Read by QxMD icon Read


Celestial R Jones-Paris, Sayan Paria, Taloa Berg, Juan Saus, Gautam Bhave, Bibhash C Paria, Billy G Hudson
Basement membranes (BMs) are specialized extracellular scaffolds that influence behaviors of cells in epithelial, endothelial, muscle, nervous, and fat tissues. Throughout development and in response to injury or disease, BMs are fine-tuned with specific protein compositions, ultrastructure, and localization. These features are modulated through implements of the BM toolkit that is comprised of collagen IV, laminin, perlecan, and nidogen. Two additional proteins, peroxidasin and Goodpasture antigen-binding protein (GPBP), have recently emerged as potential members of the toolkit...
September 9, 2016: Matrix Biology: Journal of the International Society for Matrix Biology
William F Clark, Shih-Han S Huang, Michael W Walsh, Myriam Farah, Ainslie M Hildebrand, Jessica M Sontrop
The purpose of this review is to examine the evidence supporting the application of plasma exchange in renal disease. Our review focuses on the following 6 most common renal indications for plasma exchange based on 2014 registry data from the Canadian Apheresis Group: (i) thrombotic thrombocytopenic purpura (TTP)/hemolytic uremic syndrome; (ii) renal transplantation, (iii) anti-neutrophil cytoplasm antibodies-associated vasculitis, (iv) cryoglobulinemia, (v) focal segmental glomerulosclerosis, and (vi) Goodpasture syndrome...
November 2016: Kidney International
Mary H Foster
Basement membrane components are targets of autoimmune attack in diverse diseases that destroy kidneys, lungs, skin, mucous membranes, joints, and other organs in man. Epitopes on collagen and laminin, in particular, are targeted by autoantibodies and T cells in anti-glomerular basement membrane glomerulonephritis, Goodpasture's disease, rheumatoid arthritis, post-lung transplant bronchiolitis obliterans syndrome, and multiple autoimmune dermatoses. This review examines major diseases linked to basement membrane autoreactivity, with a focus on investigations in patients and animal models that advance our understanding of disease pathogenesis...
August 2, 2016: Matrix Biology: Journal of the International Society for Matrix Biology
Rui Fernandes, Sara Freitas, Pedro Cunha, Gloria Alves, Jorge Cotter
BACKGROUND: Goodpasture's syndrome, a rare disease, is an organ-specific autoimmune disease mediated by anti-glomerular basement membrane antibodies. Its pathology is characterized by crescentic glomerulonephritis with linear immunofluorescent staining for immunoglobulin G on the glomerular basement membrane. Although rare, a few cases with absence of circulating anti-glomerular membrane antibodies have been described. CASE PRESENTATION: The objective of this clinical case report is to describe and discuss a case of a 27-year-old white man who was hospitalized with a 1-year history of weight loss and a 1-month history of hemoptysis, with aggravation  the day before, having developed dyspnea and cough in the previous 24 hours...
2016: Journal of Medical Case Reports
Mary H Foster, Elizabeth S Buckley, Benny J Chen, Kwan-Ki Hwang, Amy G Clark
Autoantibodies mediate organ destruction in multiple autoimmune diseases, yet their origins in patients remain poorly understood. To probe the genetic origins and structure of disease-associated autoantibodies, we engrafted immunodeficient mice with human CD34+ hematopoietic stem cells and immunized with the non-collagenous-1 (NC1) domain of the alpha3 chain of type IV collagen. This antigen is expressed in lungs and kidneys and is targeted by autoantibodies in anti-glomerular basement membrane (GBM) nephritis and Goodpasture syndrome (GPS), prototypic human organ-specific autoimmune diseases...
August 2016: Molecular Immunology
Mary H Foster
Animal models are indispensable for the study of glomerulonephritis, a group of diseases that destroy kidneys but for which specific therapies do not yet exist. Novel interventions are urgently needed, but their rational design requires suitable in vivo platforms to identify and test new candidates. Animal models can recreate the complex immunologic microenvironments that foster human autoimmunity and nephritis and provide access to tissue compartments not readily examined in patients. Study of rat Heymann nephritis identified fundamental disease mechanisms that ultimately revolutionized our understanding of human membranous nephropathy...
