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idiopathic membranous

Claudio Bazzi, Tomoko Usui, Virginia Rizza, Daniela Casellato, Maurizio Gallieni, Masaomi Nangaku
BACKGROUND: The clinical course of idiopathic membranous nephropathy (IMN) varies from spontaneous remission of nephrotic syndrome (NS) to end-stage renal disease (ESRD). Selecting patients with high risk of progression for immunosuppressive therapy is mandatory. METHODS: 86 IMN subjects were followed for median of 69 months (range 6-253). Receiver operating characteristic curve and Cox proportional hazards model were used to evaluate prognostic factors for progression, defined as ESRD or eGFR reduction ≥50% of baseline...
October 20, 2016: Nephrology
Shumpei Obata, Masato Fujikawa, Keisuke Iwasaki, Masashi Kakinoki, Osamu Sawada, Yoshitsugu Saishin, Hajime Kawamura, Masahito Ohji
PURPOSE: To investigate anatomic changes in retinal thickness (RT) and functional changes after vitrectomy for idiopathic epiretinal membranes (ERMs) with and without internal limiting membrane (ILM) peeling. METHODS: The medical records of 100 eyes of 96 patients with ERM who underwent vitrectomy and ERM removal were reviewed retrospectively. The RT was measured by optical coherence tomography, and the area was divided into 9 sections. The best-corrected visual acuity (BCVA), 9 RT areas, and incidence rates of recurrent ERM were compared between the groups with and without ILM peeling before the operation and 12 months postoperatively...
October 20, 2016: Ophthalmic Research
Daniel C Cattran, Esther D Kim, Heather Reich, Michelle Hladunewich, S Joseph Kim
Although change in proteinuria has been proposed as a surrogate for long-term prognosis in membranous nephropathy (MGN), variability in proteinuria levels and lag between these changes and acceptable end points, such as ESRD, has limited its utility. This cohort study examined the prognostic significance of remission duration in 376 patients with biopsy-proven idiopathic/primary MGN who achieved a remission after a period of nephrotic-range proteinuria. We defined complete remission (CR), partial remission (PR), and relapse as proteinuria ≤0...
October 18, 2016: Journal of the American Society of Nephrology: JASN
Malene Rask Andersen, Muhammad Farooq, Karen Koefoed, Klaus W Kjaer, Ane Simony, Sren Tvorup Christensen, Lars Allan Larsen
STUDY DESIGN: Mutation analysis of a candidate disease gene in a cohort of patients with moderate to severe Adolescent idiopathic scoliosis (AIS). OBJECTIVE: To investigate if damaging mutations in the planar cell polarity gene VANGL1 could be identified in AIS patients. SUMMARY OF BACKGROUND DATA: AIS is a spinal deformity which occurs in 1-3% of the population. The cause of AIS is often unknown, but genetic factors are important in the etiology...
October 17, 2016: Spine
Crystal N Marconett, Beiyun Zhou, Mitsuhiro Sunohara, Tiffany M Pouldar, HongJun Wang, Yixin Liu, Megan E Rieger, Evelyn Tran, Per Flodby, Kimberly D Siegmund, Edward D Crandall, Ite A Laird-Offringa, Zea Borok
Diseases involving the distal lung alveolar epithelium include chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF) and lung adenocarcinoma. Accurate labeling of specific cell types is critical for determining the contribution of each to pathogenesis of these diseases. The distal lung alveolar epithelium is comprised of two cell types, alveolar epithelial type 1 (AT1) and type 2 (AT2) cells. While cell type-specific markers, most prominently surfactant protein C (SFTPC), have allowed detailed lineage tracing studies of AT2 cell differentiation and their roles in disease, studies of AT1 cells have been hampered by lack of genes with expression unique to AT1 cells...
October 17, 2016: American Journal of Respiratory Cell and Molecular Biology
Katarzyna Osipowicz, Agnieszka Kalinska-Bienias, Cezary Kowalewski, Katarzyna Wozniak
Haemodialysis is the most frequent form of renal replacement therapy (RRT) in patients with end-stage renal disorder (ESRD). Patients with ESRD frequently develop skin problems, mainly xerosis, pruritus and hyperpigmentation, as well as bullous diseases, mainly porphyria or pseudoporphyria and, in some cases, bullous pemphigoid (BP). BP is the most common autoimmune sub-epidermal blistering disease, and it predominantly affects elderly people. Clinically, BP is characterised by generalised pruritic, bullous eruptions and urticaria-like lesions...
