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https://www.readbyqxmd.com/read/29314707/comparison-of-early-versus-late-onset-familial-mediterranean-fever
#1
Nazife Sule Yasar Bilge, Ismail Sari, Dilek Solmaz, Soner Senel, Hakan Emmungil, Levent Kilic, Sibel Yilmaz Oner, Fatih Yildiz, Sedat Yilmaz, Duygu Ersozlu Bozkirli, Muge Aydin Tufan, Sema Yilmaz, Veli Yazisiz, Yavuz Pehlivan, Cemal Bes, Gozde Yildirim Cetin, Sukran Erten, Emel Gonullu, Fezan Sahin, Servet Akar, Kenan Aksu, Umut Kalyoncu, Haner Direskeneli, Eren Erken, Mehmet Sayarlioglu, Muhammed Cınar, Timucin Kasifoglu
AIM: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease. One of the common characteristics of this disease is its young age predominance. Nearly 90% of patients experience disease flares during early adult age periods. Currently there are limited data for the comparison of early versus late onset FMF and therefore the primary aim of this study was to investigate these two subsets with regard to their certain demographic, clinical and genetic differences. METHODS: Early (≤ 20 years, Group 1) and late (> 20 years, Group 2) onset FMF patients were identified from the national FMF registry that involves 2246 patients from 15 adult rheumatology clinics located in different geographical areas of Turkey...
January 5, 2018: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29298867/repeat-doses-of-antibody-to-serum-amyloid-p-component-clear-amyloid-deposits-in-patients-with-systemic-amyloidosis
#2
Duncan B Richards, Louise M Cookson, Sharon V Barton, Lia Liefaard, Thirusha Lane, David F Hutt, James M Ritter, Marianna Fontana, James C Moon, Julian D Gillmore, Ashutosh Wechalekar, Philip N Hawkins, Mark B Pepys
Systemic amyloidosis is a fatal disorder caused by pathological extracellular deposits of amyloid fibrils that are always coated with the normal plasma protein, serum amyloid P component (SAP). The small-molecule drug, miridesap, [(R)-1-[6-[(R)-2-carboxy-pyrrolidin-1-yl]-6-oxo-hexanoyl]pyrrolidine-2-carboxylic acid (CPHPC)] depletes circulating SAP but leaves some SAP in amyloid deposits. This residual SAP is a specific target for dezamizumab, a fully humanized monoclonal IgG1 anti-SAP antibody that triggers immunotherapeutic clearance of amyloid...
January 3, 2018: Science Translational Medicine
https://www.readbyqxmd.com/read/29250691/nephrotic-syndrome-secondary-to-amyloidosis-in-a-patient-with-monoclonal-gammopathy-with-renal-significance-mgrs
#3
Andrei Niculae, Ileana Peride, Vlad Vinereanu, Daniela Rădulescu, Ovidiu Gabriel Bratu, Bogdan Florin Geavlete, Ionel Alexandru ChecheriŢă
Monoclonal gammopathy with renal significance (MGRS) is a relative new-described entity, diagnosed especially in older patients and deriving from the group with monoclonal gammopathy of undetermined significance (MGUS). Various renal lesions may arise in MGRS, according to the ultrastructural characteristics of the monoclonal immunoglobulin deposition in the kidney, from proliferative glomerulopathies and amyloidosis to light chain proximal tubulopathy and crystal-storing histiocytosis. Although both are considered premalign or non-malignant hematological conditions, kidney involvement in MGRS aggravates the prognosis of the patients and need to be treated aggressively...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/29228957/evaluation-of-the-effect-of-d-amino-acid-incorporation-into-amyloid-reactive-peptides
#4
Emily B Martin, Angela Williams, Tina Richey, Craig Wooliver, Alan Stuckey, James S Foster, Stephen J Kennel, Jonathan S Wall
BACKGROUND: Systemic amyloidoses comprise diseases characterized by the deposition of proteinaceous material known as amyloid. Currently, without performing multiple biopsies, there is no way to ascertain the extent of amyloid deposition in patients-a critical piece of information that informs prognosis and therapeutic strategies. We have developed pan-amyloid-targeting peptides for imaging amyloid and recently have adapted these for use as pre-targeting agents in conjunction with immunotherapy...
