues. Lethality problems were overcome by developmental stage- and skin-specific gene targeting or by cell grafting and organotypic (3D cocultures of normal or defective cells, which allows recapitulating BM formation de novo. Thus, evidence is accumulating that BM assembly and turnover rely on mechanical properties and composition of the adjacent ECM and the dynamics of molecular assembly, including further “minor” local components, nidogens largely functioning as catalysts or molecular adaptors and perlecan as bridging stabilizer. Collectively, orchestration of BM assembly, remodeling, and the role of individual players herein are determined by the developmental, tissue-specific, or functional context.

  11. Basement membrane and vascular remodelling in smokers and chronic obstructive pulmonary disease: a cross-sectional study

    Directory of Open Access Journals (Sweden)

    Muller H Konrad

    2010-07-01

    Full Text Available Abstract Background Little is known about airway remodelling in bronchial biopsies (BB in smokers and chronic obstructive pulmonary disease (COPD. We conducted an initial pilot study comparing BB from COPD patients with nonsmoking controls. This pilot study suggested the presence of reticular basement membrane (Rbm fragmentation and altered vessel distribution in COPD. Methods To determine whether Rbm fragmentation and altered vessel distribution in BB were specific for COPD we designed a cross-sectional study and stained BB from 19 current smokers and 14 ex-smokers with mild to moderate COPD and compared these to 15 current smokers with normal lung function and 17 healthy and nonsmoking subjects. Results Thickness of the Rbm was not significantly different between groups; although in COPD this parameter was quite variable. The Rbm showed fragmentation and splitting in both current smoking groups and ex-smoker COPD compared with healthy nonsmokers (p Conclusions Airway remodelling in smokers and mild to moderate COPD is associated with fragmentation of the Rbm and altered distribution of vessels in the airway wall. Rbm fragmentation was also present to as great an extent in ex-smokers with COPD. These characteristics may have potential physiological consequences.

  12. Suppression of Apoptosis by Basement Membrane Requires three-dimensional Tissue Organization and Withdrawal from the Cell Cycle

    Energy Technology Data Exchange (ETDEWEB)

    Boudreau, N.; Werb, Z.; Bissell, M.J.

    1995-12-28

    The basement membrane (BM) extracellular matrix induces differentiation and suppresses apoptosis in mammary epithelial cells, whereas cells lacking BM lose their differentiated phenotype and undergo apoptosis. Addition of purified BM components, which are known to induce {beta}-casein expression, did not prevent apoptosis, indicating that a more complex BM was necessary. A comparison of culture conditions where apoptosis would or would not occur allowed us to relate inhibition of apoptosis to a complete withdrawal from the cell cycle, which was observed only when cells acquired a three-dimensional alveolar structure in response to BM. In the absence of this morphology, both the G1 cyclin kinase inhibitor p21/WAF-I and positive proliferative signals including c-myc and cyclin Dl were expressed and the retinoblastoma protein (Rb) continued to be hyperphosphorylated. When we overexpressed either c-myc in quiescent cells or p21 when cells were still cycling, apoptosis was induced. In the absence of three-dimensional alveolar structures, mammary epithelial cells secrete a number of factors including transforming growth factor a and tenascin, which when added exogenously to quiescent cells induced expression of c-myc and interleukin-{beta}1-converting enzyme (ICE) mRNA and led to apoptosis. These experiments demonstrate that a correct tissue architecture is crucial for long-range homeostasis, suppression of apoptosis, and maintenance of differentiated phenotype.

  13. Giant cell tubulitis with tubular basement membrane immune deposits: a report of two cases after cardiac valve replacement surgery.

    Science.gov (United States)

    Chang, Anthony; Peutz-Kootstra, Carine J; Kowalewska, Jolanta; Logar, Christine M; Gitomer, Jeremy J; Davis, Connie L; Shankland, Stuart J; Alpers, Charles E; Smith, Kelly D

    2006-09-01

    This paper presents two elderly patients who had normal baseline renal function and had stenotic valvular lesions secondary to rheumatic fever and underwent aortic valve replacements with mechanical valves. Both patients developed acute renal failure after cardiac valve replacement procedures. The renal biopsies revealed acute granulomatous tubulointerstitial nephritis. The unique histologic features were tubular basement membrane (TBM) immune complex deposition detected by both immunofluorescence and electron microscopy and prominent multinucleated giant cells surrounding intact TBM. The temporal relationship to the surgical procedure and the subsequent recovery of the patients' renal functions upon therapy suggested that the renal failure may have been due to an allergic drug reaction from the perioperative exposure to unknown agents, such as prophylactic antibiotics and furosemide. The literature on TBM immune complex deposition was reviewed, and the pathophysiologic mechanisms that may account for the similarities between the clinicopathologic features of these two cases were examined. These two cases expand the histopathologic spectrum of previously described cases of putative drug-induced acute tubulointerstitial nephritis.

  14. Endogenous Tim-1 (Kim-1) promotes T-cell responses and cell-mediated injury in experimental crescentic glomerulonephritis.

    Science.gov (United States)

    Nozaki, Yuji; Nikolic-Paterson, David J; Snelgrove, Sarah L; Akiba, Hisaya; Yagita, Hideo; Holdsworth, Stephen R; Kitching, A Richard

    2012-05-01

    The T-cell immunoglobulin mucin 1 (Tim-1) modulates CD4(+) T-cell responses and is also expressed by damaged proximal tubules in the kidney where it is known as kidney injury molecule-1 (Kim-1). We sought to define the role of endogenous Tim-1 in experimental T-cell-mediated glomerulonephritis induced by sheep anti-mouse glomerular basement membrane globulin acting as a planted foreign antigen. Tim-1 is expressed by infiltrating activated CD4(+) cells in this model, and we studied the effects of an inhibitory anti-Tim-1 antibody (RMT1-10) on immune responses and glomerular disease. Crescentic glomerulonephritis, proliferative injury, and leukocyte accumulation were attenuated following treatment with anti-Tim-1 antibodies, but interstitial foxp3(+) cell accumulation and interleukin-10 mRNA were increased. T-cell proliferation and apoptosis decreased in the immune system along with a selective reduction in Th1 and Th17 cellular responses both in the immune system and within the kidney. The urinary excretion and renal expression of Kim-1 was reduced by anti-Tim-1 antibodies reflecting diminished interstitial injury. The effects of anti-Tim-1 antibodies were not apparent in the early phase of renal injury, when the immune response to sheep globulin was developing. Thus, endogenous Tim-1 promotes Th1 and Th17 nephritogenic immune responses and its neutralization reduces renal injury while limiting inflammation in cell-mediated glomerulonephritis.

  15. How to Study Basement Membrane Stiffness as a Biophysical Trigger in Prostate Cancer and Other Age-related Pathologies or Metabolic Diseases

    OpenAIRE

    Rodriguez-Teja, Mercedes; Breit, Claudia; Clarke, Mitchell; Talar, Kamil; Wang, Kai; Mohammad, Mohammad A.; Pickwell, Sage; Etchandy, Guillermina; Stasiuk, Graeme J.; Sturge, Justin

    2016-01-01

    Here we describe a protocol that can be used to study the biophysical microenvironment related to increased thickness and stiffness of the basement membrane (BM) during age-related pathologies and metabolic disorders (e.g. cancer, diabetes, microvascular disease, retinopathy, nephropathy and neuropathy). The premise of the model is non-enzymatic crosslinking of reconstituted BM (rBM) matrix by treatment with glycolaldehyde (GLA) to promote advanced glycation endproduct (AGE) generation via th...

  16. Intraepithelial expression of perlecan, a basement membrane-type heparan sulfate proteoglycan reflects dysplastic changes of the oral mucosal epithelium.

    Science.gov (United States)

    Ikarashi, Terué; Ida-Yonemochi, Hiroko; Ohshiro, Kazufumi; Cheng, Jun; Saku, Takashi

    2004-02-01

    Intercellular deposition of perlecan, a major heparan sulfate proteoglycan (HSPG) of the basement membrane, is known to result in characteristic stellate reticulum-like structures in ameloblastomas or tooth germs. Although enlargement of the intercellular space is one of the histological characteristics of epithelial dysplasia of oral mucosa, the mode of expression of perlecan is poorly understood in these epithelial lesions. Eighty-two biopsy specimens consisting of normal and hyperplastic epithelium, epithelial dysplasia, and squamous cell carcinomas were examined for both perlecan core protein and heparan sulfate (HS) chains by immunohistochemistry and in situ hybridization. In normal and hyperplastic epithelium, perlecan core protein and HS chains were localized in the cell border of parabasal cells and lower prickle cells, and HS chains were also found in basal cells. With an increase in the severity of epithelial dysplasia, the core protein was heavily and extensively deposited in the interepithelial space as well as in the cytoplasm of epithelial cells from the basal to the surface layers. Its gene expression was confirmed in the cells around the protein deposits. On the other hand, HS chains were enhanced in mild dysplasia, but decreased in moderate and severe dysplasias. In squamous cell carcinomas, either the core protein or HS chains were found scarcely in tumor cells but abundantly in the stromal space. The findings indicate that perlecan is localized in the intercellular space of the oral epithelia, and that it is over-expressed in dysplastic epithelial cells and is deposited in their interepithelial space, which results in the histology of reduction of cellular cohesion.

  17. Pathological patterns of mesangioproliferative glomerulonephritis seen at a tertiary care center

    Directory of Open Access Journals (Sweden)

    Mokhtar Ghadeer A.

    2014-04-01

    Full Text Available Background: Mesangioproliferative glomerulonephritis (MesPGN is a common morphological pattern that encompasses several groups of renal diseases including IgA nephropathy (IgAN, IgM nephropathy (IgMN, lupus nephritis (LN, C1q nephropathy (C1qN and other entities. Objectives: The aim of this study was to analyze the pathological findings and the clinical features of cases of MesPGN seen at the king Abdulaziz University, in Saudi Arabia. Patients and Methods: A total of 750 percutaneous native renal biopsies were seen at our institution from January 2000 to December 2011. All the cases diagnosed as MesPGN on light microscopy (LM were retrieved from the archives of pathology. The pathological features and the clinical data of these cases were reviewed. The clinical data was available for 80 cases only. Results: A total of 103 cases (14% met the inclusion criteria for the diagnosis of MesPGN. The most common diagnostic entity was IgMN (46.6% followed by IgAN (30% along with few cases of class II LN, C1qN, minimal change disease (MCD, Alport’s syndrome, focal segmental glomerulosclerosis (FSGS, thin basement membrane disease (TBMD, and fibrillary glomerulonephritis. The most common clinical presentation was nephrotic syndrome seen in 71% of 80 cases, followed by hematuria (14%. Histologically, focal mesangial proliferation was seen in 62% while diffuse proliferation was seen in 38% of the cases. Conclusion: Mesangioproliferative glomerulonephritis is an important cause of nephrotic syndrome in young adults in the western region of Saudi Arabia. Future studies from the region are needed to elucidate the clinical relevance of mesangial cell proliferation to the end stage kidney diseases.

  18. Normal and tumor-derived myoepithelial cells differ in their ability to interact with luminal breast epithelial cells for polarity and basement membrane deposition

    Energy Technology Data Exchange (ETDEWEB)

    Gudjonsson, Thorarinn; Ronnov-Jessen, Lone; Villadsen, Rene; Rank, Fritz; Bissell, Mina J.; Petersen, Ole William

    2001-10-04

    The signals that determine the correct polarity of breast epithelial structures in vivo are not understood. We have shown previously that luminal epithelial cells can be polarized when cultured within a reconstituted basement membrane gel. We reasoned that such cues in vivo may be given by myoepithelial cells. Accordingly, we used an assay where luminal epithelial cells are incorrectly polarized to test this hypothesis. We show that culturing human primary luminal epithelial cells within collagen-I gels leads to formation of structures with no lumina and with reverse polarity as judged by dual stainings for sialomucin, epithelial specific antigen or occludin. No basement membrane is deposited, and {beta}4-integrin staining is negative. Addition of purified human myoepithelial cells isolated from normal glands corrects the inverse polarity, and leads to formation of double-layered acini with central lumina. Among the laminins present in the human breast basement membrane (laminin-1, -5 and -10/11), laminin-1 was unique in its ability to substitute for myoepithelial cells in polarity reversal. Myoepithelial cells were purified also from four different breast cancer sources including a biphasic cell line. Three out of four samples either totally lacked the ability to interact with luminal epithelial cells, or conveyed only correction of polarity in a fraction of acini. This behavior was directly related to the ability of the tumor myoepithelial cells to produce {alpha}-1 chain of laminin. In vivo, breast carcinomas were either negative for laminin-1 (7/12 biopsies) or showed a focal, fragmented deposition of a less intensely stained basement membrane (5/12 biopsies). Dual staining with myoepithelial markers revealed that tumorassociated myoepithelial cells were either negative or weakly positive for expression of laminin-1, establishing a strong correlation between loss of laminin-1 and breast cancer. We conclude that the double-layered breast acinus may be

  19. cDNA cloning of the basement membrane chondroitin sulfate proteoglycan core protein, bamacan: a five domain structure including coiled-coil motifs

    DEFF Research Database (Denmark)

    Wu, R R; Couchman, J R

    1997-01-01

    obtained cDNA clones encoding the entire bamacan core protein of Mr = 138 kD, which reveal a five domain, head-rod-tail configuration. The head and tail are potentially globular, while the central large rod probably forms coiled-coil structures, with one large central and several very short interruptions....../translation product from a full-length bamacan cDNA. The unusual structure of this proteoglycan is indicative of specific functional roles in basement membrane physiology, commensurate with its distinct expression in development and changes in disease models....

  20. Distribution, ultrastructural localization, and ontogeny of the core protein of a heparan sulfate proteoglycan in human skin and other basement membranes

    DEFF Research Database (Denmark)

    Horiguchi, Y; Couchman, J R; Ljubimov, A V

    1989-01-01

    A variety of heparan sulfate proteoglycans (HSPG) have been identified on cell surfaces and in basement membrane (BM). To more fully characterize HSPG in human skin BM, we used two monoclonal antibodies (MAb) directed against epitopes of the core protein of a high molecular weight HSPG isolated...... from murine EHS tumor. Indirect immunofluorescence revealed linear distribution of HSPG within all skin BM, and within BM of all other human organs investigated. In a study of the ontogeny of HSPG in human skin BM, HSPG was detectable as early as 54 gestational days, comparable with other ubiquitous BM...

  1. Acute glomerulonephritis mimicking nephrotic syndrome

    African Journals Online (AJOL)

    2016-01-04

    Jan 4, 2016 ... Abstract: Background: Acute post infectious glomerulonephritis. (APIGN) describes a wide range of glomerulonephritis character- ized by an immunologic response of the kidney to varieties of infec- tious agent commonly bacteria. It is characterized by an abrupt on- set of haematuria, moderate oedema ...

  2. Clinical spectrum and outcomes of crescentic glomerulonephritis: A single center experience

    Directory of Open Access Journals (Sweden)

    S K Rampelli

    2016-01-01

    Full Text Available There is limited data on the etiology, clinical and histopathological spectrum and outcomes of crescentic glomerulonephritis (CrGN in adult Indian population. This prospective study was done to evaluate the etiology, clinicohistological patterns and predictors of outcome of CrGN in South Indian population. All the patients received standard protocol based immunosuppression in addition to supportive care. Immune-complex glomerulonephritis (ICGN was the most common etiology (n = 31; 77.5% followed by pauci-immune glomerulonephritis (PauciGN; n = 8; 20% and anti-glomerular basement membrane disease (n = 1; 2.5%. The most common etiology of ICGN was IgA nephropathy (n = 11; 27.5% followed by lupus nephritis (n = 7; 17.5% and post-infectious glomerulonephritis (PIGN (n = 7; 17.5%. The patients with PauciGN were significantly older compared to those with ICGN (44.5 ± 15 years vs. 31.8 ± 11 years; P = 0.01. The patients with PauciGN presented with significantly higher serum creatinine (9.7 ± 4.4 vs. 6.6 ± 3.3 mg/dl; P = 0.03. The histopathologic parameters of ICGN and PauciGN were comparable except for a higher proportion of sclerosed glomeruli in ICGN. At the end of 3 months follow-up, only two patients went into complete remission (5.4%. Majority of the patients had end-stage renal failure (48.6% and were dialysis dependent and seven patients (18.9% expired. There was no signifi difference in the renal survival (10.9 ± 1.9 vs. 9.6 ± 3.3 months or patient survival (17.5 ± 2.1 vs. 17.3 ± 4.3 months. The parameters associated with adverse outcomes at 3 months were hypertension (odds ratio [OR]: 0.58; confidence interval [CI]: 0.36–0.94, need for renal replacement therapy (OR: 0.19; CI: 0.04–0.9, serum creatinine at admission (P = 0.019, estimated glomerular filtration rate (P = 0.022 and percentage of fibrocellular crescents (P = 0.022.

  3. Collagen metabolism and basement membrane formation in cultures of mouse mammary epithelial cells: Induction of assembly on fibrillar type I collagen substrata

    International Nuclear Information System (INIS)

    David, G.; van der Schueren, B.; van den Berghe, H.; Nusgens, B.; Van Cauwenberge, D.; Lapiere, C.

    1987-01-01

    Collagen metabolism was compared in cultures of mouse mammary epithelial cells maintained on plastic or fibrillar type I collagen gel substrata. The accumulation of dialysable and non-dialysable [ 3 H]hydroxyproline and the identification of the collagens produced suggest no difference between substrata in the allover rates of collagen synthesis and degradation. The proportion of the [ 3 H]collagen which accumulates in the monolayers of cultures on collagen, however, markedly exceeds that of cultures on plastic. Cultures on collagen deposit a sheet-like layer of extracellular matrix materials on the surface of the collagen fibers. Transformed cells on collagen produce and accumulate more [ 3 H]collage, yet are less effective in basement membrane formation than normal cells, indicting that the accumulation of collagen alone and the effect of interstitial collagen thereupon do not suffice. Thus, exogenous fibrillar collagen appears to enhance, but is not sufficient for proper assembly of collagenous basement membrane components near the basal epithelial cell surface

  4. Stratum, a Homolog of the Human GEF Mss4, Partnered with Rab8, Controls the Basal Restriction of Basement Membrane Proteins in Epithelial Cells

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    Olivier Devergne

    2017-02-01

    Full Text Available The basement membrane (BM, a sheet of extracellular matrix lining the basal side of epithelia, is essential for epithelial cell function and integrity, yet the mechanisms that control the basal restriction of BM proteins are poorly understood. In epithelial cells, a specialized pathway is dedicated to restrict the deposition of BM proteins basally. Here, we report the identification of a factor in this pathway, a homolog of the mammalian guanine nucleotide exchange factor (GEF Mss4, which we have named Stratum. The loss of Stratum leads to the missecretion of BM proteins at the apical side of the cells, forming aberrant layers in close contact with the plasma membrane. We found that Rab8GTPase acts downstream of Stratum in this process. Altogether, our results uncover the importance of this GEF/Rab complex in specifically coordinating the basal restriction of BM proteins, a critical process for the establishment and maintenance of epithelial cell polarity.

  5. Post-streptococcal glomerulonephritis

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    Odalovic A.

    2014-01-01

    Full Text Available Post-streptococcal glomerulonephritis (PSGN is a frequent cause of acute nephritis in children. This case study was done with the aim to point out that the infections caused by Group A streptococci, in spite of antibiotic era, are still present in the population. An 8-year old boy was admitted in our hospital with a two-day history of fewer, tonsillopharyngitis. After hospital admission, patient was treated with penicillin during the period of 10 days, antihypertensive medications (captopril, furosemide, including restricted diet of salt. After the treatment, patient became better. On demission it was found proteinuria and microhematuria PSGN is very serious disease, which leaves severe complications if the valid therapy with penicillin is not used in propriety time, during the recommended period of 10 days.

  6. Glomerulonephritis-induced changes in kidney gene expression in rats

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    Mira Pavkovic

    2015-12-01

    Full Text Available We investigated a glomerulonephritis (GN model in rats induced by nephrotoxic serum (NTS which contains antibodies against the glomerular basement membrane (GBM. The anti-GBM GN model in rats is widely used since its biochemical and histopathological characteristics are similar to crescentic nephritis and Goodpasture's disease in humans (Pusey, 2003 [2]. Male Wistar Kyoto (WKY and Sprague–Dawley (SD rats were dosed once with 1, 2.5 and 5 ml/kg nephrotoxic serum (NTS or 1.5 and 5 ml/kg NTS, respectively. GN and tubular damage were observed histopathologically in all treated rats after 14 days. To obtain insight into molecular processes during GN pathogenesis, mRNA expression was investigated in WKY and SD kidneys using Affymetrix's GeneChip Rat genome 230_2.0 arrays (GSE64265. The immunopathological processes during GN are still not fully understood and likely involve both innate and adaptive immunity. In the present study, several hundred mRNAs were found deregulated, which functionally were mostly associated with inflammation and regeneration. The β-chain of the major histocompatibility complex class II RT1.B (Rt1-Bb and complement component 6 (C6 were identified as two mRNAs differentially expressed between WKY and SD rat strains which could be related to known different susceptibilities to NTS of different rat strains; both were increased in WKY and decreased in SD rats (Pavkovic et al., 2015 [1]. Increased Rt1-Bb expression in WKY rats could indicate a stronger and more persistent cellular reaction of the adaptive immune system in this strain, in line with findings indicating adaptive immune reactions during GN. The complement cascade is also known to be essential for GN development, especially terminal cascade products like C6.

  7. Immunohistochemical distribution of laminin-332 and collagen type IV in the basement membrane of normal horses and horses with induced laminitis.

    Science.gov (United States)

    Visser, M B; Pollitt, C C

    2011-07-01

    The basement membrane (BM) is a thin layer of extracellular matrix that regulates cell functions as well as providing support to tissues of the body. Primary components of the BM of epithelial tissues are laminin-332 (Ln-332) and collagen type IV. Equine laminitis is a disease characterized by destruction and dislocation of the hoof lamellar BM. Immunohistochemistry was used to characterize the distribution of Ln-332 and collagen type IV in the organs of normal horses and these proteins were found to be widespread. Analysis of a panel of tissue samples from horses with experimentally-induced laminitis revealed that Ln-332 and collagen type IV degradation occurs in the skin and stomach in addition to the hoof lamellae. These findings suggest that BM degradation is common to many epithelial tissues during equine laminitis and suggests a role for systemic trigger factors in this disease. Crown Copyright © 2010. Published by Elsevier Ltd. All rights reserved.

  8. Immunohistochemical localization of chondroitin sulfate, chondroitin sulfate proteoglycan, heparan sulfate proteoglycan, entactin, and laminin in basement membranes of postnatal developing and adult rat lungs

    DEFF Research Database (Denmark)

    Sannes, P L; Burch, K K; Khosla, J

    1993-01-01

    , and laminin. A monoclonal antibody specific for the glycosaminoglycan portion (CS) of CSPG and a monoclonal antibody against the core protein of CSPG were used in an immunoperoxidase sequence to stain extracellular matrix (ECM) components of pulmonary basement membranes (BMs). Anti-CS stained airway BM...... alveolar, airway, and vascular BMs, in addition to smooth muscle external laminae (EL), in the adult and developing rat. Immunostaining for CSPG required hyaluronidase digestion, whereas CS staining was lost with the same treatment. A polyclonal antibody to the core protein of HSPG was found...... with CSPG, except that entactin showed particular affinity for EL. These results offer a more detailed perspective on previous survey observations of CSPG, HSPG, and entactin in the rat lung, and describe the immunoreactivity of CS for the first time.(ABSTRACT TRUNCATED AT 250 WORDS)...

  9. In vitro blood-brain barrier models for drug research: state-of-the-art and new perspectives on reconstituting these models on artificial basement membrane platforms.

    Science.gov (United States)

    Banerjee, Jayati; Shi, Yejiao; Azevedo, Helena S

    2016-09-01

    In vitro blood-brain barrier (BBB) models are indispensable screening tools for obtaining early information about the brain-penetrating behaviour of promising drug candidates. Until now, in vitro BBB models have focused on investigating the interplay among cellular components of neurovascular units and the effect of fluidic sheer stress in sustaining normal BBB phenotype and functions. However, an area that has received less recognition is the role of the noncellular basement membrane (BM) in modulating BBB physiology. This review describes the state-of-the-art on in vitro BBB models relevant in drug discovery research and highlights their strengths, weaknesses and the utility potential of some of these models in testing the permeability of nanocarriers as vectors for delivering therapeutics to the brain. Importantly, our review also introduces a new concept of engineering artificial BM platforms for reconstituting BBB models in vitro. Copyright © 2016 Elsevier Ltd. All rights reserved.

  10. Reciprocal interactions between Beta1-integrin and epidermal growth factor in three-dimensional basement membrane breast cultures: A different perspective in epithelial biology

    Energy Technology Data Exchange (ETDEWEB)

    Wang, F.; Weaver, V.M.; Petersen, O.W.; Larabell, C.A.; Dedhar, S.; Briand, P.; Lupu, R.; Bissell, M.J.

    1998-09-30

    Anchorage and growth factor independence are cardinal features of the transformed phenotype. Although it is logical that the two pathways must be coregulated in normal tissues to maintain homeostasis, this has not been demonstrated directly. We showed previously that down-modulation of {beta}1-integrin signaling reverted the malignant behavior of a human breast tumor cell line (T4-2) derived from phenotypically normal cells (HMT-3522) and led to growth arrest in a threedimensional (3D) basement membrane assay in which the cells formed tissue-like acini (14). Here, we show that there is a bidirectional cross-modulation of {beta}1-integrin and epidermal growth factor receptor (EGFR) signaling via the mitogenactivated protein kinase (MAPK) pathway. The reciprocal modulation does not occur in monolayer (2D) cultures. Antibodymediated inhibition of either of these receptors in the tumor cells, or inhibition of MAPK kinase, induced a concomitant downregulation of both receptors, followed by growth-arrest and restoration of normal breast tissue morphogenesis. Crossmodulation and tissue morphogenesis were associated with attenuation of EGF-induced transient MAPK activation. To specifically test EGFR and {beta}1-integrin interdependency, EGFR was overexpressed in nonmalignant cells, leading to disruption of morphogenesis and a compensatory up-regulation of {beta}1-integrin expression, again only in 3D. Our results indicate that when breast cells are spatially organized as a result of contact with basement membrane, the signaling pathways become coupled and bidirectional. They further explain why breast cells fail to differentiate in monolayer cultures in which these events are mostly uncoupled. Moreover, in a subset of tumor cells in which these pathways are misregulated but functional, the cells could be 'normalized' by manipulating either pathway.

  11. TH1 and TH17 cells promote crescent formation in experimental autoimmune glomerulonephritis.

    Science.gov (United States)

    Hünemörder, Stefanie; Treder, Julia; Ahrens, Stefanie; Schumacher, Valéa; Paust, Hans-Joachim; Menter, Thomas; Matthys, Patrick; Kamradt, Thomas; Meyer-Schwesinger, Catherine; Panzer, Ulf; Hopfer, Helmut; Mittrücker, Hans-Willi

    2015-09-01

    Autoimmunity against the Goodpasture antigen α3IV-NC1 results in crescentic glomerulonephritis (GN). Both antibodies and T cells directed against α3IV-NC1 have been implicated in disease development and progression. Using the model of experimental autoimmune glomerulonephritis (EAG) in DBA/1 mice, we aimed to characterize the frequency and function of α3IV-NC1-specific CD4(+) T cells in the kidneys. DBA/1 mice repeatedly immunized with human α3IV-NC1 developed necrotizing/crescentic GN. Kidneys with crescentic GN contained CD4(+) cells responding to α3IV-NC1 with the production of IFN-γ or IL-17A, demonstrating the accumulation of both α3IV-NC1-specific TH1 and TH17 cells. To test the functional relevance of TH1 and TH17 cells, EAG was induced in DBA/1 mice deficient in IFN-γR, IL-17A or IL-23p19. Mice of all knockout groups mounted α3IV-NC1 IgG, developed nephrotic range proteinuria, and IgG deposition to the glomerular basement membranes at levels similar to immunized wild-type mice. However, all knockout groups showed significantly fewer glomerular crescents and attenuated tubulointerstitial damage. Our results suggest that both α3IV-NC1-specific TH1 and TH17 cells accumulate in the kidneys and are crucial for the development of necrotizing/crescentic GN. Copyright © 2015 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

  12. Chronic granulomatous disease associated with chronic glomerulonephritis

    DEFF Research Database (Denmark)

    Frifelt, J J; Schønheyder, Henrik Carl; Valerius, Niels Henrik

    1985-01-01

    A boy with chronic granulomatous disease (CGD) developed glomerulonephritis at the age of 12 years. The glomerulonephritis progressed to terminal uraemia at age 15 when maintenance haemodialysis was started. The clinical course was complicated by pulmonary aspergillosis and Pseudomonas septicaemia...

  13. Products of the unc-52 gene in Caenorhabditis elegans are homologous to the core protein of the mammalian basement membrane heparan sulfate proteoglycan.

    Science.gov (United States)

    Rogalski, T M; Williams, B D; Mullen, G P; Moerman, D G

    1993-08-01

    Mutations in the unc-52 gene of Caenorhabditis elegans affect attachment of the myofilament lattice to the muscle cell membrane. Here, we demonstrate that the unc-52 gene encodes a nematode homolog of perlecan, the mammalian basement membrane heparan sulfate proteoglycan. The longest potential open reading frame of this gene encodes a 2482-amino-acid protein with a signal peptide and four domains. The first domain is unique to the unc-52 polypeptide, whereas the three remaining domains contain sequences found in the LDL receptor (domain II) laminin (domain III) and N-CAM (domain IV). We have identified three alternatively spliced transcripts that encode different carboxy-terminal sequences. The two larger transcripts encode proteins containing all or part of domain IV, whereas the smaller transcript encodes a shortened polypeptide that completely lacks domain IV. We have determined that the disorganized muscle phenotype observed in unc-52(st196) animals is caused by the insertion of a Tc1 transposon into domain IV. Two monoclonal antibodies that recognize an extracellular component of all contractile tissues in C. elegans fail to stain embryos homozygous for a lethal unc-52 allele. We have mapped the epitopes recognized by both monoclonal antibodies to a region of domain IV in the unc-52-encoded protein sequence.

  14. Radioimmunoassay in children with glomerulonephritis

    International Nuclear Information System (INIS)

    Ametov, A.S.; Gavryushova, L.P.; Shashinka, M.; Mumladze, Eh.B.; Tvorogova, T.M.; Dakhuk, B.; Dronova, V.I.; Toritsina, D.K.; Petrova, T.V.

    1985-01-01

    Proceeding from a radioimmunoassay of various biologically active substances in the blood and urine (ACTH, cortisol, FSH, LH, prolatin, progesterone, estradiol, plasma renin activity and β 2 -microglobulin) of 220 children with glomerulonephritis change of all indices with relation to the type and gravity of glomerulonephritis as well as renal function was revealed. The nature of influence on corticosteroid and immunosuppressive therapy was shown. The authors consider it appropriate to use the determination of biologically active substances in the blood and urine for a more profound estimation of a child's status

  15. Reticular Basement Membrane Vessels Are Increased in COPD Bronchial Mucosa by Both Factor VIII and Collagen IV Immunostaining and Are Hyperpermeable

    Directory of Open Access Journals (Sweden)

    Amir Soltani

    2012-01-01

    Full Text Available Background and Objective. Using Collagen IV staining, we have previously reported that the reticular basement membrane (Rbm is hypervascular and the lamina propria (LP is hypovascular in COPD airways. This study compared Collagen IV staining with vessels marked with anti-Factor VIII and examined vessel permeability in bronchial biopsies from COPD and normal subjects using albumin staining. Results. Anti-Collagen IV antibody detected more vessels in the Rbm (P=0.002 and larger vessels in both Rbm (P<0.001 and LP (P=0.003 compared to Factor VIII. COPD airways had more vessels (with greater permeability in the Rbm (P=0.01 and fewer vessels (with normal permeability in the LP compared to controls with both Collagen IV and Factor VIII antibodies (P=0.04 and P=0.01. Conclusion. Rbm vessels were increased in number and were hyperpermeable in COPD airways. Anti-Collagen IV and anti-Factor VIII antibodies did not uniformly detect the same vessel populations; the first is likely to reflect larger and older vessels with the latter reflecting smaller, younger vessels.

  16. Reduced fibulin-2 contributes to loss of basement membrane integrity and skin blistering in mice lacking integrin α3β1 in the epidermis.

    Science.gov (United States)

    Longmate, Whitney M; Monichan, Ruby; Chu, Mon-Li; Tsuda, Takeshi; Mahoney, My G; DiPersio, C Michael

    2014-06-01

    Deficient epidermal adhesion is a hallmark of blistering skin disorders and chronic wounds, implicating integrins as potential therapeutic targets. Integrin α3β1, a major receptor in the epidermis for adhesion to laminin-332 (LN-332), has critical roles in basement membrane (BM) organization during skin development. In the current study we identify a role for α3β1 in promoting stability of nascent epidermal BMs through induction of fibulin-2, a matrix-associated protein that binds LN-332. We demonstrate that mice lacking α3β1 in the epidermis display ruptured BM beneath neo-epidermis of wounds, characterized by extensive blistering. This junctional blistering phenocopies defects reported in newborn α3-null mice, as well as in human patients with α3 gene mutations, indicating that the developmental role of α3β1 in BM organization is recapitulated during wound healing. Mice lacking epidermal α3β1 also have reduced fibulin-2 expression, and fibulin-2-null mice display perinatal skin blisters similar to those in α3β1-deficient mice. Interestingly, α3-null wound epidermis or keratinocytes also show impaired processing of the LN-332 γ2 chain, although this defect was independent of reduced fibulin-2 and did not appear to cause blistering. Our findings indicate a role for integrin α3β1 in BM stability through fibulin-2 induction, both in neonatal skin and in adult wounds.

  17. A two-dimensional model of the colonic crypt accounting for the role of the basement membrane and pericryptal fibroblast sheath.

    Directory of Open Access Journals (Sweden)

    Sara-Jane Dunn

    Full Text Available The role of the basement membrane is vital in maintaining the integrity and structure of an epithelial layer, acting as both a mechanical support and forming the physical interface between epithelial cells and the surrounding connective tissue. The function of this membrane is explored here in the context of the epithelial monolayer that lines the colonic crypt, test-tube shaped invaginations that punctuate the lining of the intestine and coordinate a regular turnover of cells to replenish the epithelial layer every few days. To investigate the consequence of genetic mutations that perturb the system dynamics and can lead to colorectal cancer, it must be possible to track the emerging tissue level changes that arise in the crypt. To that end, a theoretical crypt model with a realistic, deformable geometry is required. A new discrete crypt model is presented, which focuses on the interaction between cell- and tissue-level behaviour, while incorporating key subcellular components. The model contains a novel description of the role of the surrounding tissue and musculature, based upon experimental observations of the tissue structure of the crypt, which are also reported. A two-dimensional (2D cross-sectional geometry is considered, and the shape of the crypt is allowed to evolve and deform. Simulation results reveal how the shape of the crypt may contribute mechanically to the asymmetric division events typically associated with the stem cells at the base. The model predicts that epithelial cell migration may arise due to feedback between cell loss at the crypt collar and density-dependent cell division, an hypothesis which can be investigated in a wet lab. This work forms the basis for investigation of the deformation of the crypt structure that can occur due to proliferation of cells exhibiting mutant phenotypes, experiments that would not be possible in vivo or in vitro.

  18. Patterns of glomerulonephritis with crescents: Experience at a tertiary medical center in Saudi Arabia

    Directory of Open Access Journals (Sweden)

    Turki Al-Hussain

    2017-01-01

    Full Text Available A series of 78 cases of glomerulonephritis (GN, in which renal biopsy revealed changes of GN associated with crescent formation, were reviewed. Renal pathology findings were correlated with clinical features including patient’s age, renal function, and serologic findings. In most of the cases (71.8%, the crescents were due to immune complex-mediated GN. This was followed by pauci-immune GN (20.5% and anti-glomerular basement membrane antibody (GBM GN (7.7%. The percentage of glomeruli with crescents was the highest in cases of anti-GBM disease (mean of 93.3%, followed by pauci-immune GBM (mean of 48.2% and immune complex GN (30.9%. In cases with the pauci- immune GN, there were additional features of glomerular injury including fibrinoid necrosis, disruption of the GBM, and rupture of Bowman’s capsule. These changes were generally more pronounced in a subset of pauci-immune GN associated with serum elevation of antineutrophil cytoplasmic antibody (c-ANCA. In biopsies from patient with immune complex disease, systemic lupus erythematosus was the most common cause of crescentic GN.

  19. The association of HLA-DQB1, -DQA1 and -DPB1 alleles with anti- glomerular basement membrane (GBM disease in Chinese patients

    Directory of Open Access Journals (Sweden)

    Zhou Xu-Jie

    2011-05-01

    Full Text Available Abstract Background Human leukocyte antigen (HLA alleles are associated with many autoimmune diseases, including anti-glomerular basement membrane (GBM disease. In our previous study, it was demonstrated that HLA-DRB1*1501 was strongly associated with anti-GBM disease in Chinese. However, the association of anti-GBM disease and other HLA class II genes, including HLA-DQB1, -DQA1,-DPB1 alleles, has rarely been investigated in Asian, especially Chinese patients. The present study further analyzed the association between anti-GBM disease and HLA-DQB1, -DQA1, and -DPB1 genes. Apart from this, we tried to locate the potential risk amino acid residues of anti-GBM disease. Methods This study included 44 Chinese patients with anti-GBM disease and 200 healthy controls. The clinical and pathological data of the patients were collected and analyzed. Typing of HLA-DQB1, -DQA1 and -DPB1 alleles were performed by bi-directional sequencing of exon 2 using the SeCoreTM Sequencing Kits. Results Compared with normal controls, the prevalence of HLA-DPB1*0401 was significantly lower in patients with anti-GBM disease (3/88 vs. 74/400, p = 4.4 × 10-4, pc = 0.039. Comparing with normal controls, the combination of presence of DRB1*1501 and absence of DPB1*0401 was significantly prominent among anti-GBM patients (p = 2.0 × 10-12, pc = 1.7 × 10-10. Conclusions HLA-DPB1*0401 might be a protective allele to anti-GBM disease in Chinese patients. The combined presence of DRB1*1501 and absence of DPB1*0401 might have an even higher risk to anti-GBM disease than HLA-DRB1*1501 alone.

  20. Detection of gelatinolytic activity in developing basement membranes of the mouse embryo head by combining sensitive in situ zymography with immunolabeling.

    Science.gov (United States)

    Gkantidis, Nikolaos; Katsaros, Christos; Chiquet, Matthias

    2012-10-01

    Genetic evidence indicates that the major gelatinases MMP-2 and MMP-9 are involved in mammalian craniofacial development. Since these matrix metalloproteinases are secreted as proenzymes that require activation, their tissue distribution does not necessarily reflect the sites of enzymatic activity. Information regarding the spatial and temporal expression of gelatinolytic activity in the head of the mammalian embryo is sparse. Sensitive in situ zymography with dye-quenched gelatin (DQ-gelatin) has been introduced recently; gelatinolytic activity results in a local increase in fluorescence. Using frontal sections of wild-type mouse embryo heads from embryonic day 14.5-15.5, we optimized and validated a simple double-labeling in situ technique for combining DQ-gelatin zymography with immunofluorescence staining. MMP inhibitors were tested to confirm the specificity of the reaction in situ, and results were compared to standard SDS-gel zymography of tissue extracts. Double-labeling was used to show the spatial relationship in situ between gelatinolytic activity and immunostaining for gelatinases MMP-2 and MMP-9, collagenase 3 (MMP-13) and MT1-MMP (MMP-14), a major activator of pro-gelatinases. Strong gelatinolytic activity, which partially overlapped with MMP proteins, was confirmed for Meckel's cartilage and developing mandibular bone. In addition, we combined in situ zymography with immunostaining for extracellular matrix proteins that are potential gelatinase substrates. Interestingly, gelatinolytic activity colocalized precisely with laminin-positive basement membranes at specific sites around growing epithelia in the developing mouse head, such as the ducts of salivary glands or the epithelial fold between tongue and lower jaw region. Thus, this sensitive method allows to associate, with high spatial resolution, gelatinolytic activity with epithelial morphogenesis in the embryo.

  1. Poliomyelitis in MuLV-infected ICR-SCID mice after injection of basement membrane matrix contaminated with lactate dehydrogenase-elevating virus.

    Science.gov (United States)

    Carlson Scholz, Jodi A; Garg, Rohit; Compton, Susan R; Allore, Heather G; Zeiss, Caroline J; Uchio, Edward M

    2011-10-01

    The arterivirus lactate dehydrogenase-elevating virus (LDV) causes life-long viremia in mice. Although LDV infection generally does not cause disease, infected mice that are homozygous for the Fv1(n) allele are prone to develop poliomyelitis when immunosuppressed, a condition known as age-dependent poliomyelitis. The development of age-dependent poliomyelitis requires coinfection with endogenous murine leukemia virus. Even though LDV is a common contaminant of transplantable tumors, clinical signs of poliomyelitis after inadvertent exposure to LDV have not been described in recent literature. In addition, LDV-induced poliomyelitis has not been reported in SCID or ICR mice. Here we describe the occurrence of poliomyelitis in ICR-SCID mice resulting from injection of LDV-contaminated basement membrane matrix. After exposure to LDV, a subset of mice presented with clinical signs including paresis, which was associated with atrophy of the hindlimb musculature, and tachypnea; in addition, some mice died suddenly with or without premonitory signs. Mice presenting within the first 6 mo after infection had regions of spongiosis, neuronal necrosis and astrocytosis of the ventral spinal cord, and less commonly, brainstem. Axonal degeneration of ventral roots prevailed in more chronically infected mice. LDV was identified by RT-PCR in 12 of 15 mice with typical neuropathology; positive antiLDV immunolabeling was identified in all PCR-positive animals (n = 7) tested. Three of 8 mice with neuropathology but no clinical signs were LDV negative by RT-PCR. RT-PCR yielded murine leukemia virus in spinal cords of all mice tested, regardless of clinical presentation or neuropathology.

  2. Crescentic glomerulonephritis developing in the course of idiopathic membranoproliferative glomerulonephritis

    Directory of Open Access Journals (Sweden)

    Alok Sharma

    2013-01-01

    Full Text Available Membranoproliferative glomerulonephritis (MPGN is a rare cause of the nephrotic syndrome in adults and children. Though small focal crescents may be seen in up to 10% of cases of MPGN, the presence of more than 50% crescents (crescentic MPGN is rare. Very few cases of crescentic transformation of MPGN, documented by subsequent renal biopsies, have been described in the literature. A young female patient underwent kidney biopsy for the nephrotic-nephritic syndrome and was diagnosed as idiopathic MPGN. She was administered immunosuppressive therapy (steroids and cyclophosphamide, with which her renal functions stabilized. Six months later, she presented with features suggestive of rapidly progressive renal failure and underwent a second renal biopsy. The second biopsy showed crescentic glomerulonephritis with immune complex deposition, suggestive of MPGN. A final diagnosis of crescentic transformation of MPGN was made. Crescentic transformation of MPGN is a rare occurrence, but needs to be considered in a patient diagnosed as MPGN and presenting with rapidly progressive renal failure. The cause of such transformation remains to be elucidated.

  3. Targeted Expression of Stromelysin-1 in Mammary Gland Provides Evidence for a Role of Proteinases in Branching Morphogenesis and the Requirement for an Intact Basement Membrane for Tissue-specific Gene Expression

    Energy Technology Data Exchange (ETDEWEB)

    Sympson, Carolyn J; Talhouk, Rabih S; Alexander, Caroline M; Chin, Jennie R; Cliff, Shirley M; Bissell, Mina J; Werb, Zena

    1994-05-01

    The extracellular matrix (ECM) is an important regulator of the differentiated phenotype of mammary epithelial cells in culture. Despite the fact that ECM-degrading enzymes have been implicated in morphogenesis and tissue remodeling, there is little evidence for a direct role for such regulation in vivo. We generated transgenic mice that express autoactivated isoforms of the matrix metalloproteinase stromelysin-1, under the control of the whey acidic protein gene promoter, to examine the effect of inappropriate expression of this enzyme. Stromelysin-1 is implicated as the primary player in the loss of basement membrane and loss of function in the mammary gland during involution. The transgene was expressed at low levels in mammary glands of virgin female mice, leading to an unexpected phenotype: The primary ducts had supernumerary branches and showed precocious development of alveoli that expressed beta-casein at levels similar to that of an early- to mid-pregnant gland. Lactating glands showed high levels of transgene expression, with accumulation at the basement membrane, and a decrease in laminin and collagen IV, resulting in a loss of basement membrane integrity; this was accompanied by a dramatic alteration of alveolar morphology, with decreased size and shrunken lumina containing little beta-casein. During pregnancy, expression of endogenous whey acidic protein and beta-casein was reduced in transgenic glands, confirming the observed dependence of milk protein transcription of ECM in mammary epithelial cells in culture. These data provide direct evidence that stromelysin-1 activity can be morphogenic for mammary epithelial cells, inducing hyperproliferation and differentiation in virgin animals, and that its lytic activity can, indeed, disrupt membrane integrity and reduce mammary-specific function. We conclude that the balance of ECM-degrading enzymes with their inhibitors, and the associated regulation of ECM structure, is crucial for tissue-specific gene

  4. How to Study Basement Membrane Stiffness as a Biophysical Trigger in Prostate Cancer and Other Age-related Pathologies or Metabolic Diseases.

    Science.gov (United States)

    Rodriguez-Teja, Mercedes; Breit, Claudia; Clarke, Mitchell; Talar, Kamil; Wang, Kai; Mohammad, Mohammad A; Pickwell, Sage; Etchandy, Guillermina; Stasiuk, Graeme J; Sturge, Justin

    2016-09-20

    Here we describe a protocol that can be used to study the biophysical microenvironment related to increased thickness and stiffness of the basement membrane (BM) during age-related pathologies and metabolic disorders (e.g. cancer, diabetes, microvascular disease, retinopathy, nephropathy and neuropathy). The premise of the model is non-enzymatic crosslinking of reconstituted BM (rBM) matrix by treatment with glycolaldehyde (GLA) to promote advanced glycation endproduct (AGE) generation via the Maillard reaction. Examples of laboratory techniques that can be used to confirm AGE generation, non-enzymatic crosslinking and increased stiffness in GLA treated rBM are outlined. These include preparation of native rBM (treated with phosphate-buffered saline, PBS) and stiff rBM (treated with GLA) for determination of: its AGE content by photometric analysis and immunofluorescent microscopy, its non-enzymatic crosslinking by sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS PAGE) as well as confocal microscopy, and its increased stiffness using rheometry. The procedure described here can be used to increase the rigidity (elastic moduli, E) of rBM up to 3.2-fold, consistent with measurements made in healthy versus diseased human prostate tissue. To recreate the biophysical microenvironment associated with the aging and diseased prostate gland three prostate cell types were introduced on to native rBM and stiff rBM: RWPE-1, prostate epithelial cells (PECs) derived from a normal prostate gland; BPH-1, PECs derived from a prostate gland affected by benign prostatic hyperplasia (BPH); and PC3, metastatic cells derived from a secondary bone tumor originating from prostate cancer. Multiple parameters can be measured, including the size, shape and invasive characteristics of the 3D glandular acini formed by RWPE-1 and BPH-1 on native versus stiff rBM, and average cell length, migratory velocity and persistence of cell movement of 3D spheroids formed by PC3 cells under

  5. Glomerulonephritis

    Science.gov (United States)

    ... they're also more likely to recover quickly. Bacterial endocarditis. Bacteria occasionally can spread through your bloodstream and ... such as a damaged or artificial heart valve. Bacterial endocarditis is associated with glomerular disease, but the connection ...

  6. Nephrotic syndrome due to lupus-like glomerulonephritis in an HIV-positive patient

    NARCIS (Netherlands)

    Wiegersma, J. S.; Franssen, C. F. M.; Diepstra, A.

    2017-01-01

    Lupus nephritis, a well-known complication in systemic lupus erythematosus, is characterised by a proliferative glomerulonephritis or membranous nephropathy along with a full-house immunofluorescence pattern on renal biopsy. There are very few exceptions in which similar histopathological findings

  7. Green tea polyphenol (--epigallocatechin-3-gallate restores Nrf2 activity and ameliorates crescentic glomerulonephritis.

    Directory of Open Access Journals (Sweden)

    Ting Ye

    Full Text Available Crescentic glomerulonephritis (GN is the most severe form of GN and is associated with significant morbidity and mortality despite aggressive immunotherapy with steroids, cytotoxic drugs, and plasmapheresis. We examined the therapeutic efficacy of the green tea polyphenol (--epigallocatechin-3-gallate (EGCG, 50 mg/kg BW/day x3 weeks, a potent anti-inflammatory and anti-oxidant agent, on experimental crescentic GN induced in 129/svJ mice by administration of rabbit anti-mouse glomerular basement membrane sera. Routine histology and key molecules involved in inflammatory and redox signaling were studied. EGCG treatment significantly reduced mortality, decreased proteinuria and serum creatinine, and markedly improved renal histology when compared with vehicle-treated mice. The improvements in renal function and histology were accompanied by the restoration of Nrf2 signaling (which was impaired in vehicle-treated mice as shown by increased nuclear translocation of Nrf2 and cytoplasmic glutamate cysteine ligase catalytic subunit, glutamate cysteine ligase modifier subunit, and glutathione peroxidase. EGCG-treated mice also showed reduction in p-Akt, p-JNK, p-ERK1/2 and p-P38 as well as restoration of PPARγ and SIRT1 levels. Lower dose of EGCG (25 mg/kg BW/day x2 weeks treatment also significantly decreased proteinuria and serum creatinine, and markedly improved renal histology when compared with vehicle-treated mice. Thus, our data illustrate the efficacy of EGCG in reversing the progression of crescentic GN in mice by targeting multiple signaling and inflammatory pathways as well as countering oxidative stress.

