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M Vankalakunti, R Augustine, R Jangamani, V Siddini, R Bonu, K Babu, S H Ballal
Dense deposit disease (DDD), earlier called Type II membranoproliferative glomerulonephritis is distinct disease having frequent relapses reaching end-stage kidney disease by 10-year in up to 50%-60% of cases and high recurrence rate in the allograft. The term DDD is derived from its distinctive ribbon-like osmiophilic deposits in the lamina densa of glomerular basement membrane by electron microscopy. Pathogenetically, alternate pathway dysfunction leads to this disease, which is diagnosed by ultrastructure...
January 2018: Indian Journal of Nephrology
Masato Takei, Akira Obana, Takenori Inomata, Takao Tanaka, Tina Shiang, Yuan Bae, Tamiko Takemura, Akira Murakami
BACKGROUND: Membranoproliferative glomerulonephritis (MPGN) is characterized by mesangial cell proliferation and is classified into types I, II and III based on structural changes in the glomerular capillary walls. The drusen-like deposits of MPGN type II have been studied, but the fundus changes in MPGN type III have yet to be clarified. We report a case of MPGN type III with multiple deposits in the retinal pigment epithelium (RPE). CASE PRESENTATION: A 40-year-old Japanese woman with MPGN type III developed numerous yellow-white patches in the central macula of both eyes...
March 6, 2018: BMC Ophthalmology
Kawmadi W Gunawardena, Eranga S Wijewickrama, Carukshi Arambepola, Rushika D Lanerolle
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...
January 2018: Saudi Journal of Kidney Diseases and Transplantation
Abhilash Koratala, Xu Zeng
Membranoproliferative glomerulonephritis associated with mixed cryoglobulinemia is the most common form of kidney disease observed in relation to hepatitis C virus (HCV) infection. Rituximab, a monoclonal antibody against CD20, is an effective treatment for severe and/or refractory HCV-related vasculitis and may evade the need for dialysis as in our patient.
February 2018: Clinical Case Reports
Z Chen, Y J Yang, J Li, X P Tian
Objective: To investigate the clinical features of Takayasu's arteritis (TAK) with glomerulonephropathy and to improve physicians' understanding of this complication in patients with TAK. Methods: Clinical data were retrospectively collected including manifestations, laboratory tests, image findings and treatment of 8 patients diagnosed as Takayasu's arteritis with glomerulonephropathy from January 2002 to January 2017 in Peking Union Medical College Hospital. Results: Glomerulonephropathy was confirmed based on percutaneous renal biopsy...
February 1, 2018: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
James Goya Heaf, Alastair Hansen, Gunnar Hellmund Laier
BACKGROUND: The association of increased cancer risk with glomerulonephritis (GN) is well known, but controversy exists concerning which types of GN are involved, and the size of the association. A national registry survey was performed to assess the size of this association, and the temporal relationship of cancer diagnosis to GN diagnosis. METHODS: All patients with biopsy-proven GN between 1985 and 2015 in Denmark were extracted from The Danish Renal Biopsy Registry and the National Pathology Data Bank...
February 2, 2018: BMC Nephrology
Wei Zhang, Jeffrey H Glaze, David Wynne
IgG4-related disease (IgG4-RD) is a newly recognized immune-mediated multisystemic disease characterized by a fibro-inflammatory condition with tissue infiltration of IgG4-positive plasma cells and often associated with elevated serum IgG4 levels. Typical renal involvement of IgG4-RD presents as tubulointerstitial nephritis (TIN), membranous or membranoproliferative nephropathy. We are presenting a case with combined IgG4 membranous nephropathy and TIN, as well as a literature review on pathophysiology, diagnosis and treatment of IgG4-RD...
February 1, 2018: CEN Case Reports
Iolanda Godinho, Estela Nogueira, Sofia Jorge, António Teixeira Alves, António Gomes da Costa
BACKGROUND: Hemophagocytic syndrome (HPS) is a rare, aggressive disorder characterized by dysregulation of lymphocyte and macrophage activity, which culminates in tissue infiltration with hemophagocytosis and ultimately organ failure. Renal involvement frequently ensues and usually results in acute tubular necrosis with associated interstitial inflammation. Less frequently, glomerulopathy can also be found. CASE: We report a case of a 24-year-old Caucasian woman with previous asymptomatic hematuria, mild proteinuria, and normal renal function who presented to us with fever...