December 11, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/29226427/case-report-treatment-of-light-chain-amyloidosis-with-daratumumab-monotherapy-in-two-patients
#5
Charlotte Gran, Gösta Gahrton, Evren Alici, Hareth Nahi
Immunoglobulin light-chain amyloidosis (AL) affects multiple organs, most prominently the kidney and the heart. Renal and cardiac impairment are both associated with poor prognosis.(1) Typical treatment regimens for AL include proteasome inhibitors, alkylating agents, and steroids as well as autologous stem cell transplantation (ASCT) for younger, fit patients. Complete response after treatment is associated with a better outcome and can be measured by free light chain (FLC) reduction.(2, 3) Monoclonal antibodies such as daratumumab (Dara, human IgG1 anti-CD38) have shown promising efficacy for the treatment of relapsed and refractory multiple myeloma...
December 11, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/29217892/bowel-migration-of-dormant-chronic-ambulatory-peritoneal-dialysis-catheter-a-vexed-problem-not-avoided-by-flushing
#6
P Vincent, J Gopinathan, R Narayanan
Delayed bowel erosion by peritoneal dialysis catheter is rare with fewer than thirty cases having been reported in the literature. This complication is usually encountered when the catheter is kept dormant. Two cases have also been reported with catheters in active use. The risk factors for bowel erosion include immunosuppression, diverticulosis, and amyloidosis. An 80-year-old male with chronic kidney disease Stage 5 due to hypertensive nephrosclerosis underwent chronic ambulatory peritoneal dialysis catheter insertion...
November 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/29210840/conoscere-e-curare-il-cuore-1986-2016-how-the-heart-has-changed
#7
Alessandro Boccanelli
: The demographic transition, with longer life expectancy, is mainly due to prevention and care of atherosclerotic vascular and heart disease. The cardiovascular patient is now much older and often presents with coexisting geriatric syndromes that affect healthcare goals, outcomes, and the process of care. Numerous mechanisms at cellular level are responsible for cardiovascular aging such as replicative senescence, apoptosis, proteins misfolding, and inflammation. Aging-related modifications are increase in left-ventricular mass, increased left atrial size, myocardial collagen deposition, and calcium deposition in valvular structures and the coronary arteries...
November 27, 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/29202802/histopathology-microbiology-and-the-inflammatory-process-associated-with-sarcoptes-scabiei-infection-in-the-iberian-ibex-capra-pyrenaica
#8
José Espinosa, Arián Ráez-Bravo, Jorge R López-Olvera, Jesús M Pérez, Santiago Lavín, Asta Tvarijonaviciute, Francisco J Cano-Manuel, Paulino Fandos, Ramón C Soriguer, José Enrique Granados, Diego Romero, Roser Velarde
BACKGROUND: Sarcoptic mange has been identified as the most significant infectious disease affecting the Iberian ibex (Capra pyrenaica). Despite several studies on the effects of mange on ibex, the pathological and clinical picture derived from sarcoptic mange infestation is still poorly understood. To further knowledge of sarcoptic mange pathology, samples from ibex were evaluated from histological, microbiological and serological perspectives. METHODS: Samples of skin, non-dermal tissues and blood were collected from 54 ibex (25 experimentally infected, 15 naturally infected and 14 healthy)...
December 4, 2017: Parasites & Vectors
https://www.readbyqxmd.com/read/29196420/fibrillary-glomerulonephritis-in-primary-sjogren-s-syndrome-a-rare-cause-of-renal-failure
#9
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/29187090/outcomes-of-patients-with-familial-transthyretin-amyloidosis-after-liver-transplantation
#10
Dipti Banerjee, Lindsey E Roeker, Martha Grogan, Paul Swiecicki, John Poterucha, Julie Heimbach, Steve Zeldenrust, Morie Gertz, Brooks Edwards, Richard Daly, Kyle W Klarich, Angela Dispenzieri
BACKGROUND: Familial transthyretin amyloidosis is a disease caused by misfolded transthyretin aggregates that can impair multiple organ systems. Liver transplantation is the first-line treatment for familial transthyretin amyloidosis. RESEARCH QUESTION: Our objective is to study outcomes and survival among patients with familial transthyretin amyloidosis after transplantation. DESIGN: All patients undergoing orthotopic liver transplant for familial transthyretin amyloidosis at Mayo Clinic between 1997 and 2012 were reviewed...