September 1, 2016: American Journal of Physiology. Renal Physiology
Rafael Ponikvar, Jakob Gubenšek, Jadranka Buturović Ponikvar
In 1996 we performed tandem membrane plasma exchange-hemodialysis in a 3-year-old girl and tandem immunoadsorption-hemodialysis with citrate as the only anticoagulant in a patient with Goodpasture's syndrome. In the present study, we evaluated the feasibility, efficacy and safety of 24 tandem plasma exchange/immunoadsorption hemodialysis procedures in four different circuit setups with citrate as the only anticoagulant. In two setups, the tandem procedures were connected in series (plasma exchange hemodialysis and immunoadsorption hemodialysis), while in the other two setups they were in parallel (plasma exchange hemodialysis with independent blood circuits and plasma exchange hemodialysis with independent arterial blood lines, but with a common return line)...
June 2016: Therapeutic Apheresis and Dialysis
Antoine Huart, Anne-Gaëlle Josse, Dominique Chauveau, Jean-Michel Korach, Farhad Heshmati, Eric Bauvin, Olivier Cointault, Nassim Kamar, David Ribes, Jacques Pourrat, Stanislas Faguer
The overall and renal outcomes of patients with Goodpasture syndrome (GS), a rare autoimmune disorder characterized by circulating anti-GBM antibodies and rapidly progressive glomerulonephritis and/or pulmonary hemorrhage, have mostly been reported in small-sized cohorts or by aggregating patients receiving a variety of therapies that include aggressive (i.e., combined plasma exchanges, corticosteroids, and cyclophosphamide) and less aggressive (i.e., either plasma exchanges or immunosuppressive drugs, or no treatment)...
September 2016: Journal of Autoimmunity
S B Mandarapu, K K Mukku, S B Raju, S Chandragiri
No abstract text is available yet for this article.
May 2016: Indian Journal of Nephrology
Zhao Cui, Ming-Hui Zhao, Xiao-Yu Jia, Miao Wang, Shui-Yi Hu, Su-Xia Wang, Feng Yu, Kyle L Brown, Billy G Hudson, Vadim Pedchenko
Autoantibody against glomerular basement membrane (GBM) plays a direct role in the initiation and development of Goodpasture's (GP) disease. The principal autoantigen is the non-collagenous domain 1 (NC1) of α3 chain of collagen IV, with two immunodominant epitopes, EA-α3 and EB-α3. We recently demonstrated that antibodies targeting α5NC1 are bound to kidneys in GP patients, suggesting their pathogenic relevance. In the present study, we sought to assess the pathogenicity of the α5 autoantibody with clinical and animal studies...
June 2016: Journal of Autoimmunity
Beatrice Feragalli, Cesare Mantini, Marco Sperandeo, Michele Galluzzo, Giovanni Belcaro, Armando Tartaro, Antonio R Cotroneo
The respiratory system may be involved in all systemic vasculitides, although with a variable frequency. The aim of our review is to describe radiographic and high-resolution CT (HRCT) findings of pulmonary vasculitides and to correlate radiological findings with pathological results. Lung disease is a common feature of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitides, including granulomatosis with polyangiitis (Wegener's), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) and microscopic polyangiitis...
2016: British Journal of Radiology
F Fang, Y M Li, S T Hu, H T Wang, D G Liu, C Wang
OBJECTIVE: To improve knowledge about the clinical and pathological features of diffuse alveolar hemorrhage (DAH). METHODS: Six cases DAH with intact clinical and pathological data were retrospectively analyzed during the period from May 1999 to May 2015 in Beijing Hospital. There were altogether 2 males and 4 females, with age ranging from 32 to 68 years (mean 58.8 years). Specimens were obtained by autopsy (3 cases), open lung biopsy (2 cases) and renal biopsy (2 cases), including 1 case of open lung biopsy in 2003, renal biopsy in 2012...
January 12, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Mira Pavkovic, Björn Riefke, Anna-Lena Frisk, Ina Gröticke, Heidrun Ellinger-Ziegelbauer
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...