  8. Glomerulonephritis in Saudi Arabia: A Review

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    Mitwalli Ahmed

    2000-01-01

    Full Text Available Glomerulonephritis (GN is one of the leading causes of end-stage renal disease. A good understanding of its pattern and prevalence as well as exploration of effective therapeutic strategies for protecting the glomerulus, would have tremendous impact on public health. In the Kingdom of Saudi Arabia (KSA, focal and segmental glomerulo sclerosis (FSGS is the commonest type of primary glomerular diseases (PGD encountered in clinical practice. Its prevalence varies from less than 4% in Gizan, in the southern part of KSA, to approximately 35% in Riyadh, central Saudi Arabia. In our experience, the nephrotic syndrome was the commonest mode of presentation of FSGS. Response to corticosteroid therapy is generally poor and the mortality rate is high. Mesangioproliferative GN is the second most common GN constituting up to 25% of PGD in our experience. Other researchers from different parts of the Kingdom, however, have given prevalence rates ranging from 8 to 57.1%. The reported prevalence of Immunoglobulin-A nephropathy (IgAN in KSA ranges from 5.8% to 13.6%. It is more common in the elderly, and men are affected more often than women. In contrast to KSA, IgAN is the commonest PGD in Japan, China, Hong Kong, Singapore and Taiwan. Membranous GN (MGN is less common in KSA than encountered elsewhere, the prevalence ranging from 3.9 to 21.8%. Nephropathy secondary to systemic diseases are also common in KSA. Lupus nephritis (LN accounted for 48.5% of secondary glomerular diseases (SGD with the combination of WHO classes III and IV (aggressive types of LN accounting for 56% of all patients. LN is another disease where differences in racial susceptibility may account for the uneven distribution. Post-streptococcal GN seems to be declining in frequency in KSA, the reported prevalence ranging from 2.7% to 2.9%.

  9. Selective IgA deficiency associated with glomerulonephritis and oligoarthritis.

    OpenAIRE

    Camilleri, J P; Moore, R H; Griffiths, D F; Williams, B D

    1992-01-01

    A 59 year old woman with selective IgA deficiency associated with oligoarthritis and glomerulonephritis is described. She was seropositive for rheumatoid factor and renal histological examination showed a focal glomerulonephritis. High titre rheumatoid factor and a focal glomerulonephritis were also present in the only other well documented report of selective IgA deficiency and renal disease. Histological examination of the kidney suggested that the glomerulonephritis was mediated by immune ...

  10. Spectrum of Glomerulonephritis in Egypt

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    Barsoum Rashad

    2000-01-01

    Full Text Available 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

  11. Pretransplant interferon prevents hepatitis C virus-associated glomerulonephritis in renal allografts by HCV-RNA clearance.

    Science.gov (United States)

    Cruzado, Josep M; Casanovas-Taltavull, Teresa; Torras, Joan; Baliellas, Carme; Gil-Vernet, Salvador; Grinyó, Josep M

    2003-03-01

    The purpose of this study was to examine the effect of pretransplant interferon administration on the occurrence of post-transplant de novo glomerulonephritis in hepatitis C virus (HCV)-positive renal allografts. From December 1992 to December 2000, 78 HCV-positive patients received a renal allograft in our unit. Fifteen out of 78 received pretransplant interferon for 1 year. Hepatitis C virus was investigated by serology and qualitative polymerase chain reaction (PCR). Hepatitis C virus-related de novo glomerulonephritis (membranoproliferative or membranous) was suggested by proteinuria (>1.5 g/24 h) and/or microhematuria and always diagnosed by renal biopsy. Of 15 HCV-positive recipients who received pretransplant interferon, 10 (67%) became HCV-RNA negative at the time of transplantation and only one out of the 15 (6.7%) developed de novo glomerulonephritis (this patient was HCV-RNA positive at transplantation). Among non-interferon-treated allograft recipients, 28.7% had negative HCV-RNA and 12 out of 63 (19%) developed de novo glomerulonephritis (9, membranoproliferative; 3 membranous), all 12 having positive HCV-RNA at transplantation (p < 0.0001). In conclusion, pretransplant interferon may reduce the occurrence of post-transplant HCV-related de novo glomerulonephritis. Our results suggest that the indication for pretransplant interferon should be extended to treat all HCV-RNA positive candidates for renal transplantation.

  12. Acute Post-Streptococcal Glomerulonephritis in Children

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    M. Yu. Kagan

    2016-01-01

    Full Text Available Acute post-streptococcal glomerulonephritis is the most common form of glomerulonephritis in children, continues to be a major concern for pediatricians and nephrologists worldwide. The disease is caused by prior streptococcal infection caused by -hemolytic streptococcus of A group. Nephritis-associated plasmin receptor and streptococcal pyrogenic exotoxin B are considered to be major renotrophic antigens. Clinical findings of the disease can vary from asymptomatic gross hematuria to the full-blown acute nephritic syndrome (edema, gross hematuria, hypertension, renal failure. The main laboratory findings of acute post-streptococcal glomerulonephritis include an abnormal common urine analysis (dysmorphic erythrocytes, varying degrees of proteinuria, red blood cell casts, and segmentonuclear neutrophils, an elevated titer of antibodies to antistreptococcic antigens, and hypocomplementemia. Treatment of patients is mainly supportive and is focused upon the elimination of hypervolemia and its complications (edema, hypertension. In this situation, the most effective agents are loop diuretics and antihypertensive agents. The prognosis in children is generally favourable. The review analyses the current understanding of the pathogenesis, clinical manifestations, diagnosis, treatment and prognosis of acute post-streptococcal glomerulonephritis.

  13. The Spectrum of Glomerulonephritis in Saudi Arabia: The Results of the Saudi Registry

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    Huraib S

    2000-01-01

    Full Text Available Only few studies regarding glomerulonephritis, with relatively small numbers of patients, have so far been published from different centers in Saudi Arabia, and have reported conflicting results regarding the patterns, even in the same city. The possible reasons for these differences include the small number of patients in the different studies, differences in the indications for renal biopsies, referral bias, geographical differences, and, sometimes, the non-availability of the necessary diagnostic facilities in the reporting centers. In order to overcome these problems, a registry for glomerulonephropathy was attempted in Saudi Arabia. Six large referral hospitals from different regions of Saudi Arabia participated in this registry. Biopsy reports and clinical information of 1294 renal biopsies were obtained. There were 782 renal biopsies due to glomerulonephritis (GN accounting for 77.2% of the total biopsies. Five hundred eighty seven (72.6% were primary glomerulonephritidis. Focal and segmental glomerulosclerosis (FSGS (21.3% and membrano-proliferative glomerulonephritis (MPGN (20.7% were the most common types found in the primary glomerulonephritidis. Membranous glomerulonephritis (MGN was present in only 10.6% of the cases. IgA nephropathy was found in 6.5% of the cases. Of the secondary glomerulo-nephritides, systemic lupus erythematosus (SLE was the most common indication for biopsy (57.0% and amyloidosis was found in only 3.2% of the biopsies. In conclusion, FSGS and MPGN were the most common forms of primary glomerulonephritis in adult patients in Saudi Arabia. MGN was not as common as in the western world. SLE was the commonest cause of secondary GN. Amyloidosis was not as common as in other Arab countries. There is a need for more centers from Saudi Arabia to join this national GN registry. Similar registries can be established in different Arab countries, which all would, hopefully, lead to a Pan-Arab GN registry.

  14. Scleroderma with crescentic glomerulonephritis: a case report

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    Ramaswami Arunachalam

    2008-05-01

    Full Text Available Abstract Introduction Systemic sclerosis or scleroderma is an autoimmune rheumatic disease characterized by organ-based fibrosis. Renal involvement in scleroderma occurs mainly in the form of scleroderma renal crisis, affecting 5 to 10% of patients. It remains one of the most important and immediately life-threatening complications of scleroderma, but the prognosis improves considerably after treatment with angiotensin-converting enzyme inhibitors. Other renal pathologies can occur in scleroderma. These include scleroderma overlap syndromes with associated features of lupus nephritis, myeloperoxidase anti-neutrophil cytoplasmic antibodies (ANCA or proteinase 3 ANCA-associated glomerulonephritis, or crescentic glomerulonephritis. These alternative pathologies should be suspected in any individual patient with a differing clinical picture and the patient should be appropriately investigated. Crescentic glomerulonephritis occurs very rarely in scleroderma. This report describes a patient with scleroderma and crescentic glomerulonephritis. Case presentation A 52-year-old woman with a known history of scleroderma and hypertension on angiotensin-converting enzyme inhibitors was referred to the nephrologist because of a rapid decline in renal function. Kidney biopsy was performed which revealed immune complex type crescentic glomrulonephritis. Cytoplasmic-staining ANCA was negative. Despite immunosuppressive treatment the patient rapidly went into end-stage renal failure and is still on hemodialysis. Conclusion Scleroderma is a complex disease, and the best characterized renal involvement in scleroderma is scleroderma renal crisis. However, other renal pathologies can occur in scleroderma. These alternative pathologies should be suspected in any patient with a differing clinical picture and the patient should be appropriately investigated, as the clinical course and treatment are different from the more common scleroderma renal crisis.

  15. Prospects for Precision Medicine in Glomerulonephritis Treatment

    OpenAIRE

    Liu, Yulu Cherry; Chun, Justin

    2018-01-01

    Background: Glomerulonephritis (GN) consists of a group of kidney diseases that are categorized based on shared histopathological features. The current classifications for GN make it difficult to distinguish the individual variability in presentation, disease progression, and response to treatment. GN is a significant cause of end-stage renal disease (ESRD), and improved therapies are desperately needed because current immunosuppressive therapies sometimes lack efficacy and can lead to signif...

  16. Dermatomyositis Sine Myositis with Membranoproliferative Glomerulonephritis

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    Mohammad Bagher Owlia

    2012-01-01

    Full Text Available Dermatomyositis (DM is an autoimmune disease that is characterized by involvement of proximal musculature and skin. We report a 52-year-old woman with a 6-year history of dermatomyositis sine myositis, who developed lower extremity edema and proteinuria. Pathological examination of renal biopsy showed membranoproliferative glomerulonephritis. She received steroid, cyclophosphamide, and mycophenolate mofetil. Over the 9 to 10 months after the beginning of treatment, the proteinuria was improved.

  17. Biopsy-proven childhood glomerulonephritis in Johor.

    Science.gov (United States)

    Khoo, J J; Pee, S; Thevarajah, B; Yap, Y C; Chin, C K

    2004-06-01

    There has been no published study of biopsy-proven childhood glomerulonephritis in Malaysia. To determine the pattern of childhood glomerulonephritis in Johor, Malaysia from a histopathological perspective and the various indications used for renal biopsy in children. Retrospective study was done of all renal biopsies from children under 16 years of age, received in Sultanah Aminah Hospital, Johor between 1994 and 2001. The histopathological findings were reviewed to determine the pattern of biopsy-proven glomerulonephritis. The indications for biopsy, mode of therapy given after biopsy and the clinical outcome were studied. 122 adequate biopsies were received, 9 children had repeat biopsies. Of the 113 biopsies, minimal change disease formed the most common histopathological diagnosis (40.7%) while lupus nephritis formed the most common secondary glomerulonephritis (23.0%). The main indications for biopsy were nephrotic syndrome (50.8%), lupus nephritis (25.4%) and renal impairment (13.1%). The mode of therapy was changed in 59.8% of the children. Of 106 patients followed-up, 84 children were found to have normal renal function in remission or on treatment. 4 patients developed chronic renal impairment and 16 reached end stage renal disease. Five of the 16 children with end stage disease had since died while 11 were on renal replacement therapy. Another 2 patients died of other complications. The pattern of childhood GN in our study tended to reflect the more severe renal parenchymal diseases in children and those requiring more aggressive treatment. This was because of our criteria of selection (indication) for renal biopsy. Renal biopsy where performed appropriately in selected children may not only be a useful investigative tool for histological diagnosis and prognosis but may help clinicians plan the optimal therapy for these children.

  18. Formation of tight junctions between neighboring podocytes is an early ultrastructural feature in experimental crescentic glomerulonephritis

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    Succar L

    2016-11-01

    Full Text Available Lena Succar,1 Ross A Boadle,2 David C Harris,1,3 Gopala K Rangan1,3 1Centre for Transplant and Renal Research, The Westmead Institute for Medical Research, The University of Sydney, 2Electron Microscopy Laboratory, Institute of Clinical Pathology and Medical Research, Westmead Hospital, 3Department of Renal Medicine, Westmead Hospital, Western Sydney Local Health District, Westmead, Sydney, NSW, Australia Purpose: In crescentic glomerulonephritis (CGN, the development of cellular bridges between podocytes and parietal epithelial cells (PECs triggers glomerular crescent formation. However, the sequential changes in glomerular ultrastructure in CGN are not well defined. This study investigated the time course of glomerular ultrastructure in experimental CGN. Methods: Transmission electron microscopy (TEM was performed using kidney samples from rats with nephrotoxic serum nephritis (NSN from day 1 to day 14. Morphometric analysis was conducted on randomly selected glomeruli captured on TEM digital images. Results: On day 1 of NSN, there was widespread formation of focal contacts between the cell bodies of neighboring podocytes, and tight junctions were evident at the site of cell-to-cell contact. This was confirmed by the increased expression of the tight junction molecule, zonula occludens-1 (ZO-1, which localized to the points of podocyte cell–cell body contact. On day 2, the interpodocyte distance decreased and the glomerular basement membrane thickness increased. Foot process effacement (FPE was segmental on day 3 and diffuse by day 5, accompanied by the formation of podocyte cellular bridges with Bowman’s capsule, as confirmed by a decrease in podocyte-to-PEC distance. Fibrinoid necrosis and cellular crescents were evident in all glomeruli by days 7 and 14. In vitro, the exposure of podocytes to macrophage-conditioned media altered cellular morphology and caused an intracellular redistribution of ZO-1. Conclusion: The formation of tight

  19. Cortical blindness in a boy with acute glomerulonephritis.

    Science.gov (United States)

    Yang, Min-Hsien; Sheu, Ji-Nan; Wang, Shuoh-Jyh

    2003-01-01

    Post-infectious acute glomerulonephritis with hypertensive encephalopathy is characterized by episodic hypertension with headache, vomiting, and hematuria. The association between hypertensive encephalopathy and cortical blindness in children with acute glomerulonephritis is extremely rare. We report the case of a 10-year-old boy with acute glomerulonephritis who presented with gross hematuria, headache, vomiting, and oliguria, and developed transient cortical blindness as a complication of hypertensive encephalopathy. No occurrence of seizure was observed during the clinical course. T2-weighted cranial magnetic resonance imaging showed a high-intensity signal over cortical and subcortical areas of bilateral occipital regions. His vision recovered fully, 2 days after receiving antihypertensive therapy. The patient was well without complaint at 1-year follow-up. This case highlights the possibility that cortical blindness may develop as a complication of acute glomerulonephritis in children. Prevention of the occurrence of neurological deficits in children with acute glomerulonephritis and hypertensive encephalopathy requires careful evaluation and appropriate management of hypertension.

  20. Colorectal laterally spreading tumors show characteristic expression of cell polarity factors, including atypical protein kinase C λ/ι, E-cadherin, β-catenin and basement membrane component.

    Science.gov (United States)

    Ichikawa, Yasushi; Nagashima, Yoji; Morioka, Kaori; Akimoto, Kazunori; Kojima, Yasuyuki; Ishikawa, Takashi; Goto, Ayumu; Kobayashi, Noritoshi; Watanabe, Kazuteru; Ota, Mitsuyoshi; Fujii, Shoichi; Kawamata, Mayumi; Takagawa, Ryo; Kunizaki, Chikara; Takahashi, Hirokazu; Nakajima, Atsushi; Maeda, Shin; Shimada, Hiroshi; Inayama, Yoshiaki; Ohno, Shigeo; Endo, Itaru

    2014-09-01

    Colorectal flat-type tumors include laterally spreading tumors (LSTs) and flat depressed-type tumors. The former of which shows a predominant lateral spreading growth rather than an invasive growth. The present study examined the morphological characteristics of LSTs, in comparison with polypoid- or flat depressed-type tumors, along with the expression of atypical protein kinase C (aPKC) λ/ι, a pivotal cell polarity regulator, and the hallmarks of cell polarity, as well as with type IV collagen, β-catenin and E-cadherin. In total, 37 flat-type (24 LSTs and 13 flat depressed-type tumors) and 20 polypoid-type colorectal tumors were examined. The LSTs were classified as 15 LST adenoma (LST-A) and nine LST cancer in adenoma (LST-CA). An immunohistochemical examination was performed on aPKC λ/ι, type IV collagen, β-catenin and E-cadherin. The LST-A and -CA showed a superficial replacing growth pattern, with expression of β-catenin and E-cadherin in the basolateral membrane and type IV collagen along the basement membrane. In addition, 86.6% of LST-A and 55.6% of LST-CA showed aPKC λ/ι expression of 1+ (weak to normal intensity staining in the cytoplasm compared with the normal epithelium). Furthermore, ~45% of the polypoid-type adenomas showed 2+ (moderate intensity staining in the cytoplasm and/or nucleus) and 66.7% of the polypoid-type cancer in adenoma were 3+ (strong intensity staining in the cytoplasm and nucleus). A statistically significant positive correlation was observed between the expression of aPKC λ/ι and β-catenin (r=0.842; P<0.001), or type IV collagen (r=0.823; P<0.001). The LSTs showed a unique growth pattern, different from the expanding growth pattern presented by a polypoid tumor and invasive cancer. The growth characteristics of LST appear to be caused by adequate coexpression of β-catenin, type IV collagen and aPKC λ/ι.

  1. Hypocomplementemic Urticarial Vasculitis Syndrome With Crescentic Glomerulonephritis.

    Science.gov (United States)

    Salim, Sohail Abdul; Yousuf, Tauqeer; Patel, Asha; Fülöp, Tibor; Agarwal, Mohit

    2018-02-01

    Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disease characterized by multiple organ system involvement, including renal disease, with low complement levels. We report the case of a 31-year-old woman who presented with nonspecific symptoms including fatigue, diarrhea, macular rash and abdominal pain with acute renal failure leading to end-stage kidney disease. Laboratory results showed hematuria, nephrotic range proteinuria, worsening creatinine and low C1q levels. Left kidney biopsy showed proliferative glomerulonephritis with crescent formation. She was treated with 6 months of intravenous cyclophosphamide, followed by 2 doses of intravenous rituximab (1g each), thereafter maintained on mycophenolate mofetil and glucocorticoid-based therapy. She experienced a full recovery of renal function after 12 months of dialysis dependence. Hypocomplementemic urticarial vasculitis syndrome with crescentic glomerulonephritis is a rare disease with only 5 other reported cases in literature. In our case, we document a delayed but excellent renal recovery during a 2-year follow-up. Copyright © 2018 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

  2. Ameliorative effects of arctiin from Arctium lappa on experimental glomerulonephritis in rats.

    Science.gov (United States)

    Wu, Jian-Guo; Wu, Jin-Zhong; Sun, Lian-Na; Han, Ting; Du, Jian; Ye, Qi; Zhang, Hong; Zhang, Yu-Guang

    2009-11-01

    Membranous glomerulonephritis (MGN) remains the most common cause of adult-onset nephrotic syndrome in the world and up to 40% of untreated patients will progress to end-stage renal disease. Although the treatment of MGN with immunosuppressants or steroid hormones can attenuate the deterioration of renal function, numerous treatment-related complications have also been established. In this study, the ameliorative effects of arctiin, a natural compound isolated from the fruits of Arctium lappa, on rat glomerulonephritis induced by cationic bovine serum albumin (cBSA) were determined. After oral administration of arctiin (30, 60, 120 mg/kgd) for three weeks, the levels of serum creatinine (Scr) and blood urea nitrogen (BUN) and 24-h urine protein content markedly decreased, while endogenous creatinine clearance rate (ECcr) significantly increased. The parameters of renal lesion, hypercellularity, infiltration of polymorphonuclear leukocyte (PMN), fibrinoid necrosis, focal and segmental proliferation and interstitial infiltration, were reversed. In addition, we observed that arctiin evidently reduced the levels of malondialdehyde (MDA) and pro-inflammatory cytokines including interleukin-6 (IL-6) and tumor necrosis factor (TNF-alpha), suppressed nuclear factor-kappaB p65 (NF-kappaB) DNA binding activity, and enhanced superoxide dismutase (SOD) activity. These findings suggest that the ameliorative effects of arctiin on glomerulonephritis is carried out mainly by suppression of NF-kappaB activation and nuclear translocation and the decreases in the levels of these pro-inflammatory cytokines, while SOD is involved in the inhibitory pathway of NF-kappaB activation. Arctiin has favorable potency for the development of an inhibitory agent of NF-kappaB and further application to clinical treatment of glomerulonephritis, though clinical studies are required.

  3. Study of glomerulonephritis evolution in Castilla-La Mancha (GLOMANCHA) between 1994 and 2008.

    Science.gov (United States)

    Conde Olasagasti, José Luis; Acevedo Ribó, Mercedes; Roca Muñoz, Ana; Vozmediano Poyatos, Carmen; Rivera, Francisco; Illescas Fernández-Bermejo, María Luisa; López Rubio, Esperanza; Usón Carrasco, Javier; Tallón Lobo, Serafín; Quiroga, Borja; de Arriba, Gabriel

    2016-01-01

    Renal biopsy registries allow histopathological data to be collected to improve knowledge of different pathologies and their natural history. To analyse the data of the Castilla La Mancha Glomerulonephritis Registry (GLOMANCHA) and the evolution of the different biopsy-proven pathologies between 1994 and 2008. The 6 most common biopsy-proven pathologies were collected during the 14 years of the study (941 biopsies) in the 5 participant centres of the autonomous community. In 2008, we assessed patient renal survival and mortality and we evaluated associated factors to each situation for each pathology. Of the 941 biopsies, 59% belonged to men, with a mean age of 48±18 years. At the time of the biopsy, the median glomerular filtration rate was 50.3 (25.5-76.3) ml/min/1,73 m(2) and median proteinuria was 3.4 (1.5-6.4) grams per day. The most common pathology were lupus nephropathy, followed by focal segmental glomerulosclerosis and membranous nephropathy. Lupus nephropathy and minimal change disease achieved the best renal prognosis during follow-up (mean 7.3±4.8 years). Rapidly progressive glomerulonephritis type 3 and focal segmental glomerulosclerosis had the worst renal prognosis. In addition, rapidly progressive glomerulonephritis type 3 presented the worst vital prognosis. In GLOMANCHA, we demonstrate the poor prognosis of rapidly progressive glomerulonephritis type 3, in contrast to minimal change disease or lupus nephropathy. Renal function is an independent predictor of renal survival and mortality in this study. Copyright © 2016 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

  4. The Spectrum of Glomerulonephritis in Adults in Madinah Munawarah Region

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    Bernieh Bassam

    2000-01-01

    Full Text Available To evaluate the patterns of glomerular diseases in the Madinah region in Saudi Arabia we reviewed 85 biopsies from native kidneys of adult patients presenting with clinical and or biological features suggestive of glomerular disease. Only light microscopy was used to evaluate these biopsies. There were 62 males and 23 females with age ranging from 12 to 80 years (mean 28 + 13.4 years. The main clinical presenting features were nephrotic syndrome in 68 patients (80%, renal insufficiency in 31 (36.5%, hypertension in 25 (24% and/or hematuria in 19 (22.4%. Histopathology revealed minimal change disease in 25 patients (29%, mesangioproliferative glomerulonephritis (GN in 13 (15.3%, focal segmental glomerulosclerosis in 13 (15.3%, membranoproliferative GN in seven (8.2%, membranous GN in three (3.5%, diffuse proliferative GN in five (5.9%, crescentic GN in four (4.7%, lupus nephritis in 13 (15.3%, and amyloidosis in two (2.4%. In conclusion, Minimal change disease was the most common type of primary GN in our patients. The mean age for this group was 27.2 (range 13-43 years. Lupus nephritis was the commonest secondary GN in the Madinah region.

  5. Measure Guideline: Basement Insulation Basics

    Energy Technology Data Exchange (ETDEWEB)

    Aldrich, R.; Mantha, P.; Puttagunta, S.

    2012-10-01

    This guideline is intended to describe good practices for insulating basements in new and existing homes, and is intended to be a practical resources for building contractors, designers, and also to homeowners.

  6. Viruses in the Oceanic Basement.

    Science.gov (United States)

    Nigro, Olivia D; Jungbluth, Sean P; Lin, Huei-Ting; Hsieh, Chih-Chiang; Miranda, Jaclyn A; Schvarcz, Christopher R; Rappé, Michael S; Steward, Grieg F

    2017-03-07

    Microbial life has been detected well into the igneous crust of the seafloor (i.e., the oceanic basement), but there have been no reports confirming the presence of viruses in this habitat. To detect and characterize an ocean basement virome, geothermally heated fluid samples (ca. 60 to 65°C) were collected from 117 to 292 m deep into the ocean basement using seafloor observatories installed in two boreholes (Integrated Ocean Drilling Program [IODP] U1362A and U1362B) drilled in the eastern sediment-covered flank of the Juan de Fuca Ridge. Concentrations of virus-like particles in the fluid samples were on the order of 0.2 × 10 5 to 2 × 10 5  ml -1 ( n = 8), higher than prokaryote-like cells in the same samples by a factor of 9 on average (range, 1.5 to 27). Electron microscopy revealed diverse viral morphotypes similar to those of viruses known to infect bacteria and thermophilic archaea. An analysis of virus-like sequences in basement microbial metagenomes suggests that those from archaeon-infecting viruses were the most common (63 to 80%). Complete genomes of a putative archaeon-infecting virus and a prophage within an archaeal scaffold were identified among the assembled sequences, and sequence analysis suggests that they represent lineages divergent from known thermophilic viruses. Of the clustered regularly interspaced short palindromic repeat (CRISPR)-containing scaffolds in the metagenomes for which a taxonomy could be inferred (163 out of 737), 51 to 55% appeared to be archaeal and 45 to 49% appeared to be bacterial. These results imply that the warmed, highly altered fluids in deeply buried ocean basement harbor a distinct assemblage of novel viruses, including many that infect archaea, and that these viruses are active participants in the ecology of the basement microbiome. IMPORTANCE The hydrothermally active ocean basement is voluminous and likely provided conditions critical to the origins of life, but the microbiology of this vast habitat is not

  7. Cardiovascular function in chronic glomerulonephritis: mechanocardiographic study.

    Science.gov (United States)

    Ishikawa, H; Sumoto, H; Hasegawa, S; Nagao, M; Matsumoto, Y; Botan, Y; Soda, T

    1979-02-01

    The relations between mechanocardiographic measurements, types of chronic glomerulonephritis (CGN) and clinical data were examined in 70 cases of CGN (20 cases of the latent type, 6 cases of the nephritic type, 14 cases of the nephrotic type, 10 cases of the hypertensive type and 20 cases of the endstage). (1) In the different types of CGN, mechanocardiographic abnormalities increased in the order: latent type less than nephritic type less than nephrotic type less than hypertensive type less than endstage. (2) In the endstage, prolongations of ICT, ICT2 and PEP (p less than 0.01), shortening of LVET (p less than 0.05) and decrease of LVET/PEP (p less than 0.01) were observed. These abnormalities may be related to decreased myocardial contractility. (3) Analysis of the relations between mechanocardiographic measurements and results of various tests in all the cases showed that ICT, ICT2 and PEP were most nearly related to GFR, DBP, gamma-globulin and serum sodium and Q-T, Q-II/Q-T and (Q-T) -- (Q-II) were nearly related to Ht and age. The changes in cardiac function may be entirely due to increased blood pressure, decreased renal function, electrolyte disturbance, dysproteinemia and anemia associated with CGN. Abnormality in cardiac function seems to depend on the relative abnormalities of these factors and the extents of their influences.

  8. Viruses in the Oceanic Basement

    Directory of Open Access Journals (Sweden)

    Olivia D. Nigro

    2017-03-01

    Full Text Available Microbial life has been detected well into the igneous crust of the seafloor (i.e., the oceanic basement, but there have been no reports confirming the presence of viruses in this habitat. To detect and characterize an ocean basement virome, geothermally heated fluid samples (ca. 60 to 65°C were collected from 117 to 292 m deep into the ocean basement using seafloor observatories installed in two boreholes (Integrated Ocean Drilling Program [IODP] U1362A and U1362B drilled in the eastern sediment-covered flank of the Juan de Fuca Ridge. Concentrations of virus-like particles in the fluid samples were on the order of 0.2 × 105 to 2 × 105 ml−1 (n = 8, higher than prokaryote-like cells in the same samples by a factor of 9 on average (range, 1.5 to 27. Electron microscopy revealed diverse viral morphotypes similar to those of viruses known to infect bacteria and thermophilic archaea. An analysis of virus-like sequences in basement microbial metagenomes suggests that those from archaeon-infecting viruses were the most common (63 to 80%. Complete genomes of a putative archaeon-infecting virus and a prophage within an archaeal scaffold were identified among the assembled sequences, and sequence analysis suggests that they represent lineages divergent from known thermophilic viruses. Of the clustered regularly interspaced short palindromic repeat (CRISPR-containing scaffolds in the metagenomes for which a taxonomy could be inferred (163 out of 737, 51 to 55% appeared to be archaeal and 45 to 49% appeared to be bacterial. These results imply that the warmed, highly altered fluids in deeply buried ocean basement harbor a distinct assemblage of novel viruses, including many that infect archaea, and that these viruses are active participants in the ecology of the basement microbiome.

  9. Acute post-streptococcal glomerulonephritis followed shortly by acute rheumatic fever.

    OpenAIRE

    Kwong, Y. L.; Chan, K. W.; Chan, M. K.

    1987-01-01

    A 16 year old girl with post-streptococcal glomerulonephritis developed acute rheumatic fever 19 days afterwards. Previous publications on concurrent post-streptococcal glomerulonephritis and acute rheumatic fever are reviewed.

  10. Pathological features of glomerulonephritis in Jakarta

    Directory of Open Access Journals (Sweden)

    Sutisna Himawan

    2002-03-01

    Full Text Available All cases of renal biopsies received during a 10-year period from 1990-1999 were collected and analyzed. There were a totat of 1344 cases, comprising 390 pediatric cases, 9 I 8 adult cases and 36 cases of unknown age. Immunofluorescence microscopy was performed on 1089 cases (81.0%. The purpose of this study is to have an overview of the pattem and spectrum of glomerular diseases in Indonesia, especially in Jakarta and surroundings, with special emphasis on the cases with nephrotic syndrome, lupus nephritis and IgA nephropathy, and to compare the findings with previous reports from Indonesia and afew other countries. There were 250 cases of childhood nephrotic syndrome and 479 adult cases. The most frequent histopathological appearance in both groups was minimal change disease, i.e. 58.2% and 44.7% respectively. Males were more often affected than females with a ratio of 2.0:1 for children and 1.4:1 for adults. Lupus nephritis comprised 124 cases, among which three cases were not representative. The male to female ratio was 1:7.9. Most cases were in the fourth decade, i.e. 47 cases (38.5%, and the most frequent histopathological appearance was WHO class IV with 71 cases (58.7%. There were 97 cases of IgA nephropathy with an age range between 3 to 58 years. The peak incidence was in the fourth decade with 32 cases (33%. The male to female ratio was L7: I. The most frequent histopathological appearances were diffuse sclerosing lesion 34 cases (35% and mesangial proliftrative lesion 33 cases (34%. (Med J Indones 2002; 11: 24-9Keywords: renal biopsy, pathological features, glomerulonephritis, nephrotic syndrome, lupus nephritis, IgA nephropathy

  11. Glomerulonephritis with Acute Renal Failure Related to Osteomyelitis

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    Chang-I Ho

    2008-06-01

    Full Text Available We report a case of acute renal failure in which renal biopsy showed immune complex-mediated proliferative glomeru-lonephritis. The patient had been afflicted with progressive lower back pain for 2 months. No fever was noted. Magnetic resonance imaging of the lumbar spine demonstrated characteristic findings of vertebral osteomyelitis and bilateral psoas abscesses. Culture of the computed tomography-guided needle aspirated specimens grew Staphylococcus aureus. The patient was treated with prolonged antibiotic therapy. The lower back pain improved gradually. Furthermore, significant improvement in renal function occurred approximately 2 weeks after the beginning of antibiotic treatment. The pathologic findings and clinical causal relationship between the bacterial osteomyelitis and the glomerulonephritis suggests a close association of the 2 conditions. Although glomerulonephritis is well documented to be related to a vari-ety of infectious diseases, its association with osteomyelitis has rarely been reported. We review the clinicopathologic characteristics of another 3 cases previously reported in the literature.

  12. Experimental glomerulonephritis induced by hydrocarbon exposure: A systematic review

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    Ravnskov Uffe

    2005-12-01

    Full Text Available Abstract Background Much epidemiological evidence suggests that hydrocarbon exposure may induce glomerulonephritis and worsen its course in many patients. The mechanisms are unknown, however, no specific microscopic pattern has been identified, and it has also been argued that hydrocarbon exposure causes tubular damage mainly. Studying experimental animals may best answer these questions, and as no systematic review of glomerulonephritis produced experimentally by hydrocarbon exposure has been performed previously, I found it relevant to search for and analyse such studies. Methods Animal experiments having mimicked human glomerulonephritis by hydrocarbon exposure were sought on Medline and Toxnet Results Twenty-six experiments using thirteen different hydrocarbons were identified. Several human subtypes were observed including IgA nephritis, mesangial, proliferative and extracapillary glomerulonephritis, focal and focal-segmental sclerosis, minimal change nephropathy, anti-GBM and anti-TBM nephritis, and glomerulonephritis associated with peiarteritis nodosa. Glomerular proteinuria was seen in 10/12 experiments that included urine analyses, and renal failure in 5/8 experiments that included measurements of glomerular function. All experiments resulted in various degrees of tubular damage as well. In most studies, where the animals were examined at different times during or after the exposure, the renal microscopic and functional changes were seen immediately, whereas deposits of complement and immunoglobulins appeared late in the course, if at all. Conclusion These experiments are in accord with epidemiological evidence that hydrocarbon exposure may cause glomerulonephritis and worsen renal function. Probable mechanisms include an induction of autologous antibodies and a disturbance of normal immunological functions. Also, tubular damage may increase postglomerular resistance, resulting in a glomerular deposition of macromolecules. In most

  13. Kidney Transplantation Rates Across Glomerulonephritis Subtypes in the United States.

    Science.gov (United States)

    OʼShaughnessy, Michelle M; Liu, Sai; Montez-Rath, Maria E; Lafayette, Richard A; Winkelmayer, Wolfgang C

    2017-10-01

    Whether kidney transplantation rates differ by glomerulonephritis (GN) subtype remains largely unknown. Using the US Renal Data System, we identified all adult patients with end-stage renal disease attributed to 1 of 6 GN subtypes who initiated dialysis in the US (1996-2013). Patients with diabetic nephropathy (DN) and autosomal-dominant polycystic kidney disease (ADPKD) served as "external" non-GN comparators. Using Cox proportional hazards regression, with death considered a competing risk, we estimated hazard ratios (HRs) (95% confidence intervals [CI]) for first kidney transplantation, controlling for year, demographics, comorbidities, socioeconomic factors, and Organ Procurement Organization. Among 718 480 patients studied, unadjusted and multivariable-adjusted transplant rates differed considerably across GN subtypes. Adjusted transplant rates were highest for patients with IgA nephropathy (IgAN) (referent) and lower for all other groups: focal segmental glomerulosclerosis (HR, 0.80; 95% CI, 0.77-0.82), membranous nephropathy (HR, 0.88; 95% CI, 0.83-0.93), membranoproliferative GN (HR, 0.84; 95% CI, 0.76-0.92), lupus nephritis (HR, 0.69; 95% CI, 0.66-0.71), vasculitis (HR, 0.66; 95% CI, 0.61-0.70), DN (HR, 0.50; 95% CI, 0.47-0.52), ADPKD (HR, 0.85; 95% CI, 0.82-0.88). Reduced kidney transplantation rates among comparator groups were driven more so by lower rates of waitlisting (HRs vs IgAN, ranged from 0.49 for DN to 0.92 for membranous nephropathy or ADPKD) than by lower rates of deceased donor kidney transplantation after waitlisting (rates were only significantly lower, vs IgAN, for those with secondary GN subtypes: lupus nephritis [HR,0.91; 95% CI, 0.86-0.97], vasculitis [HR, 0.85; 95% CI, 0.76-0.94), DN [HR, 0.73; 95% CI, 0.69-0.77]). Identifying underlying reasons for apparent disease-specific barriers to kidney transplantation might inform center-specific transplant candidate selection procedures, along with national organ allocation policies, leading

  14. Descriptive analysis of glomerulonephritis by histological type and their progression among adults in a tertiary care center in Sri Lanka.

    Science.gov (United States)

    Gunawardena, Kawmadi W; Wijewickrama, Eranga S; Arambepola, Carukshi; Lanerolle, Rushika D

    2018-01-01

    Prevalence of different glomerulonephritides and their clinical course vary geographically. Our objectives are to assess the prevalence of different histological types of glomerulonephritis (GN) based on the light microscopic histology and to assess their progression according to histological type. A retrospective cross-sectional study was carried out among adult patients (>18 years) with a histological diagnosis of GN at the University Professorial Unit over a period of six months. Information including demographic data, renal biopsy findings, and progression of the disease through serum creatinine (SCr) level were collected through existing clinic records of consenting patients. Data were analyzed by Statistical Package for the Social Sciences. There were 109 patients (females = 90) with a mean age of 40.32 ± 15.24 years. The most common histological type was focal segmental glomerulosclerosis (FSGS) in 27 (24.8%) followed by minimal change disease in 25 (22.9%), mesangioproliferative glomerulonephritis (MesPGN) in 18 (16.5%), membranoproliferative glomerulonephritis in six (5.5%), membranous glomerulonephritis in three patients (2.8%), and crescentic GN in one patient (0.9%). There was a statistically significant rise in SCr level at seven years from the initial presentation in the histological types; FSGS [P = 0.04; 95% confidence interval (CI) = 0.06-1.0] and MesPGN (P = 0.03; 95% CI = 0.3-0.9). Focal segmental glomerulosclerosis was the most common histology type in the population studied. There was a statistically significant progression of FSGS and MesPGN.

  15. Typhoid glomerulonephritis and intestinal perforation in a Nigerian ...

    African Journals Online (AJOL)

    2013-08-12

    Aug 12, 2013 ... The number of children with renal complications following salmonella infection cannot be precisely defined in the sub-Saharan Africa due to scarcity of reliable data. We report a 3-year-old boy with glomerulonephritis secondary to typhoid infection and later intestinal perforation. He presented with fever, ...

  16. Antimyeloperoxidase-associated proliferative glomerulonephritis: an animal model

    NARCIS (Netherlands)

    Brouwer, E.; Huitema, M. G.; Klok, P. A.; de Weerd, H.; Tervaert, J. W.; Weening, J. J.; Kallenberg, C. G.

    1993-01-01

    To develop an animal model for antimyeloperoxidase (MPO)-associated necrotizing crescentic glomerulonephritis (NCGN), we immunized Brown Norway rats with MPO and localized a neutrophil lysosomal enzyme extract, primarily consisting of MPO and elastinolytic enzymes, plus H2O2, the substrate of MPO,

  17. ANTIMYELOPEROXIDASE-ASSOCIATED PROLIFERATIVE GLOMERULONEPHRITIS - AN ANIMAL-MODEL

    NARCIS (Netherlands)

    BROUWER, E; HUITEMA, MG; KLOK, PA; DEWEERD, H; TERVAERT, JWC; WEENING, JJ; KALLENBERG, CGM

    1993-01-01

    To develop an animal model for antimyeloperoxidase (MPO)-associated necrotizing crescentic glomerulonephritis (NCGN), we immunized Brown Norway rats with MPO and localized a neutrophil lysosomal enzyme extract, primarily consisting of MPO and elastinolytic enzymes, plus H2O2, the substrate of MPO,

  18. Typhoid glomerulonephritis and intestinal perforation in a Nigerian ...

    African Journals Online (AJOL)

    The number of children with renal complications following salmonella infection cannot be precisely defined in the sub-Saharan Africa due to scarcity of reliable data. We report a 3-year-old boy with glomerulonephritis secondary to typhoid infection and later intestinal perforation. He presented with fever, generalized body ...

  19. Pneumococcal meningitis associated with glomerulonephritis: A case report

    Directory of Open Access Journals (Sweden)

    Vuletić Biljana

    2017-01-01

    Full Text Available Introduction. Streptococcus pneumoniae is the second most common cause of meningitis in children, producing more serious complications than other bacteria. Streptococcus pneumoniae infections are a rare trigger of glomerulonephritis. We presented a case of glomerulonephritis developing concurrently with meningitis in a young male child. Case report. Gross haematuria, significant proteinuria, hypertension and decreased level of C3 alongside the signs of central nervous system involvement occurred in a male patient of 5 years and 3 months of age. Spontaneous resolution of renal affliction parameters followed the successful treatment of meningitis. The disease course was strongly suggestive of postinfectious glomerulonephritis, although it manifested at the same time as meningitis. The absence of the latent period might point to the development of IgA nephropathy, but since the renal function was stable, without any abnormalities in urine tests documented during follow-up, our opinion is that this was rather the case of postinfectious nephropathy. Conclusion. The presented case is a unique clinical form of postinfectious glomerulonephritis. An accurate diagnosis of this entity should ensure the adequate treatment and follow-up of these patients.

  20. Pauci-Immune Crescentic Glomerulonephritis: An ANCA-Associated Vasculitis

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    Rafeel Syed

    2015-01-01

    Full Text Available Rapidly progressive glomerulonephritis (RPGN is a syndrome signified by a precipitous loss of renal function, with features of glomerulonephritis including dysmorphic erythrocyturia and glomerular proteinuria. RPGN is associated with extensive crescent formation, and, thus, the clinical term RPGN is often used interchangeably with the pathologic term crescentic glomerulonephritis (CGN. From an immunopathologic standpoint, primary RPGN is divided into pauci-immune GN (PICG, anti-GBM GN, and immune complex GN. PICG, the most common etiology of primary RPGN, refers to a necrotizing glomerulonephritis with few or no immune deposits by immunofluorescence (IF or electron microscopy (EM. In most patients, pauci-immune CGN is a component of a systemic small vessel vasculitis such as granulomatosis with polyangiitis (GPA. Approximately 90% of patients with PICG have circulating ANCA antibodies, leading to the nomenclature ANCA-associated vasculitis (AAV. Recent research has identified several other antibodies associated with PICG, which is now understood to be a complex spectrum of disease with considerable overlap in terms of clinical phenotype and outcomes. In addition, several genetic and environmental factors have recently been implicated in the pathogenesis of this disorder. With new prognostic classifications, enhanced understanding of immunopathologic mechanisms, and novel treatment paradigms, clinical and experimental interest in PICG remains high.

  1. C3 glomerulonephritis: a new category of glomerulonephritis with etiopathogenic implications

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    Taborda Murillo, Alejandra

    2015-01-01

    Full Text Available Introduction: Glomerulonephritis with only deposits of C3 (GN-C3 could involve alteration on the complement alternative pathway.Objective: To describe retrospectively a series of GNC3 cases and to determine the frequency with which patients continue with renal alterations and/or hypocomplementemia. Methods: The 22 cases of GN-C3 diagnosed between 2004 and 2012 at the Department of Pathology, Faculty of Medicine, University of Antioquia (Medellin, Colombia were included. Their histological and clinical characteristics and their outcome were evaluated. Results: 14 patients were children and 12 were males. Mean age was 13 years (range: 3-65. Ten presented as a nephritic syndrome, seven as a rapidly progressive GN, three as acute renal failure, one as chronic renal failure, and one as a nephrotic-nephritic syndrome. The C3 fraction of complement was low in 21 cases. All biopsies showed proliferative GN. There was complete remission in eight patients, persistent urinalysis alterations in four, chronic renal failure in six, five of them end- stage. No follow-up was done in four. In nine patients follow-up determination of C3 serum levels was done; in all of them they normalized between 1 to 3 months after biopsy. Conclusions: GN-C3 can produce persistent or recurrent kidney alterations and end-stage renal disease. Long-term follow-up with repeated determinations of C3 is advisable.

  2. Cerebral venous thrombosis in a patient with acute postinfectious glomerulonephritis

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    S Morkhandikar

    2016-01-01

    Full Text Available Thrombosis of the cerebral venous sinuses (CVT is described in nephrotic syndrome. A 13-year-old girl was admitted with acute post-infectious glomerulonephritis (APIGN. Subsequently she developed recurrent seizures with focal neurological deficits. On evaluation, she was found to have CVT. To the best of our knowledge, this is the first report of CVT in APIGN. Identifying this complication is imperative, as timely diagnosis and treatment could be lifesaving.

  3. Pauci-immune crescentic glomerulonephritis in the Down's syndrome.

    Science.gov (United States)

    Cherif, Mejda; Hedri, Hafedh; Ounissi, Mondher; Gergah, Taher; Goucha, Rim; Barbouch, Samia; Abderrahim, Ezzedine; Maiz, Hedi Ben; Kheder, Adel

    2013-11-01

    Kidney disease is a rare complication in patients with the Down's syndrome. However, with increased survival, it appears that a growing number of these patients present with glomerulonephritis. Most cases have been reported as case reports and include lesions such as mesangiocapillary glomerulonephritis with hypo-complementemia, crescentic glomerulonephritis with anti-neutrophil cytoplasmic antibodies (ANCA), amyloidosis and immunotactoid glomerulopathy. We report the observation of a 38-year-old man with the Down's syndrome who presented with severe renal failure, proteinuria and microscopic hematuria evolving over two months. There was no history of congenital heart disease or urinary symptoms. Percutaneous renal biopsy revealed fibrous crescents, rupture of Bowman's capsule and peri-glomerular granuloma; there were no deposits on immunofluorescence study. Thoracic computerized tomography scan showed alveolar congestion. The patient tested negative for ANCA. At the time of reporting, the patient is on regular chronic hemodialysis. Our case illustrates a distinct entity that further expands the spectrum of renal disease known to occur in the Down's syndrome. Early detection of the renal disorders may prevent or slow down the progression.

  4. Hepatitis B virus-related post-infectious glomerulonephritis: A case ...

    African Journals Online (AJOL)

    Hepatitis B virus infection is an uncommon cause of acute glomerulonephritis. We present a case of acute glomerulonephritis revealing a chronic viral hepatitis B. A 45 year-old man was admitted in the nephrology department of Hassan II university hospital (Fez, Morocco) for nephritic syndrome with advanced acute kidney ...

  5. Alteration of 99mTc-DMSA biodistribution in glomerulonephritis

    International Nuclear Information System (INIS)

    Rajic, M.; Bogicevic, M.; Ilic, S.; Vlajkovic, M.; Antic, S.; Mitic, B.; Avramovic, M.; Mitic-Zlatovic, M.; Stefanovic, V.

    2002-01-01

    The aim of this study was to assess the relation between 99T c-DMSA biodistribution and its reliability as a marker of renal function in patients with glomerular kidney diseases. Sixty-seven patients involved in this study were classified into two groups according to 99T c-DTPA clearance and serum creatinine values: the 1. group consisted of 42 patients without renal failure while the 2nd group included 25 patients with renal failure. 99T c-DMSA biodistribution was determined by measuring kidney, blood and urine activity at 2 h and 4 h. The results, compared with those of 23 healthy volunteers, indicated the quantitative alteration of 99T c-DMSA distribution in both glomerulonephritis patient groups. In reference to the control mean values of 2 h and 4 h, in patients without renal failure, kidney activity was found decreased to 52% and 57%, while the blood activity increase of 37% and 44% was recorded together with the urine activity increase of 38% and 23%. In patients with renal failure the alterations of renal and blood activity were more remarkable, but the urine loss was found to be unchanged. It is suggested that these biodistribution changes originate mainly from tubular impairment. However, in glomerulonephritis patients, altered glomerular filtration might considerably affect biodistribution of this radiopharmaceutical and limits its suitability for precise quantitative estimation of renal function. (author)

  6. Renal effects of renal x irradiation and induced autoallergic glomerulonephritis

    International Nuclear Information System (INIS)

    Rappaport, D.S.

    1977-01-01

    This study was conducted to determine what, if any, influence a single large x-ray exposure of kidney has on the development and course of an experimental autoallergic glomerulonephritis (EAG) in rats. The EAG was induced by immunization with B. pertussis vaccine and homogenate of homologous kidney tissue and Freund's complete adjuvant. Rats were either immunized, sham-immunized, irradiated (1500 R to right kidney temporarily exteriorized), sham-irradiated, or both immunized and irradiated. Immunized-irradiated animals were irradiated either 4 or 2 weeks prior to, concurrently with, or 1 or 2 weeks after immunization, and were sacrificed at 2, 4, 6, 10, or 14 weeks post-immunization. Immunized-only and sham-immunized-only animals were sacrificed at corresponding post-immunization times, and irradiated-only and sham-irradiated-only animals were sacrificed at corresponding post-irradiation times. Progressive arteriolonephrosclerosis (ANA) was observed in right (irradiated) kidneys following x irradiation. The experimental autoallergic glomerulonephritis (EAG) was observed in both kidneys following immunization. The histopathological changes associated with EAG were distinct from those associated with ANS

  7. Immuno-histochemistry analysis of Helicobacter pylori antigen in renal biopsy specimens from patients with glomerulonephritis

    Directory of Open Access Journals (Sweden)

    Qian Li

    2013-01-01

    Full Text Available This study was conducted to investigate the relationship between Helicobacter pylori infection and three varieties of glomerulonephritis. Renal biopsy specimens from patients with Henoch Schonlein Purpura nephritis (HSPN; n = 10, membranous nephropathy (MN; n = 9 and lupus nephritis (LN; n = 27 were studied using immuno-histochemical labeling to clarify the etiological significance of H. pylori antigen in this disease. Immuno-histochemical labeling was performed using a mixture of anti-H. pylori-antibody-positive serum from nine volunteers; a mixture of anti-H. pylori-antibody-negative serum from nine volunteers was used as control. Staphylococci protein-A labeled by horseradish peroxidase was used as the second antibody in this study. A total of 34 of the 48 specimens revealed positive reaction with the anti-H. pylori-positive serum and five of the 48 specimens revealed positive reaction with the anti-H. pylori-negative serum. Positive reaction against anti-H. pylori-positive serum was seen in 10/10 patients with HSPN, six of nine patients with MN and 18/27 patients with LN. Statistical analysis showed that the difference of the positive reaction between anti-H. pylori-positive and negative sera was significant (χ 2 = 36.318, P = 0.000. Our study indicates that H. pylori infection may be associated with the development and/or progression of HSPN, MN and LN.

  8. [Post-transplant recurrence of glomerulonephritis: a complex clinical case].

    Science.gov (United States)

    Bonucchi, Decenzio; Leonelli, Marco; Damiano, Francesca; Granito, Maria; Ghiandai, Giulia; De Amicis, Sara; Americo, Claudio; Ligabue, Giulia; Albertazzi, Vittorio; Cappelli, Gianni

    2010-01-01

    Lupus nephritis (LN) seldom recurs in a grafted kidney. By contrast, primary membranoproliferative glomerulonephritis (MPGN), which has been included, along with hemolytic uremic syndrome and age-related maculopathy, among the complement dysregulation diseases, has a high recurrence rate and is considered a contraindication to living-donor kidney transplant because of the poor prognosis. We report the case of a young girl with LN-related chronic renal failure who underwent a living donor transplant from her mother. After four months she had a recurrence that did not match the criteria for LN. Graft biopsies and revision of the clinical course pointed to type II MPGN on the basis of a lack of ARA criteria, persistent isolated low C3 levels, and response to plasma therapy. If confirmed by genetic analysis, the patient might benefit from treatment with the monoclonal antibody against the C5-C9 complex, eculizumab.