September 2017: Progress in Transplantation
https://www.readbyqxmd.com/read/29173691/clinical-outcomes-and-survival-in-aa-amyloidosis-patients
#11
Yavuz Ayar, Alparslan Ersoy, Mustafa Ferhat Oksuz, Gokhan Ocakoglu, Berna Aytac Vuruskan, Abdülmecit Yildiz, Emel Isiktas, Aysegül Oruc, Sedat Celikci, Ismail Arslan, Ahmet Bilgehan Sahin, Mustafa Güllülü
AIM: Amyloid A amyloidosis is a rare complication of chronic inflammatory conditions. Most patients with amyloid A amyloidosis present with nephropathy and it leads to renal failure and death. We studied clinical characteristics and survival in patients with amyloid A amyloidosis. METHODS: A total of 81 patients (51 males, 30 females) with renal biopsy proven amyloid A amyloidosis were analyzed retrospectively. The patients were divided into good and poor outcomes groups according to survival results...
November 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/29155738/imaging-amyloid-tissues-stained-with-luminescent-conjugated-oligothiophenes-by-hyperspectral-confocal-microscopy-and-fluorescence-lifetime-imaging
#12
Sofie Nyström, Marcus Bäck, K Peter R Nilsson, Per Hammarström
Proteins that deposit as amyloid in tissues throughout the body can be the cause or consequence of a large number of diseases. Among these we find neurodegenerative diseases such as Alzheimer's and Parkinson's disease afflicting primarily the central nervous system, and systemic amyloidosis where serum amyloid A, transthyretin and IgG light chains deposit as amyloid in liver, carpal tunnel, spleen, kidney, heart, and other peripheral tissues. Amyloid has been known and studied for more than a century, often using amyloid specific dyes such as Congo red and Thioflavin T (ThT) or Thioflavin (ThS)...
October 20, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29153137/ckd-stage-v-in-al-amyloidosis-is-it-too-late-to-treat-maybe-not
#13
Nelson Leung
Ig light chain amyloidosis is a protein misfolding disease capable of causing multiorgan failure. Organ failure can be stopped by reducing the production of Ig light chain. The current study by Rezk et al. found that even patients with advanced chronic kidney disease can benefit if the serum free light chain can be substantially reduced within the first 3 months. This, if confirmed, can have a huge impact in the management of these patients.
December 2017: Kidney International
https://www.readbyqxmd.com/read/29142973/renal-amyloidosis-associated-with-5-novel%C3%A2-variants-in-the-fibrinogen-a-alpha-chain-protein
#14
Dorota Rowczenio, Maria Stensland, Gustavo A de Souza, Erik H Strøm, Janet A Gilbertson, Graham Taylor, Nigel Rendell, Shane Minogue, Yvonne A Efebera, Helen J Lachmann, Ashutosh D Wechalekar, Philip N Hawkins, Ketil R Heimdal, Kristian Selvig, Inger K Lægreid, Nathalie Demoulin, Selda Aydin, Julian D Gillmore, Tale N Wien
Introduction: Fibrinogen A alpha chain amyloidosis is an autosomal dominant disease associated with mutations in the fibrinogen A alpha chain (FGA) gene, and it is the most common cause of hereditary renal amyloidosis in the UK. Patients typically present with kidney impairment and progress to end-stage renal disease over a median time of 4.6 years. Methods: Six patients presented with proteinuria, hypertension, and/or lower limb edema and underwent detailed clinical and laboratory investigations...
May 2017: KI Reports
https://www.readbyqxmd.com/read/29136724/-expert-consensus-for-the-diagnosis-and-treatment-of-patients-with-renal-impairment-of-multiple-myeloma
#15
(no author information available yet)
Renal impairment (RI) is a common complication of multiple myeloma (MM), which is presented as chronic kidney disease (CKD) or acute kidney injury (AKI). The typical pathological feature is cast nephropathy. Presently international system staging (ISS) is used in evaluating MM. Although the classic Durie-Salmon staging system could be still used in clinical practice, it may miss out some patients with renal impairment. For evaluations of RI in MM patients with CKD, it's recommended to assess the estimated glomerular filtration rate (eGFR) by creatinine based formula CKD-epidemiology collaboration (EPI) or modification of diet in renal disease(MDRD) and to stage the renal injuries according to 2013 Kidney Disease Improving Global Outcomes (KDIGO) CKD guidelines...
