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amyloidosis kidney

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https://www.readbyqxmd.com/read/28729034/prolonged-renal-survival-in-light-chain-amyloidosis-speed-and-magnitude-of-light-chain-reduction-is-the-crucial-factor
#1
Tamer Rezk, Helen J Lachmann, Marianna Fontana, Sajitha Sachchithanantham, Shameem Mahmood, Aviva Petrie, Carol J Whelan, Jennifer H Pinney, Darren Foard, Thirusha Lane, Taryn Youngstein, Ashutosh D Wechalekar, Paul Bass, Philip N Hawkins, Julian D Gillmore
Renal involvement causing progressive chronic kidney disease (CKD) is present in 70% of patients with systemic Ig light-chain (AL) amyloidosis at diagnosis. Chemotherapy that substantially suppresses free light chain production is associated with improved patient survival, but its benefit in delaying the onset of renal replacement therapy among patients who present with established advanced CKD has not been studied. To evaluate this, we studied 1000 patients enrolled in the prospective UK AL amyloidosis chemotherapy study (ALchemy)...
July 17, 2017: Kidney International
https://www.readbyqxmd.com/read/28686525/kidney-biopsy-in-aa-amyloidosis-impact-of-histopathology-on-prognosis
#2
Zeynep Kendi Celebi, Saba Kiremitci, Bengi Ozturk, Serkan Akturk, Siyar Erdogmus, Neval Duman, Kenan Ates, Sehsuvar Erturk, Gokhan Nergizoglu, Sim Kutlay, Sule Sengul, Arzu Ensari, Kenan Keven
In AA amyloidosis, while kidney biopsy is widely considered for diagnosis by clinicians, there is no evidence that the detailed investigation of renal histopathology can be utilized for the prognosis and clinical outcomes. In this study, we aimed to obtain whether histopathologic findings in kidney biopsy of AA amyloidosis might have prognostic and clinical value. This is a retrospective cohort study that included 38 patients who were diagnosed with AA amyloidosis by kidney biopsy between 2005 and 2013.The kidney biopsy specimens of patients were evaluated and graded for several characteristics of histopathological lesions and their relationship with renal outcomes...
July 7, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28678923/cardiac-amyloidosis-and-its-new-clinical-phenotype-heart-failure-with-preserved-ejection-fraction
#3
Evandro Tinoco Mesquita, Antonio José Lagoeiro Jorge, Celso Vale Souza, Thais Ribeiro de Andrade
Heart failure with preserved ejection fraction (HFpEF) is now an emerging cardiovascular epidemic, being identified as the main phenotype observed in clinical practice. It is more associated with female gender, advanced age and comorbidities such as hypertension, diabetes, obesity and chronic kidney disease. Amyloidosis is a clinical disorder characterized by the deposition of aggregates of insoluble fibrils originating from proteins that exhibit anomalous folding. Recently, pictures of senile amyloidosis have been described in patients with HFpEF, demonstrating the need for clinical cardiologists to investigate this etiology in suspect cases...
June 29, 2017: Arquivos Brasileiros de Cardiologia
https://www.readbyqxmd.com/read/28675980/a-combined-deposition-of-lanthanum-and-%C3%AE-2-microglobulin-related-amyloid-in-the-gastroduodenal-mucosa-of-hemodialysis-dependent-patients-an-immunohistochemical-electron-microscopic-and-energy-dispersive-x-ray-spectrometric-analysis
#4
Takayoshi Komatsu-Fujii, Hideyuki Onuma, Youichi Miyaoka, Noriyoshi Ishikawa, Asuka Araki, Nahoko Ishikawa, Tomohiko Yamamoto, Tomoko Mishiro, Kyoichi Adachi, Yoshikazu Kinoshita, Pamela Tauchi-Nishi, Riruke Maruyama
Lanthanum carbonate (LC) is a new type of phosphate adsorbent used to treat patients with hyperphosphatemia caused by chronic kidney diseases. Recent studies have pointed out that lanthanum deposition can be found in the cytoplasm of histiocytes in the gastroduodenal mucosa of these patients. On the other hand, it is well known that patients on long-term hemodialysis can develop deposition of β2-microglobulin-related amyloid (Aβ2M) mainly around joints. However, involvement of the gastrointestinal tract by hemodialysis-associated amyloidosis has been thought to be rare, and therefore only Aβ2M, if any, has been reported to accumulate in the vascular walls of the submucosa and muscularis propria...
July 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28636627/plasma-cell-neoplasia-after-kidney-transplantation-french-cohort-series-and-review-of-the-literature
#5
Raphaël Kormann, Hélène François, Thibault Moles, Jacques Dantal, Nassim Kamar, Karine Moreau, Thomas Bachelet, Anne-Elisabeth Heng, Antoine Garstka, Charlotte Colosio, Didier Ducloux, Johnny Sayegh, Benjamin Savenkoff, Denis Viglietti, Rebecca Sberro, Eric Rondeau, Julie Peltier
Although post-transplant lymphoproliferative disorder (PTLD) is the second most common type of cancer in kidney transplantation (KT), plasma cell neoplasia (PCN) occurs only rarely after KT, and little is known about its characteristics and evolution. We included twenty-two cases of post-transplant PCN occurring between 1991 and 2013. These included 12 symptomatic multiple myeloma, eight indolent myeloma and two plasmacytomas. The median age at diagnosis was 56.5 years and the median onset after transplantation was 66...