  9. [Rapidly progressive glomerulonephritis: a diagnostic and therapeutic emergency].

    Science.gov (United States)

    Halfon, Matthieu; Teta, Daniel; Rotman, Samuel; Pruijm, Menno; Humbert, Antoine

    2014-02-26

    Rapidly progressive glomerulonephritis (RPG) is a rare clinical syndrome characterized by kidney damage that can lead to irreversible kidney failure. RPG can be caused by primary glomerular disease or can be part of a systemic autoimmune disorder. All RPG have a similar pathophysiology (proliferation of cells in Bowman's capsule and formation of crescents) and clinical evolution (rapidly progressive kidney failure with proteinuria and an active urine sediment). Immunosuppressive therapy and sometimes plasma exchanges are required. Overall- and kidney survival are closely linked to the blood creatinine level at presentation, the percentage of damaged glomeruli, and to the underlying cause. RPG is therefore a diagnostic and therapeutic emergency that needs quick referral to a nephrologist.

  10. Membranoproliferative Glomerulonephritis and X-Linked Agammaglobulinemia: An Uncommon Association

    Directory of Open Access Journals (Sweden)

    Vasco Lavrador

    2014-01-01

    Full Text Available Introduction. X-linked agammaglobulinemia (XLA is a primary immunodeficiency characterized by agammaglobulinemia requiring replacement treatment with immunoglobulin. The association of XLA and membranoproliferative glomerulonephritis (MPGN is unexpected and, to our knowledge, only one case was previously published. Case Report. The authors report the case of a 10-year-old boy with family history and prenatal diagnosis of XLA, treated from birth with intravenous immunoglobulin replacement therapy. He presented with pneumonia, macroscopic hematuria, nephrotic proteinuria, hypoalbuminemia, and hypercholesterolemia with normal renal function and serum complement levels. Renal histology showed immune complex mediated MPGN. He was started on high dose prednisolone and ramipril and switched to weekly subcutaneous immunoglobulin. After a 4-month treatment, hematuria and proteinuria significantly improved and prednisolone was gradually tapered without relapse. Conclusion. The pathogenic process underlying MPGN development in this patient is unknown but residual humoral immunity might play an important role. Thus, this case highlights the risk of autoimmune disorders among patients with XLA.

  11. [Primary chronic polyarthritis with kidney involvement (mesangiocapillary glomerulonephritis)].

    Science.gov (United States)

    Bürkle, P A

    1979-01-01

    A 34 year old white male patient suffering from seropositive "probable" rheumatoid arthritis developed a severe hypocomplementemic mesangiocapillary glomerulo-nephritis. Rheumatoid factors (Latex test, Waaler-Rose titer) and IgM were markedly elevated in the serum. The third component of complement (C3) was markedly depressed, while the fourth component (C4) was within normal range. The rapid progression of the disease forced us to start an immunosuppressive drug therapy using azathioprine and steroids. Despite marked clinical improvement, e.g. normalisation of complement components, renal function, the disappearance of rheumatoid factor and proteinuria, the second biopsy taken two years later showed unchanged histological and immuno-histological changes of the glomerula.

  12. Renal effects of renal x irradiation and induced autoallergic glomerulonephritis

    International Nuclear Information System (INIS)

    Rappaport, D.S.; Casarett, G.W.

    1979-01-01

    This study was conducted to determine what influence a single large x-ray exposure of kidney has on the development and course of an experimental autoallergic glomerulonephritis (EAG) in rats. EAG was induced in female Sprague-Dawley rats by immunization with Bordetella pertussis vaccine and homogenate of homologous kidney tissue and Freund's complete adjuvant. Progressive arteriolonephrosclerosis (ANS) was observed in right (irradiated) kidneys following unilateral renal irradiation (1500 rad). Rats were either immunized, sham-immunized, irradiated, sham-irradiated, or both immunized and irradiated. Light and immunofluorescent microscopic observation, urine protein content, and kidney weights were evaluated. In immunized-irradiated animals the effects of irradiation and immunization were largely additive. Immunization did not considerably influence the development and course of ANS and irradiation did not considerably influence the development and course of EAG

  13. Post-streptococcal glomerulonephritis leading to posterior reversible encephalopathy syndrome: a case report.

    Science.gov (United States)

    Adikari, Madura; Priyangika, Dilani; Marasingha, Indika; Thamotheram, Sharmila; Premawansa, Gayani

    2014-09-13

    Posterior reversible encephalopathy syndrome is a clinical radiographic syndrome of heterogeneous etiologies. Developing hypertensive encephalopathy following post-streptococcal glomerulonephritis is a known but uncommon manifestation and developing posterior reversible encephalopathy syndrome in such a situation is very rare. We report a case with contrast-enhanced computed tomography and magnetic resonance imaging findings of posterior reversible encephalopathy syndrome in the background of acute post-streptococcal glomerulonephritis. A thirteen-year-old Sri Lankan boy presented with a focal fit by way of secondary generalization with duration of 10 minutes, and developed 2 similar fits subsequently following admission. He later developed severe hypertension with evidence of glomerulonephritis, which was diagnosed as acute post-streptococcal glomerulonephritis. A contrast-enhanced computed tomography imaging of brain done on day-3 revealed non-enhancing low-attenuating areas in fronto-parietal regions. A T2 weighted film of magnetic resonance imaging was done on day-10 of the admission and found to have linier sub-cortical hyper intensities in both parietal regions which were compatible with the radiological diagnosis of posterior reversible encephalopathy syndrome. Post-streptococcal glomerulonephritis is an important cause of acute nephritic syndrome especially in children. This case report illustrates a rare association of posterior reversible encephalopathy syndrome in a patient with post-streptococcal glomerulonephritis.

  14. Does Alport syndrome affect the basement membrane of peritoneal vessels?

    NARCIS (Netherlands)

    Sampimon, Denise E.; Vlijm, Anniek; Struijk, Dirk G.; Krediet, Raymond T.

    2010-01-01

    Alport syndrome and encapsulating peritoneal sclerosis (EPS) are both rare diseases. Their joint occurrence is highly unlikely. Two patients at our center with Alport syndrome developed EPS. We therefore hypothesized that Alport syndrome might predispose to the development of EPS and that this

  15. Basement Construction of Measurement Standardization for Thermal Property and Basement Preparation of Industrial Technology

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Kweon Ho; Song, Kee Chan; Park, Chang Je

    2007-02-15

    There are three main categories in this report : 1)Basement construction of measurement standardization for nuclear material thermal property, 2) Reliability evaluation of measurement instrument, and 3) Standardization and industrial propagation.

  16. Clinicopathologic spectrum of crescentic glomerulonephritis: A hospital-based study

    Directory of Open Access Journals (Sweden)

    Tauhidul Alam Choudhury

    2014-01-01

    Full Text Available Recent data regarding the clinical and histopathologic spectrum of crescentic glomerulonephritis (CSGN among the Indian adult population is unknown. Our aim is to study the clinicopathological features and outcome of CSGN. It is a retrospective observational study from a tertiary care hospital in India over 3.5 years. Biopsy-proven cases of CSGN (i.e., >50% crescents in glomeruli were included in the study. Cases with insufficient data were excluded. There were 34 cases of CSGN, accounting for an incidence of 5.5% among kidney biopsies. The mean age was 32.2 ± 16.09 years, with male to female ratio of 12:22. Clinical presentations of CSGN include rapidly progressive glomerulonephritis in 23 (67.7%, chronic renal failure (CRF in seven (20.5%, nephrotic syndrome in two (5.8% and acute nephritic syndrome in two (5.8% patients. The immunological profile of CSGN showed MPO-ANCA in nine (26.4%, PR3-ANCA in one (2.9%, both PR3 and MPO-ANCA in one (2.9%, anti-GBM antibody in five (14.7% and lupus nephritis in six (17.6% patients. All the three antibodies were present in one patient. The percentage of glomeruli showing crescents were 100% in nine (26.4% and ≥80% in seven (20.5% patients. Type of crescents seen were cellular in 11 (32.3% and fibrocellular in 22 (64.7% patients and fibrous in one (2.9% patient. Interstitial fibrosis was found in seven (20.5% patients. Dialysis dependency was seen in 11 (32.3% patients. After 3 months of follow-up, mortality was seen in three (8.8%, remission in eight (23.5%, CRF in 15 (44.1% and ESRD in five (14.7% patients. CSGN carries a poor prognosis. The disorder may have an insidious onset and a slowly progressive course. ANCA, anti-GBM-antibody and anti-dsDNA can coexist in CSGN.

  17. Glomerulonephritis disease pattern at Saudi tertiary care center.

    Science.gov (United States)

    AlMatham, Khalid I; AlFayez, Ali F; AlHarthi, Raghdah A; AlMutairi, Falah S; Alrasheedi, Fahad S; Mustafa, Abdulrehman; Ahmed, Mustafa; AlMatouq, Basim A; AlRowaei, Fadil A

    2017-11-01

    To assess changes in the pattern of glomerular diseases to help guide optimal allocation of resources, to focus future reasearch, and improve outcomes. Methods: A retrospective chart review was conducted on kidney biopsies taken between 2007 and 2016 at a single tertiary care center in Saudi Arabia (King Fahad Medical City, Riyadh) to evaluate the prevalence and pattern of glomerulonephritis (GN). Results: The most common primary GN in 102 biopsies from adult patients with a mean age of 28.9 ± 13.6 years and 40.2% female, was focal and segmental glomerulosclerosis (35.3%). Among 64 patients with systemic lupus erythematosus associated nephritis, of whom most (82.8%) were female, lupus nephritis (LN) 4 (46.9%), and (LN) 3 (32.8%) were the most common lupus nephritis classes. Conclusion: Establishing prospective GN registries from which robust diagnosis, treatment, and outcomes data can be acquired is warranted; however, registry development and maintenance are often precluded by resource limitations. Accordingly, retrospective analysis of administrative data will continue to provide important complementary information on GN epidemiology.

  18. Mixed cryoglobulinemia and secondary membranoproliferative glomerulonephritis associated with ehrlichiosis.

    Science.gov (United States)

    Caster, Dawn J; Summersgill, James T; Paueksakon, Paisit; Massung, Robert F; Shieh, Wun-Ju; McLeish, Kenneth R

    2014-11-01

    Ehrlichiosis is a tick-borne disease with diverse clinical presentations, ranging in severity from a flu-like illness with fever and myalgias to a serious systemic disease with multisystem organ failure. Nephrotic syndrome has been reported previously in two cases of human ehrlichiosis. A kidney biopsy revealed minimal change disease in one of those patients. Herein, we present the case of a 40-year-old man with ehrlichiosis who developed nephrotic syndrome, cryoglobulinemia, and secondary membranoproliferative glomerulonephritis (MPGN). The patient originally presented with shortness of breath, diffuse myalgias, headache, and lower extremity edema. He subsequently developed acute kidney injury and underwent kidney biopsy which showed MPGN and acute tubular injury. A tick-borne disease panel was positive for IgM and IgG to Ehrlichia chaffeensis. Serum testing revealed type 3 mixed cryoglobulinemia with no evidence of hepatitis C infection. The cryoprecipitate contained IgM and IgG antibodies to E. chaffeensis. Cryoglobulinemia is frequently associated with infections, particularly hepatitis C; however, our case is the first to describe ehrlichiosis associated with cryoglobulinemia and secondary MPGN.

  19. Direct immunofluorescence and immunohistochemistry in diagnostics of glomerulonephritis.

    Science.gov (United States)

    Karasalihović, Zinaida; Iljazović, Ermina; Ferluga, Dusan; Cickusić, Elmir; Mustedanagić Mujanović, Jasminka; Stahov, Jugoslav; Skaljić, Ina

    2008-02-01

    The needle biopsies from 60 transplanted and native kidneys have been processed and a prospective analysis of pattern, intensity and distribution of immunoglobulin deposits (IgA, IgG and IgM) and complement components (C3c and C1q) identified in these lesions has been carried out by immunohistochemistry with three step immunoperoxidase, in the period from 2000 to 2004. Those deposits were previously detected and analyzed by immunofluorescence. The samples consisted of 30 renal biopsies, previously diagnosed with glomerulonephritis and positive immunofluorescence and 30 renal biopsies without morphologic changes and deposits on immunofluorescence. 78,7% of the analyzed samples showed the identical results of the deposits of immunoglobulin and components of the complement with both, immunohistochemistry and immunofluorescence method. Sensitivity of the immunohistochemistry method with three step immunoperoxidase for all analyzed immunoglobulin and complement components is high (0,93), while specificity for the same method is 0,79. Standardized method of the three step immunoperoxidase on the paraffin embedded, formalin fixed needle renal biopsies could successfully replace the immunofluorescence method in diagnostic of GN, with the emphasis on a follow up and control of each single step in the procedure of the method.

  20. Comparative Proteomic Analysis of Peritoneal Dialysate from Chronic Glomerulonephritis Patients

    Directory of Open Access Journals (Sweden)

    Hsin-Yi Wu

    2013-01-01

    Full Text Available Peritoneal dialysis (PD frequently contributes to peritoneal damage which cannot be easily identified without invasive techniques, implying the urgent need for biomarkers and revealing mechanisms. Chronic glomerulonephritis (CGN is one of the leading causes of receiving dialysis treatment. Here, we attempted to analyze the peritoneal dialysate collected from CGN patients when they receive continuous ambulatory peritoneal dialysis (CAPD treatment for the first time and after a year to reveal the protein changes that resulted from PD. Proteins were displayed by two-dimensional gel electrophoresis (2DE. Altered gel spots were digested followed by liquid chromatography-tandem mass spectrometry (LC-MS/MS analysis for protein identification. Eight proteins were found to have differential expression levels between two groups. Their differential expressions were validated by Western blots in other sets of peritoneal dialysates. Proteins identified with higher levels in the first-time dialysate suggested their dominant appearance in CGN patients, while those that showed higher levels in peritoneal dialysate collected after one year may result from initial peritoneal inflammation or changes in the permeability of the peritoneum to middle-sized proteins. All the identified proteins may provide a perceptiveness of peritoneal changes caused by PD and may function as potential biomarkers or drug targets.

  1. [Rapidly progressive ANCA positive glomerulonephritis as the presenting feature of infectious endocarditis].

    Science.gov (United States)

    Hanf, W; Serre, J-E; Salmon, J-H; Fabien, N; Ginon, I; Dijoud, F; Trolliet, P

    2011-12-01

    The association of positive cytoplasmic antineutrophil antibody (ANCA) necrotizing crescentic glomerulonephritis with endocarditis raises diagnostic issues. Indeed, it is often difficult to determine if the kidney injury is either secondary to an infectious disease or caused by an ANCA-associated small vessel vasculitis. We report a 59-year-old man admitted in nephrology for acute glomerular syndrome in whom the renal biopsy showed a crescentic necrotizing glomerulonephritis. A diagnosis of vasculitis was initially considered in the presence of high titer of ANCA (anti-proteinase 3). Because of associated Staphyloccocus aureus endocarditis the patient received both corticosteroids and antibiotics that allowed remission of both kidney injury and endocarditis. The renal presentation and the disappearance of ANCA support the infectious etiology of this glomerulonephritis rather than an ANCA-associated small vessel vasculitis. It is important to be cautious in the presence of ANCA positive extracapillary glomerulonephritis and endocarditis should be ruled out before initiation of corticosteroids that may be nevertheless necessary in severe acute glomerulonephritis. Copyright © 2011 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  2. Complement factor H deficiency and endocapillary glomerulonephritis due to paternal isodisomy and a novel factor H mutation

    DEFF Research Database (Denmark)

    Schejbel, L; Schmidt, I M; Kirchhoff, Eva Maria

    2011-01-01

    Complement factor H (CFH) is a regulator of the alternative complement activation pathway. Mutations in the CFH gene are associated with atypical hemolytic uremic syndrome, membranoproliferative glomerulonephritis type II and C3 glomerulonephritis. Here, we report a 6-month-old CFH-deficient child...

  3. Glomerulonephritis in schistosomiasis with mesangial IgM deposits

    Directory of Open Access Journals (Sweden)

    Ageu Godoy Magalhães Filho

    1981-06-01

    Full Text Available Twenty one cases of hepatoesplenic schistosomiasis patients without clinical and laboratory evidence of renal disease, were studied by surgical biopsies using light microscopy and immunofluorescence. The cases were classified histologically as: normal pattern (6 cases; minimal changes (6 cases; and mesangial proliferative glomerulonephritis (9 cases. By the immunofluorescence microscopy using anti IgM, IgG, IgA and C3, the predominant finding in all biopsies, except the normal cases, was granular deposits of IgM in the mesangium along with C3. On the other hand, IgG was present in all cases including normal biopsies along the capillary walls. However IgG was also present in the mesangium only in cases with glomerular lesions. This finding may well be similar to that recently described as IgM mesangial nephropathy. According to our cases a mesangial proliferative glomerulonephritis, characterized by segmental cell proliferation and deposition of IgM in the mesangium, is probably the entity found in the early stages of mansonic schistosomiasis.Com o objetivo de observar as lesões renais iniciais na esquistossomose mansoni, foram selecionados 21 casos da forma hepato-esplênica, com idade média de 12 anos e que não apresentavam sinais clínicos ou laboratoriais de alterações renais. As biópsias foram realizadas durante esplenectomia e em seguida examinadas à microscopia ótica e à microscopia de fluorescência, sendo empregados conjugados anti IgG, IgM, IgA e C3, isoladamente. No exame microscópico, os 21 casos foram classificados em: 6 casos normais; 6 casos com lesões glomerulares consideradas mínimas e 9 casos com lesões mesangiais discretas ou moderadas. À imunofluorescência constatou-se presença de depósitos de IgM e de C3 predominantemente de localização mesangial, tanto nos casos de lesões mínimas, como nos casos de proliferação mesangial mais acentuada (Tabelas II e III. Constatou-se aind a depósitos de IgG em todos

  4. Predictors of outcome in idiopathic rapidly progressive glomerulonephritis (IRPGN

    Directory of Open Access Journals (Sweden)

    Leontsini Maria

    2006-11-01

    Full Text Available Abstract Background Small vessel vasculitides are known to follow a devastating course towards end-stage renal disease, unless treated with immunosuppressive regiments. We investigated the value of clinical, histological and immunohistochemical parameters as predictors of outcome at diagnosis in patients with pauci immune necrotizing glomerulonephritis. Methods In 34 patients the percentage and evolution stage of crescents, the presence of glomerular necrosis, the degree or severity of arteriosclerosis, as well as the extent of tubulointerstitial infiltration, interstial fibrosis and tubular atrophy were assessed. Monoclonal antibodies were used to identify infiltrating macrophages, α-SMA(+ and PCNA(+ cells, the expression of integrins α3β1 and LFA-1β, the adhesion molecule ICAM-1, the growth factor TGF-β1 and the terminal complement component C5b-9. Results 24 pts (70.6% showed a complete or partial response to the treatment. The follow-up period was 20 ± 22 months. At multivariate analysis, serum CRP (p = 0.024, the intensity of tubular expression of C5b-9 (p Conclusion Patients with ANCA-associated renal vasculitis seem to respond better to the treatment. Acute phase reactants, such as CRP, implying a more intense parenchymal inflammatory reaction, as well as the intensity of the de novo expression of C5b-9 and the glomerular and tubular expression of α3β1 integrin predict the response to therapy. The severity of TIN lesions and of the tubulo-interstitial TGF-β1 and C5b-9 expression predict an unfavourable outcome.

  5. A Case of ANCA Negative Pauci-immune Crescentic Glomerulonephritis in Mixed Connective Tissue Disease

    Directory of Open Access Journals (Sweden)

    Neena Mampilly

    2015-04-01

    Full Text Available Pauci-immune crescentic glomerulonephritis (CrGN is one of the most common causes of rapidly progressive glomerulonephritis. The majority of patients with pauciimmune CrGN have circulating antineutrophil cytoplasmic autoantibodies (ANCA. Approximately 10% of systemic vasculitides patients test negative for ANCA. Majority of the ANCA negative pauciimmune CrGN described in the literature have not shown associations with connective tissue diseases. Only isolated case reports of associations with systemic lupus erythematosus and scleroderma have been described. We report the case of a 24 yr lady who was diagnosed to have a pauciimmune crescentic glomerulonephritis on renal biopsy, and was found to be ANCA negative. Subsequently her collagen profile revealed a mixed connective tissue disease, even though she did not have any other systemic manifestations of the disease at presentation. We presume that antiendothelial antibodies (AECA may have a role in the pathogenesis of the disease.

  6. Structural and Geophysical Characterization of Oklahoma Basement

    Science.gov (United States)

    Morgan, C.; Johnston, C. S.; Carpenter, B. M.; Reches, Z.

    2017-12-01

    Oklahoma has experienced a large increase in seismicity since 2009 that has been attributed to wastewater injection. Most earthquakes, including four M5+ earthquakes, nucleated at depths > 4 km, well within the pre-Cambrian crystalline basement, even though wastewater injection occurred almost exclusively in the sedimentary sequence above. To better understand the structural characteristics of the rhyolite and granite that makeup the midcontinent basement, we analyzed a 150 m long core recovered from a basement borehole (Shads 4) in Rogers County, NE Oklahoma. The analysis of the fracture network in the rhyolite core included measurements of fracture inclination, aperture, and density, the examination fracture surface features and fill minerology, as well as x-ray diffraction analysis of secondary mineralization. We also analyzed the highly fractured and faulted segments of the core with a portable gamma-ray detector, magnetometer, and rebound hammer. The preliminary analysis of the fractures within the rhyolite core showed: (1) Fracture density increasing with depth by a factor of 10, from 4 fractures/10m in the upper core segment to 40 fracture/10m at 150 m deeper. (2) The fractures are primarily sub-vertical, inclined 10-20° from the axis of the vertical core. (3) The secondary mineralization is dominated by calcite and epidote. (4) Fracture aperture ranges from 0.35 to 2.35mm based on the thickness of secondary filling. (5) About 8% of the examined fractures display slickenside striations. (6) Increases of elasticity (by rebound hammer) and gamma-ray emissions are systematically correlated with a decrease in magnetic susceptibility in core segments of high fracture density and/or faulting; this observation suggests diagenetic fracture re-mineralization.

  7. Reliability of residential basements as blast shelters

    International Nuclear Information System (INIS)

    Longinow, A.; Mohammadi, J.

    1983-01-01

    This paper describes an analysis method for predicting the probability of failure of a wood-framed basement when subjected to a static, uniformly distributed load. The analysis considers the primary failure modes of each framing member and determines the probability of failure for each mode acting alone. The failure probability of the system as a whole is then bounded. The upper bound is determined on the assumption that the failure modes are independent, while the lower bound is determined on the assumption that the failure modes are perfectly correlated. The analysis is described with reference to an example problem

  8. Drusen-like beneath retinal deposits in type II mesangiocapillary glomerulonephritis: a review

    Directory of Open Access Journals (Sweden)

    Miguel Hage Amaro

    2015-02-01

    Full Text Available The aim of this paper is to do a review of Drusen-like beneath retinal deposits in type II mesangiocapillary glomerulonephritis. Drusenlike beneath retinal deposits in type II mesangiocapillary glomerulonephritis appear to develop at an early age, often second decade of life different of drusen from age-related macular degeneration (AMD.Long term follow-up of the cases in this disease shows in the most of them, no progression of the of drusen-like beneath retinal deposits in type II mesangiocapillary glomerulonefritis, the most of subjects retain good visual acuity and no specific treatment is indicated.

  9. [First case report of Streptococcus equi subsp. zooepidemicus post-infectious acute glomerulonephritis in France].

    Science.gov (United States)

    Le-Berre, Nicolas; Filipozzi, Pierre; Martin, Laurent; Frimat, Luc; Girerd, Sophie

    2017-02-01

    Post-infectious glomerulonephritis has become exceptional in France because streptococcus infections are well-treated. When they appear, clinical and biological symptoms are mostly typical and associate acute nephritic syndrome, acute renal failure, proteinuria, hematuria and low complement. We are reporting the first French case of acute post-infectious glomerulonephritis related to Streptococcus equi subsp. zooepidemicus, which is commonly found in horses and rarely in human pathology, and of which contamination is by direct contact with sick horses or by ingestion of non-pasteurized milk. Copyright © 2016 Association Société de néphrologie. Published by Elsevier SAS. All rights reserved.

  10. Magnetotelluric inversion for depth-to-basement estimation

    DEFF Research Database (Denmark)

    Cai, Hongzhu; Zhdanov, Michael

    2015-01-01

    The magnetotelluric (MT) method can be effectively applied for depth-to-basement estimation, because there exists a strong contrast in resistivity between a conductive sedimentary basin and a resistive crystalline basement. Conventional inversions of MT data are usually aimed at determining...... the volumetric distribution of the conductivity within the inversion domain. By the nature of the MT method, the recovered distribution of the subsurface conductivity is typically diffusive, which makes it difficult to select the sediment-basement interface. This paper develops a novel approach to 3D MT...... inversion for the depth-to-basement estimate. The key to this approach is selection of the model parameterization with the depth to basement being the major unknown parameter. In order to estimate the depth to the basement, the inversion algorithm recovers both the thickness and the conductivities...

  11. Geochemistry of the Puna Austral and Cordillera Oriental basement

    International Nuclear Information System (INIS)

    Becchio, Raul; Lucassen, Friedrich; Franz, Gerhard; Kasemann, Simone

    1998-01-01

    Major and trace elements, rare earths, and 143 Nd/ 147 Nd and, 147 Sm/ 144 Nd isotope ratios have been determined in the Puna Austral and Cordillera Oriental basement. The basement is formed by high temperature amphibolite facies rocks ranulites (750-550 degrees C) and green schists. They are represented by schists, paragneiss, orthogneiss, migmatites, few metabasites, marbles and chalcosilicatic banks. Hypotheses on the formation and evolution of the basement are presented

  12. Do food antigens play a role in the pathogenesis of some cases of human glomerulonephritis

    NARCIS (Netherlands)

    van der Woude, F. J.; Hoedemaeker, P. J.; van der Giessen, M.; DEGRAEFF, PA; DEMONCHY, J; THE, TH; VANDERHEM, GK

    Circulating immune complexes after a test meal were measured with three methods (PEG precipitation, Clq-ELISA and the indirect granulocyte phagocytosis test) in 10 controls, two symptomless persons with selective IgA deficiency and 14 patients with various types of glomerulonephritis, of which two

  13. Proliferative, necrotizing and crescentic immune complex-mediated glomerulonephritis in a cat

    Directory of Open Access Journals (Sweden)

    Carolyn Gross

    2015-09-01

    Full Text Available Case Summary A 5-year-old cat was examined for vomiting and anorexia of 2 days’ duration. Azotemia, hyperphosphatemia and hypoalbuminemia were the main biochemical findings. Serial analyses of the urine revealed isosthenuria, proteinuria and eventual glucosuria. Hyperechoic perirenal fat was detected surrounding the right kidney by ultrasonography. Histopathologic evaluation of ante-mortem ultrasound-guided needle biopsies of the right kidney was consistent with proliferative, necrotizing and crescentic glomerulonephritis with fibrin thrombi, proteinaceous and red blood cell casts, and moderate multifocal chronic-active interstitial nephritis. Owing to a lack of clinical improvement, the cat was eventually euthanized. Post-mortem renal biopsies were processed for light microscopy, transmission electron microscopy and immunofluorescence. This revealed severe focal proliferative and necrotizing glomerulonephritis with cellular crescent formation, podocyte injury and secondary segmental sclerosis. Ultrastructural analysis revealed scattered electron-dense deposits in the mesangium, and immunofluorescence demonstrated positive granular staining for λ light chains, consistent with immune complex-mediated glomerulonephritis. Severe diffuse acute tubular epithelial injury and numerous red blood cell casts were also seen. Relevance and novel information To our knowledge, this is the first report of naturally occurring proliferative, necrotizing and crescentic immune complex glomerulonephritis in a cat.

  14. [Immunoglobulin filtration and reabsorption as possible factors in the pathogenesis of chronic glomerulonephritis. Clinical, immunomorphological and histoenzymological research].

    Science.gov (United States)

    Nabokov, A V; Travkina, E S; Zel'tser, G L; Nevorotin, A I

    1992-01-01

    Kidney biopsy specimens obtained from a group of individuals with chronic glomerulonephritis (CGN) have been processed for light and electron microscopic immunolocalization of total immunoglobulins (Igs). In a few cases, acid phosphatase (ACPase), a lysosomal enzyme marker, was ultrastructurally visualized. In the glomeruli, horseradish peroxidase-stained Igs were revealed in capillary lumina, urinary spaces and in transit through occasional loci of the glomerular basal membranes while ACPase-containing lysosomes resided both within and outside the cells. In the proximal tubules, Igs were traced in the endocytic vesicles and vacuoles, the latter also being positive for ACPase. Statistically significant relationships have been revealed between the number of IGs-labeled proximal tubules and some clinical or pathomorphological stigmata of CGN, in particular, proteinuria and arterial hypertension levels, marked interstitial sclerosis, etc. The data obtained are discussed in regard to the mechanisms of increased macromolecular filtration and the different proteinuria selectivity levels as well as the development of interstitial sclerosis as a result of the elevated reabsorption and incomplete lysosomal degradation of Igs in CGN.

  15. Depth-To-Basement Mapping Using Fractal Technique: Application ...

    African Journals Online (AJOL)

    ... and can thus be obtained at source level. Application to aeromagnetic data from the Chad basin north eastern Nigeria produced a basement relief which range from depths of 2.47 km to 5.40 km with an average of 3.92 +- 0.66 km. Keywords: Fractal, depth, basement, spectra, aeromagnetic. Nigerian Journal of Physics Vol ...

  16. Investigation of the subsurface features of the basement complex of ...

    African Journals Online (AJOL)

    3D seismic reflection survey was recently carried out within the Zaria area of the basement complex of northern Nigeria, in order to investigate the complexity of the subsurface features within the basement. The geology of the survey area was characterized by gneisses and low grade meta-sedimentary rocks that form the ...

  17. Potential Development of Hydrocarbon in Basement Reservoirs In Indonesia

    Directory of Open Access Journals (Sweden)

    D. Sunarjanto

    2014-07-01

    Full Text Available DOI: 10.17014/ijog.v8i3.165Basement rocks, in particular igneous and metamorphic rocks are known to have porosity and permeability which should not be ignored. Primary porosity of basement rocks occurs as the result of rock formation. The porosity increases by the presence of cracks occurring as the result of tectonic processes (secondary porosity. Various efforts have been carried out to explore hydrocarbon in basement rocks. Some oil and gas fields proved that the basement rocks are as reservoirs which so far have provided oil and gas in significant amount. A review using previous research data, new data, and observation of igneous rocks in some fields has been done to see the development of exploration and basement reservoirs in Indonesia. A review on terminology of basement rock up till the identification of oil and gas exploration in basement rocks need to be based on the latest technology. An environmental approach is suggested to be applied as an alternative in analyzing the policy on oil and gas exploration development, especially in basement reservoirs.

  18. Basement and alluvial aquifers of Malawi: An overview of ...

    African Journals Online (AJOL)

    This paper highlights the quality of groundwater in basement and alluvial aquifers of Malawi through literature assessment. Groundwater in these aquifers serves about 60% of Malawian population. Alluvial aquifers yield high groundwater in excess of 10 L/s and more mineralized than basement aquifers. The values from ...

  19. Estimation of Magnetic Basement Depth of Oyo Area from ...

    African Journals Online (AJOL)

    The digitized magnetic intensity data of Oyo area, south western Nigeria was analyzed to estimate depths to magnetic sources as well as source locations. The total magnetic intensity values ranges from -143.8 nT to 147.0 nT suggesting contrasting rock types in the basement complex. The magnetic basement depth results ...

  20. Evidence of basement controlled faulting of cretaceous strata in the ...

    African Journals Online (AJOL)

    Evidence of basement controlled faulting of cretaceous strata in the Middle Benue Trough, Nigeria from lineament analysis of gravity data. ... anomaly map of part of the Middle Benue Trough, Nigeria, was used to investigate the genetic relationship between the basement and intra-sedimentary structures in the study area.

  1. Possibilities of seismic exploration for crystalline basement study

    Directory of Open Access Journals (Sweden)

    А. Н. Телегин

    2017-03-01

    Full Text Available Possibilities of seismic methods of reflected and refracted waves have been examined for the purposes of detailed study on crystalline basement structure. Investigation of depth and structure of the basement plays an important role in the exploration of various deposits. Sedimentary cover is usually associated with oil and gas reserves. Ore deposits are formed in the basement rocks, basement splits and structure of its surface have a genetic relation not only to ore minerals, but also to oil resources. Reflection seismology is one of the main seismic methods of investigating structural geometry of the sedimentation mass, forecasting its material composition and possible hydrocarbon reserves. However, its possibilities for investigating crystalline basement are limited. Basing on many years’ experience of reflection seismology and physical modeling it has been identified that actual roughness of basement surface limits the obtainable amount of waves reflected from it. Possibilities of reflection seismology for basement structure study are mostly related to investigation of discontinuous faults as diffraction objects using diffracted waves. Method of refracted waves combined with modern procedures and material processing aimed at getting dynamic seismic sections holds much significance for the basement study, especially in the process of surface mapping and, to a lesser extent, in investigating discontinuous faults. Combining seismic methods of reflected and refracted waves in basement study increases reliability of forecasting its geological structure: in particular, its surface can be well defined by means of refraction seismology, and zones of discontinuous faults are identified from diffraction objects using both reflection and refraction methods. As a result of applying both reflection and refraction seismology, an opportunity arises to carry out detailed analysis of basement structure and to predict its oil and gas content.

  2. Possibilities of radioisotope renal clearances without blood sampling in patients with chronic glomerulonephritis

    International Nuclear Information System (INIS)

    Shejretova, E.; Velikov, P.

    1987-01-01

    Two clearance methods are proposed: 1) Effective renal plasma flow determination with 131 J-hippuran, and 2) glomeruli filtration determination with 169 Yb-EDTA. Thirty one adult patients with chronic glomerulonephritis and 59 without clinical and laboratory evidence of urinary tract disease comprised the study group. Statistically significant decrease in the effective renal plasma flow (p<0,001) and of glomerular filtration (p<0,01) was recorded in comparison to the control group, which is an evidence of the good diagnostic possibilities of the methods. The significant difference between the group of patients with elevated blood urea levels or pathologic Zimnincki's test and reduced glomerular filtration, compared to those with normal glomerular filtration, is an indication for the high diagnostic value of the glomerular filtration method. The clearance methods without blood sampling are particularly useful in the diagnosis and follow-up of patients with chronic glomerulonephritis, because of the burdenless technique, lack of blood sampling, and sufficient accuracy

  3. Plasma Exchange for Renal Vasculitis and Idiopathic Rapidly Progressive Glomerulonephritis: A Meta-analysis

    DEFF Research Database (Denmark)

    Walsh, Michael; Catapano, Fausta; Szpirt, Wladimir

    2010-01-01

    BACKGROUND:: Plasma exchange may be effective adjunctive treatment for renal vasculitis. We performed a systematic review and meta-analysis of randomized controlled trials of plasma exchange for renal vasculitis. STUDY DESIGN:: Systematic review and meta-analysis of articles identified from...... electronic databases, bibliographies, and studies identified by experts. Data were abstracted in parallel by 2 reviewers. SETTING & POPULATION:: Adults with idiopathic renal vasculitis or rapidly progressive glomerulonephritis. SELECTION CRITERIA FOR STUDIES:: Randomized controlled trials that compared...... standard care with standard care plus adjuvant plasma exchange in adult patients with either renal vasculitis or idiopathic rapidly progressive glomerulonephritis. INTERVENTION:: Adjuvant plasma exchange. OUTCOME:: Composite of end-stage renal disease or death. RESULTS:: We identified 9 trials including...

  4. X-ray diagnosis of predisposition to pulmonary nephrotic edema in patients with glomerulonephritis

    International Nuclear Information System (INIS)

    Kamenetskij, M.S.; Pervak, M.B.; Tkachenko, G.D.; Vernikov, B.L.

    1995-01-01

    To develop criteria for determining predisposition to pulmonary edema in patients with glomerulonephritis, clinical, laboratory and X-ray examinations were made in 697 patients with glomerulonephritis at different stages of its development. X-ray examination included chest tele X-ray and its densitometric analysis. Twenty two patients underwent computerized tomography with gistographic analysis. In 106 patients, X-ray findings were compared with the volume of circulating blood, cardiac and stroke indices. CHanges in the lungs and pleural cavities were found in 22,7%, pulmonary edema was revealed in 15,7% of the patients. The prognostically unfavourable criteria for the development of pulmonary edema were found to be Stage 2 pulmonary venous hypertension with hypervolemia and peripheral edemas

  5. Forty years abuse of baking soda, rhabdomyolysis, glomerulonephritis, hypertension leading to renal failure: a case report.

    Science.gov (United States)

    Forslund, Terje; Koistinen, Arvo; Anttinen, Jorma; Wagner, Bodo; Miettinen, Marja

    2008-01-01

    We present a patient who had ingested sodium bicarbonate for treatment of alcoholic dyspepsia during forty years at increasing doses. During the last year he had used more than 50 grams daily. He presented with metabolic alkalosis, epileptic convulsions, subdural hematoma, hypertension and rhabdomyolysis with end stage renal failure, for which he had to be given regular intermittent hemodialysis treatment. Untreated hypertension and glomerulonephritis was probably present prior to all these acute incidents. Examination of the kidney biopsy revealed mesangial proliferative glomerulonephritis and arterial wall thickening causing nephrosclerosis together with interstitial calcinosis. The combination of all these pathologic changes might be responsible for the development of progressive chronic renal failure ending up with the need for continuous intermittent hemodialysis treatment.

  6. Complementing the patient: a complement component deficiency in a patient with recurrent infections and glomerulonephritis.

    Science.gov (United States)

    Sinclair, David; Wilde, Graeme; Bex, Samantha; Peters, Sheila

    2005-01-01

    We present a case showing the investigation of a 7-year-old girl with empyema and glomerulonephritis whose "immunological" defect was a single complement component (C2) deficiency which prevented her from activating her classical complement pathway. A defect in complement function should be suspected in any patient with severe or recurring pyogenic infections. Investigations of "? immune deficiency" should always include tests to assess the patency of the patient's complement system.

  7. Granulomatosis with Polyangiitis Presenting as Pauci-Immune Crescentic Glomerulonephritis in Pregnancy

    OpenAIRE

    Kunjal, Ryan; Makary, Raafat; Poenariu, Andreea

    2016-01-01

    Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophili...

  8. Mesangioproliferative glomerulonephritis in a patient with Kimura′s disease presenting as Nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Surendra Singh Rathore

    2015-01-01

    Full Text Available Kimura′s disease is a rare chronic eosinophilic inflammatory disorder of unknown etiology. Majority of cases have been reported from South East Asia, while sporadic occurrences have been reported worldwide, including the Indian subcontinent. Nephrotic syndrome may be the presenting manifestation of Kimura′s disease, and a variety of renal lesions are observed histologically in such patients. We herein describe a case of steroid-responsive mesangioproliferative glomerulonephritis related to kimura′s disease.

  9. Acute postinfectious glomerulonephritis with a large number of crescents caused by Mycoplasma pneumoniae

    Directory of Open Access Journals (Sweden)

    Xinghua Chen

    2015-01-01

    Full Text Available Mycoplasma pneumoniae-induced acute postinfectious glomerulonephritis has various pathological changes and relatively poor prognosis. It often occurs in children, barely in adults. Currently, no clear treatment guidelines have been established for its treatment using glucocorticoid and immunosuppressive. In this study, we report an adult who admitted to our hospital due to fever and gross hematuria. The patient presented with nephritic syndrome and renal failure and confirmed to have M. pneumoniae infection by serum detection and acute postinfectious glomerulonephritis with a large number of crescents by renal biopsy. He was given glucocorticoid, immunosuppressive agent combined with hemodialysis as well as other supportive treatment. Three months later, his renal function became normal, urine protein level decreased to 0.4 g/24 h, and the C3 complement increased to normal level. In conclusion, glucocorticoid and immunosuppressive treatment should be given to patients with M. pneumoniae-infection induced glomerulonephritis after confirmed to have a large number of crescents by renal biopsy and the treatment could improve the prognosis.

  10. Granulomatosis with polyangiitis: rapidly progressive necrotizing glomerulonephritis in a pediatric patient

    Directory of Open Access Journals (Sweden)

    Luna M

    2014-04-01

    Full Text Available Mariana Luna,1 Victoria Bocanegra,3 Patricia G Vallés1,21Nephrology Division, Pediatric Department, Dr Humberto Notti Pediatric Hospital, Mendoza, Argentina; 2Pathophysiology Area, Pathology Department, School of Medicine, National Cuyo University, Mendoza, Argentina; 3National Council of Scientific and Technical Research of Argentina (CONICET, Buenos Aires, ArgentinaAbstract: Granulomatosis with polyangiitis (GPA is associated with a broad range of clinical manifestations including renal disease. It is a systemic vasculitis that is rarely encountered in children. We present a 14-year-old girl who suffered from pharyngitis 1 week before admittance to hospital. She was admitted for macroscopic hematuria and oliguria, under the possibility of nephritic syndrome. Renal failure with rapidly progressive glomerulonephritis occurred within 24 hours. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies (c-ANCA with antiproteinase 3 specificity and renal biopsy revealed pauci-immune crescentic focal necrotizing glomerulonephritis. Treatment including methylprednisolone and cyclophosphamide intravenous pulses allowed renal recovery after 3 weeks. The clinical, hematological, and biochemical parameters improved substantially, achieving remission. Granulomatosis with polyangiitis, although rare in children, should be considered in the above clinical scenario. This case underlines that knowledge of renal histology diagnosis and early aggressive immunosuppressive therapy are essential for the management of these patients.Keywords: acute renal failure, vasculitis, crescentic pauci-immune glomerulonephritis-Type-C, antineutrophil cytoplasmic antibodies (c-ANCA-macroscopic hematuria

  11. Acute Glomerulonephritis: A 7 Years Follow up of Children in Center of Iran

    Directory of Open Access Journals (Sweden)

    Mohsen Akhavan Sepahi

    2011-06-01

    Full Text Available Acute glomerulonephritis (AGN is a type of renal disease which indicates the inflammation of glomerulus and nephrons. This study was carried on 94 children, <15 years old with the diagnosis of AGN who were admitted to Qom and Yazd's hospitals between 2000 and 2006. Data were collected using hospital records on admission, progression notes and outpatient follow up. Among 94 patients, 55.3% were male and 44.6% were female. Mean age of patients was 8.2±2.7 years old. Acute post streptococcal glomerulonephritis (APSGN was reported in 92.5%, membranoproliferative glomerulonephritis in 4.2%, hemolytic uremic syndrome in 2.1% and IgA nephropathy in 1.06%. There was no significant differences between GN types and gender (P=0.54. Clinical manifestation included edema in 68.8%, oliguria in 36.3%, gross hematuria in 69.1%, HTN in 61.7% and anuria in 1.06%. Microscopic hematuria was detected in all patients. In the time of follow up none of patients had hypertension, 3.1% had proteinuria and 6.3% had microscopic hematuria. APSGN is the most common causes of AGN in Qom and Yazd's children. Early diagnosis and treatment of APSGN may protect children from long term morbidity and mortality and improve quality of life.

  12. Pauci-immune crescentic glomerulonephritis in the Down′s syndrome

    Directory of Open Access Journals (Sweden)

    Mejda Cherif

    2013-01-01

    Full Text Available Kidney disease is a rare complication in patients with the Down′s syndrome. However, with increased survival, it appears that a growing number of these patients present with glomerulonephritis. Most cases have been reported as case reports and include lesions such as mesangiocapillary glomerulonephritis with hypo-complementemia, crescentic glomerulonephritis with anti-neutrophil cytoplasmic antibodies (ANCA, amyloidosis and immunotactoid glomerulopathy. We report the observation of a 38-year-old man with the Down′s syndrome who presented with severe renal failure, proteinuria and microscopic hematuria evolving over two months. There was no history of congenital heart disease or urinary symptoms. Percutaneous renal biopsy revealed fibrous crescents, rupture of Bowman′s capsule and peri-glomerular granuloma; there were no deposits on immunofluorescence study. Thoracic computerized tomography scan showed alveolar congestion. The patient tested negative for ANCA. At the time of reporting, the patient is on regular chronic hemodialysis. Our case illustrates a distinct entity that further expands the spectrum of renal disease known to occur in the Down′s syndrome. Early detection of the renal disorders may prevent or slow down the progression.

  13. Sodium fire in the ILONA basement

    International Nuclear Information System (INIS)

    Klemm, H.

    1993-05-01

    The report describes the reasons, the course, the fire fighting measures and the consequences of the sodium fire, which damaged severely the 5 MW sodium test facility ILONA in Bergisch-Gladbach, Germany, in 1990. The accident occurred in the process of transferring Na from one tank containing 0.5 m''3 Na into another one with 6 m''3 Na, which were connected via sodium and gas exchange pipes. The 6 m''3 tank was also fitted with a dipping-pipe for the later purpose of filling or evacuation. The pipe was closed by a plug. The two tanks were flooded with argon. The leakage with consequent Na fire started during the preheating process of the 6 m''3 tank, and later investigations showed that Na had leaked from the dipping-pipe after a too high pressure built-up. Whether that happened because of the failure of a pressure compensation valve or because of a blockage of the pipe connecting the two tanks, could not be clarified after the accident because of the damages caused by the fire. The later analysis showed that about 4500 kg out of the originally 5820 kg had leaked from the tank during a time of about 5 hours. A total of 1344 kg Na were deposited as combustion product aerosols (carbonate and bi-carbonate) in the building and about 930 kg Na were released from the building to the atmosphere. On the basis of the temperature difference between 400 deg C in the basement and 20 deg C at the outlet and a height difference of 30 m, the gas stream was estimated to 4 m''3 per sec. The aerosol clouds left the building via the natural draught stack. They were quickly transformed into carbonate and bi-carbonate, which do not represent a risk for the people, the animals or the vegetation in the surroundings

  14. Building America Top Innovations 2012: Basement Insulation Systems

    Energy Technology Data Exchange (ETDEWEB)

    none,

    2013-01-01

    This Building America Top Innovations profile describes research on basement insulation, which identifies the wall installation methods and materials that perform best in terms of insulation and water resistance.

  15. Groundwater Exploration in the Basement Complex Around Chibok ...

    African Journals Online (AJOL)

    admin

    ABSTRACT: A geophysical survey employing Schlumberger electrode configuration using vertical electrical sounding (VES) method ... Keywords: Hydrogeology, Basement Complex, Alluvium, Colluviums, Electric resistivity, Chibok. INTRODUCTION .... unconcentrated surface runoff or sheet erosion. It is not concentrated ...

  16. Basement configuration of KG offshore basin from magnetic anomalies

    Digital Repository Service at National Institute of Oceanography (India)

    Subrahmanyam, V.; Swamy, K.V.; Raj, N.

    basement is faulted along the NW-SE direction with the upthrown side lying to the north of the anomaly trend of this region. The coincidence of magnetizations observed through the present interpretation with that of charnockites of neighbouring EGMB...

  17. Frictional Behavior of Altered Basement Approaching the Nankai Trough

    Science.gov (United States)

    Saffer, D. M.; Ikari, M.; Rooney, T. O.; Marone, C.

    2017-12-01

    The frictional behavior of basement rocks plays an important role in subduction zone faulting and seismicity. This includes earthquakes seaward of the trench, large megathrust earthquakes where seamounts are subducting, or where the plate interface steps down to basement. In exhumed subduction zone rocks such as the Shimanto complex in Japan, slivers of basalt are entrained in mélange which is evidence of basement involvement in the fault system. Scientific drilling during the Nankai Trough Seismogenic Zone Experiment (NanTroSEIZE) recovered basement rock from two reference sites (C0011 and C0012) located seaward of the trench offshore the Kii Peninsula during Integrated Ocean Discovery Program (IODP) Expeditions 322 and 333. The basement rocks are pillow basalts that appear to be heterogeneously altered, resulting in contrasting dense blue material and more vesicular gray material. Major element geochemistry shows differences in silica, calcium oxides and loss-on-ignition between the two types of samples. Minor element geochemistry reveals significant differences in vanadium, chromium, and barium. X-ray diffraction on a bulk sample powder representing an average composition shows a phyllosilicate content of 20%, most of which is expandable clays. We performed laboratory friction experiments in a biaxial testing apparatus as either intact sample blocks, or as gouge powders. We combine these experiments with measurements of Pennsylvania slate for comparison, including a mixed-lithology intact block experiment. Intact Nankai basement blocks exhibit a coefficient of sliding friction of 0.73; for Nankai basement powder, slate powder, slate blocks and slate-on-basement blocks the coefficient of sliding friction ranges from 0.44 to 0.57. At slip rates ranging from 3x10-8 to 3x10-4 m/s we observe predominantly velocity-strengthening frictional behavior, indicating a tendency for stable slip. At rates of < 1x10-6 m/s some velocity-weakening was observed, specifically in

  18. Basement Fault Reactivation by Fluid Injection into Sedimentary Reservoirs

    Science.gov (United States)

    Peter, Eichhubl; Fan, Zhiqiang; Zhu, Cheng

    2017-04-01

    Many suspected injection-induced earthquakes occur in crystalline basement rather than in the overlying sedimentary injection reservoir. To address why earthquakes nucleate in the basement rather than the injection layer we investigate the relationship between pore pressure diffusion, rock matrix deformation, and induced fault reactivation through 3D fully coupled poroelastic finite element models. These models simulate the temporal and spatial perturbation of pore pressure and solid stresses within a basement fault that extends into overlying sedimentary layers and that is conductive for flow along the fault but a barrier for flow across. We compare the effects of direct pore pressure communication and indirect poroelastic stress transfer from the injection reservoir to the fault on increasing the Coulomb failure stress that could reactivate the basement fault for normal, reverse, and strike-slip faulting stress regimes. Our numerical results demonstrate that volumetric expansion of the reservoir causes a bending of the fault near the injector and induces shear tractions along the downdip direction of the fault in the basement. These induced shear tractions act to increase the Coulomb failure stress for a normal faulting stress regime, and decrease the Coulomb failure stress for a reverse faulting regime. For a strike-slip faulting stress regime, the induced shear tractions increase the Coulomb failure stress both in the reservoir and basement. The induced normal traction on the fault reduces the Coulomb failure stress in all three tectonic regimes, but is larger in the reservoir than in the basement due to the more pronounced poroelastic effect in the reservoir. As a result, strike-slip stress regimes favor fault reactivation in the basement. Whereas the magnitude of the direct pore pressure increase exceeds the magnitude of induced poroelastic stress change, the poroelastic stress change increases the Coulomb failure stress in the basement fault for the normal

  19. Rapidly progressive glomerulonephritis in adolescents – aetiology and treatment based on case reports

    Directory of Open Access Journals (Sweden)

    Beata Banaszak

    2017-06-01

    Full Text Available Rapidly progressive glomerulonephritis is a disease characterised by an abrupt drop in glomerular filtration rate in a short period of time, which is caused by crescent formation seen in at least 50% of glomeruli. Two cases presented below illustrate rapid progressive glomerulonephritis in adolescents as a disease of heterogeneous aetiology that can develop both in the course of primary glomerulopathies and glomerulopathies secondary to systemic vasculitis. In the first case of an 11-year-old girl, nephritic syndrome with renal failure was accompanied by the presence of anti-myeloperoxidase antibodies in the serum, which in combination with the histopathological picture of the kidneys indicating pauci-immune rapidly progressive glomerulonephritis was the basis for the diagnosis of renal limited vasculitis. In the second case of a 16-year-old boy, an adverse course of acute post-streptococcal glomerulonephritis with features of severe and persistent glomerular filtration impairment was an indication for the verification of the diagnosis and identification of rapidly progressive glomerulonephritis based on a biopsy examination. Prompt diagnosis and inclusion of combined immunosuppressive therapy provided the chance to preserve renal function.