October 17, 2016: International Wound Journal
Mercedes Acevedo Ribó, Francisco Javier Ahijado Hormigos, Francisco Díaz, Marta Romero Molina, Maria Angeles Fernandez Rojo, Maria Antonia Garcia Rubiales, Eugenio García Diaz
This case report describes a patient with a previous history of autoimmune pancreatitis secondary to IgG4-related disease, who developed an overt nephrotic syndrome due to membranous nephropathy, surprisingly idiopathic. In all the previously described cases with both concurrent diseases, membranous nephropathy was considered to be secondary to the IgG4-related disease based on the absence of anti-PLA2R1 autoantibodies, and nephrotic syndrome usually remitted after treatment with steroids alone. However, in our patient positivity of serum anti-PLA2R1 autoantibodies together with a normal serum IgG4 level, and the absence of the other most commonly associated diseases were compatible with an idiopathic membranous nephropathy...
October 14, 2016: Clinical Nephrology
Sayaka Myojin, Takeru Yoshimura, Shigeo Yoshida, Atsunobu Takeda, Yusuke Murakami, Yoichi Kawano, Yuji Oshima, Tatsuro Ishibashi, Koh-Hei Sonoda
PURPOSE: The analysis of gene expression in idiopathic epiretinal membranes (iERMs) may help elucidate ERM formation and its pathology. Here, we conducted a case-control study, in order to determine the expression levels of cytokines and other genes in eyes with macular hole (MH) or iERM. METHODS: Twenty eyes, obtained from seven male and 13 female patients, were included in the study. The average age of the study subjects was 69.1 ± 7.67 years, and 15 eyes had iERM, while five eyes had MH...
2016: PloS One
Ivana Dedinská, Daniel Svetlík, Katarina Adamicova, Katarina Machalekova, Pavel Makovicky, Alena Jezikova, Ludovít Laca, Juraj Miklusica, Peter Galajda, Marian Mokan
Treatment of retroperitoneal fibrosis usually involves corticosteroids with or without other immunomodulating medications or tamoxifen.  Rituximab, a monoclonal antibody that specifically targets CD20 on the surface of B-cells, is effective in achieving complete remission of proteinuria in patients with idiopathic membranous nephropathy. We describe a case of a 45 years old man with idiopathic membranous glomerulonephritis (with proteinuria of more than 30 grams/24 hours) and simultaneously with idiopathic retroperitoneal fibrosis (with large number of cells CD20 in the histologic image)...
September 2016: Iranian Journal of Kidney Diseases
Simone Donati, Simona Maria Caprani, Francesco Semeraro, Riccardo Vinciguerra, Gianni Virgili, Francesco Testa, Francesca Simonelli, Claudio Azzolini
PURPOSE: To analyze functional and morphological findings after surgery for idiopathic epiretinal macular membrane (IEMM). MATERIALS AND METHODS: Twenty eyes of 20 patients affected by IEMM underwent 23-Gauge surgery. Morphological and functional examinations were assessed at baseline and at 30, 90, and 180 days after surgery. SD-OCT evaluated foveal morphology and thickness, photoreceptor inner/outer segment junction, and external limiting membrane. Functional assessment evaluated visual acuity, retinal sensitivity, and fixation patterns...
August 10, 2016: Seminars in Ophthalmology
K Dahan
Membranous nephropathy (MN) accounts for about 20% of cases of nephrotic syndrome in the adult. Thickening of glomerular capillary walls results from subepithelial formation of immune deposits containing IgG and the membrane attack complex of complement, which is the major mediator of proteinuria, and antigens. Idiopathic forms of MN (IMN) represent 70 to 80% of all cases. A major breakthrough was the identification of the podocyte antigen PLA2R as the target of circulating antibodies in about 70% of IMN, which confirmed that the disease was auto-immune in nature...
May 25, 2016: La Revue de Médecine Interne
Bindu Rajesh, Anantharaman Giridhar
PURPOSE: To report the occurrence of macular telangiectasia (Mac Tel) Type 2 in a 28-year-old lady. METHODS: Retrospective case report. RESULTS: A 28-year-old obese diabetic lady presented with mild blurring of vision and reading difficulty for 2 months. Clinical examination revealed the presence of myopic refraction and features suggestive of macular telangiectasia Type 2 on fundus evaluation. Fundus fluorescein angiography and optical coherence tomography confirmed the diagnosis of Mac Tel Type 2, Stage 3...
September 28, 2016: Retinal Cases & Brief Reports
Claire Dossier, Agnès Jamin, Georges Deschênes
Steroid sensitive nephrotic syndrome is marked by a massive proteinuria and loss of podocytes foot processes. The mechanism of the disease remains debated but recent publications suggest a primary role of EBV. EBV replication in the peripheral blood is found in 50% of patients during the first flare of the disease. The genetic locus of steroid sensitive nephrotic syndrome was also identified as influencing antibodies directed against EBNA1. EBV is able to establish, latent benign infection in memory B cells that display phenotypes similar to antigen-selected memory B cells...
September 28, 2016: Pediatric Research
Alastair J Rankin, Emily P McQuarrie, Jonathan G Fox, Colin C Geddes, Bruce MacKinnon
BACKGROUND: The reported incidence of venous thromboembolism (VTE) in patients with nephrotic syndrome (NS) varies widely, as does the approach to prophylactic anticoagulation. We aimed to assess the incidence of VTE in patients with primary NS in order to inform a sample size calculation to determine if a future clinical trial will ever be feasible. METHODS: All adults undergoing native renal biopsy for NS between 2008 and 2013 yielding a diagnosis of primary glomerulonephritis were identified...