January 19, 2018: Clinical Nephrology
Omer Shahab, Pegah Golabi, Zobair M Younossi
Hepatitis C virus (HCV) infection affects many organs in the body, including the liver, kidneys, skin, joints and others. Although the hepatic manifestation of HCV has been widely studied, the extrahepatic manifestaions of HCV have not been fully appreciated. Studies have shown that patients with HCV have a higher risk of chronic kidney disease and end-stage renal disease, as well as poorer outcomes after kidney transplantation. Given these findings, it is important to screen HCV patients for presence of renal impairement in a timely manner...
January 10, 2018: Minerva Gastroenterologica e Dietologica
Cristiane Bitencourt Dias, Leonardo Testagrossa, Lectícia Jorge, Denise Malheiros, Viktoria Woronik
BACKGROUND: New classification for membranoproliferative glomerulonephritis has been proposed in the literature. The aim of this study was to compare the clinical, biochemical, etiology and renal biopsy findings of these patients grouped by immunofluorescence as proposed by the new classification. METHODS: Patients with renal biopsy-proven membranoproliferative glomerulonephritis unrelated to systemic lupus erythematosus, diagnosed between 1999 and 2014. The patients were divided according to immunofluorescence: Immunoglobulin positive group, C3 positive only and negative immunofluorescence group...
October 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
Washington Luis Conrado Dos-Santos, Glória Maria Maranhão Sweet, Labene Gondim Azevêdo, Maria Brandão Tavares, Maria Fernanda Sanches Soares, Caroline Vilas Boas de Melo, Márcia Fernanda Melo Carneiro, Rilma Ferreira de Souza Santos, Márcia Cristina Conrado, Daniela Teixeira Leal Braga, Marcia Carvalho Bessa, Nathanael de Freitas Pinheiro Junior, Marília Bahiense-Oliveira
INTRODUCTION: A report on the prevalence of glomerular disease diagnosed via renal biopsy in Salvador, BA, Brazil was published in 1973 and showed a predominance of membranoproliferative glomerulonephritis, which was frequently associated with hepatosplenic schistosomiasis. OBJECTIVE: In this study, we investigate the potential changes in the distribution of glomerular diseases after a period of important epidemiological transition in Brazil. METHODS: Pathology reports of all patients subjected to kidney biopsy from 2003 to 2015 in a referral nephrology service were reviewed...
October 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
Ahmad M Mansour, Luiz H Lima, J Fernando Arevalo, Miguel Hage Amaro, Virginia Lozano, Alaa Bou Ghannam, Errol W Chan
Purpose: To assess the evolution of retinal findings in patients with membranoproliferative glomerulonephritis (MPGN) by funduscopy, intravenous fluorescein angiography and optical coherence tomography. Observations: Three women and one man were followed for a period of 1.5-37 years. Four patients (8 eyes) had drusen detected at first fundus exam at age 24, 29, 50 and 55. Three patients (6 eyes) had diffuse thickening of Bruch's membrane, and two patients (3 eyes) had detachment of the retinal pigment epithelium with serous retinal detachment...
September 2017: American Journal of Ophthalmology Case Reports
Mathieu P Rodero, Alessandra Tesser, Eva Bartok, Gillian I Rice, Erika Della Mina, Marine Depp, Benoit Beitz, Vincent Bondet, Nicolas Cagnard, Darragh Duffy, Michael Dussiot, Marie-Louise Frémond, Marco Gattorno, Flavia Guillem, Naoki Kitabayashi, Fabrice Porcheray, Frederic Rieux-Laucat, Luis Seabra, Carolina Uggenti, Stefano Volpi, Leo A H Zeef, Marie-Alexandra Alyanakian, Jacques Beltrand, Anna Monica Bianco, Nathalie Boddaert, Chantal Brouzes, Sophie Candon, Roberta Caorsi, Marina Charbit, Monique Fabre, Flavio Faletra, Muriel Girard, Annie Harroche, Evelyn Hartmann, Dominique Lasne, Annalisa Marcuzzi, Bénédicte Neven, Patrick Nitschke, Tiffany Pascreau, Serena Pastore, Capucine Picard, Paolo Picco, Elisa Piscianz, Michel Polak, Pierre Quartier, Marion Rabant, Gabriele Stocco, Andrea Taddio, Florence Uettwiller, Erica Valencic, Diego Vozzi, Gunther Hartmann, Winfried Barchet, Olivier Hermine, Brigitte Bader-Meunier, Alberto Tommasini, Yanick J Crow
Microbial nucleic acid recognition serves as the major stimulus to an antiviral response, implying a requirement to limit the misrepresentation of self nucleic acids as non-self and the induction of autoinflammation. By systematic screening using a panel of interferon-stimulated genes we identify two siblings and a singleton variably demonstrating severe neonatal anemia, membranoproliferative glomerulonephritis, liver fibrosis, deforming arthropathy and increased anti-DNA antibodies. In both families we identify biallelic mutations in DNASE2, associated with a loss of DNase II endonuclease activity...