  20. Discussion on the basement topography and its relation with the uranium mineralization in Xiangshan basin

    International Nuclear Information System (INIS)

    Long Qihua; Liu Qingcheng

    2002-01-01

    The depth of the basement and the relation between the basement relief shape and uranium mineralization are discussed by forward and inverse computation for large-scale gravity data in Xiangshan basin. The difference of basement topography result in the inhomogeneous distribution of uranium mineralization. The margin of the basement upheaval section and the variation place of basement topography are the favorable place for uranium mineralization. It's helpful to prospect deep and blind uranium deposit in Xiangshan basin

  1. Basement depressurization using dwelling mechanical exhaust ventilation system

    International Nuclear Information System (INIS)

    Collignan, B.; O'Kelly, P.; Pilch, E.

    2004-01-01

    The mechanical ventilation exhaust system is commonly used in France to generate air renewal into building and especially into dwelling. It consists of a permanent mechanical air extraction from technical rooms (kitchen, bathrooms and toilets) using a unique fan connected to exhaust ducts. Natural air inlets in living room and bed rooms ensure an air flow from living spaces towards technical rooms. To fight against radon into building, the most recognised efficient technique is the Soil Depressurization System (S.D.S.) consisting in depressurizing the house basement. The aim of this study is to test the ability of the dwelling mechanical ventilation system to depressurize the basement in conjunction with air renewal of a house. For that purpose, a S.D.S. has been installed in an experimental house at CSTB during its construction. At first, tests undertaken with a variable velocity fan connected to the S.D.S. have characterised the permeability of the basement. It is shown that basement can be depressurized adequately with a relatively low air flow rate. At a second stage, S.D.S. has been connected to the exhaust ventilation fan used for the mechanical ventilation of the house. Results obtained show the ability of such ventilation system to generate sufficient depressurization in the basement and to ensure simultaneously adequate air change rate in the dwelling. (author)

  2. Temporal Changes in Post-Infectious Glomerulonephritis in Japan (1976-2009.

    Directory of Open Access Journals (Sweden)

    Joichi Usui

    Full Text Available The incidence of post-infectious glomerulonephritis (PIGN in developed countries has decreased over the last 50 years. Here we identified the trends of the incidence of PIGN in Japan during the past four decades.We explored the frequency, clinicopathological findings, and prognosis of PIGN based on 6,369 cases from the Renal Biopsy Database of our institute in the Kanto region of Japan, diagnosed histologically from 1976 to 2009.The numbers of PIGN cases were 131 (2.1% in total, and 2.4%, 1.1%, 2.6% and 2.1% identified in the 1970s, 1980s, 1990s, and 2000s, respectively. Acute glomerulonephritis (AGN, including post-streptococcal glomerulonephritis (PSGN, accounted for almost all of the PIGN cases in the 1970s, but decreased to approx. 40%-50% since the 1990s. In the 1990s, Staphylococcus aureus infection-related nephritis (SARN showed a rapid increase in rate, reaching 30%. The incidence of hepatitis C virus infection-associated GN (HCVGN has increased since the 1990s. The average age at onset rose from 33 to 51 years over the study period. These transitions can be summarized as increases in SARN and HCVGN and decreases in PSGN and other types of AGN, since SARN and HCVGN have older onsets compared to PSGN and other AGN types. The clinicopathological features were marked for each PIGN. Regarding the prognosis, the renal death rates of both the SARN and HCVGN groups were significantly higher than those of other PIGN.Based on our analysis of the Renal Biopsy Database, the incidence of PIGN in Japan reached its peak in the 1990s. The temporal changes in the incidence of PIGN reflected the trends in infectious diseases of each decade and the continual aging of the population, with a related higher susceptibility to infections.

  3. Renal outcome and risk factors for end-stage renal disease in pediatric rapidly progressive glomerulonephritis.

    Science.gov (United States)

    Piyaphanee, Nuntawan; Ananboontarick, Chompoonut; Supavekin, Suroj; Sumboonnanonda, Achra

    2017-03-01

    Rapidly progressive glomerulonephritis (RPGN), defined as acute nephritic syndrome with dramatic loss of renal function within a few months, is associated with crescentic glomerulonephritis (CresGN), which requires ≥50% crescents on pathology. The disease characteristics and renal outcome in children with RPGN, however, will differ according to the percentage of crescents. To evaluate the renal outcomes and factors associated with end-stage renal disease (ESRD), this retrospective cohort study assessed children aged ≤15 years with RPGN at a tertiary medical center. Of 67 patients with RPGN, 32 (47.8%) were male; mean age was 10.6 ± 3.0 years; median follow up was 1.1 years (range, 0.02-9.17 years) and 24 (35.8%) progressed to ESRD. Post-infectious glomerulonephritis was the most frequent cause of RPGN (50.7%). The incidence of ESRD was significantly higher in patients with ≥50% than disease etiology, serum creatinine >3 mg/dL, need for acute dialysis, ≥80% crescents and ≥20% tubular atrophy and interstitial fibrosis (TA/IF) were associated with ESRD. On multivariate analysis, need for acute dialysis (HR, 2.8; 95% CI: 1.1-7.3, P = 0.041) and ≥20% TA/IF (HR, 4.8; 95% CI: 1.4-16.1, P = 0.011) were independent risk factors for the development of ESRD. Approximately one-third of children with RPGN developed ESRD; and need for acute dialysis and TA/IF ≥20% were independent risk factors for ESRD. © 2016 Japan Pediatric Society.

  4. Hydralazine-induced pauci-immune glomerulonephritis: intriguing case series with misleading diagnoses

    Directory of Open Access Journals (Sweden)

    Faizan Babar

    2016-04-01

    Full Text Available Hydralazine has been used since the 1950s for the management of hypertension. Evidence for hydralazine-associated vasculitis dates to pre-ANCA (antineutrophil cytoplasmic antibodies era. This abstract describes two cases of ANCA-positive pauci-immune glomerulonephritis (GN in challenging scenarios where diagnosis was misconstrued. A comprehensive literature review was done to understand the pathogenesis of drug-induced pauci-immune GN. We have described key diagnostic features that are helpful in distinguishing idiopathic ANCA vasculitis from drug-induced vasculitis. Additionally, we have also described different treatments meant to provide therapy options with the least side effects.

  5. Sickle cell disease complicated by post-streptococcal glomerulonephritis, cerebral hemorrhage and reversible posterior leucoencephalopathy syndrome.

    Science.gov (United States)

    Pashankar, Farzana D; Ment, Laura R; Pearson, Howard A

    2008-04-01

    A patient with homozygous hemoglobin SS disease presented with an intracerebral hemorrhage complicating reversible posterior leucoencephalopathy syndrome (RPLS), secondary to hypertension associated with acute post-streptococcal glomerulonephritis (APSGN). Distinguishing potentially reversible causes of central nervous system events from primary cerebral infarction or hemorrhage in patients with sickle cell disease is important because the management and prognosis of these complications is very different. Similarly, because of the difference in prognosis between APSGN and other forms of sickle cell nephropathy, it is also important to differentiate these conditions. (c) 2008 Wiley-Liss, Inc.

  6. Diffuse alveolar hemorrhage in a patient with acute poststreptococcal glomerulonephritis caused by impetigo.

    Science.gov (United States)

    Yoshida, Masahiro; Yamakawa, Hideaki; Yabe, Masami; Ishikawa, Takeo; Takagi, Masamichi; Matsumoto, Kei; Hamaguchi, Akihiko; Ogura, Makoto; Kuwano, Kazuyoshi

    2015-01-01

    We herein report a case of pulmonary renal syndrome with nephritis in a 17-year-old boy with diffuse alveolar hemorrhage (DAH) associated with acute poststreptococcal glomerulonephritis (APSGN). The patient exhibited hemoptysis two weeks after developing impetigo, and DAH was diagnosed on bronchoscopy. Respiratory failure progressed, and high-dose methylprednisolone therapy was administered; the respiratory failure regressed immediately after the onset of therapy. Streptococcus pyogenes was detected in an impetigo culture, and, together with the results of the renal biopsy, a diagnosis of APSGN was made. This case demonstrates the effects of high-dose methylprednisolone therapy in improving respiratory failure.

  7. Effects of receptor-mediated endocytosis and tubular protein composition on volume retention in experimental glomerulonephritis

    DEFF Research Database (Denmark)

    Kastner, Christian; Pohl, Marcus; Sendeski, Mauricio

    2009-01-01

    Human glomerulonephritis (GN) is characterized by sustained proteinuria, sodium retention, hypertension, and edema formation. Increasing quantities of filtered protein enter the renal tubule, where they may alter epithelial transport functions. Exaggerated endocytosis and consequent protein...... endocytosis. Mice displayed salt retention and elevated systolic blood pressure when proteinuria had reached 10-15 mg/24 h. Surface expression of proximal Na(+)-coupled transporters and water channels was in part [Na(+)-P(i) cotransporter IIa (NaPi-IIa) and aquaporin-1 (AQP1)] increased by megalin deficiency...... mechanism of channel activation which may involve the action of filtered plasma proteases....

  8. Nephrotic Syndrome Secondary to Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits of Lambda Light Chain

    Directory of Open Access Journals (Sweden)

    Seongseok Yun

    2014-01-01

    Full Text Available We describe a rare case of a 46-year-old woman with history of refractory nephrotic syndrome and hypertension who presented with worsening proteinuria and kidney function. Work-up for both autoimmune and infectious diseases and hematologic malignancies including multiple myeloma were negative. Kidney biopsy demonstrated glomerular sclerotic change with lambda light chain deposits in the subendothelial space, which is consistent with proliferative glomerulonephritis with monoclonal immunoglobulin deposit (PGNMID. The patient was treated with bortezomib and dexamethasone without clinical improvement and eventually became hemodialysis dependent.

  9. Crescentic Glomerulonephritis in IgA Multiple Myeloma: A Case Report

    Directory of Open Access Journals (Sweden)

    Grace T. Moscoso-Solorzano

    2011-09-01

    Full Text Available Background: There are few reports of glomerulonephritis (GN with crescents and a rapidly progressive course that lead to a diagnosis of a previously unsuspected B-cell dyscrasia. Case Presentation: We report a case of rapidly progressive GN: the patient showed no evidence of etiology at the time of biopsy and was diagnosed as IgA multiple myeloma (MM during investigation based on a renal biopsy. He presented diffuse proliferative and exudative GN and marked plasma cell infiltration of the kidney. Conclusion: The present case raises the possibility that proliferative GN with crescents may be a rare mode of presentation of MM.

  10. Urinary enzymatic markers (N-acetyl-beta-D-glucosaminidase) in assessing the tubulointerstitial compartment in chronic glomerulonephritis related to odontogenic foci.

    Science.gov (United States)

    Velciov, Silvia; Gluhovschi, Gheorghe; Timar, Romulus; Gluhovschi, Cristina; Petrica, Ligia; Bob, Flaviu; Bozdog, Gheorghe; Pricop, Marius; Gluhovschi, Adrian; Cornianu, Marioara; Potencz, Elena; Timar, Bogdan; Kaycsa, Adriana

    2016-02-01

    Chronic glomerulonephritis is related to focus infection. Odontogenic foci are frequently involved in glomerulonephritis. The relationship with the odontogenic focus infection can be demonstrated by the occurrence or aggravation of the symptoms of glomerulonephritis: proteinuria, haematuria, high blood pressure and oedema. Glomerular impairment in glomerulonephritis occurs together with inflammatory alterations of the tubulointerstitial compartment that can play an important part in the evolution of the disease. Tubular urinary markers can indicate the activation of this compartment during an infection of a focus, an odontogenic focus in our study.The paper aims at demonstrating the relationship between the odontogenic focus infection and tubulointerstitial lesions, assessed by a tubular urinary marker, N-acetyl beta-D glucosaminidase (NAG).We investigated the urinary N-acetyl beta-D glucosaminidase of 20 patients with chronic glomerulonephritis who presented odontogenic focus infections, comparing them with patients with chronic glomerulonephritis without odontogenic foci and of 20 controls, clinically healthy persons.Chronic glomerulonephritis patients with odontogenic focus infection presented clearly increased values as compared to clinically healthy control persons of urinary N-acetyl beta-D glucosaminidase.These patients underwent surgical intervention on the odontogenic focus under antibacterial prophylactic treatment. In 75% cases, the values of N-acetyl beta-D glucosaminidase diminished, indicating the favourable effect of the treatment of the odontogenic focus on the tubulointerstitial compartment in patients with chronic glomerulonephritis. In 25% cases this therapeutic treatment was associated with an increase of the values of urinary N-acetyl beta-D glucosaminidase, expressing its unfavourable effect on chronic glomerulonephritis.Urinary N-acetyl beta-D glucosaminidase indicated an etiopathogenetic relationship between the odontogenic focus and the

  11. Basement structures over Rio Grande Rise from gravity inversion

    Science.gov (United States)

    Constantino, Renata; Hackspacker, Peter Christian; Anderson de Souza, Iata; Sousa Lima Costa, Iago

    2017-04-01

    In this study, we show that from satellite-derived gravity field, bathymetry and sediment thicknesses, it is possible to give a 3-D model of the basement over oceanic areas, and for this purpose, we have chosen the Rio Grande Rise, in South Atlantic Ocean, to build a gravity-equivalent basement topography. The advantages of the method applied in this study are manifold: does not depend directly on reflection seismic data; can be applied quickly and with fewer costs for acquiring and interpreting the data; and as the main result, presents the physical surface below the sedimentary layer, which may be different from the acoustic basement. We evaluated the gravity effect of the sediments using the global sediment thickness model of NOAA, fitting a sediment compaction model to observed density values from Deep Sea Drilling Program (DSDP) reports. The Global Relief Model ETOPO1 and constraining data from seismic interpretation on crustal thickness are integrated in the gravity inversion procedure. The modeled Moho depth values vary between 6 to 27 km over the area, being thicker under the Rio Grande Rise and also in the direction of São Paulo Plateau. The inversion for the gravity-equivalent basement topography is applied for a gravity residual data, which is free from the gravity effect of sediments and from the gravity effect of the estimated Moho interface. A description of the basement depth over Rio Grande Rise area is unprecedented in the literature, however, our results could be compared to in situ data, provided by DSDP, and a small difference of only 9 m between our basement depth and leg 516 F was found. Our model shows a rift crossing the entire Rio Grande Rise deeper than previously presented in literature, with depths up to 5 km in the East Rio Grande Rise (ERGR) and deeper in the West Rio Grande Rise (WRGR), reaching 6.4 km. We find several short-wavelengths structures not present in the bathymetry data. Seamounts, guyots and fracture zones are much more

  12. C3 glomerulonephritis and autoimmune disease: more than a fortuitous association?

    Science.gov (United States)

    Alexander, Mariam P; Fervenza, Fernando C; De Vriese, An S; Smith, Richard J H; Nasr, Samih H; Cornell, Lynn D; Herrera Hernandez, Loren P; Zhang, Yuzhou; Sethi, Sanjeev

    2016-04-01

    C3 glomerulonephritis (C3GN) results from genetic or acquired dysregulation of the alternative complement pathway. A subset of patients may have clinical and biochemical characteristics compatible with an autoimmune disorder. We studied a cohort of 85 patients with confirmed C3GN (2007-2014), of which ten patients (3 male, 7 female; mean age 38.5 years) had an associated autoimmune disorder. All patients had abnormal ANA titers, 6 also had positive ds-DNA titers. At the time of presentation with C3GN, all 7 female patients had autoimmune-related presentations. Of the 3 male patients, only 1 patient had autoimmune-related presentations. Kidney biopsy showed predominantly mesangial proliferative or membranoproliferative glomerulonephritis. In 5 patients, the alternative pathway was evaluated. All had allele variants/polymorphisms associated with C3GN. One patient was also positive for C3Nefs. Treatment varied form conservative management to the use of prednisone alone or with cytotoxic therapy. Mean serum creatinine decreased from 2.0 to 1.4 mg/dL while proteinuria decreased from 2300 to 994 mg/24 h in 8 patients with follow-up. The study highlights the association between C3GN and autoimmune disorders, particularly in female patients. The study suggests that an autoimmune milieu may act as a trigger for the development of C3GN in genetically susceptible patients. Short-term prognosis of C3GN associated with autoimmune disorders appears excellent.

  13. Concomitant Granulomatosis with Polyangiitis and C3 Glomerulonephritis Causing Renal Failure.

    Science.gov (United States)

    Chaudhuri, Aadel A; Davis, Jason T

    2016-02-05

    A 75-year-old male with no prior history of vasculitis or renal deficiency presented with a history of 36 hours of anterior epistaxis, one month of worsening shortness of breath, severe anemia requiring transfusion, thrombocytopenia, coagulopathy with INR 4.9, and renal failure requiring hemodialysis. A peripheral blood smear revealed no evidence of microangiopathic hemolytic anemia. We performed an autoimmune workup, which revealed high levels of serum serine protease 3 antibody (C-ANCA), elevated rheumatoid factor, low serum C3, and normal levels of serum C4. We performed a renal biopsy and then performed light microscopy, immunofluorescence, and electron microscopy on the resulting samples. This revealed that approximately half of the sampled glomeruli were globally sclerotic, consistent with severe renal disease. Among the non-sclerotic glomeruli, several demonstrated diffuse granular mesangial staining for C3, while other glomeruli had small crescents, consistent with a mixed picture of C3 glomerulonephritis (C3GN) and crescentic glomerulonephritis. The patient responded well to treatment with cyclophosphamide and prednisone, with a resolution of his acute issues, significant improvement in kidney function, and was eventually weaned from routine hemodialysis. In summary, this is a unique case of a patient presenting with features of both granulomatosis with polyangiitis (GPA) and C3GN.

  14. Renal progenitor cells contribute to hyperplastic lesions of podocytopathies and crescentic glomerulonephritis.

    Science.gov (United States)

    Smeets, Bart; Angelotti, Maria Lucia; Rizzo, Paola; Dijkman, Henry; Lazzeri, Elena; Mooren, Fieke; Ballerini, Lara; Parente, Eliana; Sagrinati, Costanza; Mazzinghi, Benedetta; Ronconi, Elisa; Becherucci, Francesca; Benigni, Ariela; Steenbergen, Eric; Lasagni, Laura; Remuzzi, Giuseppe; Wetzels, Jack; Romagnani, Paola

    2009-12-01

    Glomerular injury can involve excessive proliferation of glomerular epithelial cells, resulting in crescent formation and obliteration of Bowman's space. The origin of these hyperplastic epithelial cells in different glomerular disorders is controversial. Renal progenitors localized to the inner surface of Bowman's capsule can regenerate podocytes, but whether dysregulated proliferation of these progenitors contributes to crescent formation is unknown. In this study, we used confocal microscopy, laser capture microdissection, and real-time quantitative reverse transcriptase-PCR to demonstrate that hypercellular lesions of different podocytopathies and crescentic glomerulonephritis consist of three distinct populations: CD133(+)CD24(+)podocalyxin (PDX)(-)nestin(-) renal progenitors, CD133(+)CD24(+)PDX(+)nestin(+) transitional cells, and CD133(-)CD24(-)PDX(+)nestin(+) differentiated podocytes. In addition, TGF-beta induced CD133(+)CD24(+) progenitors to produce extracellular matrix, and these were the only cells to express the proliferation marker Ki67. Taken together, these results suggest that glomerular hyperplastic lesions derive from the proliferation of renal progenitors at different stages of their differentiation toward mature podocytes, providing an explanation for the pathogenesis of hyperplastic lesions in podocytopathies and crescentic glomerulonephritis.

  15. Granulomatosis with Polyangiitis Presenting as Pauci-Immune Crescentic Glomerulonephritis in Pregnancy

    Directory of Open Access Journals (Sweden)

    Ryan Kunjal

    2016-01-01

    Full Text Available Antineutrophil cytoplasmic antibody (ANCA associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophilia. During her course she also developed one episode of small volume hemoptysis with right upper lobe infiltrates on CT Chest. There were no cutaneous manifestations of vasculitis or upper respiratory symptoms. Renal biopsy revealed a pauci-immune crescentic glomerulonephritis (PICGN. The diagnosis was consistent with granulomatosis with polyangiitis (GPA. Management initially comprised teratogen sparing agents; steroids, intravenous immunoglobulin; and plasma exchange. The response was suboptimal and she became dependent on daily renal replacement therapy. Ultimately the pregnancy was terminated allowing for traditional treatment approaches with dramatic effect. This is the first case of GPA presenting as PICGN in pregnancy and highlights the challenges of its management.

  16. Granulomatosis with Polyangiitis Presenting as Pauci-Immune Crescentic Glomerulonephritis in Pregnancy.

    Science.gov (United States)

    Kunjal, Ryan; Makary, Raafat; Poenariu, Andreea

    2016-01-01

    Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophilia. During her course she also developed one episode of small volume hemoptysis with right upper lobe infiltrates on CT Chest. There were no cutaneous manifestations of vasculitis or upper respiratory symptoms. Renal biopsy revealed a pauci-immune crescentic glomerulonephritis (PICGN). The diagnosis was consistent with granulomatosis with polyangiitis (GPA). Management initially comprised teratogen sparing agents; steroids, intravenous immunoglobulin; and plasma exchange. The response was suboptimal and she became dependent on daily renal replacement therapy. Ultimately the pregnancy was terminated allowing for traditional treatment approaches with dramatic effect. This is the first case of GPA presenting as PICGN in pregnancy and highlights the challenges of its management.

  17. Cancer in ANCA-Associated Glomerulonephritis: A Registry-Based Cohort Study

    Directory of Open Access Journals (Sweden)

    Sanjeevan Sriskandarajah

    2017-01-01

    Full Text Available Background. Immunosuppressive therapy for antineutrophil cytoplasmic antibody-associated vasculitis has been associated with increased malignancy risk. Objectives. To quantify the cancer risk associated with contemporary cyclophosphamide-sparing protocols. Methods. Patients from the Norwegian Kidney Biopsy Registry between 1988 and 2012 who had biopsy-verified pauci-immune glomerulonephritis and positive antineutrophil cytoplasmic antibody (ANCA serology were included. Standardised incidence ratios (SIRs were calculated to compare the study cohort with the general population. Results. The study cohort included 419 patients. During 3010 person-years, cancer developed in 41 patients (9.79%; the expected number of cancer cases was 37.5 (8.95%. The cohort had SIRs as follows: 1.09, all cancer types (95% CI, 0.81 to 1.49; 0.96, all types except nonmelanoma skin cancer (95% CI, 0.69 to 1.34; 3.40, nonmelanoma skin cancer (95% CI, 1.62 to 7.14; 3.52, hematologic cancer (95% CI, 1.32 to 9.37; 2.12, posttransplant cancer (95% CI, 1.01 to 4.44; and 1.53, during the 1–5-year follow-up after diagnosis (95% CI, 1.01 to 2.32. Conclusions. Cancer risk did not increase significantly in this cohort with ANCA-associated glomerulonephritis. However, increased risk of nonmelanoma skin cancer, posttransplant cancer, and hematologic cancer indicates an association between immunosuppression and malignancy.

  18. Membranoproliferative glomerulonephritis presenting as arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis: a case report.

    Science.gov (United States)

    Park, Chuiyoung; Choi, Seung Won; Kim, Misung; Park, Jongha; Lee, Jong Soo; Chung, Hyun Chul

    2014-10-22

    Hypocomplementemic urticarial vasculitis syndrome is a rare disorder characterized by chronic urticarial vasculitis, arthralgia, arthritis, and hypocomplementemia. Previously, only six patients with concomitant hypocomplementemic urticarial vasculitis syndrome, Jaccoud's arthropathy, and valvular heart disease have been reported. A 30-year-old Korean man presented with hypocomplementemic urticarial vasculitis syndrome. In addition to urticarial cutaneous lesions, he experienced polyarthralgia and arthritis that resulted in progressive deformity of the joints of both hands, cardiac valvulopathy with mitral, tricuspid, and aortic regurgitation, and intermittent neck swelling with laryngeal edema. He also developed nephritis with azotemia. His renal biopsy results revealed membranoproliferative glomerulonephritis, type I. He showed a partial response to a combination therapy of steroid, cyclophosphamide, and mycophenolate mofetil. We describe, to the best of our knowledge, the first case of glomerulonephritis presenting a arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis syndrome. A combination of corticosteroids, cyclophosphamide, and mycophenolate mofetil appear to be a safe and effective treatment for nephropathy, however are less effective for cutaneous vasculitis, cardiac valvulopathy, and arthropathy.

  19. Clinicoradiological assessment of the efficacy of pathogenetic therapy of glomerulonephritis in children

    International Nuclear Information System (INIS)

    Bagdasarova, I.V.; Mil'ko, V.I.; Moskalenko, N.I.; Tanasichuk-Gazhieva, N.V.

    1987-01-01

    Renoscintigraphy and hepatobiliscintigraphy were employed for the assessment of kidney and liver function in children with glomerulonephritis at the stages of pathogenetic therapy with chlorambucil and prednisolone. Noticeable disturbance of nephron and hepatocytic function at the climax of clinical manifestations of the nephrotic syndrome was established. The use of maximum and maintenance doses of prednisolone and chlorabucil resulted in significant improvement of clinical and radionuclide indices. However the effective renal plasma flow and the rate of passage of the radionuclide from the plasma into the proximal canalicular cell did not return to normal even in the catamnesis 1 year after the discontinuation of treatment in 35% of the patients with chronic glomerulonephritis. The use of maximum doses of cytostatics in 98.5% of the children produced no damaging effect on the hepatocyte, in 1.5% of the children the authors observed the development of drug hepatitis. Hypokinetic dyskinesia of the gall bladder was detected in the overwhelming majority of the patients therefore drugs improving bile secretion should be recommended as part of treatment

  20. Petrology and geotechnic setting of the basement comple rocks ...

    African Journals Online (AJOL)

    The basement complex, located at the south-eastern borders of Ogoja town in the south-eastern part of Nigeria, consists of two varieties of migmatitic gneisses, namely banded and augen gneisses, which, in some cases, are inter-layered with amphibolite. Many concordant to discordant quartzofeldspathic veins in these ...

  1. Groundwater Exploration in the Basement Complex Around Chibok ...

    African Journals Online (AJOL)

    A geophysical survey employing Schlumberger electrode configuration using vertical electrical sounding (VES) method was carried out around Chibok area within the Basement Complex of north-eastern Nigeria using an ABEM SAS 300C Terrameter with a view to exploring groundwater within the study area.

  2. Source parameter imaging from areomagnetic data of the basement ...

    African Journals Online (AJOL)

    Source parameter imaging from areomagnetic data of the basement rocks in part of the middle Benue trough, Nigeria. GC Onyedim, EA Ariyibi, MO Awoyemi, JB Arubayi, OM Afolabi. Abstract. No Abstract. Journal of Mining and Geology Vol. 42 (2) 2006: pp. 165-173. Full Text: EMAIL FULL TEXT EMAIL FULL TEXT

  3. Aeromagnetic imaging of the basement morphology in part of the ...

    African Journals Online (AJOL)

    Aeromagnetic imaging of the basement morphology in part of the middle Benue trough, Nigeria. GC Onyedim, MO Awoyemi, EA Ariyibi, JB Arubayi. Abstract. No Abstract. Journal of Mining and Geology Vol. 42 (2) 2006: pp. 157-163. Full Text: EMAIL FULL TEXT EMAIL FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD ...

  4. Geoelectric characterization of Aquifer types in the basement ...

    African Journals Online (AJOL)

    Geoelectric characterization of Aquifer types in the basement complex terrain of parts of Osun state,Nigeria. S Bayode, JS Ojo, MO Olorunfemi. Abstract. No Abstract. Global Journal of Pure and Applied Sciences Vol. 12(3) 2006: 377-385. Full Text: EMAIL FULL TEXT EMAIL FULL TEXT · DOWNLOAD FULL TEXT ...

  5. Hydraulic characteristics of a typical basement complex aquifer in ...

    African Journals Online (AJOL)

    Hydraulic characteristics of a typical basement complex aquifer in Ajaokuta, southwestern Nigeria. CC Osadebe, JO Fatoba, S Obrike. Abstract. No Abstract. Ife Journal of Science Vol. 7(2) 2005: 297-303. Full Text: EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT.

  6. an investigation of basement fracture pattern in a part of ...

    African Journals Online (AJOL)

    DR ANIFOWOSE

    The basement complex of southwestern Nigeria has undergone severe tectonic deformation over the geologic past, resulting in various degrees of fracturing and folding, even to the extent of complete obliteration of primary structures except for some places. This study focuses on Akoko area which is dominated by hills of ...

  7. Magnetic basement in the central Bay of Bengal

    Digital Repository Service at National Institute of Oceanography (India)

    Sarma, K.V.L.N.S.; Ramana, M.V.; Ramprasad, T.; Desa, M.; Subrahmanyam, V.; Krishna, K.S.; Rao, M.M.M.

    . The N10-12 degrees W trending subsurface 85 degrees E Ridge buried under 2 to 3 km thick sediments is a prominent tectonic feature. Offshore basins characterised by deeper magnetic basement (approx. 9 km) and 100-200 km wide are present on either sides...

  8. Basement configuration of KG offshore basin from magnetic anomalies

    Indian Academy of Sciences (India)

    Marine magnetic anomalies along three representative profiles falling between shelf break and continent–ocean boundary in the offshore Krishna–Godavari basin were quantitatively interpreted for understandingthe nature and structure of the magnetic basement using inversion technique. The interpretation of ...

  9. Membranoproliferative glomerulonephritis in a patient with congenital deficiency of the third component of complement: effect of treatment with plasma.

    Science.gov (United States)

    Roord, J J; van Diemen-van Steenvoorde, R A; Schuurman, H J; Rijkers, G T; Zegers, B J; Gmelig Meyling, F H; Stoop, J W

    1989-05-01

    A 21-year-old woman with a known congenital complement component 3 (C3) deficiency developed membranoproliferative glomerulonephritis. The kidney biopsy exhibited deposits of immunoglobulins and complement components despite the C3 deficiency. The administration of fresh frozen plasma was without therapeutic benefit. Corticosteroid treatment was followed by an improvement in kidney function.

  10. Urinary Concentration of Monocyte Chemoattractant Protein-1 in Idiopathic Glomerulonephritis: A Long-Term Follow-Up Study

    OpenAIRE

    Tofik, Rafid; Ohlsson, Sophie; Bakoush, Omran

    2014-01-01

    Background Monocyte chemoattractant protein-1 (MCP-1), which is up regulated in kidney diseases, is considered a marker of kidney inflammation. We examined the value of urine MCP-1 in predicting the outcome in idiopathic glomerulonephritis. Methods Between 1993 and 2004, 165 patients (68 females) diagnosed with idiopathic proteinuric glomerulopathy and with serum creatinine

  11. Genetic and pharmacological inhibition of microRNA-92a maintains podocyte cell cycle quiescence and limits crescentic glomerulonephritis

    OpenAIRE

    Henique, Carole; Bollée, Guillaume; Loyer, Xavier; Grahammer, Florian; Dhaun, Neeraj; Camus, Marine; Vernerey, Julien; Guyonnet, Léa; Gaillard, François; Lazareth, Hélène; Meyer, Charlotte; Bensaada, Imane; Legrès, Luc; Satoh, Takashi; Akira, Shizuo

    2017-01-01

    Crescentic rapidly progressive glomerulonephritis (RPGN) represents the most aggressive form of acquired glomerular disease. While most therapeutic approaches involve potentially toxic immunosuppressive strategies, the pathophysiology remains incompletely understood. Podocytes are glomerular epithelial cells that are normally growth-arrested because of the expression of cyclin-dependent kinase (CDK) inhibitors. An exception is in RPGN where podocytes undergo a deregulation of their differenti...

  12. RENAL ISCHEMIA-REPERFUSION INJURY CONTRIBUTES TO RENAL DAMAGE IN EXPERIMENTAL ANTI-MYELOPEROXIDASE-ASSOCIATED PROLIFERATIVE GLOMERULONEPHRITIS

    NARCIS (Netherlands)

    BROUWER, E.; Klok, P.A; HUITEMA, M.G.; Weening, J.J.; Kallenberg, Cees

    The occurrence of focal fibrinoid necrosis of capillary loops in the very early stages of ANCA-associated necrotizing crescentic glomerulonephritis (NCGN) and the increased prevalence of this disease at older age suggest that renal ischemia may play an additional role in its pathophysiology. In the

  13. Hepatitis B reactivation in HBsAg-negative/HBcAb-positive patients receiving immunosuppressive therapy for glomerulonephritis: a retrospective analysis.

    Science.gov (United States)

    Fang, Jing; Li, Wenge; Peng, Xiangxin; Tan, Zhao; Tan, Min; Zhang, Cong; Wang, Wenbo; Xu, Zhihong; Zhou, Gumin

    2017-03-01

    The aim of this study is to investigate the prevalence and risk factors for hepatitis B virus (HBV) reactivation in HBsAg-negative/HBcAb-positive patients receiving immunosuppressive therapy for glomerulonephritis. We performed a retrospective study of 745 HBsAg-negative/HBcAb-positive patients undergoing immunosuppressive therapy for glomerulonephritis from years 2003 to 2012 at the department of nephrology, China-Japan Friendship Hospital, Beijing, China. The patients were divided into HBV reactivation group (n = 27) and non-HBV reactivation group (n = 718). The prevalence of HBV reactivation in patients receiving immunosuppressive therapy for glomerulonephritis was up to 3.62% in serological status of HBsAg-negative/HBcAb-positive. HBV reactivation was associated with several findings: greater proportion of lupus nephritis (25.93 vs. 9.61%, p = 0.014), much higher percentage of HBsAb-negative (74.07 vs. 23.82%, p HBcAb-positive glomerulonephritis patients treated with immunosuppressant, and the prevalence was up to 3.62%. Patients with serological status of HBsAb-negative, more than 1 year of immunosuppressive therapy, and combined immunosuppressant are independent risk factors for HBV reactivation.

  14. Study on the changes of serum IL-2, IL-6 and TNF-α levels in patients with chronic glomerulonephritis

    International Nuclear Information System (INIS)

    Xie Jianping; Feng Jiangchao; Zhang Guoyuan; Xiong Gang; Tu Lirong; Xia Chengyun

    2003-01-01

    Objective: To investigate the changes of serum IL-2, IL-6 and TNF-α levels in patients with chronic glomerulonephritis of various types and stages. Methods: Serum IL-2, IL-6 and TNF-α levels were measured with RIA in 71 patients with chronic glomerulonephritis and 36 controls. Results: 1) Serum levels of IL-6 and TNF-α were significantly higher in patients with chronic glomerulonephritis than those in controls (p<0.01) but serum IL-2 levels were significantly lower in the patients (p<0.01). 2)Serum IL-6 and TNF-α levels were positively correlated to BUN levels (p<0.01). 3)There were no significant differences of these cytokines levels among the different types of patients (i.e. general type n=18, nephrotic type n=33 and hypertensive type n=20). Conclusion: These cytokines participated in the pathogenesis of chronic glomerulonephritis. Monitoring the changes of their serum levels was helpful for the management of the disease

  15. Antineutrophil cytoplasmic antibody–negative pauci-immune glomerulonephritis with massive intestinal bleeding

    Directory of Open Access Journals (Sweden)

    Miyeon Kim

    2015-09-01

    Full Text Available A 61-year-old woman was admitted to hospital because of generalized edema and proteinuria. Her renal function deteriorated rapidly. Serum immunoglobulin and complement levels were within normal ranges. An autoantibody examination showed negative for antinuclear antibody and antineutrophil cytoplasmic antibody. Histologic examination of a renal biopsy specimen revealed that all of the glomeruli had severe crescent formations with no immune deposits. The patient was treated with steroid pulse therapy with cyclophosphamide followed by oral prednisolone. Fifteen days later, she experienced massive recurrent hematochezia. Angiography revealed an active contrast extravasation in a branch of the distal ileal artery. We selectively embolized with a permanent embolic agent. On the 45th hospital day, the patient suddenly lost consciousness. Brain computed tomography showed intracerebral hemorrhage. We report a case of antineutrophil cytoplasmic antibody–negative pauci-immune glomerulonephritis with massive intestinal bleeding and cerebral hemorrhage.

  16. Polyarthritis and membranoproliferative glomerulonephritis as paraneoplastic manifestation of Hodgkin's lymphoma: A case report and literature review.

    Science.gov (United States)

    Erlij, Daniel; Calderón, Beatriz; Rivera, Angela; Mella, Cristián; Valladares, Ximena; Roessler, Emilio; Rivera, María Teresa; Méndez, Gonzalo

    2016-01-01

    Paraneoplastic syndromes can be presented in multiple ways, which include endocrinological, hematologic, rheumatologic and nephrologic manifestations. While most of the publications described solid tumors as responsible for these manifestations, hematologic neoplasms are important cause to consider as part of the differential diagnosis. We report the case of a 46 year-old man with seronegative symmetric polyarthritis of large and small joints associated with membranoproliferative glomerulonephritis with deposits of immune complexes and acute impairment of renal function, as part of a paraneoplastic syndrome secondary of a classical Hodgkin lymphoma with bone marrow invasion, which reversed completely with chemotherapy treatment. Copyright © 2015 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  17. Plasma Exchange for Renal Vasculitis and Idiopathic Rapidly Progressive Glomerulonephritis: A Meta-analysis

    DEFF Research Database (Denmark)

    Walsh, Michael; Catapano, Fausta; Szpirt, Wladimir

    2010-01-01

    .9). LIMITATIONS:: Although the primary result was statistically significant, there is insufficient statistical information to reliably determine whether plasma exchange decreases the composite of end-stage renal disease or death. CONCLUSIONS:: Plasma exchange may decrease the composite end point of end......BACKGROUND:: Plasma exchange may be effective adjunctive treatment for renal vasculitis. We performed a systematic review and meta-analysis of randomized controlled trials of plasma exchange for renal vasculitis. STUDY DESIGN:: Systematic review and meta-analysis of articles identified from...... electronic databases, bibliographies, and studies identified by experts. Data were abstracted in parallel by 2 reviewers. SETTING & POPULATION:: Adults with idiopathic renal vasculitis or rapidly progressive glomerulonephritis. SELECTION CRITERIA FOR STUDIES:: Randomized controlled trials that compared...

  18. Erythropoietin deficiency in acute crescentic glomerulonephritis and in total bilateral renal cortical necrosis

    DEFF Research Database (Denmark)

    Thaysen, J H; Nielsen, O J; Brandi, L

    1991-01-01

    Six patients with acute renal failure, in five cases due to acute crescentic glomerulonephritis and in one case due to total bilateral renal cortical necrosis, were studied. All had serum erythropoietin (EPO) concentrations in the normal range, despite a relatively severe anaemia. Half...... treated with rhEPO (Eprex). In marked contrast to untreated controls, both patients responded with vigorous reticulocytosis and normalization of haemoglobin levels while they were still in severe renal failure. These results are similar to our previous findings in patients with acute renal failure due...... of EPO is not only a permanent and irreversible feature of severe chronic renal failure, but that it is also present, usually in a transient and reversible form, in different types of acute renal failure....

  19. Posterior reversible encephalopathy syndrome and acute post-streptococcal glomerulonephritis mimicking breakthrough seizures

    Directory of Open Access Journals (Sweden)

    Kamille Abdool

    2015-05-01

    Full Text Available We report the case of a 14-year-old boy with a past history of primary generalized seizures, who had been seizure-free for 2 years on sodium valproate and presented with generalized tonic clonic seizures suggestive of breakthrough seizures. Examination revealed hypertension, impetiginous lesions of the lower limbs, microscopic hematuria, elevated antistreptolysin O titre and low complement levels consistent with acute post-streptococcal glomerulonephritis. Cranial magnetic resonance imaging (MRI demonstrated changes consistent with posterior reversible encephalopathy syndrome. Hypertension was controlled with intravenous nitroglycerin followed by oral captopril and amlodipine. Brain MRI changes returned normal within 2 weeks. The nephritis went in to remission within 2 months and after 8 months the patient has been seizure free again. Posterior reversible encephalopathy syndrome appeared to have neither short nor intermediate effect on seizure control in this patient. The relationship between posterior reversible encephalopathy syndrome and seizures is reviewed.

  20. Posterior Reversible Encephalopathy Syndrome and Acute Post-Streptococcal Glomerulonephritis Mimicking Breakthrough Seizures.

    Science.gov (United States)

    Abdool, Kamille; Ramcharan, Kanterpersad; Bhagwandass, Neal; Persad, Navindra; Temull, Vasant; Seegobin, Karan; Mike, Cassie

    2015-03-23

    We report the case of a 14-year-old boy with a past history of primary generalized seizures, who had been seizure-free for 2 years on sodium valproate and presented with generalized tonic clonic seizures suggestive of breakthrough seizures. Examination revealed hypertension, impetiginous lesions of the lower limbs, microscopic hematuria, elevated anti-streptolysin O titre and low complement levels consistent with acute post-streptococcal glomerulonephritis. Cranial magnetic resonance imaging (MRI) demonstrated changes consistent with posterior reversible encephalopathy syndrome. Hypertension was controlled with intravenous nitroglycerin followed by oral captopril and amlodipine. Brain MRI changes returned normal within 2 weeks. The nephritis went in to remission within 2 months and after 8 months the patient has been seizure free again. Posterior reversible encephalopathy syndrome appeared to have neither short nor intermediate effect on seizure control in this patient. The relationship between posterior reversible encephalopathy syndrome and seizures is reviewed.

  1. [Membranous nephropathy associated to autoimmune thyroiditis, chronic pancreatitis and suprarrenal insufficiency].

    Science.gov (United States)

    Merino, J L; Fernández Lucas, M; Teruel, J L; Valer, P; Moreira, V; Arambarri, M; Ortuño, J

    2004-01-01

    A 33 year old female was admitted to the hospital to study aedema and bocio, A nephrotic syndrome was diagnosed and the renal biopsy demonstrated membranous glomerulonephritis, stage II. She was also diagnosed of Hashimoto's autoinmmune thyroiditis: TSH (41.5 uUl/ml), T4 (0.07 ng/dl), antithyroglobuline (1/2560) and antimicrosome (1/6400). Four year latter she was diagnosed of autoinmmune pancreatitis, without evidence of diabetes mellitus or exocrine pancreatic insufficiency. Eight years latter she was diagnosed of primary autoimmune suprarrenal insufficiency: basal cortisol: 2.7 mcg/dl, post ACTH estimulated cortisol: 5.6 mcg/dl, antinuclear antibody (1/160) and antiparietal (1/320). We present a pluriglandular autoimmune syndrome with membranous glomerulonephritis, thyroiditis, pancreatitis and suprarrenal insufficiency. To the best of our knowledge this complex syndrome has not been previously described.

  2. [The role of renal anemia and cardiovascular disease in the progression of chronic glomerulonephritis].

    Science.gov (United States)

    Murkamilov, I T; Gordeev, I G; Kaliev, R R

    To study the rate of chronic glomerulonephritis progression when added by anemia and cardiovascular disease (CVD). 231 patients (133 men and 98 women) with predialysis chronic glomerulonephritis (CGN) were examined. The patients' mean age of was 35.8±11.8 years; the disease duration was 1 to 17 years. The disease onset was the date when urinalysis showed evidence of persistent proteinuria and (or) hematuria. Besides, the time when anemia developed and the clinical and instrumental signs of CVD appeared was taken as the initial reference point; the time when end-stage renal failure was diagnosed was taken to be the endpoint. Red blood cell counts with the inclusion of its indices, hemoglobin concentration, hematocrit values, daily proteinuria values, and glomerular filtration rate were analyzed. The biochemical parameters included the concentrations of electrolytes, creatinine, fibrinogen, iron, cholesterol, total protein and C-reactive protein (CRP). Electrocardiography and echocardiography, bicycle ergometry and 24-hour ECG monitoring were used to detect CVD. The presence of anemia and CVD in patients with predialysis CGN versus those without anemia and CVD was associated with an increase in the concentrations of CRP [36.2 and 12.6%; respectively; (p<0.05)], creatinine [123.0 (83.2-217.0) and 86.5 (72.0-128.5) µmol/L; (p<0.05)] and a decrease in GFR [65.4 (30.8-95.5) and 92.7 (64.5-122.3) ml/min; (p<0.05)]. The 8-year survival of patients with CGN concurrent with CVD was noted to be 58% whereas the renal survival in patients with CGN + CVD + anemia was shorter and the 6-year survival in this case was as high as 52%. Anemia and CVD in patients with CGN serve as additional independent factors for the progression of the underlying disease. The concurrence of CGN, anemia, and CVD substantially reduces the predialysis period.

  3. Treatment with Quinoline-3-carboxamide does not successfully prevent immune-mediated glomerulonephritis in mice.

    Science.gov (United States)

    Draibe, Juliana; Pepper, Ruth J; Salama, Alan D

    Quinoline-3-carboximide compounds, such as paquinimod, which targets the protein S100A9, have demonstrated efficacy in treating autoimmune diseases. S100A9, in association with S100A8, forms the heterodimer S100A8/S100A9, known as calprotectin; that has been shown to be upregulated in numerous inflammatory disorders. We had previously demonstrated protection from glomerular disease in S100A9-deficient mice. The aim of this study was to assess the efficacy of paquinimod in the prevention and treatment of experimental glomerulonephritis. Nephrotoxic nephritis (NTN) was induced in C57BL/6 mice according to our standard protocol. Mice were treated with different doses of paquinimod either at disease induction (prevention group) or two days following induction (therapeutic group) and sacrificed 8 days following induction. Disease was assessed histologically (number of glomerular crescents, degree of glomerular thrombosis, number of infiltrating leucocytes and calprotectin expression) and biochemically (serum creatinine and urea levels, and urinary levels of protein). Neither treatment with low (0.5mg/kg) or high (25mg/kg) doses of paquinimod, given preventatively or therapeutically, led to disease attenuation, as assessed by biochemical or histological parameters. Additionally, we found trends for an increase in renal glomerular calprotectin expression in the high dose groups, suggesting a possible feedback regulation of calprotectin expression. Our results show that paquinimod does not successfully prevent or treat mice with NTN. Other models of immune-mediated glomerulonephritis need to be tested to investigate the therapeutic potential of this compound in renal disease. Copyright © 2016 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

  4. C3 deposits worsens the prognosis in type iii extracapillary glomerulonephritis.

    Science.gov (United States)

    Sánchez-Agesta Martínez, Marina; Rabasco Ruiz, Cristina; Sánchez Sánchez, Rafael; Ortega Salas, Rosa; López Andreu, María; Aljama García, Pedro; Espinosa Hernández, Mario

    Type iii extracapillary glomerulonephritis (PEGN) is a common cause of rapidly progressive glomerulonephritis and it is usually associated with circulating anti-neutrophil cytoplasmic antibodies (ANCAs). Recent evidence points to complement activation as an important factor in the pathogenesis of PEGN. The aim of the present study was to assess the value of C3 deposits in the prognosis of PEGN. All patients diagnosed of PEGN from 1995 to 2015 (n=72) were included in this study. Progression of renal disease in patients with positive staining for C3 by immunofluorescence was compared with those with negative staining. Mean follow up was 73 months. Progression to end-stage renal disease in relation to clinical and histological variables was analyzed. Positive staining for C3 was observed in 22 out of the 72 patients (30.5%). At the time of diagnosis, patients with C3 deposits had higher serum creatinine concentration than those without C3 staining (5.00 vs. 3.85mg/dl, P=0.050). Renal survival at 10 years was 36.9% in patients with positive C3 staining vs. 64.4% in patients with negative staining (P=0.005). Mortality at 10 years was higher in patients with C3 deposits than in patients without deposits (77 vs. 49.3%). Thus, our study shows that PEGN with deposits of C3 is associated with worse renal prognosis and greater mortality. These results would support the hypothesis that activation of the alternative pathway complement may play an important role in the generation of renal injury associated with PEGN. Copyright © 2017 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

  5. Poor renal outcome of antineutrophil cytoplasmic antibody negative Pauci-immune glomerulonephritis in Taiwanese.

    Science.gov (United States)

    Hung, Peir-Haur; Chiu, Yen-Ling; Lin, Wei-Chou; Chiang, Wen-Chih; Chen, Yung-Ming; Lin, Shuei-Liong; Wu, Kwan-Dun; Tsai, Tun-Jun

    2006-10-01

    Pauci-immune glomerulonephritis (GN) is an important cause of crescentic GN, acute renal failure and mortality. However, data are limited on the clinical presentation and outcome in antineutrophil cytoplasmic antibody (ANCA) negative patients, especially in Asians. This retrospective study analyzed medical records and pathology slides of patients who received renal biopsy between February 1998 and October 2004. Enzyme-linked immunosorbent assay was used routinely for ANCA testing in all patients. Among 637 patients with biopsy-proven GN included in this study, 88 (13.8%) had glomerular crescent formation. Among them, pauci-immune crescentic glomerulonephritis (PICGN) (42 patients, 47.2%) and lupus nephritis (25 patients, 28.4%) were the most common pathologic diagnoses. Lupus patients were younger (p = 0.028), while PICGN patients had more chronic lesions (p < 0.001), extensive glomerular crescents (p < 0.001), less severe proteinuria (p < 0.001) and poorer renal survival (p = 0.0017). Among the PICGN patients, 62.5% had a positive ANCA test, 80% had myeloperoxidase-ANCA and 20% had proteinase-3-ANCA. Subgroup analysis showed that ANCA negativity was associated with less crescent formation (p < 0.001) but more chronic glomerular lesions (p < 0.001) and a trend toward worse renal outcome (p = 0.055). This study illustrates the necessity for pathologic diagnosis of pauci-immune GN despite ANCA negativity. The poor prognosis associated with ANCA negativity in this study may be partly due to delayed diagnosis since these patients frequently lacked systemic involvement.