November 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/29133141/abdominal-fat-pad-excisional-biopsy-for-the-diagnosis-and-typing-of-systemic-amyloidosis
#16
Yessica Garcia, A Bernard Collins, James R Stone
In the past, the diagnosis and typing of amyloidosis often required an invasive biopsy of an internal organ, such as the heart or kidneys. Abdominal fat pad excisional biopsy (FPEB) offers a less invasive approach, but the sensitivity of this technique has been unclear. To determine the sensitivity of FPEB for immunoglobulin light chain (AL) and transthyretin (ATTR) amyloidosis, we performed a retrospective clinicopathologic analysis of 97 patients who had undergone FPEB, of which 16 were positive for amyloid...
November 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/29114004/dysproteinemias-and-glomerular-disease
#17
Nelson Leung, Maria E Drosou, Samih H Nasr
Dysproteinemia is characterized by the overproduction of an Ig by clonal expansion of cells from the B cell lineage. The resultant monoclonal protein can be composed of the entire Ig or its components. Monoclonal proteins are increasingly recognized as a contributor to kidney disease. They can cause injury in all areas of the kidney, including the glomerular, tubular, and vascular compartments. In the glomerulus, the major mechanism of injury is deposition. Examples of this include Ig amyloidosis, monoclonal Ig deposition disease, immunotactoid glomerulopathy, and cryoglobulinemic GN specifically from types 1 and 2 cryoglobulins...
January 6, 2018: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29093404/nationwide-survey-of-741-patients-with-systemic-amyloid-light-chain-amyloidosis-in-japan
#18
Chihiro Shimazaki, Hiroyuki Hata, Sinsuke Iida, Mitsuharu Ueda, Nagaaki Katoh, Yoshiki Sekishima, Shuichi Ikeda, Masahide Yazaki, Wakaba Fukushima, Yukio Ando
Objective To retrospectively investigate the clinical manifestations of systemic amyloid light-chain (AL) amyloidosis in Japanese patients and the treatment strategy for the condition. Methods We conducted a survey of Japanese AL patients, who were treated between January 1, 2012, and December 31, 2014. Results A total of 741 AL amyloidosis patients were included in this study (436 men and 305 women; median age: 65 years old, range: 31-93). The most frequently affected organ was the kidneys (n=542), followed by the heart (n=252), gastrointestinal (GI) tract (n=164), autonomic nervous system (n=131), liver (n=71), and peripheral nervous system (n=71)...
November 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29089309/vlitl-is-a-major-cross-%C3%AE-sheet-signal-for-fibrinogen-a%C3%AE-chain-frameshift-variants
#19
Cyrille Garnier, Fatma Briki, Brigitte Nedelec, Patrick Le Pogamp, Ahmet Dogan, Nathalie Rioux-Leclercq, Renan Goude, Caroline Beugnet, Laurent Martin, Marc Delpech, Franck Bridoux, Gilles Grateau, Jean Doucet, Philippe Derreumaux, Sophie Valleix
The first case of hereditary fibrinogen Aα-chain amyloidosis was recognized more than 20 years ago, but disease mechanisms still remain unknown. Here we report detailed clinical and proteomics studies of a French kindred with a novel amyloidogenic fibrinogen Aα-chain frameshift variant, Phe521Leufs, causing a severe familial form of renal amyloidosis. Next, we focused our investigations to elucidate the molecular basis that render this Aα-chain variant amyloidogenic. We show that a 49-mer peptide derived from the C-terminal part of the Phe521Leufs-chain is deposited as fibrils in the patient's kidneys, establishing that only a small portion of Phe521Leufs directly contributes to amyloid formation in vivoIn silico analysis indicated that this 49-mer Aα-chain peptide contained a motif (VLITL), with a high intrinsic propensity for β-aggregation at residues 44-48 of human renal fibrils...
October 31, 2017: Blood
https://www.readbyqxmd.com/read/29068987/membranous-nephropathy-in-a-patient-with-ankylosing-spondylitis-a-case-report
#20
Ruiying Chen, Fang Li, Qionghong Xie, Jun Xue, Lingyun Lai, Shaojun Liu, Liyin Zhang, Chuanming Hao
RATIONALE: Renal complications in ankylosing spondylitis (AS) were rarely observed, and proteinuria associated with AS can be seen often due to amyloidosis in this kind of complications, while membranous nephropathy (MN) is seldom considered. This article reports a case of coexistence of AS and MN, to provide the exact relationship of these 2 entities and recognized some causes of renal involvement in AS. PATIENT CONCERNS: A 44-year-old female presented with pain of the left leg for 4 years and pedal edema for 2 weeks...
October 2017: Medicine (Baltimore)
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