December 2015: Genomics Data
Bethel Shiferaw, Viktor Miro, Carroll Smith, Jagadish Akella, Walter Chua, Zae Kim
Goodpasture's disease is an uncommon composite of features including renal failure with pulmonary hemorrhage secondary to an autoimmune response that specifically targets these organ systems. We present a case of particular interest in regards to atypical presentation, and the uncommon treatment that the patient underwent. A 65-year-old Afghani female arrived with complaints of nausea, vomiting, loss of appetite, malaise, decreased urine output, exertional dyspnea, and cough. The patient presented initially with renal failure and unexpectedly developed respiratory failure after hemodialysis...
January 2016: Journal of Clinical Medicine Research
Piero Marson, Maria Teresa Gervasi, Tiziana Tison, Anna Colpo, Giustina De Silvestro
It is widely known that pregnancy does not represent a contraindication to therapeutic apheresis (TA) techniques. In fact, since the first experiences of TA in pregnancy for the prevention of hemolytic disease of the newborn, several diseases are at present treated with TA, mainly within 6 clinical categories: (a) TA is a priority and has no alternative equally effective treatment (e.g., thrombotic thrombocytopenic purpura); (b) TA is a priority but there are alternative therapies not contraindicated in pregnancy (e...
December 2015: Transfusion and Apheresis Science
Ewout J Hoorn, Noor E Taams, Tiina Hurskainen, Mahdi Salih, Jan J Weening, Marcel F Jonkman, Hendri H Pas, Marco W J Schreurs
A 75-year-old man presented with a blistering skin disease and nephrotic syndrome. Bullous pemphigoid was diagnosed by linear immunoglobulin G (IgG) and C3 staining along the basement membrane zone of a skin biopsy specimen and by the presence of circulating IgG recognizing the 180-kDa bullous pemphigoid antigen (BP180; type XVII collagen). A kidney biopsy specimen showed endocapillary inflammation without crescents. Direct immunofluorescence showed strong IgG and C3 staining in a combined granular and linear pattern along the glomerular basement membrane...
February 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Sarah Friend, Stephen James Carlan, Jack Wilson, Mario Madruga
BACKGROUND: Active Goodpasture disease (GD) in pregnancy is extremely rare and can result in significant maternal and fetal morbidity. Relapse of GD in a subsequent pregnancy has not been reported. Factors associated with a relapse of GD are unknown. CASE: A woman in her second pregnancy presented with clinical findings of preeclampsia and biopsy evidence of GD in the third trimester. Her first puerperium was also complicated by GD, requiring temporary hemodialysis...
September 2015: Journal of Reproductive Medicine
M Manera, C Borreca, B S Dezfuli
Histopathology, histochemistry and immunohistochemistry of the integument of European eel, Anguilla anguilla (Linnaeus, 1758), infected by Myxidium sp. are reported. Skin samples from affected and unaffected eels were dissected, formalin fixed, paraffin embedded, sectioned and stained with H&E, Periodic acid-Schiff's staining method, Alcian Blue 8 GX pH 2.5/Periodic acid-Schiff's and McCallum-Goodpasture's Gram stain. Moreover, immunohistochemistry was performed using a primary polyclonal laminin antibody. Histologically, cysts (diameter 2-3 mm) were observed mainly under the scale pockets, encircled by a thin collagen layer, lined by elongated, flattened fibroblasts and containing bipolar, PAS- and Gram-positive spores with opposite polar capsules...
July 2016: Journal of Fish Diseases
Antonio Amoroso
There are many evidences that the HLA genes are risk factors for kidney complex disease for the pathogenesis of which an abnormal response of the immune system is involved. In this review, we present the latest knowledge about the genetics of the HLA complex, the molecules encoded by the HLA genes, their polymorphism and the physiological role of the HLA system in the defense against infections. It then addresses the issue of the association between specific alleles of HLA and renal disorders. Many kidney diseases have been described associated with HLA...
2015: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
Marie Bourgault, Damien Sarret, Pierre Isnard, Marion Rabant, Jacques Labaye
We report the case of a patient suffering from atypical hemolytic uremic syndrome with inaugural intra-alveolar hemorrhage. Clinical features and detection of circulating anti-glomerular basal membrane antibodies first raise the possibility of a Goodpasture syndrome. Renal biopsy allows to correct the diagnosis. Partial remission is obtained thanks to specific care and eculizumab infusions.
December 2015: Néphrologie & Thérapeutique
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"