  6. Ultrastructural immunocytochemical localization of chondroitin sulfate proteoglycan in Bruch's membrane of the rat

    DEFF Research Database (Denmark)

    Lin, W L; Essner, E; McCarthy, K J

    1992-01-01

    Two monoclonal antibodies (Mab 4D5 and 2D6) raised against the core protein of a basement membrane chondroitin sulfate proteoglycan from Reichert's membrane of the rat, were used for ultrastructural immunoperoxidase localization of this protein in Bruch's membrane of the rat. Immunoreactivity for...

  7. Value of /sup 113m/In-DTPA computer scintigraphy of the kidneys before and after heparin treatment in chronic glomerulonephritis

    International Nuclear Information System (INIS)

    Alekse, R.; Brezinskis, G.; Lazovskis, I.

    1986-01-01

    In 70 patients with morphologically confirmed diagnosis of chronic glomerulonephritis functional computer scintigraphy of the kidneys was performed prior to and following treatment with heparin. Improvement in scintigraphic parameters following heparin therapy was apparent, as the reduction of time indices of radiopharmaceutical passage and the decrease of spastic excretory disturbances. A certain deterioration of scintigraphic parameters after heparin therapy was found in patients with fibroblastic and focal sclerotic glomerulonephritis with renal insufficiency. (author)

  8. Membranoproliferative glomerulonephritis in the setting of multicentric angiofollicular lymph node hyperplasia (Castleman's disease) complicated by Evan's syndrome.

    Science.gov (United States)

    Gakiopoulou, Hariklia; Korkolopoulou, Penelope; Paraskevakou, Helen; Marinaki, Smaragdi; Voulgarelis, Michael; Stofas, Anastasios; Lelouda, Maria; Lazaris, Andreas C; Boletis, John; Patsouris, Efstratios

    2010-06-01

    Systemic Castleman's disease is a lymphoproliferative disorder with various clinical presentations and incompletely understood aetiology. The authors report on a rare case of the plasma cell variant of Castleman's disease associated with autoimmune haemolytic anaemia and autoimmune thrombocytopenia (Evan's syndrome) and complicated by mixed nephrotic-nephritic syndrome and acute renal failure due to an underlying glomerulopathy with microscopic and immunofluorescence findings suggestive of membranoproliferative glomerulonephritis (MPGN) type I. Immunocomplexed glomerulonephritis is rare in Castleman's disease, while, to the best of our knowledge, constellation of all these autoimmune phenomena is reported for the first time suggesting that apart from the putative role of VEGF and IL-6 in the pathogenesis of the disease, a more generalised immunological disturbance occurs, probably through autoantibodies induced by active polyclonal B cells raised from Castleman's disease tumour.

  9. Anti-neutrophil cytoplasmic antibody negative crescentic paucimmune glomerulonephritis in a case of scleroderma with systemic lupus erythematosus overlap

    Directory of Open Access Journals (Sweden)

    Rohit Tewari

    2016-01-01

    Full Text Available Renal Involvement in scleroderma is a known problem and the manifestations are well described. Renal involvement in systemic lupus erythematosus (SLE is also well known. However, in scleroderma and SLE overlap syndrome, the renal findings may vary being a combination of features of immune complex mediated glomerulonephritis as well as thrombotic microangiopathy. We report a case in which the renal manifestation in such a situation was of a focal necrotising pauci-immune glomerulonephritis with crescents, anti-neutrophil cytoplasmic antibody negative. To the best of our knowledge, such manifestations have not been described before. Renal dysfunction in a normotensive setting in such a case should direct one towards evaluation for other causes and should prompt a kidney biopsy. This would be valuable in delineating the pathological process in the kidney and would help in guiding therapy.

  10. ANCA-associated pauci-immune glomerulonephritis in a patient with bacterial endocarditis: a challenging clinical dilemma

    OpenAIRE

    Cervi, Andrea; Kelly, Dylan; Alexopoulou, Iakovina; Khalidi, Nader

    2017-01-01

    Purpose: We report the case of a 59-year-old man with chronic hepatitis B and C infection presenting with acute kidney injury and enterococcus faecalis-infective endocarditis (IE). An elevated proteinase-3 (PR3)-ANCA and pauci-immune glomerulonephritis (GN) on renal biopsy were discovered, corresponding to ANCA-mediated GN. We conducted a literature review to assess the role of ANCA in IE and treatment implications. Methods: On systematic review of the literature, we found five previous cases...

  11. Urinary concentration of monocyte chemoattractant protein-1 in idiopathic glomerulonephritis: a long-term follow-up study.

    Science.gov (United States)

    Tofik, Rafid; Ohlsson, Sophie; Bakoush, Omran

    2014-01-01

    Monocyte chemoattractant protein-1 (MCP-1), which is up regulated in kidney diseases, is considered a marker of kidney inflammation. We examined the value of urine MCP-1 in predicting the outcome in idiopathic glomerulonephritis. Between 1993 and 2004, 165 patients (68 females) diagnosed with idiopathic proteinuric glomerulopathy and with serum creatinine 150 µmol/L at diagnosis were selected for the study. Urine concentrations of MCP-1 were analyzed by ELISA in early morning spot urine samples collected on the day of the diagnostic kidney biopsy. The patients were followed until 2009. The progression rate to end-stage kidney disease was calculated using Kaplan-Meier survival analysis. End-stage kidney disease (ESKD) was defined as the start of kidney replacement therapy during the study follow-up time. Patients with proliferative glomerulonephritis had significantly higher urinary MCP-1 excretion levels than those with non-proliferative glomerulonephritis (pexcretion group and 29.9% in the low MCP-1 excretion group. However, after adjustment for confounding variables such as glomerular filtration rate (GFR) and proteinuria, there was no significant association between urine MCP-1 concentration and progression to ESKD, (HR=1.75, 95% CI=0.64-4.75, p=0.27). Our findings indicate that progression to end-stage kidney disease in patients with idiopathic glomerulopathies is not associated with urine MCP-1 concentrations at the time of diagnosis.

  12. ANC A-Associated Glomerulonephritis: Relationship of main ANCA subtypes to renal outcome, age and sex of patients

    International Nuclear Information System (INIS)

    Rais-Jalali, G.; Khajehdehi, P.

    1999-01-01

    Antineutrophil cytoplasmic antibodies (ANCA) have been proven to be useful diagnostic tool in patients with systemic vasculitis with systemic vasculitis and glomerulonephritis. These antibodies exist in two types, a cytoplasmic pattern (cANCA) and a perineuclear pattern (pANCA). The effect of the main ANCA subtypes on renal outcome and its relationship to demographic findings and clinical features of patients with ANCA-associated glomerulonephritis has not been adequately studied. In this prospective study, we compared the clinical features at presentation and the renal outcome after 1 year of follow-up between two group of patients with cANCA (n=22) and pANCA (n=29) consecutively encountered over a one year period. At presentation, rapidly progressive glomerulonephritis (RPGN), and after 1 year of follow-up, end stage renal disease (ESRD) were seen more commonly in patients with pANCA than cases with cANCA (P=0.001 and P=0.04, respectively). Seropositivity for cANCA was more common in male and pANCA in female patients (P=0.05). Occurrence of the pulmonary-renal syndrome or extra-renal manifestations, such as sinusitis and skin rash, did not differ significantly among the two groups of patients with cANCA and pANCA. Patients with pANCA present more frequently with RPGN, leading to a poorer renal survival compared to cases with cANCA. RPGN and pANCA are more common in females. (author)

  13. Geoelectrical characterisation of basement aquifers: the case of Iberekodo, southwestern Nigeria

    Science.gov (United States)

    Aizebeokhai, Ahzegbobor P.; Oyeyemi, Kehinde D.

    2018-03-01

    Basement aquifers, which occur within the weathered and fractured zones of crystalline bedrocks, are important groundwater resources in tropical and subtropical regions. The development of basement aquifers is complex owing to their high spatial variability. Geophysical techniques are used to obtain information about the hydrologic characteristics of the weathered and fractured zones of the crystalline basement rocks, which relates to the occurrence of groundwater in the zones. The spatial distributions of these hydrologic characteristics are then used to map the spatial variability of the basement aquifers. Thus, knowledge of the spatial variability of basement aquifers is useful in siting wells and boreholes for optimal and perennial yield. Geoelectrical resistivity is one of the most widely used geophysical methods for assessing the spatial variability of the weathered and fractured zones in groundwater exploration efforts in basement complex terrains. The presented study focuses on combining vertical electrical sounding with two-dimensional (2D) geoelectrical resistivity imaging to characterise the weathered and fractured zones in a crystalline basement complex terrain in southwestern Nigeria. The basement aquifer was delineated, and the nature, extent and spatial variability of the delineated basement aquifer were assessed based on the spatial variability of the weathered and fractured zones. The study shows that a multiple-gradient array for 2D resistivity imaging is sensitive to vertical and near-surface stratigraphic features, which have hydrological implications. The integration of resistivity sounding with 2D geoelectrical resistivity imaging is efficient and enhances near-surface characterisation in basement complex terrain.

  14. A fall-out shelter or basement structure

    International Nuclear Information System (INIS)

    Blatchford, J.M.A.

    1982-01-01

    A strong structure of precast concrete for use as a fallout shelter, tank, basement structure, blast-proof garage is described. It consists of several upright, concrete wall elements, including L-shaped sections, and at least one concrete roof element. The concrete elements are preferably encased in, and may also be supported on, a concrete surround which is formed in situ and which may be secured to the elements by projecting links. The structure may be assembled as an underground or above-ground building. This invention provides a strong structure of quite large span which is relatively simple in construction and can be rapidly assembled. (U.K.)

  15. Air exchange rates and migration of VOCs in basements and residences.

    Science.gov (United States)

    Du, L; Batterman, S; Godwin, C; Rowe, Z; Chin, J-Y

    2015-12-01

    Basements can influence indoor air quality by affecting air exchange rates (AERs) and by the presence of emission sources of volatile organic compounds (VOCs) and other pollutants. We characterized VOC levels, AERs, and interzonal flows between basements and occupied spaces in 74 residences in Detroit, Michigan. Flows were measured using a steady-state multitracer system, and 7-day VOC measurements were collected using passive samplers in both living areas and basements. A walk-through survey/inspection was conducted in each residence. AERs in residences and basements averaged 0.51 and 1.52/h, respectively, and had strong and opposite seasonal trends, for example, AERs were highest in residences during the summer, and highest in basements during the winter. Airflows from basements to occupied spaces also varied seasonally. VOC concentration distributions were right-skewed, for example, 90th percentile benzene, toluene, naphthalene, and limonene concentrations were 4.0, 19.1, 20.3, and 51.0 μg/m(3), respectively; maximum concentrations were 54, 888, 1117, and 134 μg/m(3). Identified VOC sources in basements included solvents, household cleaners, air fresheners, smoking, and gasoline-powered equipment. The number and type of potential VOC sources found in basements are significant and problematic, and may warrant advisories regarding the storage and use of potentially strong VOCs sources in basements. Few IAQ studies have examined basements. A sizable volume of air can flow between the basement and living area, and AERs in these two zones can differ considerably. In many residences, the basement contains significant emission sources and contributes a large fraction of VOC concentrations found in the living area. Exposures can be lowered by removing VOC sources from the basement; other exposure management options, such as local ventilation or isolation, are unlikely to be practical. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  16. Changes in the clinical presentation of immunoglobulin A nephropathy: data from the Spanish Registry of Glomerulonephritis.

    Science.gov (United States)

    Gutiérrez, Eduardo; Praga, Manuel; Rivera, Francisco; Sevillano, Angel; Yuste, Claudia; Goicoechea, Marian; López-Gómez, Juan M

    2018-03-01

    Immunoglobulin A nephropathy (IgAN) is the most common glomerulonephritis in the world, but there is little epidemiological data about possible changes in its presentation over the years. Available information about the influence of age on the form of clinical presentation is also scarce. The aim of the study was to analyse all renal biopsies performed between 1994 and 2013 and recorded in the Spanish Registry of Glomerulonephritis with a histological diagnosis of IgAN. The study was divided into five 4-year periods (1994-97, 1998-2001, 2002-05, 2006-09 and 2010-13) and patients were divided into four age groups: ≤16, 17-44, 45-64 and ≥65 years. From 20.974 renal biopsies recorded, 2961 (14.1%) corresponded to IgAN. The prevalence of IgAN remained stable, but a significant increase in age [from 37.6 (SD 17.7) in 1994-97 to 44.9 (SD 16.8) years in 2010-13; P = 0.001] and worse renal function at presentation [from serum creatinine (SCr) 1.9 (SD 1.9) in 1994-97 to 2.3 (SD 2.1) mg/dL in 2010-13; P = 0.001] were observed over the years. Nephrotic-range proteinuria and acute kidney injury (AKI) as forms of presentation were significantly more common among patients ≥65 years (17.7% and 43.2%, respectively) as compared with the other age groups [≤16 (11.4% and 13.1%, respectively), 17-44 (13.1% and 13%, respectively) and 45-64 (12.1% and 21.3%, respectively)]. Blood pressure, SCr and proteinuria were also significantly higher at presentation among elderly patients. Although the prevalence of IgAN in Spain has remained stable over the years, patients are significantly older and present with significantly worse renal function in the last years. The incidence of nephrotic-range proteinuria (17.7%) and AKI (43.2%) as forms of presentation is remarkable among patients ≥65 years of age. © The Author 2017. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

  17. Meningitis granulomatosa, glomerulonefritis rápidamente progresiva y vasculitis Granulomatous meningitis, crescentic glomerulonephritis and vasculitis

    Directory of Open Access Journals (Sweden)

    Ana Ludueña

    2011-08-01

    Full Text Available El compromiso meníngeo es una manifestación infrecuente de la granulomatosis de Wegener. Puede manifestarse como cefalea con hiperproteinorraquia y engrosamiento de la duramadre con aspecto granulomatoso, que se observa en la resonancia magnética. Presentamos un varón de 57 años con granulomatosis de Wegener que debutó con compromiso de vías aéreas superiores, oídos, órbitas y meningitis granulomatosa asintomática y que posteriormente evolucionó con mononeuritis múltiple y glomerulonefritis crescéntica ANCA positiva. La presencia de ANCA y el compromiso sistémico (vías aéreas superiores, oído, órbitas, nervios periféricos, duramadre y glomerulonefritis rápidamente progresiva permitieron en este caso llegar a un diagnóstico de certeza e iniciar el tratamiento inmunosupresor combinado (corticoides y ciclofosfamida. Evolucionó con remisión clínica y serológica (negativización de ANCA, pero persistiendo leve deterioro secuelar auditivo y de la función renal, sin recidiva de la enfermedad de base.Meningeal involvement is an infrequent manifestation of Wegener's granulomatosis. Clinical manifestations can be headache with high protein level in the cerebrospinal fluid and an enhanced MRI signal of granulomatous thickening of the duramater in the brain. We report a 57 year-old male with Wegener granulomatosis with onset manifestations of asymptomatic granulomatous meningitis, upper respiratory tract, ears and orbits involvement. He progressively developed ANCA positive multiple mononeuritis and crescentic glomerulonephritis. The diagnostic confirmation of Wegener's granulomatosis based on a positive ANCA test and on the evidence of systemic disease (crescentic glomerulonephritis and involvement of the upper respiratory tract, ears, orbits, peripheral nerves and duramater allowed a prompt initiation of aggressive immunosuppressive treatment with systemic cyclophosphamide and high - dosis corticosteroids. The patient entered

  18. Wegener's granulomatosis: report of a case with rapidly progressive glomerulonephritis and diabetes mellitus.

    Science.gov (United States)

    Kakoi, H; Hiraide, F; Nishizawa, S; Inouye, T; Yoshizawa, N

    1986-01-01

    A patient with the classic form of Wegener's granulomatosis with severe dabetes mellitus and rapidly progressive glomerulonephritis is described. This 61-year-old male presented with epistaxis and nasal pain and obstruction. The nasal cavities were filled with crusts covering eroded mucosa. The diagnosis was made by biopsy of nasal and bronchial mucosa, and laboratory data. The epistaxis was stopped by 10 Gy irradiation over the nasal cavities. The patient had severe diabetes mellitus. His blood sugar was not controlled by diet and insulin injection. His general condition worsened rapidly as the growth of granuloma in the nose and lung. Accordingly, prednisolone therapy reinitiated to suppress the granuloma although it has a reverse effect on diabetes mellitus. Approximately one month after admission, he died of acute renal failure. Autopsy was carried out. Granulomatous lesions were noted in the nasal cavities, lungs and spleen. Many petechiae were found macroscopically over the cortex of the kidney. Hyalinization or sclerosis with crescent formation was found microscopically in estimated 85% of the glomeruli. Immunohistologic analysis of the renal tissue demonstrated an irregular linear pattern deposition of IgG, IgA and C3 and a granular pattern deposition of IgM and C1q.

  19. Membranoproliferative glomerulonephritis and acute renal failure in a patient with chronic lymphocytic leukemia: Response to obinutuzumab.

    Science.gov (United States)

    Jain, Punit; Kanagal-Shamanna, Rashmi; Wierda, William; Ferrajoli, Alessandra; Keating, Michael; Jain, Nitin

    2017-09-01

    Membranoproliferative glomerulonephritis (MPGN) is a common extramedullary renal presentation in chronic lymphocytic leukemia (CLL) and can present with either a frank renal failure or proteinuria. One of its etiologies has been attributed to a paraneoplastic, immune complex phenomenon occurring in CLL. Although there is no standard of care in such patients, use of anti-CD20 monoclonal antibodies like rituximab have been used before in such patients with variable responses. Obinutuzumab is a novel, type II, immunoglobulin-G1 monoclonal antibody with a higher efficacy than rituximab and has an established safely profile in patients with comorbidities and poor renal functions. There are no such reported cases of MPGN in CLL being treated with obinutuzumab. We used the standard doses of obinutuzumab in our elderly patient (78-year-old woman) with high-risk CLL due to an underlying TP53 mutation, along with a MPGN-related acute renal failure. The patient achieved complete remission after six cycles of obinutuzumab; however, she remained positive for minimal residual disease on flow cytometry. Her renal function improved completely, suggesting a complete response of her underlying MPGN. Obinutuzumab has an established safety profile in patients with CLL, but our case is the first reported case of a paraneoplastic, immune complex-mediated MPGN in CLL being treated with obinutuzumab. Obinutuzumab should be explored as a potential option in patients with CLL and MPGN. Copyright © 2016 King Faisal Specialist Hospital & Research Centre. Published by Elsevier B.V. All rights reserved.

  20. Rhino-orbitocerebral mucormycosis in a patient with idiopathic crescentic glomerulonephritis

    Directory of Open Access Journals (Sweden)

    Suzan Sanavi

    2013-01-01

    Full Text Available Mucormycosis, caused by mucorales, is an acute, rapidly progressive infection associated with high mortality. Rhino-orbitocerebral infection is the most common variant and is generally seen in association with immune deficiency syndromes. Prompt medical treatment of this infection and debridement decreases the mortality rate. We describe a 47-year-old man with crescentic glomerulonephritis on maintenance prednisolone therapy. He had earlier received steroid and cyclophosphamide pulse therapies. Renal functions improved following immunosuppressive treatment. In the third month of maintenance therapy, he presented to us with left-sided facial swelling and bloody nasal discharge. He had high blood sugar and acidic blood pH (ketoacidosis, probably due to steroid therapy. Magnetic resonance imaging of the head and sinuses showed inflammation and mass in the ethmoid sinus and nose with partial septal destruction, proptosis, global destruction of the left eye, brain infarction and carotid artery obliteration. Endoscopic biopsy of the sinuses revealed severe tissue necrosis. Samples of nasal discharge and biopsy tissue showed aseptate hyphae on light microscopy and culture, compatible with Rhizopus. The patient was treated with amphotericin B and multiple wound debridements along with ethmoidectomy and enucleation of the left eye. He was discharged in good general condition but with mild right hemiparesis. On follow-up examination at one year, there were no signs of fungal infection or renal dysfunction.

  1. Severe glomerulonephritis complicated by coagulopathy: treatment with anticoaguland and immunosuppresive drugs.

    Science.gov (United States)

    Robson, A M; Cole, B R; Kienstra, R A; Kissane, J M; Alkjaersig, N; Fletcher, A P

    1977-06-01

    Serial determinations, using plasma fibrinogen gel chromatography as well as standard methodology, demonstrated that six children with severe glomerulonephritis, characterized on renal biopsy by glomerular necrosis and crescent formation, had persistent evidence of intravascular coagulation. Based on these observations, therapy with anticoagulants and azathicoagulants and azathioprine was instituted for one year; treatment with anticoagulants was continued for a second year. Anticoagulant therapy was initiated with heparin, followed by oral anticoagulation with phenindione and dipyridamole. In contrast to our earlier experience with similar patients, each of the present patients improved. Urinalyses returned to normal and glomerular filtration rates to near normal values in all patients at the end of the treatment period and have remained so for up to 3.9 years since treatment has been completed. Post-treatment biopsies showed remarkable improvement, with virtually no glomerulosclerosis even in patients who had had a high incidence of glomerular crescents before treatment. It is suggested that the therapeutic regimen favorably influenced the natural history of disease and that plasma fibrinogen chromatographic findings may be helpful in selecting patients likely to benefit from the use of anticoagulant therapy.

  2. Membranoproliferative glomerulonephritis with isolated C3 deposits: case report and literature review.

    Science.gov (United States)

    Darouich, Sihem; Goucha, Rym; Jaafoura, Mohamed Habib; Zekri, Semy; Kheder, Adel; Ben Maiz, Hédi

    2011-02-01

    Membranoproliferative glomerulonephritis with isolated C3 deposits (MPGNC3) is an uncommon condition characterized by overt glomerular C3 deposits in the absence of immunoglobulins and intramembranous dense deposits. Here the authors describe the clinical and morphological features of primary MPGNC3 in a 13-year-old boy and critically review the previously published cases. The patient presented with nephrotic syndrome and microscopic hematuria. Blood tests revealed very low circulating C3 levels. The renal biopsy exhibited subendothelial, subepithelial, and mesangial deposits, with C3 but not immunoglobulins seen on immunofluorescence. This case and the review of the literature indicate that the serum complement profile with decreased levels of C3 and normal levels of classical pathway components together with glomerular deposits containing exclusively complement C3 is highly suggestive of alternative pathway activation. The diagnosis of acquired and/or genetic complement abnormalities in some cases supports that complement dysregulation is implicated in the pathogenesis of MPGNC3. Such data show great promise to provide new therapy strategies based on modulation of the complement system activity.

  3. A Case of Thyroid Storm Associated with Cardiomyopathy and Poststreptococcal Glomerulonephritis

    Directory of Open Access Journals (Sweden)

    Lisa J. Underland

    2016-01-01

    Full Text Available Thyroid storm has a high mortality rate and is often associated with a precipitating factor such as intercurrent illness or infection. It is rare in pediatric patients. Cardiac disease in hyperthyroidism mostly manifests itself as tachycardia but more serious cardiac findings have also been described. A 5-year-old male with recent strep throat infection presented with dilated cardiomyopathy, hematuria, and symptoms and lab findings consistent with severe hyperthyroidism. He was diagnosed with thyroid storm secondary to concurrent Graves’ disease and poststreptococcal glomerulonephritis (PSGN. After starting the treatment with methimazole and a beta-blocker, his cardiac disease gradually improved and the PSGN resolved over time. There are no specific pediatric criteria for thyroid storm. Adult criteria can be difficult to apply to pediatric cases. Criteria for diagnosis of thyroid storm are less clear for pediatric patients. Dilated cardiomyopathy is a rare cardiac manifestation of hyperthyroidism. PSGN is due to glomerular immune complexes and can complicate group A strep infection. Providers should be aware of cardiac disease as a complication of hyperthyroidism. PSGN should not mechanistically be related to hyperthyroidism but can precipitate the signs of thyroid storm such as hypertension. This association has not been previously reported in the literature.

  4. Acute Glomerulonephritis in a Child with Chlamydia pneumoniae Infection: A Case Report

    Directory of Open Access Journals (Sweden)

    Giovanna Vitaliti

    2013-01-01

    Full Text Available Background. Infectious diseases seem to be an important and independent risk factor for renal failure, but the underlying mechanism of renal involvement during some kinds of infectious diseases is still unclear, even if the literature data report immunomediated and/or autoimmune mechanisms to explain the pathogenic relationship between the two diseases. In paediatric patients, Chlamydia pneumoniae is a rare cause of renal complications and it may manifest in several ways, mainly involving the respiratory system, even if also renal and glomerulalr complications, have been described. Case Diagnosis/Treatment. Herein we report a case of a 3-year-old child who developed an acute glomerulonephritis that was chronologically, clinically, and biologically related to a previous Chlamydia pneumoniae infection. On our knowledge, in the literature it is the youngest patient with renal involvement during course of Chlamydia pneumoniae infection ever reported. Conclusions. The present case supports the hypothesis of a rather close causal relationship between this infective agent and renal and glomerular symptoms occurred in this child, during an acute episode of respiratory disease.

  5. HENOCH SCHONLEIN PURPURA ASSOCIATED WITH ACUTE POSTSTREPTOCOCCAL GLOMERULONEPHRITIS: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Deborah Melati

    2015-05-01

    Full Text Available Acute post-streptococcal glomerulonephritis (APSGN is one of the most common renal disease resulting from a prior infection with group A â-hemolytic streptococcus (GAS. Henoch Schonlein Purpura (HSP is a systemic disease with frequent renal involvement, its etiology is still unknown but several infections have been described as trigger includingGAS infection. A 4 year 10 month old Balinese boy presented with full blown acute nephritic syndrome, an elevation in serum creatinine and four fold increase of anti streptolysin-O, also low serum levels of complement C3 with normal C4 confirmed the diagnosis of APSGN. During hospitalization he developed palpable purpura, gastrointestinal symptoms as well as leucytoclastic vasculitis in skin biopsy conclude HSP diagnosis.He was treated with anti-hypertensions and metylprednisolone intravenous. The prognosis of the patient was excellent, he showed normal physical examination with normal complete blood count and urinalysis after 3 months follow up. We conclude that both APSGN and HSP could appear concurrently after GAS infection. [MEDICINA 2014;45:102-7]  

  6. Membranoproliferative glomerulonephritis. A prospective clinical trial of platelet-inhibitor therapy

    International Nuclear Information System (INIS)

    Donadio, J.V. Jr.; Anderson, C.F.; Mitchell, J.C.; Holley, K.E.; Ilstrup, D.M.; Fuster, V.; Chesebro, J.H.

    1984-01-01

    Forty patients with Type I membranoproliferative glomerulonephritis were treated for one year with dipyridamole, 225 mg per day, and aspirin, 975 mg per day, in a prospective, randomized, double-blind, placebo-controlled study. At the base line, the half-life of 51 Cr-labeled platelets was reduced in 12 of 17 patients. The platelet half-life became longer and renal function stabilized in the treated group, as compared with the placebo group, suggesting a relation between platelet consumption and the glomerulopathy. The glomerular filtration rate, determined by iothalamate clearance, was better maintained in the treated group (average decrease, 1.3 ml per minute per 1.73 m 2 of body-surface area per 12 months) than in the placebo group (average decrease, 19.6). Fewer patients in the treated group than in the placebo group had progression to end-stage renal disease (3 of 21 after 62 months as compared with 9 of 19 after 33 months). The data suggest that dipyridamole and aspirin slowed the deterioration of renal function and the development of end-stage renal disease

  7. Kidney Transplant Recipients With Primary Membranous Glomerulonephritis Have a Higher Risk of Acute Rejection Compared With Other Primary Glomerulonephritides

    Directory of Open Access Journals (Sweden)

    Tripti Singh, MD

    2017-11-01

    Conclusions. Patients with MN have higher incidence of acute rejection after kidney transplant but have similar 10-year allograft survival in comparison to the other glomerular diseases like IgAN, FSGS, and LN.

  8. Tectonic and neotectonic implications of a new basement map of the Lower Tagus Valley, Portugal

    OpenAIRE

    Carvalho, João P. G.; Rabeh, Taha; Dias, Rui; Dias, Ruben P.; Pinto, Carlos C.; Oliveira, José Tomás; Cunha, Teresa Arriaga; Borges, José Fernando

    2014-01-01

    In this paper we present a new basement (defined here as Paleozoic, Precambrian and Mesozoic igneous rocks) map of the Lower Tagus Valley area. This map is a contribution to the understanding of the structural evolution of the top of the basement in the Lower Tagus Valley area during the Mesozoic and Cenozoic Eras. The map was produced using aeromagnetic, well, seismic reflection and geological outcrop data. It shows unprecedented details of the geometry of the basement rock's sur...

  9. Rapidly progressive antineutrophil cytoplasm antibodies associated with pulmonary-renal syndrome in a 10-year-old girl

    OpenAIRE

    Blanco Filho,Fermin; Ernesto,Luci Carla; Rosa,Mônica Assis; Stuginski,Luis Antônio; Zlochevsky,Eliana Regina; Blanco,Fernando

    2001-01-01

    CONTEXT: The term pulmonary-renal syndrome has been used frequently to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. The classic example of this type of vasculitis is Goodpasture´s syndrome, a term used to describe the association of pulmonary hemorrhage, glomerulonephritis and the presence of circulating antiglomerular basement membrane antibodies (anti-GBM). Among the several types of systemic vasculitides th...

  10. Structural analysis of a fractured basement reservoir, central Yemen

    Science.gov (United States)

    Veeningen, Resi; Rice, Hugh; Schneider, Dave; Grasemann, Bernhard; Decker, Kurt

    2013-04-01

    The Pan-African Arabian-Nubian Shield (ANS), within which Yemen lies, formed as a result of Neoproterozoic collisional events between c. 870-550 Ma. Several subsequent phases of extension occurred, from the Mesozoic (due to the breakup of Gondwana) to the Recent (forming the Gulf of Aden and the Red Sea). These resulted in the formation of numerous horst- and-graben structures and the development of fractured basement reservoirs in the southeast part of the ANS. Two drill cores from the Mesozoic Marib-Shabwa Basin, central Yemen, penetrated the upper part of the Pan-African basement. The cores show both a lithological and structural inhomogeneity, with variations in extension-related deformation structures such as dilatational breccias, open fractures and closed veins. At least three deformation events have been recognized: D1) Ductile to brittle NW-SE directed faulting during cooling of a granitic pluton. U-Pb zircon ages revealed an upper age limit for granite emplacement at 627±3.5 Ma. As these structures show evidence for ductile deformation, this event must have occurred during the Ediacaran, shortly after intrusion, since Rb/Sr and (U-Th)/He analyses show that subsequent re-heating of the basement did not take place. D2) The development of shallow dipping, NNE-SSW striking extensional faults that formed during the Upper Jurassic, simultaneously with the formation of the Marib-Shabwa Basin. These fractures are regularly cross-cut by D3. D3) Steeply dipping NNE-SSW to ENE-WSW veins that are consistent with the orientation of the opening of the Gulf of Aden. These faults are the youngest structures recognized. The formation of ductile to brittle faults in the granite (D1) resulted in a hydrothermally altered zone ca. 30 cm wide replacing (mainly) plagioclase with predominantly chlorite, as well as kaolinite and heavy element minerals such as pyrite. The alteration- induced porosity has an average value of 20%, indicating that the altered zone is potentially a

  11. Rac1 is essential for basement membrane-dependent epiblast survival

    DEFF Research Database (Denmark)

    He, Xiaowen; Liu, Jie; Qi, Yanmei

    2010-01-01

    biological process are largely unknown. Here we demonstrate that Rac1 ablation in embryonic stem cell-derived embryoid bodies (EBs) leads to massive apoptosis of epiblast cells in contact with the BM. Expression of wild-type Rac1 in the mutant EBs rescues the BM-contacting epiblast, while expression...... of a constitutively active Rac1 additionally blocks the apoptosis of inner cells and cavitation, indicating that the spatially regulated activation of Rac1 is required for epithelial cyst formation. We further show that Rac1 is activated through integrin-mediated recruitment of the Crk-DOCK180 complex and mediates BM...

  12. Structural analysis of how podocytes detach from the glomerular basement membrane under hypertrophic stress

    Directory of Open Access Journals (Sweden)

    Wilhelm eKriz

    2014-12-01

    Full Text Available Podocytes are lost by detachment from the GBM as viable cells; details are largely unknown. We studied this process in the rat after growth stimulation with FGF-2. Endothelial and mesangial cells responded by hyperplasia, podocytes underwent hypertrophy, but, in the long run, developed various changes that could either be interpreted showing progressing stages in detachment from the GBM or stages leading to a tighter attachment by foot process effacement (FPE. This occurred in microdomains within the same podocyte; thus features of detachment and of reinforced attachment may simultaneously be found in the same podocyte.(1 Initially, hypertrophied podocytes underwent cell body attenuation and formed large pseudocysts, i.e. expansions of the subpodocyte space.(2 Podocytes entered the process of FPE starting with the retraction of foot processes and the replacement of the slit diaphragm by occluding junctions thereby sealing the filtration slits. Successful completion of this process led to broad attachments of podocyte cell bodies to the GBM. (3 Failure of sealing the slits led to gaps of varying width between retracting foot processes facilitating the outflow of the filtrate from the GBM.(4 Since those gaps are frequently overarched by broadened primary processes the drainage of the filtrate into the Bowman's space may be hindered leading to the formation of small pseudocysts associated with bare areas of GBM.(5 The merging of pseudocysts created a system of communicating chambers through which the filtrate has to pass to reach Bowman's space. Multiple flow resistances in series likely generated an expansile force on podocytes contributing to detachment.(6 Such a situation appears to proceed to complete disconnection generally of a group of podocytes owing to the junctional connections between them. (7 Since such groups of detaching podocytes generally make contact to parietal cells, they start the formation of tuft adhesions to Bowman's capsule.

  13. Histopathological and ultrastructural analysis of vestibular endorgans in Meniere's disease reveals basement membrane pathology

    Directory of Open Access Journals (Sweden)

    McCall Andrew A

    2009-06-01

    Full Text Available Abstract Background We report the systematic analysis of the ultrastructural and cytological histopathology of vestibular endorgans acquired from labyrinthectomy in Meniere's disease. Methods 17 subjects with intractable Meniere's disease and ipsilateral non-serviceable hearing presenting to the Neurotology Clinic from 1997 to 2006 who chose ablative labyrinthectomy (average age = 62 years; range 29–83 years participated. The average duration of symptoms prior to surgery was 7 years (range 1–20 years. Results Nearly all vestibular endorgans demonstrated varying degrees of degeneration. A monolayer of epithelial cells occurred significantly more frequently in the horizontal cristae (12/13 = 92% (p Conclusion Systematic histopathological analysis of the vestibular endorgans from Meniere's disease demonstrated neuroepithelial degeneration which was highly correlated with an associated BM thickening. Other findings included hair cell and supporting cell microvessicles, increased intercellular clear spaces in the stroma, and endothelial cell vacuolization and stromal perivascular BM thickening.

  14. In vivo imaging of basement membrane movement: ECM patterning shapes Hydra polyps.

    Science.gov (United States)

    Aufschnaiter, Roland; Zamir, Evan A; Little, Charles D; Özbek, Suat; Münder, Sandra; David, Charles N; Li, Li; Sarras, Michael P; Zhang, Xiaoming

    2011-12-01

    Growth and morphogenesis during embryonic development, asexual reproduction and regeneration require extensive remodeling of the extracellular matrix (ECM). We used the simple metazoan Hydra to examine the fate of ECM during tissue morphogenesis and asexual budding. In growing Hydra, epithelial cells constantly move towards the extremities of the animal and into outgrowing buds. It is not known, whether these tissue movements involve epithelial migration relative to the underlying matrix or whether cells and ECM are displaced as a composite structure. Furthermore, it is unclear, how the ECM is remodeled to adapt to the shape of developing buds and tentacles. To address these questions, we used a new in vivo labeling technique for Hydra collagen-1 and laminin, and tracked the fate of ECM in all body regions of the animal. Our results reveal that Hydra 'tissue movements' are largely displacements of epithelial cells together with associated ECM. By contrast, during the evagination of buds and tentacles, extensive movement of epithelial cells relative to the matrix is observed, together with local ECM remodeling. These findings provide new insights into the nature of growth and morphogenesis in epithelial tissues.

  15. FIBROBLAST GROWTH FACTOR-2 DURING POSTNATAL DEVELOPMENT OF THE TRACHEAL BASEMENT MEMBRANE ZONE. (R827442)

    Science.gov (United States)

    The perspectives, information and conclusions conveyed in research project abstracts, progress reports, final reports, journal abstracts and journal publications convey the viewpoints of the principal investigator and may not represent the views and policies of ORD and EPA. Concl...

  16. Matrigel Basement Membrane Matrix influences expression of microRNAs in cancer cell lines

    International Nuclear Information System (INIS)

    Price, Karina J.; Tsykin, Anna; Giles, Keith M.; Sladic, Rosemary T.; Epis, Michael R.; Ganss, Ruth; Goodall, Gregory J.; Leedman, Peter J.

    2012-01-01

    Highlights: ► Matrigel alters cancer cell line miRNA expression relative to culture on plastic. ► Many identified Matrigel-regulated miRNAs are implicated in cancer. ► miR-1290, -210, -32 and -29b represent a Matrigel-induced miRNA signature. ► miR-32 down-regulates Integrin alpha 5 (ITGA5) mRNA. -- Abstract: Matrigel is a medium rich in extracellular matrix (ECM) components used for three-dimensional cell culture and is known to alter cellular phenotypes and gene expression. microRNAs (miRNAs) are small, non-coding RNAs that regulate gene expression and have roles in cancer. While miRNA profiles of numerous cell lines cultured on plastic have been reported, the influence of Matrigel-based culture on cancer cell miRNA expression is largely unknown. This study investigated the influence of Matrigel on the expression of miRNAs that might facilitate ECM-associated cancer cell growth. We performed miRNA profiling by microarray using two colon cancer cell lines (SW480 and SW620), identifying significant differential expression of miRNAs between cells cultured in Matrigel and on plastic. Many of these miRNAs have previously been implicated in cancer-related processes. A common Matrigel-induced miRNA signature comprised of up-regulated miR-1290 and miR-210 and down-regulated miR-29b and miR-32 was identified using RT-qPCR across five epithelial cancer cell lines (SW480, SW620, HT-29, A549 and MDA-MB-231). Experimental modulation of these miRNAs altered expression of their known target mRNAs involved in cell adhesion, proliferation and invasion, in colon cancer cell lines. Furthermore, ITGA5 was identified as a novel putative target of miR-32 that may facilitate cancer cell interactions with the ECM. We propose that culture of cancer cell lines in Matrigel more accurately recapitulates miRNA expression and function in cancer than culture on plastic and thus is a valuable approach to the in vitro study of miRNAs.

  17. FLUORESCENCE OVERLAY ANTIGEN MAPPING OF THE EPIDERMAL BASEMENT-MEMBRANE ZONE .1. GEOMETRIC ERRORS

    NARCIS (Netherlands)

    BRUINS, S; DEJONG, MCJM; HEERES, K; WILKINSON, MHF; JONKMAN, MF; VANDERMEER, JB

    To identify in tissue sections the relative positions of antigen distributions close to the resolving power of the microscope, we have developed the fluorescence overlay antigen mapping (FOAM) procedure. As this technique makes high demands on the geometric fidelity of the overlay image, it is

  18. FLUORESCENCE OVERLAY ANTIGEN MAPPING OF THE EPIDERMAL BASEMENT-MEMBRANE ZONE .2. COLOR FIDELITY

    NARCIS (Netherlands)

    BRUINS, S; DEJONG, MCJM; HEERES, K; WILKINSON, MHF; JONKMAN, MF; VANDERMEER, JB

    In this second report on the fluorescence overlay antigen mapping (FOAM) technique, we highlight some of the errors that may influence faithful color rendition of slide preparations using triple antigen immunofluorescence staining. Reliable interpretation of multicolor fluorescence images requires

  19. Dye Transport across the Retinal Basement Membrane of the Blowfly Calliphora erythrocephala

    NARCIS (Netherlands)

    Weyrauther, E.; Roebroek, J.G.H.; Stavenga, D.G.

    In the blowfly, Calliphora erythrocephala, transport of dye into or out of the retina, following injection into the eye or thorax, was investigated, mainly by microspectrophotometry and fluorimetry. After injection into the eye, Phenol Red, Trypan Blue, Lucifer Yellow and 9-amino-acridine were

  20. CORRELATIVE LIGHT AND IMMUNOHISTOLOGICAL STUDY OF THE BASEMENT MEMBRANE OF THE HUMAN SQUAMOUS CERVICAL CARCINOMA

    OpenAIRE

    Masato, YAMASAKI; Gaiko, UEDA; Masaki, INOUE; Yoshiaki, TANAKA; IKeiichi, KURACH; Department of Obstetrics and Gynecology, Osaha University Medical School; Department of Obstetrics and Gynecology, Osaha University Medical School; Department of Obstetrics and Gynecology, Osaha University Medical School; Department of Obstetrics and Gynecology, Osaha University Medical School; Department of Obstetrics and Gynecology, Osaha University Medical School

    1980-01-01

    子宮頚部扁平上皮癌に基底膜は存在するか否か光顕・電顕的に多くの研究がなされてきた.1968年Beutner et al.はbullous pemphigioid患者血清が扁平上皮に対する抗基底膜抗体を含むことが明らかにされた.今回,我々は子宮頚部の上皮内癌3例,微小浸潤癌1例,浸潤癌27例を用い,凍結切片を作製し,抗基底膜抗体を含むbullous pemphigioid患者血清を用い蛍光抗体法による基底膜の観察を行なった.更に同一症例について,PAS及びMucin染色を行なった.蛍光抗体法による基底膜の観察では上皮内癌3例及び正常上皮では全て連続した蛍光の線が認められた.浸潤癌27例中7例に陽性で,keratinizing type 1/3, large cell type 6/23, small cell type 1/1例に陽性所見を認めた.CPL分類ではC型3/9,P型5/16,L型0/2が陽性であった.間質細胞反応との関連では細胞浸潤の軽度のもの5/16例,中等度・高度のもの3/11例に陽性であった.PAS及びMucin染色との比較検討を行なうと,PAS陽性基底膜例では3...

  1. Formation evaluation of fractured basement, Cambay Basin, India

    International Nuclear Information System (INIS)

    Gupta, Saurabh Datta; Farooqui, M Y; Chatterjee, Rima

    2012-01-01

    Unconventional reservoirs such as fractured basalts, shale gas and tight sand are currently playing an important role in producing a significant amount of hydrocarbon. The Deccan Trap basaltic rocks form the basement of the Cambay Basin, India, and hold commercially producible hydrocarbon. In this study two wells drilled through fractured basalts are chosen for evaluating the lithology, porosity and oil saturation of the reservoir sections. Well logs, such as gamma ray, high resolution resistivity, litho density, compensated neutron and elemental capture spectroscopy, have been used in cross-plotting techniques for lithology and mineral identification. Formation micro imagery log data have been analysed to quantify the fractures and porosity in the fractured reservoirs for a well in the south Ahmedabad block of the Cambay Basin. The results of the analysis of two wells are presented and discussed and they are found to be in good agreement with geological and production data. (paper)

  2. ENFORCEMENT OF FINANCIAL BASEMENTS AS A FACTOR OF TERRITORIES DEVELOPMENT

    Directory of Open Access Journals (Sweden)

    E.N. Sidorova

    2007-06-01

    Full Text Available Article contains description of structure of regional finance resources, discloses the sources of financing, describes the role of budgeting. Problems and possible ways of solution of inter-budget relationships optimisation are described with the purpose of increasing of financial prosperity of territories. Overall role of optimisation as one of the most important factors of strengthening of financial basement of territories is described along with the necessity of considering the budget process as stimulated factor for regional economic systems development. Suggestions on substitution of cost method of budget resources management by the model of outcomes management and further development of mechanisms of territorial bodies interaction with economic entities on the base of state-private partnership were proposed.

  3. Age of Izu-Bonin-Mariana arc basement

    Science.gov (United States)

    Ishizuka, Osamu; Hickey-Vargas, Rosemary; Arculus, Richard J.; Yogodzinski, Gene M.; Savov, Ivan P.; Kusano, Yuki; McCarthy, Anders; Brandl, Philipp A.; Sudo, Masafumi

    2018-01-01

    Documenting the early tectonic and magmatic evolution of the Izu-Bonin-Mariana (IBM) arc system in the Western Pacific is critical for understanding the process and cause of subduction initiation along the current convergent margin between the Pacific and Philippine Sea plates. Forearc igneous sections provide firm evidence for seafloor spreading at the time of subduction initiation (52 Ma) and production of "forearc basalt". Ocean floor drilling (International Ocean Discovery Program Expedition 351) recovered basement-forming, low-Ti tholeiitic basalt crust formed shortly after subduction initiation but distal from the convergent margin (nominally reararc) of the future IBM arc (Amami Sankaku Basin: ASB). Radiometric dating of this basement gives an age range (49.3-46.8 Ma with a weighted average of 48.7 Ma) that overlaps that of basalt in the present-day IBM forearc, but up to 3.3 m.y. younger than the onset of forearc basalt activity. Similarity in age range and geochemical character between the reararc and forearc basalts implies that the ocean crust newly formed by seafloor spreading during subduction initiation extends from fore- to reararc of the present-day IBM arc. Given the age difference between the oldest forearc basalt and the ASB crust, asymmetric spreading caused by ridge migration might have taken place. This scenario for the formation of the ASB implies that the Mesozoic remnant arc terrane of the Daito Ridges comprised the overriding plate at subduction initiation. The juxtaposition of a relatively buoyant remnant arc terrane adjacent to an oceanic plate was more favourable for subduction initiation than would have been the case if both downgoing and overriding plates had been oceanic.

  4. Cocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis

    Directory of Open Access Journals (Sweden)

    F.V. Veronese

    2016-01-01

    Full Text Available Levamisole has been increasingly used as an adulterant of cocaine in recent years, emerging as a public health challenge worldwide. Levamisole-associated toxicity manifests clinically as a systemic vasculitis, consisting of cutaneous, hematological, and renal lesions, among others. Purpura retiform, cutaneous necrosis, intravascular thrombosis, neutropenia, and less commonly crescentic nephritis have been described in association with anti-neutrophil cytoplasmic antibodies (ANCAs and other autoantibodies. Here we report the case of a 49-year-old male who was a chronic cocaine user, and who presented spontaneous weight loss, arthralgia, and 3 weeks before admission purpuric skin lesions in the earlobes and in the anterior thighs. His laboratory tests on admission showed serum creatinine of 4.56 mg/dL, white blood count 3,800/μL, hemoglobin 7.3 g/dL, urinalysis with 51 white blood cells/μL and 960 red blood cells/μL, and urine protein-to-creatinine ratio 1.20. Serum ANCA testing was positive (>1:320, as well as serum anti-myeloperoxidase and anti-proteinase 3 antibodies. Urine toxicology screen was positive for cocaine and levamisole, with 62.8% of cocaine, 32.2% of levamisole, and 5% of an unidentified substance. Skin and renal biopsies were diagnostic for leukocytoclastic vasculitis and pauci-immune crescentic glomerulonephritis, respectively. The patient showed a good clinical response to cocaine abstinence, and use of corticosteroids and intravenous cyclophosphamide. Last serum creatinine was 1.97 mg/dL, white blood cell count 7,420/μL, and hemoglobin level 10.8 g/dL. In levamisole-induced systemic vasculitis, the early institution of cocaine abstinence, concomitant with the use of immunosuppressive drugs in severe cases, may prevent permanent end organ damage and associate with better clinical outcomes.

  5. Acute glomerulonephritis in children: A seven-year experience in a tertiary health center

    Directory of Open Access Journals (Sweden)

    Ali Güneş

    2012-09-01

    Full Text Available Objectives: The aim of this study was to examine clinicaland laboratory characteristics of children with AGN in atertiary university hospital.Materials and methods: Hospital records of childrenwhich hospitalized with AGN diagnosis in Pediatric NephrologyUnit, between 2004 and 2011, were retrospectivelyreviewed. Patients were divided into threesubgroups as age 10 years.Characteristics at hospital admission including, bloodpressure, diuresis state, serum urea, creatinine and electrolytelevels, complement factors C3 and C4, completeblood count and urine analysis results were recorded.Data about primary infection, treatments, disease courseand outcome were recorded and analyzed.Results: Totally 220 children (157 male, 71.2%; 63 female,28.8% were included. Probable etiological infectionswere tonsillopharyngitis in 133 (60.5%, pyodermain 12 (5.5%, other infections in 27 (12.2% children. In48 (21.8 children with AGN, no primary infection weredetected. Edema was found in 81.4% of patients, hypertensionin 73.6%, and pulmonary stasis in 22.6%. Low C3levels were found in 89.1%, azotemia in 45.9%, hematuriain 95.4%, nephritic proteinuria in 80.5%, and leukocyturiain 37.6% of patients. Diuresis was enabled withinone week in 82%. Additional vasodilators were given to45 (20.5% patients, and hemodialysis was performedto three. Except for frequency of low C3 (p=0.018 andanemia (p=0.006, no significant differences were foundin clinical and laboratory findings between age groups(p>0.05.Conclusions: No difference was found in clinical andlaboratory features of AGN based on age groups, excludinglow C3 and anemia frequency. J Clin Exp Invest 2012;3 (3: 357-362Key words: Acute glomerulonephritis, childhood, clinicalfindings, laboratory findings, age grops

  6. Cocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis.

    Science.gov (United States)

    Veronese, F V; Dode, R S O; Friderichs, M; Thomé, G G; da Silva, D R; Schaefer, P G; Sebben, V C; Nicolella, A R; Barros, E J G

    2016-01-01

    Levamisole has been increasingly used as an adulterant of cocaine in recent years, emerging as a public health challenge worldwide. Levamisole-associated toxicity manifests clinically as a systemic vasculitis, consisting of cutaneous, hematological, and renal lesions, among others. Purpura retiform, cutaneous necrosis, intravascular thrombosis, neutropenia, and less commonly crescentic nephritis have been described in association with anti-neutrophil cytoplasmic antibodies (ANCAs) and other autoantibodies. Here we report the case of a 49-year-old male who was a chronic cocaine user, and who presented spontaneous weight loss, arthralgia, and 3 weeks before admission purpuric skin lesions in the earlobes and in the anterior thighs. His laboratory tests on admission showed serum creatinine of 4.56 mg/dL, white blood count 3,800/μL, hemoglobin 7.3 g/dL, urinalysis with 51 white blood cells/μL and 960 red blood cells/μL, and urine protein-to-creatinine ratio 1.20. Serum ANCA testing was positive (>1:320), as well as serum anti-myeloperoxidase and anti-proteinase 3 antibodies. Urine toxicology screen was positive for cocaine and levamisole, with 62.8% of cocaine, 32.2% of levamisole, and 5% of an unidentified substance. Skin and renal biopsies were diagnostic for leukocytoclastic vasculitis and pauci-immune crescentic glomerulonephritis, respectively. The patient showed a good clinical response to cocaine abstinence, and use of corticosteroids and intravenous cyclophosphamide. Last serum creatinine was 1.97 mg/dL, white blood cell count 7,420/μL, and hemoglobin level 10.8 g/dL. In levamisole-induced systemic vasculitis, the early institution of cocaine abstinence, concomitant with the use of immunosuppressive drugs in severe cases, may prevent permanent end organ damage and associate with better clinical outcomes.

