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Fibrillary glomerulonephritis

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https://www.readbyqxmd.com/read/29423350/primary-psychiatric-disorder-masking-the-diagnosis-of-neuropsychiatric-lupus-in-a-patient-with-altered-mental-status-a-case-report
#1
Osman Perez, Kairavee Dave, Aimee Almanzar, Tajul Prodhan, Livasky Concepion
Neuropsychiatric systemic lupus erythematosus (NPSLE) has a wide variety of neurologic and psychiatric features. NPSLE symptoms and the psychotic features of primary psychiatric disorders often overlap with each other. These psychotic features often mask and delay the diagnosis of NPSLE. We present the case of a 59-year-old female previously diagnosed with bipolar disorder and generalized anxiety disorder presenting with altered mental status (AMS), subsequently diagnosed with neuropsychiatric lupus. Initially, medication overdose was suspected as an empty bottle of trazodone was found beside her...
October 23, 2017: Curēus
https://www.readbyqxmd.com/read/29340314/dnajb9-is-a-specific-immunohistochemical-marker-for-fibrillary-glomerulonephritis
#2
Samih H Nasr, Julie A Vrana, Surendra Dasari, Frank Bridoux, Mary E Fidler, Sihem Kaaki, Nathalie Quellard, Alexia Rinsant, Jean Michel Goujon, Sanjeev Sethi, Fernando C Fervenza, Lynn D Cornell, Samar M Said, Ellen D McPhail, Loren P Herrera Hernandez, Joseph P Grande, Marie C Hogan, John C Lieske, Nelson Leung, Paul J Kurtin, Mariam P Alexander
Introduction: Fibrillary glomerulonephritis (FGN) is a rare disease with unknown pathogenesis and a poor prognosis. Until now, the diagnosis of this disease has required demonstration of glomerular deposition of randomly oriented fibrils by electron microscopy that are Congo red negative and stain with antisera to Igs. We recently discovered a novel proteomic tissue biomarker for FGN, namely, DNAJB9. Methods: In this work, we developed DNAJB9 immunohistochemistry and tested its sensitivity and specificity for the diagnosis of FGN...
January 2018: KI Reports
https://www.readbyqxmd.com/read/29327821/chronic-kidney-disease-in-patients-with-chronic-hepatitis-c-virus-infection
#3
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
https://www.readbyqxmd.com/read/29310419/proliferative-lupus-nephritis-in-the-absence-of-overt-systemic-lupus-erythematosus-a-historical-study-of-12-adult-patients
#4
MULTICENTER STUDY
Maxime Touzot, Cécile Saint-Pastou Terrier, Stanislas Faguer, Ingrid Masson, Hélène François, Lionel Couzi, Aurélie Hummel, Nathalie Quellard, Guy Touchard, Noémie Jourde-Chiche, Jean-Michel Goujon, Eric Daugas
Severe lupus nephritis in the absence of systemic lupus erythematosus (SLE) is a rare condition with an unclear clinical presentation and outcome.We conducted a historical observational study of 12 adult (age >18 years) patients with biopsy-proven severe lupus nephritis or lupus-like nephritis without SLE immunological markers at diagnosis or during follow-up. Excluded were patients with chronic infections with HIV or hepatitis B or C; patients with a bacterial infectious disease; and patients with pure membranous nephropathy...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29225801/histologic-regression-of-fibrillary-glomerulonephritis-the-first-report-of-biopsy-proven-spontaneous-resolution-of-disease
#5
Miroslav Sekulic, Samih H Nasr, Joseph P Grande, Lynn D Cornell
Fibrillary glomerulonephritis (FGN) is a rare immune complex type glomerulonephritis characterized by glomerular deposition of randomly oriented fibrils measuring 10-30 nm in thickness, and typically presents with proteinuria with or without renal insufficiency and hematuria. We present a case in which a patient initially presented at age 41 years with nephrotic-range proteinuria and hypertension; a kidney biopsy showed FGN. The patient was treated with angiotensin receptor blockage only, without immunosuppression as per patient preference, and the level of protein in the urine improved...
December 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/29196420/fibrillary-glomerulonephritis-in-primary-sjogren-s-syndrome-a-rare-cause-of-renal-failure
#6
Rahul Sehgal, Syed M Sajjad, Jiwan K Thapa
Renal involvement in primary Sjogren's syndrome (pSS) varies in severity and prevalence. Although previously felt to be uncommon, kidneys can be involved in up to 25%-30% of pSS patients. Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease that can occur in association with another autoimmune condition or malignancy. The diagnosis relies on renal biopsy findings of haphazardly arranged fibrils in all glomerular compartments and distinction from other forms of fibrillary glomerulopathies such as renal amyloidosis and immunotactoid glomerulopathy...