December 19, 2017: Nature Communications
Yoshito Yamaguchi, Kunihiko Maeda, Katsuyuki Nagatoya, Atsushi Yamauchi
A 53-year-old man presented with proteinuria and hematuria. No significant abnormality was detected in his physical examination or laboratory tests, including evidence of paraprotein in serum and urine. Renal biopsy revealed mesangial proliferation, thickened glomerular basement membranes, and spike formation. Immunofluorescence revealed deposition of immunoglobulin (Ig) M heavy chain, kappa (κ) light chain, and complement component C3 along capillary walls in the glomeruli. Light chain staining indicated significant restriction, because only κ chain, not lambda chain, was present in glomeruli...
December 11, 2017: CEN Case Reports
Naoki Nakagawa, Naoyuki Hasebe, Motoshi Hattori, Michio Nagata, Hitoshi Yokoyama, Hiroshi Sato, Hitoshi Sugiyama, Akira Shimizu, Yoshitaka Isaka, Shoichi Maruyama, Ichiei Narita
BACKGROUND: The incidence and age distribution of membranoproliferative glomerulonephritis (MPGN) vary throughout the world by race and ethnicity. We sought to evaluate the clinical features, pathogenesis, and age distribution of MPGN among a large nationwide data from the Japan Renal Biopsy Registry (J-RBR). METHODS: A cross-sectional survey of 593 patients with MPGN (types I and III) registered in the J-RBR between 2007 and 2015 was conducted. Clinical parameters, and laboratory findings at diagnosis were compared between children (< 20 years), adults (20-64 years), and elderly patients (≥ 65 years)...
December 6, 2017: Clinical and Experimental Nephrology
Homare Shimohata, Kentaro Ohgi, Hiroshi Maruyama, Yasunori Miyamoto, Mamiko Takayashu, Kouichi Hirayama, Masaki Kobayashi
In 2004, the novel category of monoclonal IgG deposition disease has been proposed and termed "proliferative glomerulonephritis with monoclonal IgG deposits" (PGNMID). This disease is characterized by membranoproliferative glomerulonephritis and staining for a single light-chain isotype and gamma heavy-chain subclass. A 76-year-old male who had monoclonal gammopathy was referred to our hospital because of proteinuria. The renal biopsy showed diffuse thickening of the glomerular capillary walls with focal mesangial proliferation...
2017: Case Reports in Nephrology
Farah Daccueil, Naveed N Masani, Vivette D D'Agati, Shayan Shirazian
No abstract text is available yet for this article.
December 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Nicola Pirozzi, Antonella Stoppacciaro, Paolo Menè
Recently, a number of reports have described dominant C3 deposits in renal biopsies of patients with infection-related glomerulonephritis (GN). While acute post-infectious GN and membranoproliferative GN are commonly characterized by immune deposits containing C3 and/or C4, the absence of immunoglobulin (Ig) and/or immune complexes at light or electron microscopy is a rather unusual observation. Dominant C3 deposition is believed to result from the alternative pathway of complement activation via the C3bBb "tickover" convertase...
November 18, 2017: Journal of Nephrology
Olga Balafa, Georgios Liapis
Nodular glomerulosclerosis -as it was originally described by Kimmelstiel and Wilson- is the main pathognomonic lesion of diabetic nephropathy. However, this particular pattern can also be seen in other conditions such as membranoproliferative glomerulonephritis, light or heavy chain deposition disease, amyloidosis, fibrillary and immunotactoid glomerulonephritis and Takayasu's arteritis. History of diabetes as well as other clinical and pathological features are of crucial importance in order to determine the precise cause of nodular glomerulosclerosis...
November 10, 2017: Minerva Medica
Sam Sedaghat, Eleène Sahmila Heshmati, Thomas Frese, Wilma Gertrud Rasche, Filip Barinka, Florian Fahr, Joachim Beige, Stephan Schiekofer, Franz Maximilian Rasche
INTRODUCTION: The possible confounding influence of investigator-related preferences, available histological techniques, and healthcare systems on the frequencies and incidences of primary and secondary nephropathies was evaluated in this long-term observation. MATERIALS AND METHODS: The observation time from 1983 to 2010 was divided in regard to the political regimes: a) prior to and after German reunification: German Democratic Republic (GDR, period 1 from 1983 to 1990)/Federal Republic of Germany (FRG, period 2 from 1990 to 2010); and the two heads of the division of nephrology, b) conductor 1 (1983 - 2006) and conductor 2 (2006 - 2010)...
December 2017: Clinical Nephrology
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