  7. ABIN1 Determines Severity of Glomerulonephritis via Activation of Intrinsic Glomerular Inflammation.

    Science.gov (United States)

    Korte, Erik A; Caster, Dawn J; Barati, Michelle T; Tan, Min; Zheng, Shirong; Berthier, Celine C; Brosius, Frank C; Vieyra, Mark B; Sheehan, Ryan M; Kosiewicz, Michele; Wysoczynski, Marcin; Gaffney, Patrick M; Salant, David J; McLeish, Kenneth R; Powell, David W

    2017-12-01

    Transcription factor NF-κB regulates expression of numerous genes that control inflammation and is activated in glomerular cells in glomerulonephritis (GN). We previously identified genetic variants for a NF-κB regulatory, ubiquitin-binding protein ABIN1 as risk factors for GN in systemic autoimmunity. The goal was to define glomerular inflammatory events controlled by ABIN1 function in GN. Nephrotoxic serum nephritis was induced in wild-type (WT) and ubiquitin-binding deficient ABIN1[D485N] mice, and renal pathophysiology and glomerular inflammatory phenotypes were assessed. Proteinuria was also measured in ABIN1[D485N] mice transplanted with WT mouse bone marrow. Inflammatory activation of ABIN1[D472N] (D485N homolog) cultured human-derived podocytes, and interaction with primary human neutrophils were also assessed. Disruption of ABIN1 function exacerbated proteinuria, podocyte injury, glomerular NF-κB activity, glomerular expression of inflammatory mediators, and glomerular recruitment and retention of neutrophils in antibody-mediated nephritis. Transplantation of WT bone marrow did not prevent the increased proteinuria in ABIN1[D845N] mice. Tumor necrosis factor-stimulated enhanced expression and secretion of NF-κB-targeted proinflammatory mediators in ABIN1[D472N] cultured podocytes compared with WT cells. Supernatants from ABIN1[D472N] podocytes accelerated chemotaxis of human neutrophils, and ABIN1[D472N] podocytes displayed a greater susceptibility to injurious morphologic findings induced by neutrophil granule contents. These studies define a novel role for ABIN1 dysfunction and NF-κB in mediating GN through proinflammatory activation of podocytes. Copyright © 2017 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.

  8. Use of C4d as a diagnostic tool to classify membranoproliferative glomerulonephritis.

    Science.gov (United States)

    Gupta, Nirupama; Wakefield, Dara N; Clapp, William L; Garin, Eduardo H

    Membranoproliferative glomerulonephritis (MPGN type I, II and III) was reclassified in 2013 as MPGN and C3 glomerulopathy (C3G) based on the complement system activation mechanism. To evaluate whether C4d, a component of the classical pathway, could be a diagnostic tool in differentiating between MPGN and C3G. We conducted a retrospective study of 15 MPGN type I, II and III and 13 minimal change disease (MCD) patients diagnosed between 2000 and 2012. C4d staining using the peroxidase method was employed. Using the 2013 C3G consensus classification, the 15 MPGN types I, II and III biopsies were re-classified as MPGN (8) and C3G (7). Following C4d staining, of the 8 biopsies diagnosed as MPGN, 4 had classical pathway involvement [C1q (+), C3 (+), C4d (+)]; two had lectin pathway involvement [C1q (-), C3 (+), C4d (+)]; and, two were reclassified as C3G because the absence of C4d and C1q suggested the presence of the alternative pathway [C1q (-), C3 (+), C4d (-)]. Three of the seven C3G biopsies presented classical pathway involvement and were reclassified as MPGN. The alternative pathway was present in one of the other 4 biopsies considered to be C3G. Two C3G biopsies involved the lectin pathway and the one case of dense deposit disease had lectin pathway involvement. C4d staining may help to differentiate between MPGN and C3G. In addition, the lectin pathway could play a role in the pathogenesis of these glomerulopathies. Copyright © 2016 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

  9. Post-Infectious Glomerulonephritis in Pediatric Patients over Two Decades: Severity-Associated Features.

    Science.gov (United States)

    Dagan, Rona; Cleper, Roxana; Davidovits, Miriam; Sinai-Trieman, Levana; Krause, Irit

    2016-06-01

    The incidence of post-infectious glomerulonephritis (PIGN) has decreased over the last decades. As a result, recent epidemiological data from industrialized countries are scarce. To evaluate patterns of PIGN in children and detect possible predictors of disease severity. We collected clinical and laboratory data of patients with PIGN admitted to Schneider Children's Medical Center during 1994-2011. Diagnostic criteria included presence of hematuria with/without other features of nephritic syndrome along with hypocomplementemia and/or microbiological/serological evidence of streptococcal infection. Patients with other diseases (systemic lupus erythematosus, vasculitis, etc.) were excluded from the study. A total of 125 patients with a mean age of 5.8 ± 3.3 years (range 1.5-17.6), of whom 16% were nephritic syndrome was diagnosed in 51 patients (41.1%) and 28 (22.9%) had nephritic syndrome with nephrotic-range proteinuria. Depressed C3 complement levels were associated with the presence of nephritic syndrome (OR 0.73, 95% CI 0.60-0.88, P = 0.001) as well as older age (OR 1.24, Cl 1.08-1.43, P = 0.001). At last follow-up (mean 42 months) all examined patients (100 of 125) had normal renal function, 6 had hypertension, and 1 had proteinuria. PIGN remains an important cause of glomerular disease in children and may affect very young patients. Nephrotic-range proteinuria with hypoalbuminemia seems to be more frequent than previously reported. Hypocomplementemia is associated with a more severe disease course, namely, azotemia and nephritic syndrome.

  10. AUTOANTIBODIES TO THE LAMININ P1 FRAGMENT IN HGCL2-INDUCED MEMBRANOUS GLOMERULOPATHY

    NARCIS (Netherlands)

    ATEN, J; VENINGA, A; COERS, W; SONNENBERG, A; TIMPL, R; CLAESSEN, N; VANEENDENBURG, JDH; DEHEER, E; WEENING, JJ

    Exposure to mercuric chloride induces the development of a membranous glomerulopathy with high proteinuria in DZB rats, in which immunoglobulin (Ig)G1 and IgG2a bound in the glomeruli were previously found to react with laminin of the EHS tumor and several unidentified glomerular basement membrane

  11. Urinary concentration of monocyte chemoattractant protein-1 in idiopathic glomerulonephritis: a long-term follow-up study.

    Directory of Open Access Journals (Sweden)

    Rafid Tofik

    Full Text Available BACKGROUND: Monocyte chemoattractant protein-1 (MCP-1, which is up regulated in kidney diseases, is considered a marker of kidney inflammation. We examined the value of urine MCP-1 in predicting the outcome in idiopathic glomerulonephritis. METHODS: Between 1993 and 2004, 165 patients (68 females diagnosed with idiopathic proteinuric glomerulopathy and with serum creatinine <150 µmol/L at diagnosis were selected for the study. Urine concentrations of MCP-1 were analyzed by ELISA in early morning spot urine samples collected on the day of the diagnostic kidney biopsy. The patients were followed until 2009. The progression rate to end-stage kidney disease was calculated using Kaplan-Meier survival analysis. End-stage kidney disease (ESKD was defined as the start of kidney replacement therapy during the study follow-up time. RESULTS: Patients with proliferative glomerulonephritis had significantly higher urinary MCP-1 excretion levels than those with non-proliferative glomerulonephritis (p<0.001. The percentage of patients whose kidney function deteriorated significantly was 39.0% in the high MCP-1 excretion group and 29.9% in the low MCP-1 excretion group. However, after adjustment for confounding variables such as glomerular filtration rate (GFR and proteinuria, there was no significant association between urine MCP-1 concentration and progression to ESKD, (HR=1.75, 95% CI=0.64-4.75, p=0.27. CONCLUSION: Our findings indicate that progression to end-stage kidney disease in patients with idiopathic glomerulopathies is not associated with urine MCP-1 concentrations at the time of diagnosis.

  12. Fractional excretions of albumin and IgG are the best predictors of progression in primary glomerulonephritis.

    Science.gov (United States)

    McQuarrie, Emily P; Shakerdi, Loai; Jardine, Alan G; Fox, Jonathan G; Mackinnon, Bruce

    2011-05-01

    Proteinuria is the most sensitive predictor of development of progressive renal insufficiency, with increasing focus on the composition of proteinuria, particularly high molecular weight proteins such as immunoglobulin G (IgG) (molecular weight 150 kDa). Differing methods of assessing excretion of proteinuria molecules have limited interpretation of results. We aimed to assess the utility of available indices of IgG, total proteinuria and albumin excretions as predictors of chronic kidney disease (CKD) progression in patients with primary glomerulonephritis. We recruited 97 patients with primary glomerulonephritis and measured 24-h urinary protein excretion, 24-h urinary albumin excretion, selectivity index, albumin:creatinine ratio, urinary IgG:creatinine ratio, fractional excretion of albumin (FE Alb) and fractional excretion of IgG (FE IgG) at baseline. The composite endpoint was developing stage 5 CKD, requiring RRT or death. Receiver operating characteristics curve analysis was used to assess the value of each measure in predicting outcome. From this analysis, high- and low-risk patient groups according to each measure were established. These were then tested using Kaplan-Meier and Cox survival analysis. During a median follow-up of 7.07 years, 23 patients developed the primary endpoint. FE IgG and FE Alb were the most sensitive predictive tests. The hazard ratios (HR) of developing the primary endpoint using FE IgG [HR 37.1 (95% CI 8.6-158.8)] and FE Alb [HR 35.2 (95% CI 8.2-150.8)] cut-offs were double those using the other measures. FE IgG and FE Alb are superior to conventional measures of proteinuria in predicting outcome in patients with primary glomerulonephritis, possibly because they are more accurate indicators of impairment of glomerular permselectivity. FE Alb should be used, in conjunction with other measures of proteinuria, in future studies of prediction of CKD progression.

  13. Geology of the Pan-African basement Complex in Ube-Wulko area ...

    African Journals Online (AJOL)

    The Ube-Wulko area of southeast Akwanga falls within the Pan-African remobilized Basement Complex of northcentral Nigeria. It consists of intensely multi-deformed high grade polymetamorphic basement rocks, predominantly composed of migmatitic gneisses and schists and subordinate quartzites, marbles, and ...

  14. Basement radon entry and stack driven moisture infiltration reduced by active soil depressurization

    Science.gov (United States)

    C.R. Boardman; Samuel V. Glass

    2015-01-01

    This case study presents measurements of radon and moisture infiltration from soil gases into the basement of an unoccupied research house in Madison, Wisconsin, over two full years. The basement floor and exterior walls were constructed with preservative-treated lumber and plywood. In addition to continuous radon monitoring, measurements included building air...

  15. Basement Surface Faulting and Topography for Savannah River Site and Vicinity

    International Nuclear Information System (INIS)

    Cumbest, R.J.

    1998-01-01

    This report integrates the data from more than 60 basement borings and over 100 miles of seismic reflection profiling acquired on the Savannah River Site to map the topography of the basement (unweathered rock) surface and faulting recorded on this surface

  16. ANALYSIS OF THE RELATIONSHIP BETWEEN RENAL REMODELING AND VASCULAR ADHESION-1 PROTEIN (VAP-1 IN CHRONIC PRIMARY GLOMERULONEPHRITIS

    Directory of Open Access Journals (Sweden)

    H. Z. Gadaborsheva

    2017-01-01

    Full Text Available Goal. Determination of the relationship between the concentration of VAP-1 and the clinical and morphological parameters of the structural reorganization of the renal tissue.Materials and methods. The study included 80 patients with primary chronic glomerulonephritis in the period of exacerbation. The predominant morphological variants were IgA-nephropathy (42 patients and focal-segmental glomerulosclerosis (16 patients. All patients underwent general clinical examination, with the establishment of stages of CKD, and nephrobiopsy was performed. Based on the results of morphological analysis, the parameters of remodeling of renal tissue were taken into account. A blood sample was taken to study the concentration of Vascular Adhesion-1 protein (VAP-1. Nine months after the patients were admitted to the study and treated according to standard therapy, several indicators were re-recorded.Results. It was found that the age, duration of the disease, urea, proteinuria of a single and a daily portion of urine were inversely proportional to the level of VAP-1. Moreover, a signifi cant difference was found between the groups of patients ranked by median VAP-1, by prevalence in the group ofpatients with higher VAP-1 concentration of nephritic syndrome, IgA nephropathy, cases with mesangium expansion, mesangial and endothelial hypercellularity, endothelial swelling, IgA immune deposits In mesangium and capillary loops.Conclusion. As a result of the study, the prognostic signifi cance of VAP-1 concentration in patients with chronic glomerulonephritis was demonstrated. The formulas and the table of risk stratifi cation for the development of morphological reconstruction of kidney tissue are presented. It was found that the concentration of VAP-1 is statistically signifi cantly increased in the early stages of glomerulonephritis, with the predominance of infl ammatory and proliferative changes. At the stage of fi brous restructuring of renal

  17. CLINICAL SIGNIFICANCE OF SERUM AND URINARY HEAT SHOCK PROTEIN 70 LEVEL ASSESSMENT IN PATIENTS WITH CHRONIC GLOMERULONEPHRITIS

    Directory of Open Access Journals (Sweden)

    N. V. Chebotaryova

    2014-01-01

    Full Text Available The levels of urinary heat shock protein-70 and serum antibodies to the heat shock protein-70 were assessed in 79 patients with different course variants of the chronic glomerulonephritis. The indices studied were higher in patients with an active course of the disease (expressed urinary and nephritic syndromes, especially if associated with transient renal disturbances which reflected the degree of lesion and activation of the renal self-defence system. Decrease of the serum level of antibodies to the heat shock protein-70 was an unfavorable factor of the nephrotic syndrome persistence despite the immunosuppressive therapy.

  18. Three-Dimensional Inversion of Magnetotelluric Data for the Sediment–Basement Interface

    DEFF Research Database (Denmark)

    Cai, Hongzhu; Zhdanov, Michael

    2016-01-01

    and a resistive basement. Conventional inversions of MT data are aimed at determining the volumetric distribution of the conductivity within the inversion domain. The recovered distribution of the subsurface conductivity is typically diffusive, which makes it difficult to select the sediment-basement interface....... This letter develops a novel approach to 3-D MT inversion for the depth-to-basement estimate. The key to this approach is selection of the model parameterization, with the depth to basement being the major unknown parameter. In order to estimate the depth to the basement, the inversion algorithm recovers both...... the thickness and the conductivities of the sedimentary basin. The forward modeling is based on the integral equation approach. The inverse problem is solved using a regularized conjugate gradient method. The Fréchet derivative matrix is calculated based on quasi-Born approximation. The developed method...

  19. A case of lupus-like glomerulonephritis in an HIV patient with nephrotic range proteinuria, purpura, and elevated IgA level.

    Science.gov (United States)

    Yang, Jihyun; Seo, Min Young; Kim, Ki Tae; Lee, Jun Yong; Kim, Sun-Chul; Kim, Myung-Gyu; Jo, Sang-Kyung; Cho, Won-Yong; Kim, Hyoung-Kyu; Won, Nam Hee; Cha, Ran-Hui; Cho, Eunjung

    2014-01-01

    Human immunodeficiency virus (HIV) infection is growing medical concern worldwide. There are many types of glomerulonephritis which are associated with HIV infection. We report a case of a 53-year-old Korean man with an HIV infection, who was developed nephritic range proteinuria and purpura with elevated IgA level rasing a possibility of Henoch-Schölein Purpura (H-S purpura). However, renal biopsy showed "lupus-like feature" glomerulonephritis without clinical or serologic evidence of systemic lupus erythematosus. Although baseline renal function was maintained without further need for maintenance dialysis following anti-retroviral therapy (ART) and steroid, patient died from uncontrolled gastrointestinal bleeding.

  20. Membrane dynamics

    DEFF Research Database (Denmark)

    Bendix, Pól Martin

    2015-01-01

    Current topics include membrane-protein interactions with regard to membrane deformation or curvature sensing by BAR domains. Also, we study the dynamics of membrane tubes of both cells and simple model membrane tubes. Finally, we study membrane phase behavior which has important implications...

  1. Cooperation of ETV6/RUNX1 and BCL2 enhances immunoglobulin production and accelerates glomerulonephritis in transgenic mice

    Science.gov (United States)

    Bauer, Eva; Schlederer, Michaela; Scheicher, Ruth; Horvath, Jaqueline; Aigner, Petra; Schiefer, Ana-Iris; Kain, Renate; Regele, Heinz; Hoermann, Gregor; Steiner, Günter; Kenner, Lukas; Sexl, Veronika; Villunger, Andreas; Moriggl, Richard; Stoiber, Dagmar

    2016-01-01

    The t(12;21) translocation generating the ETV6/RUNX1 fusion gene represents the most frequent chromosomal rearrangement in childhood leukemia. Presence of ETV6/RUNX1 alone is usually not sufficient for leukemia onset, and additional genetic alterations have to occur in ETV6/RUNX1-positive cells to cause transformation. We have previously generated an ETV6/RUNX1 transgenic mouse model where the expression of the fusion gene is restricted to CD19-positive B cells. Since BCL2 family members have been proposed to play a role in leukemogenesis, we investigated combined effects of ETV6/RUNX1 with exogenous expression of the antiapoptotic protein BCL2 by crossing ETV6/RUNX1 transgenic animals with Vav-BCL2 transgenic mice. Strikingly, co-expression of ETV6/RUNX1 and BCL2 resulted in significantly shorter disease latency in mice, indicating oncogene cooperativity. This was associated with faster development of follicular B cell lymphoma and exacerbated immune complex glomerulonephritis. ETV6/RUNX1-BCL2 double transgenic animals displayed increased B cell numbers and immunoglobulin titers compared to Vav-BCL2 transgenic mice. This led to pronounced deposition of immune complexes in glomeruli followed by accelerated development of immune complex glomerulonephritis. Thus, our study reveals a previously unrecognized synergism between ETV6/RUNX1 and BCL2 impacting on malignant disease and autoimmunity. PMID:26919255

  2. Cooperation of ETV6/RUNX1 and BCL2 enhances immunoglobulin production and accelerates glomerulonephritis in transgenic mice.

    Science.gov (United States)

    Bauer, Eva; Schlederer, Michaela; Scheicher, Ruth; Horvath, Jaqueline; Aigner, Petra; Schiefer, Ana-Iris; Kain, Renate; Regele, Heinz; Hoermann, Gregor; Steiner, Günter; Kenner, Lukas; Sexl, Veronika; Villunger, Andreas; Moriggl, Richard; Stoiber, Dagmar

    2016-03-15

    The t(12;21) translocation generating the ETV6/RUNX1 fusion gene represents the most frequent chromosomal rearrangement in childhood leukemia. Presence of ETV6/RUNX1 alone is usually not sufficient for leukemia onset, and additional genetic alterations have to occur in ETV6/RUNX1-positive cells to cause transformation. We have previously generated an ETV6/RUNX1 transgenic mouse model where the expression of the fusion gene is restricted to CD19-positive B cells. Since BCL2 family members have been proposed to play a role in leukemogenesis, we investigated combined effects of ETV6/RUNX1 with exogenous expression of the antiapoptotic protein BCL2 by crossing ETV6/RUNX1 transgenic animals with Vav-BCL2 transgenic mice. Strikingly, co-expression of ETV6/RUNX1 and BCL2 resulted in significantly shorter disease latency in mice, indicating oncogene cooperativity. This was associated with faster development of follicular B cell lymphoma and exacerbated immune complex glomerulonephritis. ETV6/RUNX1-BCL2 double transgenic animals displayed increased B cell numbers and immunoglobulin titers compared to Vav-BCL2 transgenic mice. This led to pronounced deposition of immune complexes in glomeruli followed by accelerated development of immune complex glomerulonephritis. Thus, our study reveals a previously unrecognized synergism between ETV6/RUNX1 and BCL2 impacting on malignant disease and autoimmunity.

  3. Pregnancy and glomerulonephritis. A review Glomerulonefritis y embarazo. Revisión de tema

    Directory of Open Access Journals (Sweden)

    Luis Fernando Arias Restrepo

    2008-06-01

    pregestational stage, gestationand puerperium, and the indications for dialysis and renal biopsy. Materials and methods: Analysis of textual revisions published between 1980 and 1992, and the publications of Medline between 1993 and 2007 (Pregnancy and Glomerulonephritis. Results: Few randomized controlled studies were found. Most studies were cross-sectional and cases series, but they allow the assertion that glomerulonephritis adversely affects gestational outcomes and that pregnancy favors renal damage and the lupus flare. Conclusion: The incidence of exacerbation of glomerulonephritis is higher during pregnancy and the perinatal and maternal outcomes are affected, especially in the presence of preeclampsia or antiphospholipid syndrome.

  1. Membranous nephropathy

    Science.gov (United States)

    ... check for hepatitis B, hepatitis C, and syphilis Complement levels Cryoglobulin test Treatment The goal of treatment ... not as helpful for people with membranous nephropathy. Medicines used treat membranous nephropathy include: Angiotensin-converting enzyme ( ...

  2. Fault assessment for basement reservoir compartmentalization: Case study at Northeast Betara gas field, South Sumatra Basin

    Science.gov (United States)

    Risyad, M.; Suta, I. N.; Haris, A.

    2017-07-01

    Northeast Betara field is situated on the northern part of prolific South Sumatra Basin. It has produced gas from Lower Talang Akar Formation sandstone and over 90 wells have been drilled. A 3D seismic data was acquired in 2000 and reprocessed in 2012 to enhance the subsurface image. In 2013 an exploratory well NEB Base-1 was drilled and made gas and condensate discovery from the subsequent pre-tertiary basement which is confirmed as granite. The well proved fractured basement reservoir play on paleo high of the structure. The absence of full-diameter conventional core prompts well logs and seismic data analysis by using a workstation. Main methods for fracture prediction have been seismic attributes extraction and structural geology studies of basement provided by image logs on a few exploration wells. Ant tracking attribute is widely employed to image seismic event discontinuities due to extensive faults which generated the natural fractures. Delineations well NEB Base-2 was drilled on second paleo high and unfortunately, it did not find any gas indication from pre-tertiary basement target. Seismic structural interpretation and seismic attributes are conducted to image distribution of event discontinuities related to faults or fracture. We found that compartmentalization on basement involved old faults and both paleo high have undergone different structural history and stress character which resulted in separated fractures distribution.

  3. BASECALC - software for residential basement and slab-on-grade heat-loss analysis

    Energy Technology Data Exchange (ETDEWEB)

    Beausoleil-Morrison, I. [Natural Resources Canada, Ottawa, ON (Canada). Office of Energy Efficiency

    1999-11-01

    BASECALC models heat losses from residential building basements and slabs-on-grade by calculating heat loss based on thermal and physical properties of individual construction, insulation type and location, ground properties and weather. BASECALC provides bilingual software and numerical, graphical and parametric output. It can model above-grade and below-grade heat losses and include the effects of thermal bridging between the basement and the main-floor envelope. This is then validated against the National Research Council of Canada`s Mitalas method. The software can calculate insulation configurations with code implications and carry out batch processing for multiple runs. It includes libraries of common insulation and construction materials. BASECALC has the potential in other applications to develop more accurate basement algorithms for building simulation programs, establish building and energy code requirements for basement and slab-on-grade insulation, assess and demonstrate the performance of new products and novel insulation placements, and validate and calibrate basement heat-loss predictions from building simulation programs.

  4. The metamorphic basement of the Cordillera Frontal of Mendoza: New geochronologic and isotopic data

    International Nuclear Information System (INIS)

    Basei, Miguel; Ramos, Victor A.; Vujovich, Graciela I.; Poma, Stella

    1998-01-01

    The metamorphic rocks of the Cordillera Frontal exposed in the Cordon del Portillo, Mendoza were examined by Rb/Sr geochronology and Nd/Sm isotopic analysis. The Rb/Sr data defined a Devonian age for the last metamorphic episode, similar to the previous K/Ar and Ar/Ar ages obtained in this region and western Precordillera. The isotopic analysis identified three sets of model ages: 1.- The oldest corresponds to a set of meta sedimentary rocks with a model age of 1,400 to 1,700 Ma; 2.- A monzogranodiorite with a model age of 1,000 Ma; and 3.- Metabasites with model ages between 577 and 330 Ma. These rocks are interpreted as 1.- A typical Grenvillian derived basement; 2.- Late Paleozoic granitoids derived from a different Proterozoic basement; and 3.- Some Eopaleozoic metabasites tectonically inter fingered with the Grenvillian basement. These new data are coherent with the existence of a Laurentia derived terrane, Chilenia, that was separated by oceanic rocks from the basement of Pre cordillera during Eopaleozoic times. This last basement known as the Cuyania terrane, was also derived from Laurentia. (author)

  5. Characteristic aerial and ground radioactives of basement and sedimentary rocks in (Egypt): relations and natural cycles across geologic time

    International Nuclear Information System (INIS)

    Ammar, A.A.

    1998-01-01

    Each geologic unit, exposure, formation or rock group of the exposed precambrian I (igneous and metamorphic) basement complex (Upper proterozoic) and phanerozoic cover sediments in an area covering about 4500 km2 located in the central eastern desert of egypt, has been found to possess certain radioactivity characteristics and levels. Minimum values of radiation are generally associated with the first basement volcanics, maximum values are correlated are correlated with the third basement plutonites and phosphate formation (upper cretaceous), while intermedialte values are connected with the first basement sediments and upper eocene-quaternary fifth detrital-calcareous-evaporite sediments. Therefore, the radioactivities of various rock groups of the precambrian I basement complex - except the first basement sediments sediments- correlate progressively well with their chronology. Generally, the increase of radioactivity within this complex is connected with the transition into final stages of the magmatic evolution

  6. Dissolution of biogenic ooze over basement edifices in the equatorial Pacific with implications for hydrothermal ventilation of the oceanic crust

    Science.gov (United States)

    Bekins, B.A.; Spivack, A.J.; Davis, E.E.; Mayer, L.A.

    2007-01-01

    Recent observations indicate that curious closed depressions in carbonate sediments overlying basement edifices are widespread in the equatorial Pacific. A possible mechanism for their creation is dissolution by fluids exiting basement vents from off-axis hydrothermal flow. Quantitative analysis based on the retrograde solubility of calcium carbonate and cooling of basement fluids during ascent provides an estimate for the dissolution capacity of the venting fluids. Comparison of the dissolution capacity and fluid flux with typical equatorial Pacific carbonate mass accumulation rates shows that this mechanism is feasible. By maintaining sediment-free basement outcrops, the process may promote widespread circulation of relatively unaltered seawater in the basement in an area where average sediment thicknesses are 300-500 m. The enhanced ventilation can explain several previously puzzling observations in this region, including anomalously low heat flux, relatively unaltered seawater in the basement, and aerobic and nitrate-reducing microbial activity at the base of the sediments. ?? 2007 The Geological Society of America.

  7. A case of rheumatic fever with acute post-streptococcal glomerulonephritis and nephrotic syndrome caused by a cutaneous infection with beta-hemolytic streptococci

    Directory of Open Access Journals (Sweden)

    Carsten Sauer Mikkelsen

    2010-01-01

    Full Text Available A middle-aged patient of Greenlandic origin was referred for skin infection of the leg. An initial minor trauma of the skin of the distal right lower extremity was complicated by bullous erysipelas which cultured positive for group A β-hemolytic streptococci (GABHS. The clinical condition deteriorated and necrotizing fasciitis developed despite relevant surgical and antibiotic treatment. Approximately 3 weeks later, the patient developed arthralgia, impaired renal function with azotemia, hypertension and severe nephrotic syndrome with periorbital and peripheral edema. A kidney biopsy demonstrated endocapillary glomerulonephritis. Concomitantly, carditis with chest pain, moderately reduced left ventricular ejection fraction and mitral regurgitation were noted. The patient had no signs of pharyngitis in the whole period. The patient thus contracted poststreptococ glomerulonephritis and furthermore she fulfilled the criteria of acute rheumatic fever following a GABHS skin infection. We suggest a possible relation between a virulent GABHS clone causing NF and ARF.

  8. Inversion of gravity data in the Big Bear Lake Area to recover depth to basement using Cauchy-type integrals

    DEFF Research Database (Denmark)

    Cai, Hongzhu; Zhdanov, Michael

    2014-01-01

    One of the important applications of the gravity method is evaluation of the depth to the basement, which is characterized by a significant density contrast with the sedimental layeres. We have introduced recently a new method of modeling and inversion of potential field data generated by a density...... response caused by sediment-basement interface with variable density in depth. We have also developed the inversion of gravity data to recover the depth to basement given the density profile with depth....

  9. Henoch-Schönlein purpura in an older man presenting as rectal bleeding and IgA mesangioproliferative glomerulonephritis: a case report

    OpenAIRE

    Howarth Charles B; Jirajariyavej Teeranun; Cheungpasitporn Wisit; Rosen Raquel M

    2011-01-01

    Abstract Introduction Henoch-Schönlein purpura is the most common systemic vasculitis in children. Typical presentations are palpable purpura, abdominal pain, arthritis, and hematuria. This vasculitic syndrome can present as an uncommon cause of rectal bleeding in older patients. We report a case of an older man with Henoch-Schönlein purpura. He presented with rectal bleeding and acute kidney injury secondary to IgA mesangioproliferative glomerulonephritis. Case presentation A 75-year-old Pol...

  10. Membrane Biophysics

    CERN Document Server

    Ashrafuzzaman, Mohammad

    2013-01-01

    Physics, mathematics and chemistry all play a vital role in understanding the true nature and functioning of biological membranes, key elements of living processes. Besides simple spectroscopic observations and electrical measurements of membranes we address in this book the phenomena of coexistence and independent existence of different membrane components using various theoretical approaches. This treatment will be helpful for readers who want to understand biological processes by applying both simple observations and fundamental scientific analysis. It provides a deep understanding of the causes and effects of processes inside membranes, and will thus eventually open new doors for high-level pharmaceutical approaches towards fighting membrane- and cell-related diseases.

  11. Treatment of primary chronic glomerulonephritis with Rehmannia glutinosa acteosides in combination with the angiotensin receptor blocker irbesartan: a randomized controlled trial.

    Science.gov (United States)

    Qiu, HongYu; Fu, Ping; Fan, WenXing; Zuo, Chuan; Feng, Ping; Shi, Peng; Cao, Lina; Liu, Fang; Zhou, Li; Chen, Feng; Zhong, Hui; Gou, ZhongPing; Liang, YaPing; Shi, Mei

    2014-01-01

    This study aims to assess the efficacy and safety of Rehmannia glutinosa acteosides used in combination with the angiotensin receptor blocker irbesartan to treat primary chronic glomerulonephritis. A total of 479 patients diagnosed with primary chronic glomerulonephritis were recruited from outpatient clinics and were randomly assigned to the treatment group (Rehmannia glutinosa acteosides, two 200-mg capsules, bid; and irbesartan, one 150-mg tablet, qd) or the control group (irbesartan, one 150-mg tablet, qd). The primary outcome was 24-h urinary protein. Secondary outcome measures included blood pressure, estimated glomerular filtration rate, erythrocyturia, serum alanine aminotransferase, aspartate transaminase and electrolytes. After 8 weeks of treatment, the treatment group showed a mean reduction in 24-h proteinuria of 36.42% compared to baseline, which was significantly higher than the mean reduction from baseline of 27.97% in the control group (P = 0.0278).Adverse drug reactions occurred at a similarly low rate in the treatment group (0.4%) and control group (1.2%, P = 0.3724). In the treatment of chronic glomerulonephritis, the combination of Rehmannia glutinosa acteosides and irbesartan can reduce proteinuria more effectively than irbesartan alone. Copyright © 2013 John Wiley & Sons, Ltd.

  12. Fracture Analysis of basement rock: A case example of the Eastern Part of the Peninsular Malaysia

    International Nuclear Information System (INIS)

    Shamsuddin, A; Ghosh, D

    2015-01-01

    In general, reservoir rocks can be defined into carbonates, tight elastics and basement rocks. Basement rocks came to be highlighted as their characteristics are quite complicated and remained as a significant challenge in exploration and production area. Motivation of this research is to solve the problem in some area in the Malay Basin which consist fractured basement reservoirs. Thus, in order to increase understanding about their characteristic, a study was conducted in the Eastern part of the Peninsular Malaysia. The study includes the main rock types that resemble the offshore rocks and analysis on the factors that give some effect on fracture characteristic that influence fracture systems and fracture networks. This study will allow better fracture prediction which will be beneficial for future hydrocarbon prediction in this region

  13. Modeling radon entry into houses with basements: Model description and verification

    International Nuclear Information System (INIS)

    Revzan, K.L.; Fisk, W.J.; Gadgil, A.J.

    1991-01-01

    We model radon entry into basements using a previously developed three-dimensional steady-state finite difference model that has been modified in the following ways: first, cylindrical coordinates are used to take advantage of the symmetry of the problem in the horizontal plant; second, the configuration of the basement has been made more realistic by incorporating the concrete footer; third, a quadratic relationship between the pressure and flow in the L-shaped gap between slab, footer, and wall has been employed; fourth, the natural convection of the soil gas which follows from the heating of the basement in winter has been taken into account. The temperature field in the soil is determined from the equation of energy conservation, using the basement, surface, and deep-soil temperatures as boundary conditions. The pressure field is determined from Darcy's law and the equation of mass conservation (continuity), assuming that there is no flow across any boundary except the soil surface (atmospheric pressure) and the opening in the basement shell (fixed pressure). After the pressure and temperatures field have been obtained the velocity field is found from Darcy's law. Finally, the radon concentration field is found from the equation of mass-transport. The convective radon entry rate through the opening or openings is then calculated. In this paper we describe the modified model, compare the predicted radon entry rates with and without the consideration of thermal convection, and compare the predicted rates with determined from data from 7 houses in the Spokane River valley of Washington and Idaho. Although the predicted rate is much lower than the mean of the rates determined from measurements, errors in the measurement of soil permeability and variations in the permeability of the area immediately under the basement slab, which has a significant influence on the pressure field, can account for the range of entry rates inferred from the data. 25 refs., 8 figs

  14. Hydrogeologic controls on induced seismicity in crystalline basement rocks due to fluid injection into basal reservoirs.

    Science.gov (United States)

    Zhang, Yipeng; Person, Mark; Rupp, John; Ellett, Kevin; Celia, Michael A; Gable, Carl W; Bowen, Brenda; Evans, James; Bandilla, Karl; Mozley, Peter; Dewers, Thomas; Elliot, Thomas

    2013-01-01

    A series of Mb 3.8-5.5 induced seismic events in the midcontinent region, United States, resulted from injection of fluid either into a basal sedimentary reservoir with no underlying confining unit or directly into the underlying crystalline basement complex. The earthquakes probably occurred along faults that were likely critically stressed within the crystalline basement. These faults were located at a considerable distance (up to 10 km) from the injection wells and head increases at the hypocenters were likely relatively small (∼70-150 m). We present a suite of simulations that use a simple hydrogeologic-geomechanical model to assess what hydrogeologic conditions promote or deter induced seismic events within the crystalline basement across the midcontinent. The presence of a confining unit beneath the injection reservoir horizon had the single largest effect in preventing induced seismicity within the underlying crystalline basement. For a crystalline basement having a permeability of 2 × 10(-17)  m(2) and specific storage coefficient of 10(-7) /m, injection at a rate of 5455 m(3) /d into the basal aquifer with no underlying basal seal over 10 years resulted in probable brittle failure to depths of about 0.6 km below the injection reservoir. Including a permeable (kz  = 10(-13)  m(2) ) Precambrian normal fault, located 20 m from the injection well, increased the depth of the failure region below the reservoir to 3 km. For a large permeability contrast between a Precambrian thrust fault (10(-12)  m(2) ) and the surrounding crystalline basement (10(-18)  m(2) ), the failure region can extend laterally 10 km away from the injection well. © 2013, National Ground Water Association.

  15. PURIFIED IGG F C-BINDING PROTEINS FROM M22 GROUP A STREPTOCOCCUS ARE ABLE TO INDUCE EXPERIMENTAL GLOMERULONEPHRITIS

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    L. A. Burova

    2012-01-01

    Full Text Available Abstract. Pathogenesis of acute post-streptococcal glomerulonephritis (APSGN, a major complication of group A streptococcal (GAS throat or skin disease, remains unclear. Over years, various theories were based on distinct streptococcal extracellular factors, as well as immunological mimicry of streptococci for renal tissue antigens was considered. Previously we reported that a lot of clinical GAS isolates with proven nephritogenic ability show a non-immune binding of monomeric or aggregated IgG. Moreover, using a rabbit model of APSGN, we obtained evidence for important causative role of streptococcal IgG Fc-binding proteins (IgG FcBPs belonging to the M family surface proteins. I.e., rabbits injected by whole IgG FcBP-positive streptococci showed induction of renal glomerular changes, with deposition of IgG and complement C3, resembling the picture recorded in human APSGN. These typical renal changes were always preceded by development of circulating anti-IgG antibodies. Present study was performed in the same rabbit model. Both purified IgG FcBPs isolated from type M22 GAS were found to elicit glomerular degenerative damage of renal glomeruli comparable to those caused by whole bacteria, as well as induce anti-IgG antibodies, deposition of IgG and C3 complement and production of proinflammatory cytokines (IL-1β, TNFα, IL-6 by glomerular mesangial and endothelial cells. By contrast, rabbits injected with proteins A or G, IgG FcBPs of S. aureus and group G streptococci, respectively, exhibited only low levels of circulating anti-IgG and reversible glomerular changes. In these settings, we have not observed any features of membranousproliferative glomerulonephritis (GN resembling morphological traits of acute post-streptococcal GN in humans. These data correlated with results obtained after injection of intact Staphylococcus aureus (Cowan 1 strain or group G streptococci (G148 strain. Both microbial types are known to harbor IgG Fc

  16. Autosomal dominant polycystic kidney disease with diffuse proliferative glomerulonephritis - an unusual association: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Kapoor Vinay

    2010-04-01

    Full Text Available Abstract Introduction Autosomal dominant polycystic kidney disease is an inherited disorder that is characterized by the development and growth of cysts in the kidneys and other organs. Urinary protein excretion is usually less than 1 g/24 hours in autosomal dominant polycystic kidney disease, and an association of nephrotic syndrome with this condition is considered rare. There are only anecdotal case reports of autosomal dominant polycystic kidney disease associated with nephrotic syndrome, with focal segmental glomerulosclerosis being the most commonly reported histopathological diagnosis. Nephrotic-range proteinuria in the presence of autosomal dominant polycystic kidney disease, with or without an accompanying decline in renal function, should be investigated by open renal biopsy to exclude coexisting glomerular disease. To the best of our knowledge, this is the first case of autosomal dominant polycystic kidney disease with histologically proven diffuse proliferative glomerulonephritis presenting with nephrotic-range proteinuria. No other reports of this could be found in a global electronic search of the literature. Case presentation We report the case of a 35-year-old Indo-Aryan man with autosomal dominant polycystic kidney disease associated with nephrotic syndrome and a concomitant decline in his glomerular filtration rate. Open renal biopsy revealed diffuse proliferative glomerulonephritis. An accurate diagnosis enabled us to manage him conservatively with a successful outcome, without the use of corticosteroid which is the standard treatment and the drug most commonly used to treat nephrotic syndrome empirically. Conclusion Despite the reluctance of physicians to carry out a renal biopsy on patients with autosomal dominant polycystic kidney disease, our case supports the idea that renal biopsy is needed in patients with polycystic kidney disease with nephrotic-range proteinuria to make an accurate diagnosis. It also illustrates the

  17. Application of high resolution aeromagnetic data for basement topography mapping of Siluko and environs, southwestern Nigeria

    Science.gov (United States)

    Osinowo, Olawale O.; Akanji, Adesoji O.; Olayinka, Abel I.

    2014-11-01

    The discovery of hydrocarbon in commercial quantity in the Niger Delta, southern Nigeria, has since the early fifties shifted the attention of exploration/active geological studies from the Dahomey basin and the adjacent basement terrain in south-western Nigeria towards the south and this has left some gaps in information required for the discovery and exploitation of the economic potential of the region. This study mapped the Siluko transition zone in south-western Nigeria in terms of structures, geometry and basement topography with the object of providing requisite geological information that will engender interest in the exploration and exploitation of the numerous economic potentials of south-western part of Nigeria. Acquired high resolution aeromagnetic data were filtered, processed and enhanced, the resultant data were subjected to qualitative and quantitative magnetic interpretation, depth weighting analyses and modelling to generate the subsurface basement topography across the study area. The obtained results indicate regions of high and low magnetic anomalies with residual magnetic intensity values ranging from -100.8 nT to 100.9 nT. Euler Deconvolution indicates generally undulating basement topography with depth range of 125-1812 m. The basement relief is generally gentle and flat lying within the basement terrain with depth ranging from 125 to 500 m. However the sedimentary terrain is undulating and generally steeps south, down the basin with depth range of 300-1812 m. A basement topography model of the magnetic data constrained by Euler solutions correlate positively with the geology of the study area and indicates a generally increasing sedimentary deposits' thickness southward toward the western part of Dahomey basin. The revealed basement topography and structures as well as the delineated direction of continuous increase in thickness of sedimentary deposit provide insight to the controlling factor responsible for tar sand deposit and bitumen

  18. Immunofluorescence serration pattern analysis as a diagnostic criterion in antilaminin-332 mucous membrane pemphigoid : immunopathological findings and clinical experience in 10 Dutch patients

    NARCIS (Netherlands)

    Terra, J. B.; Pas, H. H.; Hertl, M.; Dikkers, F. G.; Kamminga, N.; Jonkman, M. F.

    2011-01-01

    Background Antilaminin-332 mucous membrane pemphigoid (anti-LN-332 MMP) is a chronic subepidermal blistering disease characterized by IgG anti-epidermal basement membrane zone (BMZ) autoantibodies against laminin-332 (LN-332). Patients with anti-LN-332 MMP have an increased relative risk of

  19. Prevalence of membranoproliferative glomerulonephritis at Hospital General de México “Dr. Eduardo Liceaga” over a 5-year period

    Directory of Open Access Journals (Sweden)

    C.A. Mendoza-Cerpa

    2017-10-01

    Full Text Available Background: Membranoproliferative glomerulonephritis is a pattern of glomerular lesion with a variety of causes. It can be classified by using direct immunofluorescence into immune complex-mediated membranoproliferative glomerulonephritis and complement-mediated membranoproliferative glomerulonephritis. Objective: Determine the prevalence of membranoproliferative glomerulonephritis by biopsy (light microscopy, direct immunofluorescence and electron microscopy, categorise them according to the most recent classification, identify possible causes and determine certain epidemiological and clinical characteristics. Material and method: A descriptive, cross-sectional study was carried out, selecting renal biopsies with a membranoproliferative pattern at 5 years at Hospital General de México. Age, gender, clinical syndrome and immunofluorescence and electron microscopy results were obtained. Biopsies lacking immunofluorescence or electron microscopy results were excluded. Results: 133 biopsies diagnosed as membranoproliferative glomerulonephritis were obtained. The average annual rate was 3.37%, while average age was 31.35 ± 16 years. The disorder affected mostly women (60.15%, n = 80. Nephrotic syndrome was the most common clinical presentation (48.12%, n = 64, while autoimmune disease was the most common cause (77.77%, n = 91. Conclusions: Direct immunofluorescence is indispensable for classifying membranoproliferative glomerulonephritis. Resumen: Antecedentes: La glomerulonefritis membranoproliferativa es un patrón de lesión glomerular con diferentes etiologías. Se clasifica por inmunofluorescencia directa en: glomerulonefritis membranoproliferativa mediada por complejos inmunes y glomerulonefritis membranoproliferativa mediada por complemento. Objetivo: Determinar la prevalencia de las glomerulonefritis membranoproliferativas mediante la biopsia (microscopía de luz, inmunofluorescencia directa y microscop

  20. Genetic and pharmacological inhibition of microRNA-92a maintains podocyte cell cycle quiescence and limits crescentic glomerulonephritis.

    Science.gov (United States)

    Henique, Carole; Bollée, Guillaume; Loyer, Xavier; Grahammer, Florian; Dhaun, Neeraj; Camus, Marine; Vernerey, Julien; Guyonnet, Léa; Gaillard, François; Lazareth, Hélène; Meyer, Charlotte; Bensaada, Imane; Legrès, Luc; Satoh, Takashi; Akira, Shizuo; Bruneval, Patrick; Dimmeler, Stefanie; Tedgui, Alain; Karras, Alexandre; Thervet, Eric; Nochy, Dominique; Huber, Tobias B; Mesnard, Laurent; Lenoir, Olivia; Tharaux, Pierre-Louis

    2017-11-28

    Crescentic rapidly progressive glomerulonephritis (RPGN) represents the most aggressive form of acquired glomerular disease. While most therapeutic approaches involve potentially toxic immunosuppressive strategies, the pathophysiology remains incompletely understood. Podocytes are glomerular epithelial cells that are normally growth-arrested because of the expression of cyclin-dependent kinase (CDK) inhibitors. An exception is in RPGN where podocytes undergo a deregulation of their differentiated phenotype and proliferate. Here we demonstrate that microRNA-92a (miR-92a) is enriched in podocytes of patients and mice with RPGN. The CDK inhibitor p57 Kip2 is a major target of miR-92a that constitutively safeguards podocyte cell cycle quiescence. Podocyte-specific deletion of miR-92a in mice de-repressed the expression of p57 Kip2 and prevented glomerular injury in RPGN. Administration of an anti-miR-92a after disease initiation prevented albuminuria and kidney failure, indicating miR-92a inhibition as a potential therapeutic strategy for RPGN. We demonstrate that miRNA induction in epithelial cells can break glomerular tolerance to immune injury.

  1. CD4+ T Cell Fate in Glomerulonephritis: A Tale of Th1, Th17, and Novel Treg Subtypes

    Directory of Open Access Journals (Sweden)

    Christan F. Krebs

    2016-01-01

    Full Text Available Multiple studies have identified CD4+ T cells as central players of glomerulonephritis (GN. Cells of the Th1 and Th17 responses cause renal tissue damage, while Tregs mediate protection. Recently, a high degree of plasticity among these T cell lineages was proposed. During inflammation, Th17 cells were shown to have the potential of transdifferentiation into Th1, Th2, or alternatively anti-inflammatory Tr1 cells. Currently available data from studies in GN, however, do not indicate relevant Th17 to Th1 or Th2 conversion, leaving the Th17 cell fate enigmatic. Tregs, on the other hand, were speculated to transdifferentiate into Th17 cells. Again, data from GN do not support this concept. Rather, it seems that previously unrecognized subspecialized effector Treg lineages exist. These include Th1 specific Treg1 as well as Th17 directed Treg17 cells. Furthermore, a bifunctional Treg subpopulation was recently identified in GN, which secrets IL-17 and coexpresses Foxp3 together with the Th17 characteristic transcription factor RORγt. Similarities between these different and highly specialized effector Treg subpopulations with the corresponding T helper effector cell lineages might have resulted in previous misinterpretation as Treg transdifferentiation. In summary, Th17 cells have a relatively stable phenotype during GN, while, in the case of Tregs, currently available data suggest lineage heterogeneity rather than plasticity.

  2. C1q nephropathy and isolated CD59 deficiency manifesting as necrotizing crescentic glomerulonephritis: A rare association of two diseases

    Directory of Open Access Journals (Sweden)

    Ruchika Gupta

    2015-01-01

    Full Text Available C1q nephropathy is a recently described clinico-pathologic entity with a variable clinical presentation and pathology. Crescentic glomerulonephritis (GN has been reported in only two patients in the available literature. CD59 deficiency, along with lack of CD55, is responsible for paroxysmal nocturnal hemoglobinuria (PNH. Few cases of isolated CD59 deficiency have been described with PNH-like features. A middle-aged adult male presented with rapidly progressive renal failure. Serological investigations were negative. A renal biopsy revealed necrotizing crescentic GN with rupture of Bowman′s capsule. Immunofluorescence on the frozen sections showed dominant mesangial deposits of C1q along with IgM. Hematological work-up of the patient revealed isolated CD59 deficiency. Hence, a final diagnosis of C1q nephropathy and CD59 deficiency manifesting as crescentic GN and hemolytic anemia was made. The co-existence of two rare disorders, C1q nephropathy and CD59 deficiency, in a patient with necrotizing crescentic GN is described for the first time to the best of our knowledge. The pathogenetic link of these two entities with the clinical manifestation requires further study.

  3. Adalimumab (TNFα Inhibitor Therapy Exacerbates IgA Glomerulonephritis Acute Renal Injury and Induces Lupus Autoantibodies in a Psoriasis Patient

    Directory of Open Access Journals (Sweden)

    S. S. Wei

    2013-01-01

    Full Text Available Adalimumab (Humira is a tumour necrosis factor α (TNFα inhibitor that is approved for the treatment of rheumatoid arthritis, psoriasis, psoriatic arthritis, Crohn's disease, ankylosing spondylitis, and juvenile idiopathic arthritis (Sullivan and Preda (2009, Klinkhoff (2004, and Medicare Australia. Use of TNFα inhibitors is associated with the induction of autoimmunity (systemic lupus erythematosus, vasculitis, and sarcoidosis or sarcoid-like granulomas (Ramos-Casals et al. (2010. We report a patient with extensive psoriasis presenting with renal failure and seropositive lupus markers without classical lupus nephritis after 18 months treatment with adalimumab. He has renal biopsy proven IgA nephritis instead. Renal biopsy is the key diagnostic tool in patients presenting with adalimumab induced nephritis and renal failure. He made a remarkable recovery after adalimumab cessation and steroid treatment. To our knowledge, this is a unique case of a psoriasis patient presenting with seropositive lupus markers without classical lupus nephritis renal failure and had renal biopsy proven IgA glomerulonephritis after receiving adalimumab.

  4. Different types of glomerulonephritis associated with the dysregulation of the complement alternative pathway in 2 brothers: A case report.