December 1, 2017: Clinical Medicine & Research
https://www.readbyqxmd.com/read/29155318/kidney-pathology-after-hematologic-cell-transplantation-a-single-center-observation-study-of-indication-biopsies-and-autopsies
#7
Michael Girsberger, Jörg P Halter, Helmut Hopfer, Michael Dickenmann, Thomas Menter
Hematopoietic cell transplantation (HCT) is an increasingly used treatment for hematologic malignancies as well as for non-malignant diseases. Kidney impairment remains an important early and late posttransplant complication. Numerous histopathologic changes have been reported but the pathophysiology is still incompletely understood. Furthermore, correlations between clinical findings and morphologic changes are not well studied. Between 2000 and 2016, 17 patients after allogeneic (n=12) or autologous (n=5) HCT underwent kidney biopsy for either proteinuria or deterioration of kidney function at our center...
November 16, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29130681/nodular-glomerulosclerosis-not-always-diabetic-nephropathy
#8
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
https://www.readbyqxmd.com/read/28628381/monoclonal-immunoglobulin-associated-proliferative-glomerulonephritis-characterized-by-organized-deposits-of-striated-ultra-substructures-a-case-report
#9
Shigeo Hara, Hiroyasu Tsukaguchi, Tastufumi Oka, Makiko Kusabe, Masayuki Mizui, Kensuke Joh
We herein report the case of a 64-year-old male who presented with progressive glomerulonephritis notable for organized and striated ultra-substructures. The patient was diagnosed with hypertension and proteinuria 3 years prior to admission and subsequently developed nephrotic syndrome and impairment of renal function. Laboratory tests did not reveal any evidence of infections or autoimmune diseases. Monoclonal gammopathy was not detected in serum or urine, although a small population of abnormal plasma cell clones was detected by flow cytometry...
July 2017: Ultrastructural Pathology
https://www.readbyqxmd.com/read/28503551/a-case-of-fibrillary-glomerulonephritis-with-fibril-deposition-in-the-arteriolar-wall-and-a-family-history-of-renal-disease
#10
Kentaro Watanabe, Kentaro Nakai, Nozomi Hosokawa, Shuhei Watanabe, Keiji Kono, Shunsuke Goto, Hideki Fujii, Shigeo Hara, Shinichi Nishi
Herein, we report a case of fibrillary glomerulonephritis (FGN). FGN usually shows non-amyloidal fibrils in the mesangium and glomerular capillary walls on electron microscopy. Inherited cases of FGN have been reported in only 3 families, and the suspected genetic form was autosomal dominant. In the present case, the deposition of microfibrils in the arteriolar wall as well as the glomerulus is unique. Our patient's father died of nephrotic syndrome, and his elder brother had a biopsy-proven glomerulopathy...
January 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28356674/fibrillary-glomerulonephritis-presenting-as-crescentic-glomerulonephritis
#11
H H Shah, J Thakkar, J M Pullman, A T Mathew
Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease that commonly presents clinically with hypertension, proteinuria, microscopic hematuria, and varying degree of renal insufficiency. Histologically, FGN can present with different patterns of glomerular injury, more commonly mesangioproliferative, membranoproliferative, and membranous nephropathy. While crescent formation has been described in some kidney biopsy series of FGN, crescentic glomerulonephritis pattern of glomerular injury has been rarely described...
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28352020/profile-of-glomerular-diseases-associated-with-hepatitis-b-and-c-a-single-center-experience-from-india
#12
Nishad Raveendran, Pankaj Beniwal, Amith Vijay Leon D'Souza, Rajendra Singh Tanwar, Piyush Kimmatkar, Dhananjai Agarwal, Vinay Malhotra
Hepatitis B and C are known to affect kidneys in a number of ways. Glomerular diseases associated with hepatitis B and C include membranous nephropathy (MN), membranoproliferative glomerulonephritis (MPGN), focal segmental glomerulosclerosis, immunoglobulin A nephropathy, rarely amyloidosis, and fibrillary and immunotactoid glomerulopathy. In a retrospective analysis of kidney biopsy of 534 patients, we found 16 (2.9%) patients of hepatitis B and 11 (2.05%) patients of hepatitis C with glomerular disease. The most common form of glomerulonephritis in hepatitis B patient was MN and in hepatitis C patient was MPGN...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28161700/clinical-features-and-outcomes-of-a-racially-diverse-population-with-fibrillary-glomerulonephritis
#13
Fernanda Payan Schober, Meghan A Jobson, Caroline J Poulton, Harsharan K Singh, Volker Nickeleit, Ronald J Falk, J Charles Jennette, Patrick H Nachman, William F Pendergraft Iii
BACKGROUND: Fibrillary glomerulonephritis is characterized by randomly arranged fibrils, approximately 20 nm in diameter by electron microscopy. Patients present with proteinuria, hematuria and kidney insufficiency, and about half of the reported patients progress to end-stage kidney disease within 4 years. The dependence of patient characteristics and outcomes on race has not been explored. In this study, we describe a cohort of patients with fibrillary glomerulonephritis and compare their clinical characteristics and outcomes with those of patients previously described...