September 24, 2016: Nephron
Michael R R Böhm, Christoph Tappeiner, Marc A Breitbach, Beatrix Zurek-Imhoff, Carsten Heinz, Arnd Heiligenhaus
PURPOSE: To analyze occurrence, risk factors, and course of ocular hypotony (OH) in juvenile idiopathic arthritis-associated uveitis (JIAU). DESIGN: Cohort study METHODS: Epidemiological and ophthalmological data at baseline and during follow-up of JIAU patients with and without ocular hypotony were evaluated. RESULTS: OH developed in 57 of the 365 JIAU patients during the follow-up (mean 4.5±3.5 years). In 40 patients with follow-up ≥ 12 months, OH was unrelated to previous ocular surgery: risk factors at baseline (univariate logistic regression analysis) included longer total duration of uveitis (OR 1...
September 21, 2016: American Journal of Ophthalmology
Claudia Neul, Elke Schaeffeler, Alex Sparreboom, Stefan Laufer, Matthias Schwab, Anne T Nies
Small-molecule inhibitors of tyrosine kinases (TKIs) are the mainstay of treatment for many malignancies and represent novel treatment options for other diseases such as idiopathic pulmonary fibrosis. Twenty-five TKIs are currently FDA-approved and >130 are being evaluated in clinical trials. Increasing evidence suggests that drug exposure of TKIs may significantly contribute to drug resistance, independently from somatic variation of TKI target genes. Membrane transport proteins may limit the amount of TKI reaching the target cells...
September 19, 2016: Trends in Pharmacological Sciences
Akira Hagiwara, Takayuki Baba, Tomoaki Tatsumi, Eiju Sato, Toshiyuki Oshitari, Shuichi Yamamoto
PURPOSE: To determine the relationship between preoperative and postoperative characteristics and visual outcomes after successful surgery for unclosed macular hole (MH). METHODS: The medical charts of 166 eyes of 161 patients who underwent vitrectomy for a MH were reviewed. The MH was not closed in 9 eyes of 9 patients after the initial surgery. The second vitrectomy with additional internal limiting membrane (ILM) peeling and gas tamponade was performed. The preoperative and postoperative best-corrected visual acuity (BCVA), optical coherence tomographic images, MH size, duration of MH before the first operation, and area of ILM peeling were studied...
September 9, 2016: European Journal of Ophthalmology
Mei Chin Lim, Niketa Chandrakant Chotai, Danilo Medina Giron
We present a case of a previously healthy 50-year-old gentleman who had recurrent vomiting and abdominal pain of two-month duration. The patient was subsequently diagnosed with abdominal cocoon on computed tomography. Idiopathic sclerosing encapsulating peritonitis, also known as abdominal cocoon, is a rare cause of small bowel obstruction. Visualization of variable encasement of the small bowel loops by a characteristic membranous sac, either preoperatively with cross-sectional imaging or intraoperatively, is the key to diagnosis...
2016: Case Reports in Medicine
Sarah J Annesley, Sui T Lay, Shawn W De Piazza, Oana Sanislav, Eleanor Hammersley, Claire Y Allan, Lisa M Francione, Minh Q Bui, Zhi-Ping Chen, Kevin R W Ngoei, Flora Tassone, Bruce E Kemp, Elsdon Storey, Andrew Evans, Danuta Z Loesch, Paul R Fisher
In combination with studies of post-mortem PD brains, pharmacological and genetic models of Parkinson's Disease (PD) have suggested that two fundamental, interacting cellular processes are impaired - proteostasis and mitochondrial respiration. We have re-examined the role of mitochondrial dysfunction in lymphoblasts isolated from patients with idiopathic PD and an age-matched control group. As previously reported for various PD cell types, the production of reactive oxygen species (ROS) by PD lymphoblasts was significantly elevated...
September 16, 2016: Disease Models & Mechanisms
Li Lin, Wei Ming Wang, Xiao Xia Pan, Jing Xu, Chen Ni Gao, Wen Zhang, Hong Ren, Jing Yuan Xie, Pin Yan Shen, Yao Wen Xu, Li Yan Ni, Nan Chen
Anti-M-type phospholipase A2 receptor (anti-PLA2R) is a widely accepted biomarker for clinical idiopathic membranous neurophathy (IMN). However, its ability to differentiate between IMN and secondary MN (SMN) is controversial. The objective of this study was to assess clinical MN biomarkers in blood, tissue and urine samples from Chinese patients. In total, 195 MN patients and 70 patients with other glomerular diseases were prospectively enrolled in the study. Participants were followed up for average of 17 months (range 3-39 months)...
September 13, 2016: Oncotarget
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