    Science.gov (United States)

    Chen, Pei; Zhu, Li; Yu, Feng; Han, Sha-Sha; Meng, Si-Jun; Guo, Wei-Yi; Zhang, Hong; Song, Yan

    2017-06-01

    C3 glomerulonephritis (C3GN) and complement-mediated hemolytic uremic syndrome (HUS) both result from the abnormal regulation of the complement system. A significant number of patients with C3GN or complement-mediated HUS have mutations of more than 1 complement protein. This discovery has had a major impact on identifying the underlying cause of familial C3GN or complement-mediated HUS. We report the cases of 2 brothers (herein referred to as patient II-1 and patient II-9), both with complement disorders that differed in their clinical and genetic features. Patient II-1 clinically presented with nephrotic syndrome and acute kidney injury and pathologically presented with C3GN combined with thrombotic microangiopathy (TMA) and subacute tubulointerstitial nephritis. Meanwhile, patient II-9 clinically presented with HUS and pathologically presented with TMA combined with acute severe tubular injury. Screenings for genetic mutations contributed to complement system dysregulation were performed on patient II-1. The genome sequencing identified that patient II-1 had a heterozygous mutation in the C3 gene (c.C1774T/p.R592W). Nine other relatives of the brothers were checked for this C3 mutation and only the daughter of patient II-1 (herein referred to as patient III-2) carried it, but so far, she does not have any clinical manifestations of kidney disease. Family members with a dysregulation of the complement alternative pathway may differ in its clinical and genetic features.

  5. Forms of iron in soils on basement complex rocks of Kaduna state in ...

    African Journals Online (AJOL)

    The forms of iron extracted by different methods were studied in soils developed on four basement complex rocks within Northern Guinea Savanna of Nigeria namely: migmatite gneisses, older granite, quartzites and mica schists. The study shows that forms of iron generally decreased in the order of total elemental iron ...

  6. Improved Access To Aging Ocean Basement Biosphere For Microbial Geochemical Studies

    Science.gov (United States)

    Cowen, J. P.; Glazer, B.; Rappe, M.; Kenig, F.; Fisher, A.; Copson, D.; Harris, D.; Jolly, J.; Nuzzio, D.

    2005-12-01

    CORK observatories affixed to Integrated Ocean Drilling Program (IODP) boreholes offer an unprecedented opportunity to study biogeochemical properties and microbial diversity in circulating fluids from sediment-buried ocean basement. Preliminary 16S rRNA gene sequence data from 65 degrees C fluids escaping from the top of the over-pressured ODP borehole 1026B, on the flanks of the Juan de Fuca Ridge indicated the presence of diverse Bacteria and Archaea, including gene clones with varying degrees of relatedness to known dissimilatory nitrate reducers (with ammonia production), thermophilic sulfate reducers, and thermophilic fermentative heterotrophs, consistent with fluid chemistry measurements. However, questions remain regarding microbial community structure, key metabolic pathways and rates, and redox chemistry of the basement fluids, along with concerns for contamination issues. We describe ongoing developments intended to address key in situ analytical and sampling challenges including: 1) The new generation CORKs' dedicated microbiological/geochemical fluid delivery system specifically designed to minimize chemical contamination and surface biofouling; and 2) Development of a seafloor instrument sled for coupling to the CORK's bio-fluid delivery system for acquisition of real-time, in situ electrochemical (voltammetry) redox chemistry data on basement fluids, in addition to in situ particle filtration of basement fluids for molecular genetics, culturing and biogeochemical studies. Results of the first deployment of this instrument sled to new CORK observatory 1301A in Cascadia Basin, on the flanks of the Juan de Fuca Ridge, will be described.

  7. Basement to surface expressions and critical factors in the genesis of unconformity-related deposits

    International Nuclear Information System (INIS)

    Potter, Eric

    2014-01-01

    Two subprojects: 1) Basement to surface expressions of deep mineralization and refinement of critical factors leading to the genesis of unconformity-related uranium deposits; and 2) Recognition of uranium ore system alteration signatures in complex terranes: IOCG vs albite-hosted uranium vs volcanic-hosted uranium.

  8. KUALITAS UDARA DALAM RUANG DI DAERAH PARKIR BASEMENT DAN PARKIR UPPERGROUND (STUDI KASUS DI SUPERMARKET SEMARANG

    Directory of Open Access Journals (Sweden)

    Haryono S Huboyo

    2016-03-01

    Full Text Available Ever increasing building growth in urban areas is limited by land availability. Lack of space in this area lead to build high rise building rather landed building. In this type of building, parking area is built in the basement and or upperground inside the building. Within this enclosed space, indoor air quality might a problem. This study focus to compare emerged pollutants between basement parking area and upperground parking area in supermarket building. The dust sampler, the impinger and the CO monitor were used to measure TSP, NO2 and CO concentrations respectively in these areas during supermarket operations. In the basement area, in particular, the TSP concentrations tend to exceeds 300 µg/m3 mainly at weekend period. While for NO2 and CO concentrations still meet the air quality standard. Based on these findings it seems the main source of pollutants was derived from dust resuspension. Thus, the mitigation measures to reduce this dust resuspension should be emphasized in order to prevent air quality deterioration in the basement parking area.

  9. Location of fishpond sites in the crystalline basement area of Edo ...

    African Journals Online (AJOL)

    Location of fishpond sites in the crystalline basement area of Edo State Nigeria using geoelectric method. O Egwebe, RE Iserhien-Emekeme. Abstract. No Abstract. Nigerian Journal of Fisheries Vol. 4 (2) 2007: pp. 160-171. Full Text: EMAIL FULL TEXT EMAIL FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT.

  10. Possible options for reducing occupational dose from the TMI-2 basement

    International Nuclear Information System (INIS)

    Munson, L.F.; Harty, R.

    1985-11-01

    The major sources of exposure in the basement include the enclosed stairwell/elevator shaft structure, water and sludge in the elevator shaft, cast concrete walls, concrete floor slab, water and sludge on the floor, and activity in the paint and loose surface contamination. The sources were identified using data obtained by the utility from water processing, water and solid samples, remote video inspections and radiation monitoring with a robot, and strings of thermoluminescent dosimeters lowered from upper elevations. The area dose rates in the basement range from approximately 4 R/hr (in the NE quadrant) to over 1100 R/hr (near the enclosed stairwell/elevator shaft structure). It is estimated that the basement contains between 11,000 and 21,000 curies of 137 Cs. Specific decontamination and cleanup techniques are discussed. These techniques include flushing with water, high-pressure water blasting, leaching, scabbling and chemical cleaning. The applicability of these techniques to the major sources of radiation are discussed, and possible approaches and work sequences for basement cleanup are given

  11. Impact of climate changes during the last 5 million years on groundwater in basement aquifers.

    Science.gov (United States)

    Aquilina, Luc; Vergnaud-Ayraud, Virginie; Les Landes, Antoine Armandine; Pauwels, Hélène; Davy, Philippe; Pételet-Giraud, Emmanuelle; Labasque, Thierry; Roques, Clément; Chatton, Eliot; Bour, Olivier; Ben Maamar, Sarah; Dufresne, Alexis; Khaska, Mahmoud; Le Gal La Salle, Corinne; Barbecot, Florent

    2015-09-22

    Climate change is thought to have major effects on groundwater resources. There is however a limited knowledge of the impacts of past climate changes such as warm or glacial periods on groundwater although marine or glacial fluids may have circulated in basements during these periods. Geochemical investigations of groundwater at shallow depth (80-400 m) in the Armorican basement (western France) revealed three major phases of evolution: (1) Mio-Pliocene transgressions led to marine water introduction in the whole rock porosity through density and then diffusion processes, (2) intensive and rapid recharge after the glacial maximum down to several hundred meters depths, (3) a present-day regime of groundwater circulation limited to shallow depth. This work identifies important constraints regarding the mechanisms responsible for both marine and glacial fluid migrations and their preservation within a basement. It defines the first clear time scales of these processes and thus provides a unique case for understanding the effects of climate changes on hydrogeology in basements. It reveals that glacial water is supplied in significant amounts to deep aquifers even in permafrosted zones. It also emphasizes the vulnerability of modern groundwater hydrosystems to climate change as groundwater active aquifers is restricted to shallow depths.

  12. Uranium deposits in the metamorphic basement of the Rouergue massif. Genesis and extension of related albitization processes

    International Nuclear Information System (INIS)

    Schmitt, J.M.

    1982-02-01

    Albitization processes in the Rouergue metamorphic basement, probably Permian aged is evidenced. Late development of uranium orebodies occured within albitized zones. The detection of the latter serves as a highly valuable indirect guide for prospecting this type of deposits in a metamorphic basement [fr

  13. Cracked and full of sand: microstructural insights into how oil gets into a crystalline basement reservoir

    Science.gov (United States)

    Holdsworth, Bob; McCaffrey, Ken; Dempsey, Eddie

    2017-04-01

    The fractured Neoarchaean orthogneisses forming the 200km long, NE-SW trending Rona Ridge lie offshore along the southeast margin of the Faroe-Shetland Basin (FSB). The basement ridge was uplifted during Cretaceous-age normal faulting and is flanked and immediately overlain by Devonian to Cretaceous cover sequences. Basement-hosted oil is known to occur in significant volumes in at least two fields (Clair, Lancaster). Re-Os dating of bitumen samples from the Clair Field suggests that oil was generated in the period 64-72Ma. A new microstructural study of basement cores was carried out to assess the mechanisms and timing of oil charge and other fracture-hosted mineralization. Oil charge is everywhere associated with quartz-adularia-calcite-pyrite mineralization and is hosted in a complex mesh of interconnected shear and tensile fractures that formed during a single protracted episode of brittle deformation. This association is recognized in all basement cores containing oil and also in locally overlying well-cemented Devonian (Lower Clair Group) and Jurassic (Rona Sandstone) sequences. Mineralization and oil charge is everywhere associated with clastic sedimentary infillings which occur either as vein-hosted injected slurries, or as little deformed laminated infills in mm to dm-scale open fractures. The latter preserve delicate way-up criteria and geopetal structures. The largest accumulations of oil are found either in these poorly-cemented sedimentary infills, or in fracture-hosted vuggy cavities up to several cm across. All these features, together with the widespread development of zoned mineral cements and cockade textures suggest a low-temperature hydrothermal system that likely formed in a near surface (Jurassic source rocks located to the west in the FSB, through the basement ridge and up into the overlying Clair Group and other cover sequences during the 64-72Ma time period. Our findings have major implications for the development of fractured basement

  14. Lupus glomerulonephritis and other autoimmune glomerulonephritis

    Directory of Open Access Journals (Sweden)

    María Ruiz-Olivares

    2014-12-01

    Full Text Available La glomerulonefritis es un término empleado para expresar la proliferación e inflamación endocapilar del glomérulo renal, que clínicamente puede manifestarse de numerosas formas e incluso permanecer asintomática. En su etiología se encuentran múltiples mecanismos, como la participación de microorganismos y parásitos, aunque es destacable el mecanismo autoinmune en el que se identifican varios componentes del sistema inmune, entre ellos el sistema del complemento. Un ejemplo de este último mecanismo es la glomerulonefritis secundaria al Lupus Eritematoso Sistémico (LES, que ha sido objeto de investigación en los últimos años, y en la que se han hecho importantes avances en cuanto al descubrimiento de nuevas moléculas implicadas en el proceso etiopatogénico. Esto ha conseguido abrir una puerta a nuevas terapias que reduzcan la mortalidad y mejoren la calidad de vida. Se ha revisado la fisiopatología, clínica, diagnóstico, pronóstico y tratamientos, incluyendo los emergentes, en cuanto a glomerulonefritis haciendo hincapié en la glomerulonefritis lúpica y otras glomerulonefritis de mecanismo igualmente.

  15. Lupus glomerulonephritis and other autoimmune glomerulonephritis

    OpenAIRE

    María Ruiz-Olivares; Guillermo Arturo Cañadas-De la Fuente; José Luis Gómez-Urquiza; Rafael Fernández Castillo

    2014-01-01

    La glomerulonefritis es un término empleado para expresar la proliferación e inflamación endocapilar del glomérulo renal, que clínicamente puede manifestarse de numerosas formas e incluso permanecer asintomática. En su etiología se encuentran múltiples mecanismos, como la participación de microorganismos y parásitos, aunque es destacable el mecanismo autoinmune en el que se identifican varios componentes del sistema inmune, entre ellos el sistema del complemento. Un ejemplo de este último mec...

  16. Basement characterization and crustal structure beneath the Arabia-Eurasia collision (Iran): A combined gravity and magnetic study

    Science.gov (United States)

    Mousavi, Naeim; Ebbing, Jörg

    2018-04-01

    We present a study on the depth to basement and magnetic crustal domains beneath the Iranian Plateau by modeling aeromagnetic and gravity data. First, field processing of the aeromagnetic data was undertaken to estimate the general characteristics of the magnetic basement. Afterwards, inverse modeling of aeromagnetic data was carried out to estimate the depth to basement. The obtained model of basement was refined using combined gravity and magnetic forward modeling. Hereby, we were able to distinguish different magnetic domains in the uppermost crust (10-20 km depths) influencing the medium to long wavelength trends of the magnetic anomalies. Magnetic basement mapping shows that prominent shallow magnetic features are furthermore located in the volcanic areas, e.g. the Urumieh Dokhtar Magmatic Assemblage. The presence of ophiolite outcrops in SE Iran implies that shallow oceanic crust (with high magnetization) is the main source of one of the biggest magnetic anomalies in entire Iran area located north of the Makran.

  17. An Interactive GIS Procedure for Building and Basement Corrections in Urban Microgravity Surveys

    Science.gov (United States)

    Chasseriau, P.; Olivier, R.

    2007-12-01

    Construction of a new underground railway in Lausanne, a highly-urbanized city in Switzerland, was an opportunity to test the feasibility and reliability of microgravity surveys in urban environments. The goal of our microgravity survey was to determine the depth-to-bedrock along the project corridor. Available drilling information allowed us verify the density model obtained. The geophysical results also provided spatially exhaustive subsurface information that could not be obtained with drilling methods alone. Gravimetry is one of the rare geophysical methods that can be used in noisy urban environments. An inevitable constraint of this method is terrain correction. It is not easy to obtain a simple and accurate digital elevation model (DEM) of an urban environment considering that buildings and basements are not included. However, these structures significantly influence gravity measurements. We calculate, with software that we have developed, the influence of buildings and basements in order to correct our gravity data. Our procedure permits the integration of gravity measurements, cadastral information (building typology and geometry) and basement geometry in an Access database that allows interactive determination of the Bouguer anomaly. A geographic information system (GIS) is used to extract building geometries based on cadastral information and to correct the influence of each building using a simplified architectural style. Basement voids are then introduced in the final DEM using building outlines given by cadastral maps. The depths and altitudes of the basements are measured by visiting them, and then linking the results to a regional topographic map. All of these corrections can be calculated before the gravity acquisition has begun in order to optimize the design of the survey. The surveys are executed late at night so as to minimize the effects of traffic noise. 160 gravity measurements were carried out before and after digging of the underground

  18. Membrane paradigm

    International Nuclear Information System (INIS)

    Price, R.H.; Thorne, K.S.

    1986-01-01

    The membrane paradigm is a modified frozen star approach to modeling black holes, with particles and fields assuming a complex, static, boundary-layer type structure (membrane) near the event horizon. The membrane has no effects on the present or future evolution of particles and fields above itself. The mathematical representation is a combination of a formalism containing terms for the shear and bulk viscosity, surface pressure, momentum, temperature, entropy, etc., of the horizon and the 3+1 formalism. The latter model considers a family of three-dimensional spacelike hypersurfaces in one-dimensional time. The membrane model considers a magnetic field threading the hole and undergoing torque from the hole rotation. The field is cleaned by the horizon and distributed over the horizon so that ohmic dissipation is minimized. The membrane paradigm is invalid inside the horizon, but is useful for theoretically probing the properties of slowly evolving black holes

  19. Membrane processes

    Science.gov (United States)

    Staszak, Katarzyna

    2017-11-01

    The membrane processes have played important role in the industrial separation process. These technologies can be found in all industrial areas such as food, beverages, metallurgy, pulp and paper, textile, pharmaceutical, automotive, biotechnology and chemical industry, as well as in water treatment for domestic and industrial application. Although these processes are known since twentieth century, there are still many studies that focus on the testing of new membranes' materials and determining of conditions for optimal selectivity, i. e. the optimum transmembrane pressure (TMP) or permeate flux to minimize fouling. Moreover the researchers proposed some calculation methods to predict the membrane processes properties. In this article, the laboratory scale experiments of membrane separation techniques, as well their validation by calculation methods are presented. Because membrane is the "heart" of the process, experimental and computational methods for its characterization are also described.

  20. Primordial membranes

    DEFF Research Database (Denmark)

    Hanczyc, Martin M; Monnard, Pierre-Alain

    2017-01-01

    Cellular membranes, which are self-assembled bilayer structures mainly composed of lipids, proteins and conjugated polysaccharides, are the defining feature of cell physiology. It is likely that the complexity of contemporary cells was preceded by simpler chemical systems or protocells during...... the various evolutionary stages that led from inanimate to living matter. It is also likely that primitive membranes played a similar role in protocell 'physiology'. The composition of such ancestral membranes has been proposed as mixtures of single hydrocarbon chain amphiphiles, which are simpler versions...

  1. ANCA-associated pauci-immune glomerulonephritis in a patient with bacterial endocarditis: a challenging clinical dilemma.

    Science.gov (United States)

    Cervi, Andrea; Kelly, Dylan; Alexopoulou, Iakovina; Khalidi, Nader

    2017-01-01

    We report the case of a 59-year-old man with chronic hepatitis B and C infection presenting with acute kidney injury and enterococcus faecalis-infective endocarditis (IE). An elevated proteinase-3 (PR3)-ANCA and pauci-immune glomerulonephritis (GN) on renal biopsy were discovered, corresponding to ANCA-mediated GN. We conducted a literature review to assess the role of ANCA in IE and treatment implications. On systematic review of the literature, we found five previous cases whereby IE caused by streptococcus and bartonella species were related to ANCA vasculitis-associated GN. Most reports of IE-related GN are mediated by immune complex deposition and resolve following microbial clearance. Of the 5 cases of ANCA GN in the setting of IE, all had markedly elevated levels of PR3-ANCA with either a subacute or chronic course of infection. Patients were treated with a combination of steroids and cyclophosphamide (2/5), steroids and antibiotics alone (1/5), or with valvular replacement (2/5). Renal function was recovered in 4/5 patients. Infection is a major etiologic player in the formation of ANCA; however, the role of PR3-ANCA in IE remains unclear. Kidney biopsy is essential in differentiating IE-related GN due to infection and immune complex deposition versus ANCA-associated vasculitis. A paucity of reports on the development of GN in IE-associated ANCA vasculitis exists, highlighting the rarity of our case and lack of clear therapeutic strategies in a patient with active infection requiring immunosuppression. In this case, the patient's chronic hepatitis B and C coinfection presented a unique challenge.

  2. Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Syndrome in Patients With Biopsy-Proven Glomerulonephritis

    Science.gov (United States)

    Jarrot, Pierre-Andre; Chiche, Laurent; Hervier, Baptiste; Daniel, Laurent; Vuiblet, Vincent; Bardin, Nathalie; Bertin, Daniel; Terrier, Benjamin; Amoura, Zahir; Andrés, Emmanuel; Rondeau, Eric; Hamidou, Mohamed; Pennaforte, Jean-Loup; Halfon, Philippe; Daugas, Eric; Dussol, Bertrand; Puéchal, Xavier; Kaplanski, Gilles; Jourde-Chiche, Noemie

    2016-01-01

    Abstract The aim of the study was to report the clinical, biological, and pathological characteristics of patients with glomerulonephritis (GN) secondary to systemic lupus erythematosus (SLE)/antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) overlap syndrome. A nationwide survey was conducted to identify cases of SLE/AAV overlap syndrome. Data were collected from SLE and AAV French research groups. Inclusion criteria were diagnosis of both SLE and AAV according to international classification criteria and biopsy-proven GN between 1995 and 2014. Additional cases were identified through a systematic literature review. A cohort of consecutive biopsy-proven GN was used to study the prevalence of overlapping antibodies and/or overlap syndrome. The national survey identified 8 cases of SLE/AAV overlap syndrome. All patients were female; median age was 40 years. AAV occurred before SLE (n = 3), after (n = 3), or concomitantly (n = 2). Six patients had rapidly progressive GN and 3/8 had alveolar hemorrhage. All patients had antinuclear antibodies (ANA); 7/8 had p-ANCA antimyeloperoxidase (MPO) antibodies. Renal biopsies showed lupus nephritis (LN) or pauci-immune GN. Remission was obtained in 4/8 patients. A literature review identified 31 additional cases with a similarly severe presentation. In the GN cohort, ANCA positivity was found in 30% of LN, ANA positivity in 52% of pauci-immune GN, with no correlation with pathological findings. The estimated prevalence for SLE/AAV overlap syndrome was 2/101 (2%). In patients with GN, SLE/AAV overlap syndrome may occur but with a low prevalence. Most patients have an aggressive renal presentation, with usually both ANA and anti-MPO antibodies. Further studies are needed to assess shared pathogenesis and therapeutic options. PMID:27258503

  3. The Effects of Helicobacter pylori Eradication on Proteinuria in Patients with Primary Glomerulonephritis

    Directory of Open Access Journals (Sweden)

    Bahar Caliskan

    2014-01-01

    Full Text Available Background. Membranous nephropathy (MN is a common cause of nephrotic syndrome. In most cases it is idiopathic, while it may also be secondary to many diseases. In this study, prevalence of H. pylori infection and the effects of H. pylori eradication on proteinuria levels were investigated. Methods. Thirty five patients with MN (19 male, 12 patients with IgA nephropathy (4 male and 12 patients with focal segmental glomerulosclerosis (FSGS (8 male were studied. The presence of H. pylori antigen was investigated in renal tissues obtained by biopsy, and the effects of H. pylori eradication on proteinuria levels were investigated. Results. Immunohistochemistry with H. pylori antigen revealed no positive staining in the glomeruli of all patients. 19 patients (54% with MN, 10 (83% with IgA nephropathy and 4 (33% with FSGS were positive for H. pylori stool antigen test (P=0.045. Patients with H. pylori infection were administered eradication therapy (lansoprazole, 30 mg twice daily, plus amoxicillin, 0.75 g twice daily, plus clarithromycin, 250 mg twice daily, for 14 days. Before the eradication therapy the mean proteinuria of patients with MN, IgA nephropathy and FSGS were 2.42 ± 3.24 g/day, 2.12 ± 1.63 g/day and 1.80 ± 1.32 g/day, respectively. Three months after eradication, baseline proteinuria levels of patients with MN significantly decreased to 1.26 ± 1.73 g/day (P=0.031. In all three groups there were no significant differences with regard to serum creatinine, albumin and C-reactive protein levels before and after eradication therapy. Conclusions. The eradication of H. pylori infection may be effective to reduce proteinuria in patients with MN, while spontaneous remission of MN could not be excluded in this patient cohort. This trial is registered with NCT00983034.

  4. A Nonlinear Inversion Approach to Map the Magnetic Basement: A Case Study from Central India Using Aeromagnetic Data

    Science.gov (United States)

    Kumar, R.; Bansal, A. R.; Anand, S. P.; Rao, V. K.; Singh, U. K.

    2016-12-01

    The central India region is having complex geology covering various geological units e.g., Precambrian Bastar Craton (including Proterozoic Chhattisgarh Basin, granitic intrusions etc.) and Eastern Ghat Mobile Belt, Gondwana Godavari and Mahanadi Grabens, Late Cretaceous Deccan Traps etc. The central India is well covered by reconnaissance scale aeromagnetic data. We analyzed this data for mapping the basement by dividing into143 overlapping blocks of 100×100km using least square nonlinear inversion method for fractal distribution of sources. The scaling exponents and depth values are optimized using grid search method. We interpreted estimated depths of anomalous sources as magnetic basement and shallow anomalous magnetic sources. The shallow magnetic anomalies are found to vary from 1 to 3km whereas magnetic basement depths are found to vary from 2km to 7km. The shallowest basement depth of 2km found corresponding to Kanker granites a part of Bastar Craton whereas deepest basement depth of 7km is associated with Godavari Graben and south eastern part of Eastern Ghat Mobile Belts near the Parvatipuram Bobbili fault. The variation of magnetic basement, shallow depths and scaling exponent in the region indicate complex tectonic, heterogeneity and intrusive bodies at different depths which is due to different tectonic processes in the region. The detailed basement depth of central India is presented in this study.

  5. Membranous glomerulopathy in a patient with selective IgA deficiency: is there a link?

    Science.gov (United States)

    Vanacker, A; Van Dorpe, J; Maes, B

    2011-01-01

    We report a 42-year-old woman, who presented with proteinuria (3.85 g/day) and malleolar oedema. She had a medical history of Graves' disease, recurrent upper respiratory tract infections, episodes of Raynaud phenomenon and dysphagia. Biochemistry showed a selective IgA deficiency (SIgAD). Percutaneous renal needle biopsy showed diffuse global thickening of the glomerular basement membranes on light microscopy and granular deposits of IgG and C3 along the glomerular basement membranes on immunofluorescence. The pathological diagnosis was membranous glomerulopathy stage II. A treatment with dietary salt restriction and an angiotensin-converting enzyme inhibitor was initiated, resulting in a reduction of proteinuria. Despite the fact that selective IgA deficiency is associated with various autoimmune disorders, the association with glomerular disease is rather rare and the pathogenesis is not fully understood.

  6. Geology and petrography of basement in south extreme in Sierra Grande de San Luis, Argentina

    International Nuclear Information System (INIS)

    Morosini, A.; Ortiz Suarez, A.

    2007-01-01

    In the south of the Sierra de San Luis is recognized a basement composed by La Escalerilla and Los Puquios granites, accompanied by schist, mylonite s and mafic-ultramafic rocks. The schists, La Escalerilla granite and the mafic -ultramafic rocks are affected by a regional metamorphism in anphibolites facies, the two first present a N-S foliation di ping to east. The mylonite s correspond to a second event of deformation, distributed in thin belts of NNE-SSO direction and inclination to east, developed in green schist to anphibolites facies. Los Puquios granite represents the more young of the basement rocks and the intrusion was associated with a shear zone. Los Puquios granite forms dikes and small plutons cutting La Escalerilla granite and micas chits in high cortical levels

  7. Soil inertia and shallow basement envelope impact on cellar internal temperature

    Directory of Open Access Journals (Sweden)

    Naima Sakami

    2016-06-01

    Full Text Available This work deals with a three dimensional numerical study of heat transfer by conduction between the soil and the shallow basement in the city of Marrakech (Morocco. The heat transfer equation is solved by the finite difference method using the implicit alternative direction (ADI. The internal temperature of the cellar is computed by using energy balance equation in the cellar. The objective of this work is to evaluate the effects of the nature of the soil, the nature of the walls, the thickness of the walls of the cellar and the distance L far from the cellar on the internal temperature and the heat exchanged between the soil and the shallow basement

  8. Fractured but not fractal: Fragmentation of the gulf of suez basement

    Science.gov (United States)

    Korvin, Gabor

    1989-03-01

    Recent geophysical studies revealed that the Palaeozoic basement of the Gulf of Suez consists of an enormous number of fault blocks whose network qualitavely resembles the contraction-crack polygons which can be found in nature in a wide variety of materials and on all scales (mud cracks, hardening concrete, age cracking in paintings, etc.). The fault network of the Gulf of Suez basement forms a rather uniformly spaced polygonal pattern, most of the blocks are four-sided, the lengths of block sides parallel with the Gulf of Suez axis are exponentially distributed. The power-law size distribution associated with the fractal (scale-free) fragmentation can be possibly ruled out. The paper calls attention to the necessity of calssifying the physical processes leading to fragmentations with exponential-, lognormal-, and power-law size distributions, respectively.

  9. Utility of magnetic susceptibility values for the petrographic analysis of weathering crust basement samples

    Directory of Open Access Journals (Sweden)

    Alfredo Hernández-Ramsay

    2018-01-01

    Full Text Available This article exposes the resolving power of the magnetic susceptibility measurements in basement minerals samples of the lateritic weathering profile as an element of complementary analysis in the petrographic characterization of the rocks, and useful in the mapping of the magnetic heterogeneities of the basement. The comparison of the magnetic susceptibility data with the petrographic data of different samples revealed that even in samples that correspond to homogeneous lithotypes, great heterogeneities and differences can be manifested from the physical-mineralogical point of view. In general, a high concordance was observed between the intensity of the weathering processes in the rock samples, and the values of the magnetic susceptibility of such samples. The results support the possibility of extrapolating the composition information to samples with magnetic susceptibility measurements and without petrographic studies.

  10. Geological Structure of the Basement of Western and Eastern Parts of the West-Siberian Plain

    Science.gov (United States)

    Ivanov, Kirill S.; Erokhin, Yuriy V.; Ponomarev, Vladimir S.; Pogromskaya, Olga E.; Berzin, Stepan V.

    2016-01-01

    The U-Pb dating (SHRIMP-II on zircon) was obtained for the first time from the basement of the West Siberian Plain in the Western half of the region. It is established that a large part of the protolith of the metamorphic depth in the Shaim-Kuznetsov meganticlinorium contained sedimentary late- and middle-Devonian rocks (395-358 million years).…

  11. Equatorial Pacific gravity lineaments: interpretations with basement topography along seismic reflection lines

    Science.gov (United States)

    Mitchell, Neil C.; Davies, Huw

    2018-03-01

    The central equatorial Pacific is interesting for studying clues to upper mantle processes, as the region lacks complicating effects of continental remnants or major volcanic plateaus. In particular, the most recently produced maps of the free-air gravity field from satellite altimetry show in greater detail the previously reported lineaments west of the East Pacific Rise (EPR) that are aligned with plate motion over the mantle and originally suggested to have formed from mantle convection rolls. In contrast, the gravity field 600 km or farther west of the EPR reveals lineaments with varied orientations. Some are also parallel with plate motion over the mantle but others are sub-parallel with fracture zones or have other orientations. This region is covered by pelagic sediments reaching 500-600 m thickness so bathymetry is not so useful for seeking evidence for plate deformation across the lineaments. We instead use depth to basement from three seismic reflection cruises. In some segments of these seismic data crossing the lineaments, we find that the co-variation between gravity and basement depth is roughly compatible with typical densities of basement rocks (basalt, gabbro or mantle), as expected for some explanations for the lineaments (e.g., mantle convection rolls, viscous asthenospheric inter-fingering or extensional deformation). However, some other lineaments are associated with major changes in basement depth with only subtle changes in the gravity field, suggesting topography that is locally supported by varied crustal thickness. Overall, the multiple gravity lineament orientations suggest that they have multiple origins. In particular, we propose that a further asthenospheric inter-fingering instability mechanism could occur from pressure variations in the asthenosphere arising from regional topography and such a mechanism may explain some obliquely oriented gravity lineaments that have no other obvious origin.

  12. Gravitational sliding of the Mt. Etna massif along a sloping basement

    Science.gov (United States)

    Murray, John B.; van Wyk de Vries, Benjamin; Pitty, Andy; Sargent, Phil; Wooller, Luke

    2018-04-01

    Geological field evidence and laboratory modelling indicate that volcanoes constructed on slopes slide downhill. If this happens on an active volcano, then the movement will distort deformation data and thus potentially compromise interpretation. Our recent GPS measurements demonstrate that the entire edifice of Mt. Etna is sliding to the ESE, the overall direction of slope of its complex, rough sedimentary basement. We report methods of discriminating the sliding vector from other deformation processes and of measuring its velocity, which averaged 14 mm year-1 during four intervals between 2001 and 2012. Though sliding of one sector of a volcano due to flank instability is widespread and well-known, this is the first time basement sliding of an entire active volcano has been directly observed. This is important because the geological record shows that such sliding volcanoes are prone to devastating sector collapse on the downslope side, and whole volcano migration should be taken into account when assessing future collapse hazard. It is also important in eruption forecasting, as the sliding vector needs to be allowed for when interpreting deformation events that take place above the sliding basement within the superstructure of the active volcano, as might occur with dyke intrusion or inflation/deflation episodes.

  13. Basement membrane and interstitial proteoglycans produced by MDCK cells correspond to those expressed in the kidney cortex

    DEFF Research Database (Denmark)

    Erickson, A C; Couchman, J R

    2001-01-01

    ) cells has been utilized. Proteoglycans were prepared from conditioned medium by DEAE anion exchange chromatography. The eluted PGs were treated with heparitinase or chondroitinase ABC (cABC), separately or combined, followed by SDS-PAGE. Western blot analysis, using antibodies specific for various PG...

  14. The Mitogen-Activated Protein Kinase p38 alpha Regulates Tubular Damage in Murine Anti-Glomerular Basement Membrane Nephritis

    NARCIS (Netherlands)

    Mueller, Ralf; Daniel, Christoph; Hugo, Christian; Amann, Kerstin; Mielenz, Dirk; Endlich, Karlhans; Braun, Tobias; van der Veen, Betty; Heeringa, Peter; Schett, Georg; Zwerina, Jochen

    2013-01-01

    p38 mitogen-activated protein kinase (MAPK) is thought to play a central role in acute and chronic inflammatory responses. Whether p38MAPK plays a pathogenic role in crescentic GN (GN) and which of its four isoforms is preferentially involved in kidney inflammation is not definitely known. We thus

  15. Human Papillomavirus Species-Specific Interaction with the Basement Membrane-Resident Non-Heparan Sulfate Receptor

    Directory of Open Access Journals (Sweden)

    Kathleen F. Richards

    2014-12-01

    Full Text Available Using a cell culture model where virus is bound to the extracellular matrix (ECM prior to cell surface binding, we determined that human papillomavirus type 16 (HPV16 utilizes ECM resident laminin (LN 332 as an attachment receptor for infectious entry. In presence of LN332, soluble heparin can function as ligand activator rather than competitive inhibitor of HPV16 infection. We also show that the ability to use LN332 binding as a productive attachment step for infectious entry is not conserved amongst HPV types. In the alpha genus, species 9 members (HPV16 attach to ECM via LN332, while members of species 7 (HPV18 are completely inhibited by heparin pre-incubation due to an inability to use LN332. Since HPV species 7 and 9 are preferentially associated with adenocarcinoma and squamous cell carcinoma of the cervix, respectively, our data provide first evidence that pre-entry events may contribute to the anatomical-site preference of HPV species.

  16. AGE-modified basement membrane cooperates with Endo180 to promote epithelial cell invasiveness and decrease prostate cancer survival

    DEFF Research Database (Denmark)

    Rodriguez-Teja, Mercedes; Gronau, Julian H; Breit, Claudia

    2015-01-01

    in non-transformed PECs via a molecular mechanism linked to cancer progression. This study provides a rationale for targeting CTLD2 in Endo180 in prostate cancer and other pathologies in which increased basal lamina thickness and tissue stiffness are driving factors. © 2014 The Authors. The Journal......Biomechanical strain imposed by age-related thickening of the basal lamina and augmented tissue stiffness in the prostate gland coincides with increased cancer risk. Here we hypothesized that the structural alterations in the basal lamina associated with age can induce mechanotransduction pathways...... in prostate epithelial cells (PECs) to promote invasiveness and cancer progression. To demonstrate this, we developed a 3D model of PEC acini in which thickening and stiffening of basal lamina matrix was induced by advanced glycation end-product (AGE)-dependent non-enzymatic crosslinking of its major...

  17. Kallikrein genes are associated with lupus and glomerular basement membrane–specific antibody–induced nephritis in mice and humans

    Science.gov (United States)

    Liu, Kui; Li, Quan-Zhen; Delgado-Vega, Angelica M.; Abelson, Anna-Karin; Sánchez, Elena; Kelly, Jennifer A.; Li, Li; Liu, Yang; Zhou, Jinchun; Yan, Mei; Ye, Qiu; Liu, Shenxi; Xie, Chun; Zhou, Xin J.; Chung, Sharon A.; Pons-Estel, Bernardo; Witte, Torsten; de Ramón, Enrique; Bae, Sang-Cheol; Barizzone, Nadia; Sebastiani, Gian Domenico; Merrill, Joan T.; Gregersen, Peter K.; Gilkeson, Gary G.; Kimberly, Robert P.; Vyse, Timothy J.; Kim, Il; D’Alfonso, Sandra; Martin, Javier; Harley, John B.; Criswell, Lindsey A.; Wakeland, Edward K.; Alarcón-Riquelme, Marta E.; Mohan, Chandra

    2009-01-01

    Immune-mediated nephritis contributes to disease in systemic lupus erythematosus, Goodpasture syndrome (caused by antibodies specific for glomerular basement membrane [anti-GBM antibodies]), and spontaneous lupus nephritis. Inbred mouse strains differ in susceptibility to anti-GBM antibody–induced and spontaneous lupus nephritis. This study sought to clarify the genetic and molecular factors that may be responsible for enhanced immune-mediated renal disease in these models. When the kidneys of 3 mouse strains sensitive to anti-GBM antibody–induced nephritis were compared with those of 2 control strains using microarray analysis, one-fifth of the underexpressed genes belonged to the kallikrein gene family, which encodes serine esterases. Mouse strains that upregulated renal and urinary kallikreins exhibited less evidence of disease. Antagonizing the kallikrein pathway augmented disease, while agonists dampened the severity of anti-GBM antibody–induced nephritis. In addition, nephritis-sensitive mouse strains had kallikrein haplotypes that were distinct from those of control strains, including several regulatory polymorphisms, some of which were associated with functional consequences. Indeed, increased susceptibility to anti-GBM antibody–induced nephritis and spontaneous lupus nephritis was achieved by breeding mice with a genetic interval harboring the kallikrein genes onto a disease-resistant background. Finally, both human SLE and spontaneous lupus nephritis were found to be associated with kallikrein genes, particularly KLK1 and the KLK3 promoter, when DNA SNPs from independent cohorts of SLE patients and controls were compared. Collectively, these studies suggest that kallikreins are protective disease-associated genes in anti-GBM antibody–induced nephritis and lupus. PMID:19307730

  18. Glomerulopathy induced by immunization with a peptide derived from the goodpasture antigen α3IV-NC1.

    Science.gov (United States)

    Hopfer, Helmut; Hünemörder, Stefanie; Treder, Julia; Turner, Jan-Eric; Paust, Hans-Joachim; Meyer-Schwesinger, Catherine; Hopfer, Ulrike; Sachs, Marlies; Peters, Anett; Bucher-Kocaoglu, Biranda; Ahrens, Stefanie; Panzer, Ulf; Mittrücker, Hans-Willi

    2015-04-15

    Mouse experimental autoimmune glomerulonephritis, a model of human antiglomerular basement membrane disease, depends on both Ab and T cell responses to the Goodpasture Ag noncollagenous domain 1 of the α3-chain of type IV collagen (α3IV-NC1). The aim of our study was to further characterize the T cell-mediated immune response. Repeated immunization with mouse α3IV-NC1 caused fatal glomerulonephritis in DBA/1 mice. Although two immunizations were sufficient to generate high α3IV-NC1-specific IgG titers, Ab and complement deposition along the glomerular basement membranes, and a nephrotic syndrome, two additional immunizations were needed to induce a necrotizing/crescentic glomerulonephritis. Ten days after the first immunization, α3IV-NC1-specific CD4(+) cells producing TNF-α, IFN-γ, or IL-17A were detected in the spleen. With the emergence of necrotizing/crescentic glomerulonephritis, ∼0.15% of renal CD4(+) cells were specific for α3IV-NC1. Using peptides spanning the whole α3IV-NC1 domain, three immunodominant T cell epitopes were identified. Immunization with these peptides did not lead to clinical signs of experimental autoimmune glomerulonephritis or necrotizing/crescentic glomerulonephritis. However, mice immunized with one of the peptides (STVKAGDLEKIISRC) developed circulating Abs against mouse α3IV-NC1 first detected at 8 wk, and 50% of the mice showed mild proteinuria at 18-24 wk due to membranous glomerulopathy. Taken together, our results suggest that autoreactive T cells are able to induce the formation of pathologic autoantibodies. The quality and quantity of α3IV-NC1-specific Ab and T cell responses are critical for the phenotype of the glomerulonephritis. Copyright © 2015 by The American Association of Immunologists, Inc.

  19. High-resolution temporal analysis of deep subseafloor microbial communities inhabiting basement fluids

    Science.gov (United States)

    Jungbluth, S.; Lin, H. T.; Hsieh, C. C.; Rappe, M. S.

    2014-12-01

    The temporal variation in microbial communities inhabiting the anoxic, sediment-covered basaltic ocean basement is largely uncharacterized due to the inaccessible nature of the environment and difficulties associated with collection of samples from low-biomass microbial habitats. Here, a deep sea instrumented platform was employed on the Juan de Fuca Ridge in the summer of 2013 to collect 46 samples of basement fluids from the most recent generation of borehole observatories (U1362A and B), which feature multiple sampling horizons at a single location and fluid delivery lines manufactured using stainless steel or inert polytetrafluoroethylene (PTFE) parts. Included were three time-series deployments of the GEOmicrobe sled meant to resolve the fine-scale (i.e. hourly) temporal variation within in situ crustal microbial communities. Illumina technology was used to sequence small subunit ribosomal RNA (SSU rRNA) gene fragments from sediment, seawater, and subseafloor fluids. Similar to has been reported previously, basic differences in the three environments was observed. Fluid samples from depth horizons extending 30, 70, and ~200 meters sub-basement revealed differences in the observed microbial communities, indicating potential depth-specific zonation of microorganisms in the basaltic basement fluids. Extensive overlap between microorganisms collected from a single depth horizon but using two fluid delivery lines manufactured with different materials was observed, though some differences were also noted. Several archaeal (e.g. THSCG, MCG, MBGE, Archaeoglobus) and bacterial (e.g. Nitrospiraceae, OP8, KB1) lineages detected in previous years of basement fluid sampling nearby were found here, which further supports the notion that these microorganisms are stable residents of anoxic basaltic subseafloor fluids. Direct cell enumeration of samples collected from U1362A and U1362B revealed an elevated biomass compared to samples at these locations from previous years

  20. Microbial diversity within Juan de Fuca ridge basement fluids sampled from oceanic borehole observatories

    Science.gov (United States)

    Jungbluth, S.; Bowers, R.; Lin, H.; Hsieh, C.; Cowen, J. P.; Rappé, M.

    2012-12-01

    Three generations of sampling and instrumentation platforms known as Circulation Obviation Retrofit Kit (CORK) observatories affixed to Ocean Drilling Program (ODP) and Integrated Ocean Drilling Program (IODP) boreholes are providing unrivaled access to fluids originating from 1.2-3.5 million-years (Myr) old basaltic crust of the eastern flank of the Juan de Fuca ridge. Borehole fluid samples obtained via a custom seafloor fluid pumping and sampling system coupled to CORK continuous fluid delivery lines are yielding critical insights into the biogeochemistry and nature of microbial life inhabiting the sediment-covered basement environment. Direct microscopic enumeration revealed microbial cell abundances that are 2-41% of overlying bottom seawater. Snapshots of basement fluid microbial diversity and community structure have been obtained through small subunit ribosomal RNA (SSU rRNA) gene cloning and sequencing from five boreholes that access a range of basement ages and temperatures at the sediment-basement interface. SSU rRNA gene clones were derived from four different CORK installations (1026B, 1301A, 1362A, and 1362B) accessing relatively warmer (65°C) and older (3.5 Myr) ridge flank, and one location (1025C) accessing relatively cooler (39°C) and younger (1.2 Myr) ridge flank, revealing that warmer basement fluids had higher microbial diversity. A sampling time-series collected from borehole 1301A has revealed a microbial community that is temporally variable, with the dominant lineages changing between years. Each of the five boreholes sampled contained a unique microbial assemblage, however, common members are found from both cultivated and uncultivated lineages within the archaeal and bacterial domains, including meso- and thermophilic microbial lineages involved with sulfur cycling (e.g Thiomicrospira, Sulfurimonas, Desulfocapsa, Desulfobulbus). In addition, borehole fluid environmental gene clones were also closely related to uncultivated lineages

  1. Geochemical variability of the Yucatan basement: Constraints from crystalline clasts in Chicxulub impactites

    Science.gov (United States)

    Kettrup, B.; Deutsch, A.

    2003-07-01

    The 65 Ma old Chicxulub impact structure with a diameter of about 180 km is again in the focus of the geosciences because of the recently commenced drilling of the scientific well Yaxcopoil- 1. Chicxulub is buried beneath thick post-impact sediments, yet samples of basement lithologies in the drill cores provide a unique insight into age and composition of the crust beneath Yucatan. This study presents major element, Sr, and Nd isotope data for Chicxulub impact melt lithologies and clasts of basement lithologies in impact breccias from the PEMEX drill cores C-1 and Y-6, as well as data for ejecta material from the K/T boundaries at La Lajilla, Mexico, and Furlo, Italy. The impact melt lithologies have an andesitic composition with significantly varying contents of Al, Ca, and alkali elements. Their present day 87Sr/86Sr ratios cluster at about 0.7085, and 143Nd/144Nd ratios range from 0.5123 to 0.5125. Compared to the melt lithologies that stayed inside the crater, data for ejecta material show larger variations. The 87Sr/86Sr ratios range from 0.7081 for chloritized spherules from La Lajilla to 0.7151 for sanidine spherules from Furlo. The 143Nd/144Nd ratio is 0.5126 for La Lajilla and 0.5120 for the Furlo spherules. In an tCHUR(Nd)-tUR(Sr) diagram, the melt lithologies plot in a field delimited by Cretaceous platform sediments, various felsic lithic clasts and a newly found mafic fragment from a suevite. Granite, gneiss, and amphibolite have been identified among the fragments from crystalline basement gneiss. Their 87Sr/86Sr ratios range from 0.7084 to 0.7141, and their 143Nd/144Nd ratios range from 0.5121 to 0.5126. The TNdDM model ages vary from 0.7 to 1.4 Ga, pointing to different source terranes for these rocks. This leads us to believe that the geological evolution and the lithological composition of the Yucatàn basement is probably more complex than generally assumed, and Gondwanan as well as Laurentian crust may be present in the Yucatàn basement.

  2. Basement control in the development of the early cretaceous West and Central African rift system

    Science.gov (United States)

    Maurin, Jean-Christophe; Guiraud, René

    1993-12-01

    The structural framework of the Precambrian basement of the West and Central African Rift System (WCARS) is described in order to examine the role of ancient structures in the development of this Early Cretaceous rift system. Basement structures are represented in the region by large Pan-African mobile belts (built at ca. 600 Ma) surrounding the > 2 Ga West African, Congo and Sao Francisco cratons. Except for the small Gao trough (eastern Mali) located near the contact nappe of the Pan-African Iforas suture zone along the edge of the West African craton, the entire WCARS is located within the internal domains of the Pan-African mobile belts. Within these domains, two main structural features occur as the main basement control of the WCARS: (1) an extensive network of near vertical shear zones which trend north-south through the Congo, Brazil, Nigeria, Niger and Algeria, and roughly east-west through northeastern Brazil and Central Africa. The shear zones correspond to intra-continental strike-slip faults which accompanied the oblique collision between the West African, Congo, and Sao Francisco cratons during the Late Proterozoic; (2) a steep metamorphic NW-SE-trending belt which corresponds to a pre-Pan-African (ca. 730 Ma) ophiolitic suture zone along the eastern edge of the Trans-Saharian mobile belt. The post-Pan-African magmatic and tectonic evolution of the basement is also described in order to examine the state of the lithosphere prior to the break-up which occurred in the earliest Cretaceous. After the Pan-African thermo-tectonic event, the basement of the WCARS experienced a long period of intra-plate magmatic activity. This widespread magmatism in part relates to the activity of intra-plate hotspots which have controlled relative uplift, subsidence and occasionally block faulting. During the Paleozoic and the early Mesozoic, this tectonic activity was restricted to west of the Hoggar, west of Aïr and northern Cameroon. During the Late Jurassic

  3. OTUB1 overexpression in mesangial cells is a novel regulator in the pathogenesis of glomerulonephritis through the decrease of DCN level.

    Directory of Open Access Journals (Sweden)

    Yan Zhang

    Full Text Available BACKGROUND: OTUB1 is a member of OTUs (Ovarian-tumor-domain-containing proteases, a deubiquitinating enzymes family (DUBs, which was shown as a proteasome-associated DUB to be involved in the proteins Ub-dependent degradation. It has been reported that OTUB1 was expressed in kidney tissue. But its concrete cellular location and function in the kidney remain unclear. Decorin (DCN in mesangial cells (MC is considered to be a potentially important factor for antagonizing glomerulonephritides, and its degradation is mediated by ubiquitination. The aim of this study is to investigate the role of OTUB1 expression in MC and its relationship with DCN during glomerulonephritis. METHODOLOGY/PRINCIPAL FINDINGS: Using quantitative RT-PCR and Western blot, we demonstrated that OTUB1 mRNA and protein were constitutively expressed in cultured rat MC and found to be upregulated by the stimulation of IL-1β or ATS. OTUB1 overexpression was detected in the mesangial area of glomeruli in some immunocomplex mediated nephritides such as IgA nephropathy, acute diffuse proliferative glomerulonephritis and lupus nephritis by immunohistochemistry. The immunoprecipitation assay demonstrated that OTUB1 interacted with DCN. The overexpression of OTUB1 enhanced the ubiquitination and degradation of DCN in MC. CONCLUSION/SIGNIFICANCE: These data showed the inflammatory injury could up-regulate OTUB1 expression in MC, which might attribute the promoting effect of OTUB1 on glomerulonephritides to the decrease of DCN level.

  4. [Clinical significance of serum and urinary biomarkers for water-salt metabolism in patients with proteinuric forms of chronic glomerulonephritis].

    Science.gov (United States)

    Pulin, A A; Brovko, M Iu; Kustova, T Iu; Kozlovskaia, L V; Fomin, V V; Mukhin, N A

    2014-01-01

    To determine the nature and magnitude of changes in the detectable serum and urinary biomarkers of water-salt metabolism in patients with proteinuric forms of chronic glomerulonephritis (CGN), their importance for assessing the activity and prognosis of the disease. Forty-seven patients with CGN were examined. Group 1 included 10 patients with nephrotic syndrome (NS) and decreased renal function; Group 2 comprised 16 patients with persistent NS and normal renal function; Group 3 consisted of 10 patients with partial remission of NS; Group 4 included 11 patients with active hematuric CGN. A control group consisted of 9 healthy individuals matched for gender and age with the patients with CGN. The serum level of copeptin and the urinary excretion of aquaporine-2 (AQP-2) and kidney injury molecule-1 (KIM-1) were determined by indirect enzyme-linked immunosorbent assay (ELISA). In the NS patients with and without renal dysfunction, the serum copeptin concentration was significantly higher than that in those with partial remission of NS or hematuric CGN and in the controls. In the patients with hematuric CGN, this indicator was virtually different from that in the control group. Urinary AQP-2 excretion was significantly similar in 3 NS groups. In the patients with hematuric CGN, the urinary AQP-2 concentration was higher than that in those with NS, but it was significantly lower than in the control group. The highest urinary excretion of KIM-1 was found in the patients with NS and diminished renal function while its excretion was significantly lower in the patients with NS and stable renal function, as in those with partial remission of NS. The lowest values were seen in the patients with hematuric CGN and in the control group; the differences between these groups were statistically insignificant. Correlation analysis showed that there was an inverse correlation between serum copeptin and urinary AQP-2 levels and between urinary AQP- 2 and KIM-1 levels. Serum copeptin

  5. An overview of the British Columbia Glomerulonephritis network and registry: integrating knowledge generation and translation within a single framework.