2017: American Journal of Nephrology
https://www.readbyqxmd.com/read/27866967/cryofibrinogen-associated-glomerulonephritis
#14
Sanjeev Sethi, Ralph Yachoui, David L Murray, Jai Radhakrishnan, Mariam P Alexander
Cryofibrinogen is an under-recognized cryoprotein. Cryofibrinogen is a cryoprecipitate that develops following plasma refrigeration, but does not occur in cold serum. People with cryofibrinogenemia may be asymptomatic, but this cryoprotein can be associated with thromboembolic disease, particularly affecting the skin. Kidney manifestations are relatively uncommon, but are likely underestimated. We describe clinical features and kidney biopsy results in 2 patients with cryofibrinogen-related kidney disease. Both patients presented with proteinuria and hematuria...
February 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/27688084/-diabetic-nephropathy-in-a-non-diabetic-patient
#15
Olga Balafa, Georgios Liapis, Paraskevi Pavlakou, Georgios Baltatzis, Rigas Kalaitzidis, Moses Elisaf
Kimmelstiel and Wilson originally described nodular glomerulosclerosis as the pathognomonic lesion of diabetic nephropathy. Nevertheless, nodular glomerulosclerosis pattern can rarely occur in non-diabetic patients. In such cases, the differential diagnosis includes membranoproliferative glomerulonephritis, light or heavy chain deposition disease, amyloidosis, fibrillary and immunotactoid glomerulonephritis and chronic hypoxic or ischemic conditions. In cases that the above entities cannot be proven, the term idiopathic nodular glomerulosclerosis is given...
December 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/27542513/paraneoplastic-fibrillary-glomerulonephritis-associated-with-intrahepatic-cholangiocarcinoma-when-diagnosis-of-a-rare-kidney-disease-leads-to-successful-hepatic-cancer-treatment
#16
Gabrielle Normand, Anne Jolivot, Maud Rabeyrin, Valérie Hervieu, Pierre-Jean Valette, Jean-Yves Scoazec, Jean-Michel Gougon, Laurent Juillard, Jérôme Dumortier
A 50-year-old man presented with nephrotic syndrome. Electron microscopy analysis of a kidney biopsy specimen showed fibrillary glomerulonephritis, a rare glomerular disease, while histological analysis of a liver tumor biopsy confirmed an intrahepatic cholangiocarcinoma. The paraneoplastic nature of fibrillary glomerulonephritis is debated but after curative treatment of the hepatic nodule, remission of nephrotic syndrome was confirmed at 6-, 12- and 24-months follow-up. To our knowledge, this is the first description of a paraneoplastic fibrillary glomerulonephritis associated with a cholangiocarcinoma, supported by complete remission achieved following cancer treatment...
August 16, 2016: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/27051142/idiopathic-nodular-glomerulosclerosis-report-of-two-cases-and-review-of-literature
#17
S Chandragiri, S Raju, K K Mukku, S Babu, M S Uppin
Nodular glomerulosclerosis, a pathological finding characterized by areas of marked mesangial expansion with accentuated glomerular nodularity can be seen in a number of conditions including diabetic nephropathy, amyloidosis, light chain deposition disease, fibrillary and immunotactoid glomerulopathy, collagen type III disease, nodular membranoproliferative glomerulonephritis, and Takayasu's arteritis. Idiopathic nodular glomerulosclerosis is a diagnosis of exclusion and is reported in patients with hypertension, smoking, chronic obstructive pulmonary disease, obesity, metabolic syndrome, etc...
March 2016: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/27036111/acthar-gel-in-the-treatment-of-nephrotic-syndrome-a-multicenter-retrospective-case-series
#18
MULTICENTER STUDY
Arvind Madan, Snezana Mijovic-Das, Ana Stankovic, Geoffrey Teehan, Amber S Milward, Anupa Khastgir
BACKGROUND: Current first-line anti-proteinuric treatments for nephrotic syndrome (NS) do not produce an effective response in all patients and are not tolerated by some patients. Additional effective and tolerable treatment options in NS are strongly needed. This retrospective case series is the largest to date to examine Acthar gel (adrenocorticotropic hormone, ACTH) in patients with varied-etiology NS. METHODS: This multicenter retrospective case series included adult patients with NS (N = 44) treated with Acthar gel at 6 clinical practices...
March 31, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27022729/treatment-of-fibrillary-glomerulonephritis-by-corticosteroids-and-tripterygium-glycoside-tablets-a-case-report
#19
Zhao-Yu Lu, Hai-Feng Yang, Yu Peng, Yin Li, Zi-Chang Yin, Fu-Hua Lu, Xu-Sheng Liu
No abstract text is available yet for this article.
May 2016: Chinese Journal of Integrative Medicine
https://www.readbyqxmd.com/read/27015134/renal-pathology-in-hematopoietic-cell-transplant-recipients-a-contemporary-biopsy-nephrectomy-and-autopsy-series
#20
Brian T Brinkerhoff, Donald C Houghton, Megan L Troxell
Renal injury in hematopoietic cell transplant recipients may be related to a combination of factors including chemotherapy, radiation, infection, immunosuppressive agents, ischemia, and graft-versus-host disease, and can involve glomerular, tubulointerstitial, and vascular structures. We reviewed renal pathology from 67 patients at a single institution (2009-2014), including 14 patients with biopsy for clinical dysfunction, 6 patients with surgical kidney resection for other causes, and 47 autopsy patients...
June 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
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