    Science.gov (United States)

    Barbour, Sean; Beaulieu, Monica; Gill, Jagbir; Djurdjev, Ognjenka; Reich, Heather; Levin, Adeera

    2013-10-29

    Glomerulonephritis (GN) is a group of rare kidney diseases with a substantial health burden and high risk of progression to end-stage renal disease. Research in GN has been limited by poor availability of large comprehensive registries. Substantial variations in access to and administration of treatment and outcomes in GN have been described. Leveraging provincial resources and existing infrastructure, the British Columbia (BC) GN Network is an initiative which serves to combine research and clinical care objectives. The goal of the BC GN Network is to coordinate and improve health care, including robust data capture, on all patients with GN in BC, a Canadian province of over 4.6 million people. This provincial initiative will serve as a model for Canadian or other national and international endeavours. The BC Provincial Renal Agency (BCPRA) is the provincial governmental agency responsible for health delivery for all kidney patients in BC. The BC GN Network has been created by the BCPRA to ensure high quality and equitable access to care for all patients with GN and is a platform for evidence based clinical care programs and associated health policy. All patients with biopsy-proven GN are registered at the time of kidney biopsy into the BCPRA provincial database of kidney disease patients, forming the BC GN Registry. Thereafter, all laboratory results and renal related outcomes are captured automatically. Histology data and core clinical variables are entered into the database. Additional linkages between the GN Registry and administrative databases ensure robust capture of medications, hospital admissions, health care utilization, comorbidities, cancer and cardiac outcomes, and vital statistics. The BC GN Network and Registry is a unique model in that it combines robust data capture, data linkages, and health care delivery and evaluation into one integrated system. This model utilizes existing health infrastructure to prospectively capture population level data

  6. Goodpasture's Syndrome due to IgA in a patient with clinical diagnosis of Henoch Schonlein's purpura

    International Nuclear Information System (INIS)

    Restrepo Cesar A

    2005-01-01

    This is a case of a 23 year old woman with an initial clinical syndrome compatible with glomerulonephritis of uncertain origin, who later showed lesions of purpuric rash characteristics of Henoch- Schonlein Purpura and then complicated with a pulmonary hemorrhage and a rapidly progressive glomerulonephritis, with a mixed lung-kidney syndrome. The renal biopsy showed presence of linear deposits of immunoglobulin A and extra capillary proliferative changes. The case was concluded corresponding to Goodpasture's syndrome for antibodies antiglomerular basement membrane of the type of IgA in the context of a Henoch-Schonlein Purpura.

  7. Robotic membranes

    DEFF Research Database (Denmark)

    Ramsgaard Thomsen, Mette

    2008-01-01

    , Vivisection and Strange Metabolisms, were developed at the Centre for Information Technology and Architecture (CITA) at the Royal Danish Academy of Fine Arts in Copenhagen as a means of engaging intangible digital data with tactile physical material. As robotic membranes, they are a dual examination...

  8. Treatment strategies in mucous membrane pemphigoid

    Directory of Open Access Journals (Sweden)

    Ann G Neff

    2008-06-01

    Full Text Available Ann G Neff, Matthew Turner, Diya F MutasimDepartment of Dermatology, University of Cincinnati, Cincinnati, OH, USAAbstract: Mucous membrane pemphigoid (MMP is an autoimmune blistering disorder that is characterized by subepithelial bullae. Various basement membrane zone components have been identified as targets of autoantibodies in MMP. Considerable variability exists in the clinical presentation of MMP. Mucous membranes that may be involved include the oral cavity, conjunctiva, nasopharynx, larynx, esophagus, genitourinary tract, and anus. A multidisciplinary approach is essential in the management of MMP. Early recognition of this disorder and treatment may decrease disease-related complications. The choice of agents for treatment of MMP is based upon the sites of involvement, clinical severity, and disease progression. For more severe disease, or with rapid progression, systemic corticosteroids are the agents of choice for initial treatment, combined with steroid-sparing agents for long-term maintenance. Due to the rarity of this disease, large controlled studies comparing the efficacy of various agents are lacking.Keywords: mucous membrane pemphigoid, cicatricial pemphigoid

  9. Thermochronology and geochemistry of the Pan-African basement below the Sab'atayn Basin, Yemen

    Science.gov (United States)

    Veeningen, Resi; Rice, A. Hugh N.; Schneider, David A.; Grasemann, Bernhard

    2015-02-01

    Three important lithologies occur in two drill wells from the Pan-African basement underlying the Mesozoic Sab'atayn Basin, in a previously undocumented area of the Pan-African, 83 and 90 km NE of known exposures in Yemen. Cores from well 1 include amphibolite, with basaltic to andesitic compositions, affected by crustal contamination during emplacement into a thickened crust. Deeper in the well, an unfoliated dark red monzogranite has a U-Pb zircon age of 628.8 ± 3.1 Ma and a Rb-Sr biotite cooling age of 591.6 ± 5.8 Ma (∼300 °C). Regional constraints suggest emplacement in a transitional tectonic setting with compressional terrane amalgamation followed by extensional collapse. Sm-Nd isotope analysis yields a TDM model age of 1.24 Ga with negative εNd values, suggesting the monzogranite is part of the Al Bayda island arc terrane. Cores from well 2 contains a weakly deformed, massive (unbedded) medium grey meta-arkose exhibiting essentially no geochemical signature of weathering and with an almost pure dacitic composition. This rock may have been directly derived from an (extrusive) granitoid that was emplaced prior to, or during terrane amalgamation. A (U-Th-Sm)/He zircon age of 156 ± 14 Ma constrains the time of basement cooling to ∼180 °C, synchronous with basin formation. These lithologies provide new insights in the development of the Pan-African basement of Yemen, extending our knowledge of the nearby surface geology to the subsurface.

  10. Uplift, Emergence, and Subsidence of the Gorda Escarpment Basement Ridge Offshore Cape Mendocino, CA

    Science.gov (United States)

    Hoover, Susan M.; Tréhu, Anne M.

    2017-12-01

    The Gorda Escarpment is a topographic step that characterizes the south side of the Mendocino Transform Fault east of 126oW and forms the northern edge of the Vizcaino Block. Seismic reflection data suggest that the basement beneath the northern edge of the Vizcaino is composed of east-west trending slivers of oceanic crust that form a 15 km wide band of buried ridges we call the Gorda Escarpment Basement Ridge (GEBR) to distinguish it from the northwest-trending basement structure that characterizes most of the Vizcaino Block. The history of uplift and subsidence of the GEBR is reconstructed by combining the seismic reflection profiles with age and lithological constraints from ODP Site 1022, gravity cores, and grab samples from the northern face of the Escarpment recovered using a remotely operated vehicle. Uplift of the GEBR began prior to 6 Ma, and it was above sea level 3.7-2.5 Ma. GEBR uplift and emergence coincided with sediment deposition on the southern flank of the GEBR that we interpret as indicative of strong upwelling and turbulence in the lee of a shallow ridge and island chain. A bright reflection, interpreted to be a sill, is observed south of the shallowest part of the GEBR. We speculate that this sill may reflect a larger, hidden intrusion at depth and that thermal expansion of the crust combined with tectonic forces to drive enhanced uplift of this segment of the plate boundary. The GEBR has been subsiding since 2.7 Ma, and its shallowest point is now 1,400 m below sea level.

  11. The nature of the acoustic basement on Mendeleev and northwestern Alpha ridges, Arctic Ocean

    Science.gov (United States)

    Bruvoll, Vibeke; Kristoffersen, Yngve; Coakley, Bernard J.; Hopper, John R.; Planke, Sverre; Kandilarov, Aleksandre

    2012-01-01

    The Alpha-Mendeleev ridge complex, over 1500 km long and 250-400 km wide, is the largest submarine structure in the Arctic Ocean basin. Its origin is unknown, but often inferred to represent a large igneous province where domains of continental crust may also be a possibility. We investigate the basement geology of part of this large scale feature using 1100 km of multichannel seismic reflection data, sonobuoy recordings and marine gravity data acquired in 2005 from USCG icebreaker Healy. The sonobuoy results show top and intra-acoustic basement velocities in the range of 2.3-4.0 km/s and the seismic reflection attributes define three main acoustic facies: 1) continuous high amplitude reflections often with abrupt breaks, 3) weak wedge geometry and 3) segmented, disrupted to chaotic reflections. The acoustic characteristics and seismic velocities compare more closely with basement on Ontong Java Plateau than normal ocean crust or wedges of seaward dipping reflections at volcanic margins. The acoustic facies are interpreted to represent basalt flows and sills capping voluminous tuff deposits and possible sediments. At least two volcanic centres are identified. The upper volcanic carapace on the surveyed part of Mendeleev and northwestern Alpha ridges was emplaced during a brief igneous episode no later than Campanian (80 Ma) and most likely part of wider Late Cretaceous circum Arctic volcanism. The horst and graben morphology on Mendeleev Ridge is largely a result of post-emplacement faulting where a number of the major extensional faults remained active until a late Miocene intrusive event.

  12. Dwellings with cellars and basements. A BRE guide to radon remedial measures in existing dwellings

    International Nuclear Information System (INIS)

    Scivyer, C.R.; Jaggs, M.P.R.

    1998-01-01

    This guide is one of a series giving practical advice on methods of reducing radon levels in existing dwellings. It is intended to help surveyors, builders and householders who are trying to reduce indoor radon levels in houses with cellars or basements. The guide is based on a large body of remedial work carried out to advice given by BRE, and on discussions with others working in the field. The measures it describes are applicable, in principle, to all dwellings and other buildings of a similar scale and construction which have cellars or basements. Contact details of other organisations referred to are listed at the back of this guide. Basements and cellars are relatively uncommon in the United Kingdom, but where they do occur they are likely to be major contributors to the radon problem within a building. Experience shows that most houses with high indoor radon levels can be remedied through the use of straightforward building works. These can be carried out by a builder or householder competent in DIY. The cost should not be excessive. Before reading this guide, for background information on radon, consult Radon: a householder's guide and Radon: a guide to reducing levels M your home, obtainable from local Environmental Health Officers or from the Department of the Environment, Transport and the Regions. The Government recommends that, if the average radon concentration in a dwelling exceeds 200 Bq/m 3 (the 'action' level), measures should be taken to reduce it. This guide assumes that radon measurements have been made in the building and that the annual average indoor radon level was shown to exceed the action level

  13. Detailed petrographic-structural study of an outcrop of Crystalline Basement of Montevideo

    International Nuclear Information System (INIS)

    Pascale, A.; Oyhantçabal, P.

    2010-01-01

    Preliminary data analysis of detailed outcrop Punta Virgilo, located on the S E coast of the department of Montevideo are presented. The investigated outcrop includes gneisses, amphibolite s and several generations of pegmatite and aplite dikes of Paleoproterozoic metamorphic basement, plus a set of dikes emplaced lamprófido exhumed once the area. Petrographic and microstructural studies of metamorphic units allowed to determine the conditions of metamorphism and deformation temperature between 520-720 ° C and pressure between 2 and 6 kbar (depth of 10 to 23 km)

  14. Lead isotopic study of aplites from the Precambrian basement rocks near Ibadan, southwestern Nigeria

    International Nuclear Information System (INIS)

    Oversby, V.M.

    1975-01-01

    Pb isotopic compositions for three total-rock samples of aplite and their constituent K-feldspars from the Nigerian basement assemblage near Ibadan show lead homogenization during the Pan-African thermo-tectonic event. A secondary isochron formed by the K-feldspars data points is used to calculate a primary age of about 2750 m.y. for the aplites. The aplites do not register any Pb isotopic effect from the intrusion of granite gneiss in the area at 2330m.y. (Auth.)

  15. The First Evidence of the Precambrian Basement in the Fore Range Zone of the Great Caucasus.

    Science.gov (United States)

    Latyshev, A.; Kamzolkin, V.; Vidjapin, Y.; Somin, M.; Ivanov, S.

    2017-12-01

    Within the Great Caucasus fold-thrust belt, the Fore Range zone has the most complicated structure, and the highest degree of metamorphism was found there. This zone consists of several salients with the different composition and the structural and metamorphic evolution. The largest Blyb salient includes the metamorphic basement covered by the pack of thrusts. According to the recent isotopic data the upper levels of the Blyb metamorphic complex (BMC) are supposed to be Middle-Paleozoic (Somin, 2011). We studied zircons from the granitic intrusions located in the metamorphic rocks of the BMC. The U-Pb dating (SHRIMP II, VSEGEI, Russia) of zircons from the large Balkan metadiorite massif yielded the ages of 549±7,4, 574,1±6,7, and 567,9±6,9 Ma. All studied zircons show the high Th/U ratios and likely have the magmatic origin. This data is the first confirmation of the presence of the Precambrian basement and Vendian magmatic activity in the Fore Range zone. Zircons from the Unnamed granodiorite massif from the south of the Blyb salient yielded the age of 319±3.8 Ma (the Early Carboniferous). This fact taken together with the low grade of metamorphism in this intrusion reveals the Late Paleozoic magmatic event in the Fore Range zone. We also suggest that the Precambrian basement of the BMC, including the Balkan intrusion, is covered by so-called Armovsky nappe. This is confirmed by the field data, Middle-Paleozoic U-Pb ages and the higher degree of metamorphism of the Armovsky gneisses and schists. Thus, the BMC is not uniform but includes the blocks of the different age and metamorphic grades. Finally, we measured the anisotropy of magnetic susceptibility (AMS) of the Balkan metadiorites. The axes of AMS ellipsoid fix the conditions of the north-east compression, as well as the strain field reconstructed from the macrostructures orientation, which corresponds to the thrusts propagation. Therefore, the emplacement of the Balkan massif happened before the thrust

  16. Shock pressure estimation in basement rocks of the Chicxulub impact crater using cathodoluminescence spectroscopy of quartz

    Science.gov (United States)

    Tomioka, N.; Tani, R.; Kayama, M.; Chang, Y.; Nishido, H.; Kaushik, D.; Rae, A.; Ferrière, L.; Gulick, S. P. S.; Morgan, J. V.

    2017-12-01

    The Chicxulub impact structure, located in the northern Yucatan Peninsula, Mexico, was drilled by the joint IODP-ICDP Expedition 364 in April-May 2016. This expedition is the first attempt to obtain materials from the topographic peak ring within the crater previously identified by seismic imaging. A continuous core was successfully recovered from the peak ring at depths between 505.7 and 1334.7 mbsf. Uplifted, fractured, and shocked granitic basement rocks forming the peak ring were found below, in the impact breccia and impact melt rock unit (747.0-1334.7 mbsf; Morgan et al. 2016). In order to constrain impact crater formation, we investigated shock pressure distribution in the peak-ring basement rocks. Thin sections of the granitic rocks were prepared at intervals of 60 m. All the samples contains shocked minerals, with quartz grains frequently showing planar deformation features (PDFs). We determined shock pressures based on the cathodoluminescence (CL) spectroscopy of quartz. The strong advantage of the CL method is its applicability to shock pressure estimation for individual grains for both quartz and diaplectic SiO2 glass with high-spatial resolution ( 1 μm) (Chang et al. 2016). CL spectra of quartz shows a blue emission band caused by shock-induced defect centers, where its intensity increases with shock pressure. A total of 108 quartz grains in ten thin sections were analyzed using a scanning electron microscope with a CL spectrometer attached (an acceleration voltage of 15 kV and a beam current of 2 nA were used). Natural quartz single crystals, which were experimentally shocked at 0-30 GPa, were used for pressure calibration. CL spectra of all the quartz grains in the basement rocks showed broad blue emission band at the wavelength range of 300-500 nm and estimated shock pressures were in the range of 15-20 GPa. The result is consistent with values obtained from PDFs analysis in quartz using the universal stage (Ferrière et al. 2017; Rae et al. 2017

  17. Basement inheritance and salt tectonics in the SE Barents Sea: Insights from new potential field data

    Science.gov (United States)

    Gernigon, L.; Broenner, M.; Dumais, M. A.; Gradmann, S.; Grønlie, A.; Nasuti, A.; Roberts, D.

    2017-12-01

    The tectonic evolution of the former `grey zone' between Russia and Norway has so far remained poorly constrained due to a lack of geophysical data. In 2014, we carried out a new aeromagnetic survey (BASAR-14) in the southern part of the new Norwegian offshore territory. Caledonian and Timanian structures, highlighted by the new potential field data, dominate the basement patterns and have exerted a strong influence on the structure and development of the overlying basins and basement highs. Clearly associated with NW-SE-oriented Timanian trends, the Tiddlybanken Basin represents an atypical sag basin that developed at the southern edge of the Fedynsky High. Regional extension and rapid sedimentation initiated the salt tectonics in the Barents Sea in the Early Triassic. Some of the pillows became diapiric during the Early Triassic and rejuvenated during subsequent Jurassic-Tertiary episodes of regional extension and/or compression. At present, quite a few large diapiric salt domes along the Nordkapp and Tiddlybanken basins are relatively shallow, locally reaching the seabed and thus show a clear bathymetric and magnetic signature. Quantitative modelling along 2D seismic transects was also carried out to constrain the structural and basement composition of the study area. The predominant NE-SW Mesozoic trend of the Nordkapp Basin represents a major crustal hinge zone between the Finnmark Platform, poorly affected by major crustal deformation, and the Bjarmeland Platform where Late Palaeozoic rifting controlled the widespread accumulation of salt deposits in Late Carboniferous-Early Permian time. The entire structure and segmentation of the Nordkapp Basin have been influenced by the inherited basement configuration highlighted by the new aeromagnetic data. Both the Nordkapp and the Tiddlybanken basins appear to lie at the edge of a peculiar thick and rigid crustal feature that coincides with a highly magnetic region. The abrupt termination of the eastern Nordkapp

  18. Glomerulonefritis fibrilar: Una rara forma de enfermedad glomerular por depósitos organizados Fibrillary glomerulo-nephritis: A rare form of glomerular disease with organized deposits

    Directory of Open Access Journals (Sweden)

    Marta B. Cabrera

    2011-10-01

    diagnosis was fibrillary glomerulonephritis. Glomerular lesions with organized deposits may exhibit syndromic and pathological overlap. For this reason it is important to initially discriminate between positive and negative Congo red deposits, using, in the latter case, transmission electron microscopy to distinguish between immuno-tactoid and fibrillary glomerulonephritis. This differentiation relies not only on ultrastructural features, but on different clinical characteristics. Unlike what happens with fibrillary glomerulonephritis, the immuno-tactoid shows a strong association with lymphoproliferative processes.

  19. Glomerulonephritis (For Parents)

    Science.gov (United States)

    ... need those procedures and will be able to play sports, participate in favorite activities, and lead perfectly normal ... Kidney Diseases in Childhood When Your Child Has a Chronic Kidney Disease ...

  20. Basement structure of the United Arab Emirates derived from an analysis of regional gravity and aeromagnetic database

    Science.gov (United States)

    Ali, M. Y.; Fairhead, J. D.; Green, C. M.; Noufal, A.

    2017-08-01

    Gravity and aeromagnetic data covering the whole territory of the United Arab Emirates (UAE) have been used to evaluate both shallow and deep geological structures, in particular the depth to basement since it is not imaged by seismic data anywhere within the UAE. Thus, the aim has been to map the basement so that its structure can help to assess its control on the distribution of hydrocarbons within the UAE. Power spectrum analysis reveals gravity and magnetic signatures to have some similarities, in having two main density/susceptibility interfaces widely separated in depth such that regional-residual anomaly separation could effectively be undertaken. The upper density/susceptibility interface occurs at a depth of about 1.0 km while the deeper interface varies in depth throughout the UAE. For gravity, this deeper interface is assumed to be due to the combined effect of lateral changes in density structures within the sediments and in depth of basement while for magnetics it is assumed the sediments have negligible susceptibility and the anomalies unrelated to the volcanic/magmatic bodies result from only changes in depth to basement. The power spectrum analysis over the suspect volcanic/magmatic bodies indicates they occur at 5 km depth. The finite tilt-depth and finite local wavenumber methods were used to estimate depth to source and only depths that agree to within 10% of each other were used to generate the depth to basement map. This depth to basement map, to the west of the UAE-Oman Mountains, varies in depth from 5 km to in excess of 15 km depth and is able to structurally account for the location of the shear structures, seen in the residual magnetic data, and the location of the volcanic/magmatic centres relative to a set of elongate N-S to NE-SW trending basement highs. The majority of oilfields in the UAE are located within these basement highs. Therefore, the hydrocarbon distribution in the UAE basin appears to be controlled by the location of the

  1. A skin substitute based on human amniotic membrane.

    Science.gov (United States)

    Tauzin, Hélène; Rolin, Gwenaël; Viennet, Céline; Saas, Philippe; Humbert, Philippe; Muret, Patrice

    2014-06-01

    Human amniotic membrane (HAM) has biological properties which are useful for wound healing. HAM is notably one of the therapeutic alternatives for venous leg ulcer care. Indeed, a prospective clinical study has demonstrated that cryopreserved HAM transplantation for leg ulcer is feasible, safe and has beneficial effects: 80 % of the patients had a significant clinical response. Nevertheless, at the end of the 3-month follow-up period, only 20 % of the ulcers were totally closed. The aim of this work was to create and characterize a model of epidermized HAM. The method of HAM desepithelialization was validated by histology, immunohistochemistry and scanning electron microscopy. Then, de-epithelialized HAM was seeded with primary keratinocytes. After 21 days of culture, 15 at the air-liquid interface, the model obtained was analyzed histologically and by immunohistochemistry. The amniotic basement membrane was preserved during enzymatic desepithelialization of HAM. Primary keratinocytes proliferated on HAM: the model obtained showed involucrin expression and had a good basement membrane. As re-epithelialization is an important step for ulcer closure, a model of epidermized HAM could be used to speed up the healing of such wounds.

  2. The relationship between HBV serum markers and the clinicopathological characteristics of hepatitis B virus-associated glomerulonephritis (HBV-GN in the northeastern chinese population

    Directory of Open Access Journals (Sweden)

    Zhang Lei

    2012-09-01

    Full Text Available Abstract Background To investigate the effect of HBV markers on HBV-GN. Methods The immunohistochemistry was used to detect HBsAg and HBcAg in frozen sections of renal biopsy, the changes in HBV serum markers, renal functional parameters and clinical manifestations or symptoms were observed to analyze renal damage. Results Using renal biopsy data from 329 cases, this study found that the most common pathological subtype in HBV-GN was mesangioproliferative glomerulonephritis (MsPGN (24.9%, P P P P Conclusion Examination of HBV markers in serum and renal biopsy will be useful for clinicians to predict the renal damage in early stage when it is reversible in HBV-GN.

  3. Nanggulan Formation and Its Problem As a Basement in Kulonprogo Basin, Yogyakarta

    Directory of Open Access Journals (Sweden)

    Hill Gendoet Hartono

    2017-03-01

    Full Text Available DOI: 10.17014/ijog.4.2.71-80Nanggulan Formation consists of the oldest clastic rock sequence exposed in Kulonprogo area, Yogyakarta. This paper discusses the position of Nanggulan Formation as a basement. The method used in this research is surface and subsurface investigations based on gravity surveys. The rock assemblage is exposed and distributed partly in the east flank of Kulonprogo Mountains with weak undulated morphology. The rock sequence is composed of sand to clay grain sizes such as sandstone, quartz sandstone, calcareous sandstone, claystone, fossiliferous claystone, calcareous claystone, siltstone, and coal seam intercalations. The total thickness of the sequence is less than 200 m. Based on the fossil and palynology investigations, previous investigators concluded the age of the rock was Eocene to Middle Miocene. The geological structures developed in the rocks are the lithological stratification, fractures, folding, and faulting. The subsurface interpretation based on gravity data revealed the rock was located under the andesite breccias with 2.44 g/cc density. The density of the rock sequence was 2.63 g/cc. The gravity interpretation shows a strong indication that Nanggulan Formation underlies the andesitic breccias presumably associated with Old Andesite Formation exposed in Kulonprogo Mountains. The limited distribution, the thickness, and the closed environmental deposition of Nanggulan Formation found in the present investigation raised problems on the position of the formation as the basement of Old Andesite Formation occurring in the Kulonprogo Mountain.

  4. Geophysical evaluation of groundwater potential in part of southwestern Basement Complex terrain of Nigeria

    Science.gov (United States)

    Bayewu, Olateju O.; Oloruntola, Moroof O.; Mosuro, Ganiyu O.; Laniyan, Temitope A.; Ariyo, Stephen O.; Fatoba, Julius O.

    2017-12-01

    The geophysical assessment of groundwater in Awa-Ilaporu, near Ago Iwoye southwestern Nigeria was carried out with the aim of delineating probable areas of high groundwater potential. The area falls within the Crystalline Basement Complex of southwestern Nigeria which is predominantly underlain by banded gneiss, granite gneiss and pegmatite. The geophysical investigation involves the very low frequency electromagnetic (VLF-EM) and Vertical Electrical Sounding (VES) methods. The VLF-EM survey was at 10 m interval along eight traverses ranging between 290 and 700 m in length using ABEM WADI VLF-EM unit. The VLF-EM survey was used to delineate areas with conductive/fractured zones. Twenty-three VES surveys were carried out with the use of Campus Ohmega resistivity meter at different location and at locations areas delineated as high conductive areas by VLF-EM survey. The result of VLF-EM survey along its traverse was used in delineating high conductive/fractured zones, it is, however, in agreement with the delineation of the VES survey. The VES results showed 3-4 geoelectric layers inferred as sandy topsoil, sandy clay, clayey and fractured/fresh basement. The combination of these two methods, therefore, helped in resolving the prospecting location for the groundwater yield in the study area.

  5. 3D modeling of the Strasbourg’s Cathedral basements for interdisciplinary research and virtual visits

    Directory of Open Access Journals (Sweden)

    T. Landes

    2015-08-01

    Full Text Available On the occasion of the millennium celebration of Strasbourg Cathedral, a transdisciplinary research group composed of archaeologists, surveyors, architects, art historians and a stonemason revised the 1966-1972 excavations under the St. Lawrence’s Chapel of the Cathedral having remains of Roman and medieval masonry. The 3D modeling of the Chapel has been realized based on the combination of conventional surveying techniques for the network creation, laser scanning for the model creation and photogrammetric techniques for the texturing of a few parts. According to the requirements and the end-user of the model, the level of detail and level of accuracy have been adapted and assessed for every floor. The basement has been acquired and modeled with more details and a higher accuracy than the other parts. Thanks to this modeling work, archaeologists can confront their assumptions to those of other disciplines by simulating constructions of other worship edifices on the massive stones composing the basement. The virtual reconstructions provided evidence in support of these assumptions and served for communication via virtual visits.

  6. 3D modeling of the Strasbourg's Cathedral basements for interdisciplinary research and virtual visits

    Science.gov (United States)

    Landes, T.; Kuhnle, G.; Bruna, R.

    2015-08-01

    On the occasion of the millennium celebration of Strasbourg Cathedral, a transdisciplinary research group composed of archaeologists, surveyors, architects, art historians and a stonemason revised the 1966-1972 excavations under the St. Lawrence's Chapel of the Cathedral having remains of Roman and medieval masonry. The 3D modeling of the Chapel has been realized based on the combination of conventional surveying techniques for the network creation, laser scanning for the model creation and photogrammetric techniques for the texturing of a few parts. According to the requirements and the end-user of the model, the level of detail and level of accuracy have been adapted and assessed for every floor. The basement has been acquired and modeled with more details and a higher accuracy than the other parts. Thanks to this modeling work, archaeologists can confront their assumptions to those of other disciplines by simulating constructions of other worship edifices on the massive stones composing the basement. The virtual reconstructions provided evidence in support of these assumptions and served for communication via virtual visits.

  7. First indication of mesoproterozoic age from the western basement of Sierra de San Luis, Argentina

    International Nuclear Information System (INIS)

    Sato, A.M.; Gonzalez, P.D; Sato, K

    2001-01-01

    Mafic to ultramafic metamagmatic rocks are widespread within the western basement of Sierra de San Luis (Eastern Sierras Pampeanas), through a belt of almost 45 km long, from Villa de la Quebrada in the south up to San Francisco del Monte de Oro in the north. The northern area have been studied by Merodio et al. (1978), Kilmurray and Villar (1981), Carugno Duran (1998) and Ortiz Suarez (1999). In the southern area, the detailed structural and metamorphic study revealed the presence of komatiites preserved within amphibolites. These rocks are in association with a supracrustal sequence composed of banded iron formations (BIF), metaquartzites, micaschists and other gneissic rocks (Gonzalez, 2000). Although this basement complex shows a penetrative Famatinian (Early Paleozoic) NNE-SSW trending foliation, remnants of an older NW-SE trending structures are also found (Gonzalez and Llambias, 1998; von Gosen and Prozzi, 1998). As a result of Sm-Nd whole rock analyses of the komatiites and amphibolites from the southern area, we obtained an isochron date of 1502 Ma. This is the first mention of an Early Mesoproterozoic date from the Sierra de San Luis, and hence, in this contribution we discuss its validity, taking into consideration the geological and regional context (au)

  8. Tectonic inheritage from adjacent basement, north of the Campos Basin; Heranca tectonica no embasamento adjacente no norte da Bacia de Campos

    Energy Technology Data Exchange (ETDEWEB)

    Ferroni, Felipe R. [Universidade Estadual Paulista (UNESP), Rio Claro, SP (Brazil); Castro, Joel C. de [Universidade Estadual Paulista (UNESP), Rio Claro, SP (Brazil). Inst. de Geociencias e Ciencias Exatas. Dept. de Geologia Aplicada; Souza, Iata A. de; Castro, Joel C. de [Universidade Estadual Paulista (UNESP), Rio Claro, SP (Brazil). Inst. de Geociencias e Ciencias Exatas

    2008-07-01

    The evolution of the Atlantic Brazilian basins is a target of researches since the firth discovery of oil deposits. With the advance of the geophysical methods the understanding of the structures in depth became possible. The objective of this paper is to determine if the adjacent basement in the north of Campos Basin has significant influence in the identification of areas that can contain hydrocarbons. Therefore, lineaments had been extracted with SRTM images of continental basement and the main alignment was correlated with gravimetric anomalies map and seismic data. Eight levels on seismic data had been interpreted (basement, top rift, shallow water Albian, Albian, mid-Oligocene and mid-Miocene). In all levels were identified a fault normal system, which cut sediments since basement until the Recent. The main direction of the basement is NE-SW, and the alignments formed for basin basement faults coincide with this direction, what indicates that the system is active and also genetically related. (author)

  9. Independent associations of urine neutrophil gelatinase–associated lipocalin and serum uric acid with interstitial fibrosis and tubular atrophy in primary glomerulonephritis

    Directory of Open Access Journals (Sweden)

    Lertrit A

    2016-04-01

    Full Text Available Amornpan Lertrit,1 Suchin Worawichawong,2 Somlak Vanavanan,2 Anchalee Chittamma,2 Dittapol Muntham,3 Piyanuch Radinahamed,1 Aumporn Nampoon,4 Chagriya Kitiyakara1 1Department of Medicine, 2Department of Pathology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, 3Section for Mathematics, Faculty of Science and Technology, Rajamangala University of Technology Suvarnabhumi, Phranakhon Si Ayutthaya, 4Research Center, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand Abstract: The degree of interstitial fibrosis and tubular atrophy (IFTA is one of the strongest prognostic factors in glomerulonephritis (GN. In experimental models, high serum uric acid (UA could contribute to IFTA through direct effects on the renal tubules, but the significance of this process has not been evaluated in patients. Urine neutrophil gelatinase–associated lipocalin (NGAL is produced by renal tubules following acute or chronic damage. We investigated the relationship between UA and NGAL excretion in primary GN and tested whether these biomarkers are independently associated with IFTA. Urine and blood were collected from patients on the day of kidney biopsy. IFTA was assessed semi-quantitatively. Fifty-one patients with primary GN were enrolled. NGAL/creatinine correlated significantly with proteinuria but not with glomerular filtration rate (GFR. By contrast, UA correlated with GFR but not with proteinuria. NGAL/creatinine did not correlate with UA. Both NGAL/creatinine and UA increased with the severity of IFTA. By multivariate analysis, GFR, NGAL/creatinine, and UA were independently associated with moderate-to-severe IFTA. Combining UA and NGAL/creatinine with classical predictors (proteinuria and GFR tended to improve discrimination for moderate-to-severe IFTA. Findings that UA was unrelated to urinary NGAL excretion suggest that the two biomarkers reflect different pathways related to the development of IFTA in

  10. Dynamics of uranium ore formation in the basement and frame of the Streltsovskaya Caldera

    International Nuclear Information System (INIS)

    Petrov, V.; Schukin, S.

    2014-01-01

    The analysis of geological-geophysical, paleo-geodynamics, mineralogical, geochemical, isotope, geochronological, and thermo-baro-geochemical data allow us to offer a model of uranium ore formation dynamics in the basement and frame of the Streltsovskaya Caldera connected to activity of the fluid-conducting fault zones network with the aim to identify prospective areas The most ancient fluid-conducting structures are inter-block NE-SW, NNE-submeridional, NW-SE and, probably, WNW-sub-latitudinal faults. The oldest NE-SW faults and schistosity zones were formed during Proterozoic tectonic cycle (TC) with reactivation in T3-J2 time due to global reorganization of stress field and reactivation of tectonic movements. The NNE-submeridional and NW-SE faults were extended with increased fluid permeability during Caledonian and Variscan TCs. They also were reactivated in the process of Late Mesozoic tectonic and magmatic activation (TMA). Thus already at early stages of geotectonic evolution within the intersection of NE-SW (N-Urulyunguyevskiy fault) and NNE-submeridional (Chindachinskaya zone) faults the areas of increased fluid and magmatic activity were formed. The dynamics of fault formation in the basement and frame of the Streltsovskaya caldera and its volcano-sedimentary cover differs. In the basement and granite framework NE-SW, NNEsubmeridional and NW-SE faults are interblock structures of the I rank. Their intersection formed areas of long-term circulation of hydrothermal solutions and telescopic appearance of multi-age metasomatites that created conditions for localizing of vein-stockwork mineralization. In volcanosedimentary cover the NE-SW and NNE-submeridional faults should be considered as interblock structures of the I rank where intersections provided inflow of ore-bearing solutions and their redistribution within the cover. Here the main ore distributing role belongs to NW-SE shears. They are intrablock II rank structures which were formed due to dextral

  11. Prostate-specific membrane antigen (PSMA)-mediated laminin proteolysis generates a pro-angiogenic peptide

    Czech Academy of Sciences Publication Activity Database

    Conway, R. E.; Rojas, C.; Alt, J.; Nováková, Zora; Richardson, S. M.; Rodrick, T. C.; Fuentes, J. L.; Richardson, N. H.; Attalla, J.; Stewart, S.; Fahmy, B.; Bařinka, Cyril; Ghosh, M.; Shapiro, L. H.; Slusher, B. S.

    2016-01-01

    Roč. 19, č. 4 (2016), s. 487-500 ISSN 0969-6970 R&D Projects: GA ČR GAP301/12/1513; GA MŠk(CZ) ED1.1.00/02.0109 Institutional support: RVO:86652036 Keywords : TUMOR-ASSOCIATED NEOVASCULATURE * BASEMENT-MEMBRANE * DISTINCT ANTITUMOR PROPERTIES Subject RIV: FA - Cardiovascular Diseases incl. Cardiotharic Surgery Impact factor: 5.253, year: 2016

  12. Structural Evolution of the Eastern Sierra Madre Oriental: The Role of Basement Structures in Fold-Thrust Development

    Science.gov (United States)

    Hamade, A.; Murphy, M. A.; Hall, S.

    2006-12-01

    Investigations worldwide show that basement plays an important role in the evolution of fold-thrust belts and recognizing their structural development leads to better understanding of zones of intercontinental deformation. Two explanations have been proposed to explain uplift of basement rocks in the Sierra Madre Oriental (SMO) fold-thrust belt, 1) basement rocks are uplifted along high-angle reverse faults (Laramide-style) which postdate a thin-skinned phase of deformation; and 2) basement rocks are carried in deeper-rooted thrust sheets during the thin-skinned phase of deformation. A structural study in the SMO fold-thrust belt that integrates field mapping, structural analysis, and gravity modeling was conducted to distinguish between the two explanations. The study area lies in the southern section of the state of Nuevo Leon near the town of Aramberri. In this area the fold-thrust belt involves upper Cretaceous shale, Mesozoic carbonate rocks and crystalline basement. Thrusting is directed approximately due east. The structural style is characterized by fault bend folds. Mapping shows that evaporite rocks, at least locally, are not exploited as a regional slip surface and the thrust fault roots into the basement. Cross-sections across the study area indicate approximately 6.5 km east-west shortening (19%). Three forward 2-D structural models aid in constraining the fault geometry at depth and show the reactivation of half graben faults as a means for the main thrust to step up to shallower levels. These data are consistent with a single phase of deformation which involves movement along a thrust system that creates the structure in the study area implying magnitude of depth to detachment to be greater for this area of the SMO.

  13. Nature of the Yucatan Block Basement as Derived From Study of Granitic Clasts in the Impact Breccias of Chicxulub Crater

    Science.gov (United States)

    Vera-Sanchez, P.; Rebolledo-Vieyra, M.; Perez-Cruz, L.; Urrutia-Fucugauchi, J.

    2008-05-01

    The tectonic and petrologic nature of the basement of the Yucatan Block is studied from analyses of basement clasts present in the impact suevitic breccias of Chicxulub crater. The impact breccias have been sampled as part of the drilling projects conducted in the Yucatan peninsula by Petroleos Mexicanos, the National University of Mexico and the Chicxulub Scientific Drilling Project. Samples analyzed come mainly from the Yaxcopoil-1, Tekax, and Santa Elena boreholes, and partly from Pemex boreholes. In this study we concentrate on clasts of the granites, granodiorites and quartzmonzonites in the impact breccias. We report major and trace element geochemical and petrological data, which are compared with data from the granitic and volcanic rocks from the Maya Mountains in Belize and from the Swannee terrane in Florida. Basement granitic clasts analyzed present intermediate to acidic sub-alkaline compositions. Plots of major oxides (e.g., Al2O3, Fe2O3, TiO2 and CaO) and trace elements (e.g., Th, Y, Hf, Nb and Zr) versus silica allow separation of samples into two major groups, which can be compared to units in the Maya Mountains and in Florida basement. The impact suevitic breccia samples have been affected by alteration likely related to the hydrothermal processes associated with the crater melt sheet. Cloritization, seritization and fenitization alterations are recognized, due to the long term hydrothermalism. Krogh et al. (1993) reported U-Pb dates on zircons from the suevitic breccias, which gave dates of 545 +/- 5 Ma and 418 +/- 6 Ma, which were interpreted in terms of the deep granitic metamorphic Yucatan basement. The younger date correlates with the age for the Osceola Granite and the St. Lucie metamorphic complex of the Swannee terrane in the Florida peninsula. The intrusive rocks in the Yucatan basement may be related to approx. 418 Ma ago collisional event in the Late Silurian.

  14. The offshore basement of Perú: Evidence for different igneous and metamorphic domains in the forearc

    Science.gov (United States)

    Romero, Darwin; Valencia, Kiko; Alarcón, Pedro; Peña, Daniel; Ramos, Victor A.

    2013-03-01

    As a result of new studies carried out in the offshore of Perú during the exploration and hydrocarbon evaluation of the forearc basins, new U-Pb SHRIMP and TIMS in zircons and some Ar-Ar data were obtained in the metamorphic and igneous basement. The understanding of this basement was critical to evaluate different hypotheses that have been proposed for the tectonic evolution of pre-Andean crust of Perú. Recent research performed in the basement rocks of the Marañón Massif in northern Perú, claimed that west of this area was a basement-free region in the Paleozoic, where the arc and forearc were developed in a mafic quasi-oceanic crust. However, petrographic studies and new preliminary ages indicate, for the first time, the nature and age of this sialic basement. Reconnaissance studies were performed in several offshore islands, as the Las Hormigas de Afuera Island west of Lima, and Macabí and Lobera islands along the edge of the continental platform. These data were complemented with the studies of some cutting samples obtained in recent exploration wells in northern Perú. The results of the present work show two large crustal domains in the Peruvian offshore forearc. A northern domain contains late Paleozoic igneous rocks that appear to be the southern offshore continuation of the Amotape-Tahuin block, which is interpreted as the southernmost remnant of the Laurentia Alleghenian orogen. The central offshore domain, known as the Paracas High, corresponds to the outer shelf high of previous studies. It contains orthogneisses of Grenville-age, probably recrystallized during an Ordovician magmatic episode. The new results show that the central offshore of Perú is an extension of the Grenville-age basement affected by Famatinian, early Paleozoic magmatism, well exposed in the southern domain in the Arequipa Massif along the coast of southern Perú.

  15. An analysis of radon levels in the basements of UK workplaces and review of when employers should test.

    Science.gov (United States)

    Gooding, Tracy Dawn

    2018-01-12

    Radon is a recognised lung carcinogen. The main source of radon in UK buildings is the ground. As basements have more ground contact than other parts of a building, and often limited ventilation, there is an increased potential for high radon levels to occur. Regulations are in place in the UK to control radon exposures at work, which prompt employers to undertake risk assessments, test where necessary and take action if levels exceed certain thresholds. Both Public Health England (PHE) and the Health and Safety Executive (HSE) currently advise employers to test routinely occupied basements for radon, irrespective of geographical location; the minimum annual occupancy threshold has been established as one hour per week (approximately 50 hours per year). PHE operates a routine measurement service for employers and holds the measurement data for radon levels in the basements of more than 3500 workplaces. Typically, radon concentrations follow a lognormal distribution over a wide range, when corrections are made for seasonal variations and the contribution of outside air. Statistical tests on these data show good correspondence in most cases for workplace basements of seven different occupational sectors. Radon should be included in the risk assessments of basements of all workplace types irrespective of the radon Affected Area status (i.e. whether they are located in a geographical unit where higher proportions of houses or workplaces are likely to exceed their reference levels). As a consequence of this analysis, however, evidence is presented that supports a change in the existing protocols on when to test the radon levels in basements if a more sophisticated approach were to be adopted that takes into account the Affected Area status. © 2017 IOP Publishing Ltd.

  16. K-Ar age constrains on chemically weathered granitic basement rocks (saprolites) in Scandinavia

    Science.gov (United States)

    Margreth, Annina; Fredin, Ola; Viola, Giulio; Knies, Jochen; Sørlie, Ronald; Lie, Jan-Erik; Margrethe Grandal, Else; Zwingmann, Horst; Vogt, Christoph

    2017-04-01

    Remnants of in-situ weathered bedrock, saprolite, are found in several locations in Scandinavia. Saprolites contain important information about past climate conditions and landscape evolution, although their age and genesis are commonly difficult to constrain. It is generally thought that clay-poor, coarse-grained (arêne) saprolites, mostly occurring as thin regolith blankets or in larger outcrops, formed in temperate climate during the Cenozoic, whereas clay-rich (argillic) saprolites, commonly restricted to small, fracture-bounded outcrops, formed in (sub-)tropical climate during the Mesozoic. Recent methodological and conceptual advances in K-Ar dating of illite-bearing fault rocks have been applied to date clay-rich saprolites. To test the K-Ar dating technique for saprolites, we first selected an offshore site in the Viking Graben of the North Sea, where weathered and fractured granitic basement highs have been drilled during petroleum exploration, and an abandoned kaolin mine in Southern Sweden. Both targets provide independent age control through the presence of overlying Mesozoic sedimentary rocks. Clay-rich saprolites occurring in fractured basement rocks were additionally sampled in a joint valley landscape on the southwestern coast of Norway, which can be regarded as the possible onland correlative to the offshore basement high. In order to offer a sound interpretation of the obtained K-Ar ages, the mineralogical and chemical composition of the saprolites requires a thorough characterization. Scanning electron microscopy of thin sections, integrated by XRD and XRF analysis, reveals the progressive transformation of primary granitic rock minerals into secondary clay minerals. The authigenesis of illite is particularly important to understand, since it is the only K-bearing clay mineral that can be dated by the K-Ar method. K-feldspars and mica are the common primary K-bearing minerals, from which illite can be formed. While progressive leaching of

  17. Nature of the basement of the East Anatolian plateau: Implications for the lithospheric foundering processes

    Science.gov (United States)

    Topuz, G.; Candan, O.; Zack, T.; Yılmaz, A.

    2017-12-01

    The East Anatolian Plateau (Turkey) is characterized by (1) an extensive volcanic-sedimentary cover of Neogene to Quaternary age, (2) crustal thicknesses of 42-50 km, and (3) an extremely thinned lithospheric mantle. Its basement beneath the young cover is thought to consist of oceanic accretionary complexes of Late Cretaceous to Oligocene age. The attenuated state of the lithospheric mantle and the causes of the young volcanism are accounted for by slab steepening and subsequent break-off. We present field geological, petrological and geochronological data on three basement inliers (Taşlıçay, Akdağ and Ilıca) in the region. These areas are made up of amphibolite- to granulite-facies rocks, comprising marble, amphibolite, metapelite, quartzite and metagranite. The granulite-facies domain is equilibrated at 0.7 GPa and 800 ˚C at 83 ± 2 Ma (2σ). The metamorphic rocks are intruded by subduction-related coeval gabbroic, quartz monzonitic to tonalitic rocks. Both the metamorphic rocks and the intrusions are tectonically overlain by ophiolitic rocks. All these crystalline rocks are unconformably overlain by lower Maastrichtien clastic rocks and reefal limestone, suggesting that the exhumation at the earth's surface and juxtaposition with ophiolitic rocks occurred by early Maastrichtien. U-Pb dating on igneous zircon from metagranite yielded a protolith age of 445 ± 10 Ma (2σ). The detrital zircons from a metaquartzite point to Neoproterozoic to Early Paleozoic provenance. All these data favor a more or less continuous continental substrate to the allochthonous ophiolitic rocks beneath the young volcanic-sedimentary cover. The metamorphism and coeval magmatism can be regarded as the middle- to lower-crustal root of the Late Cretaceous magmatic arc that developed due to northward subduction along the Bitlis-Zagros suture. The presence of a continental basement beneath the young cover requires that the loss of the lithospheric mantle from beneath the East

  18. Recent understanding of the Svalbard basement in the light of new radiometric age determinations

    International Nuclear Information System (INIS)

    Ohta, Y.

    1992-01-01

    Several tectonothermal events in the pre-Carboniferous basement of Svalbard during Caledonian and Proterozoic times have been dated recently by radiometric age determinations. Three or four stages have been recognized in the Caledonian period; a post-orogentic graben formation during the Devonian, a late Caledonian event in the Middle Silurian, an earely Caledonian event in the Middle Ordovician and possibly an earliest event in the Middle to Late Cambrian. The Grenvillian event, 950-1270 Ma, has been well established by both radiometric ages and unconformities in Nordaustlandet and southwestern Spitsbergen. Sveco-Karelian ages, 1670-1750 Ma, also have been obtained from Ny Friesland, northerneastern Spitsbergen. Two even older ages (zircon U-Pb) upper intercept ages), 2.1 and 3.2 Ga, may suggest the presence of still older crust in Svalbard and adjacent areas. 40 refs., 2 figs

  19. Effects of Water and Low-Medium Temperature on Limestone from Mt Etna basement

    Science.gov (United States)

    Castagna, Angela; Ougier-Simonin, Audrey; Benson, Philip; Browning, John; Fazio, Marco; Walker, Richard; Vinciguerra, Sergio

    2017-04-01

    Mount Etna volcano, Sicily, sits atop a structurally complex sedimentary basement continuously subjected to tectonic deformation. The flyschoid formations belonging to the Appenninic-Maghrebian Chain (AMC) and making up the accretionary wedge of a regional fold-and-thrust belt lie above carbonate Hyblean Plateau (HP) sequences, belonging to the African plate. Carbonate rocks represent a major component of the sedimentary basement: they are spread throughout the AMC as continuous strata and discontinuous lenses, and are the main constituent (e.g., Comiso Limestone) of the HP foreland. Etna is an active volcanic environment, characterized by complex stress field distributions, magmatic and non-magmatic fluid circulation, and elevated temperature gradients; the edifice has been constructed at various rates and with variable distribution of effusive products. These intrinsic and extrinsic parameters are known to impact the rheological behaviour of rocks. Previous triaxial deformation studies on carbonates (Tavel Limestone, Solnhofen Limestone and Comiso Limestone) have shown the importance of temperature, and the presence of water as pore fluid, on the mechanical strength and failure mode of the rocks. However, to our knowledge, no previous studies have considered the distal heating effect of intrusions on the carbonate mechanical strength from the basement. Here we investigate the behaviour under varying P-T conditions at constant strain rate (10-5 s-1) on both dry and water saturated samples of Comiso Limestone, a low-porosity (10.2% average) carbonate rock belonging to the HP. We ran separate conventional triaxial experiments at various confining effective pressure from 0 up to 50 MPa at room temperature (20°C), in both dry and drained water-saturated conditions, using natural samples, and thermally-treated samples (150°C, 300°C, and 450°C). Acoustic Emissions and P-wave velocities were recorded during the experiments. Sample failure covers the brittle and

  20. Dynamics of Uranium Ore Formation in the Basement and Frame of the Streltsovskaya Caldera

    International Nuclear Information System (INIS)

    Petrov, V.A.; Poluektov, V.V.; Schukin, S.I.; Tolstobrov, V.A.

    2014-01-01

    Conclusions and challenges: 1. Caldera Granitic Frame: Urtuy and Bambakay Massifs: • AR-PR 800 Ma (relics?) granite-gneiss and PZ1 520 Ma (Caledonian) granites • NE-SW main fluid conducting faults; • Uraninite formation 770±35 Ma and transformation 195±79 Ma (U-Pb). 2. Caldera Basement U Deposits: • AR-PR 800 Ma (relics) granite-gneiss (Argun deposit); • PZ2 240 Ma (Variscian) granites (Antey deposit); • NNE-submeridional main fluid conducting faults; • Hydromicatization 131-139 Ma; • U (economic) ores formation 133-135 Ma 3. Long-term fluid circulation in the ore-forming fluid-magmatic system: • Chronology; • Depth (source and PT conditions); • Pathways; • Transport mechanisms; • Stress-strain-temperature field evolution