2017: PloS One
https://www.readbyqxmd.com/read/28613962/significant-association-between-renal-function-and-area-of-amyloid-deposition-in-kidney-biopsy-specimens-in-both-aa-amyloidosis-associated-with-rheumatoid-arthritis-and-al-amyloidosis
#6
Takeshi Kuroda, Naohito Tanabe, Eriko Hasegawa, Ayako Wakamatsu, Yukiko Nozawa, Hiroe Sato, Takeshi Nakatsue, Yoko Wada, Yumi Ito, Naofumi Imai, Mitsuhiro Ueno, Masaaki Nakano, Ichiei Narita
The kidney is a major target organ for systemic amyloidosis, which results in proteinuria and an elevated serum creatinine level. The clinical manifestations and precursor proteins of amyloid A (AA) and light-chain (AL) amyloidosis are different, and the renal damage due to amyloid deposition also seems to differ. The purpose of this study was to clarify haw the difference in clinical features between AA and AL amyloidosis are explained by the difference in the amount and distribution of amyloid deposition in the renal tissues...
June 14, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28584627/mixed-leukocyte-cell-derived-chemotaxin-2-and-amyloid-a-renal-amyloidosis-in-a-kazakh-german-patient
#7
Vega A Gödecke, Christoph Röcken, Lars Steinmüller-Magin, Felix Nadrowitz, Susanne V Fleig, Hermann Haller, Annette D Wagner
Leukocyte cell-derived chemotaxin 2 (LECT2)-related amyloidosis (ALECT2) constitutes a subtype of systemic amyloidosis affecting the kidney. This is the first case describing mixed ALECT2 and Amyloid A renal amyloidosis in a Kazakh-German patient. Genetic analysis shows a polymorphism in the LECT2 gene and a homozygous mutation in the SAA1 gene. Notably, our patient has a body mass index of 61 kg/m(2) and a pathological glucose tolerance test. ALECT2 was found in certain ethnic groups with a high incidence of diabetes...
April 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28578625/glomerular-lipidosis-in-dogs
#8
Rebecca A Kohnken, Hayley Amerman, Cathy A Brown, Eva Furrow, George E Lees, Rachel E Cianciolo
Glomerular lipidosis (GL) is characterized by dilated glomerular capillary loops containing lipid-laden cells (foam cells). Previously, GL was considered to be an incidental finding because affected dogs were typically not azotemic. However, the International Renal Interest Society staging system for canine chronic kidney disease has increased the awareness of other clinical parameters (eg, proteinuria and hypertension) that should be included in the assessment of renal function. As such, the aim of this study was to determine clinical abnormalities and concurrent renal lesions in dogs with GL...
January 1, 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/28550043/al-amyloidosis-patients-with-low-amyloidogenic-free-light-chain-levels-at-first-diagnosis-have-an-excellent-prognosis
#9
Tobias Dittrich, Tilmann Bochtler, Christoph Kimmich, Natalia Becker, Anna Jauch, Hartmut Goldschmidt, Anthony D Ho, Ute Hegenbart, Stefan O Schönland
The difference between involved minus uninvolved serum free light chains (dFLC) has been established as invaluable hematologic parameter in systemic light chain amyloidosis (AL). However, patients with initial dFLC below 50mg/L are currently deemed not evaluable for response to therapy. Therefore, we aimed to characterize this subgroup and to define novel hematologic response parameters. We retrospectively analyzed 783 AL patients newly diagnosed at our center between 2002 and 2016. Patients with dFLC<50 showed a smaller bone marrow plasmacytosis (7% vs...
May 26, 2017: Blood
https://www.readbyqxmd.com/read/28540891/chronic-renal-failure-an-autopsy-study
#10
Anitha Padmanabhan, Sanjay Gohil, N M Gadgil, Prerna Sachdeva
Diabetes and hypertension are at present the major causes of chronic kidney disease (CKD) and end-stage renal disease (ESRD) worldwide. The stages 0-5 of CKD are defined according to the estimated glomerular filtration rate. The term chronic renal failure (CRF) typically corresponds to CKD stages 3-5. Cardiovascular disease is the main cause of morbidity and mortality in patients of CRF and ESRD. This study was undertaken to analyze the age and sex incidence, clinical features, etiology, pathology of various organs in detail, and causes of death of CRF patients...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28536682/a-case-of-intramural-coronary-amyloidosis-associated-with-hemodialysis
#11
Faisal M Huq Ronny, George Kleinman, Paul James Kurtin, John Thomas Fallon
Dialysis-related amyloidosis predominantly occurs in osteo-articular structures and dialysis-related amyloid (DRA) substances also deposit in extra-articular tissues. Clinical manifestations of DRA include odynophagia, gastrointestinal hemorrhage, intestinal obstruction, kidney stones, myocardial dysfunction, and subcutaneous tumors. The pathological characteristics of DRA in the heart of hemodialysis patients have rarely been reported. We report the case of a 73-year-old female with a history of cerebral palsy and end-stage renal disease status post two failed renal transplants who had been on hemodialysis for 30 years...
January 2017: Autopsy & case reports
https://www.readbyqxmd.com/read/28456755/light-chain-cardiac-amyloidosis-strategies-to-promote-early-diagnosis-and-cardiac-response
#12
REVIEW
Martha Grogan, Angela Dispenzieri, Morie A Gertz
Amyloid light chain (AL) amyloidosis is a systemic disease characterised by the aggregation of misfolded immunoglobulin light chain (LC), predominantly in the heart and kidneys, causing organ failure. If untreated, the median survival of patients with cardiac AL amyloidosis is 6 months from the onset of heart failure. Protracted time to establish a diagnosis, often lasting >1 year, is a frequent factor in poor treatment outcomes. Cardiologists, to whom patients are often referred, frequently miss the opportunity to diagnose cardiac AL amyloidosis...
July 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28434330/significant-association-between-renal-function-and-area-of-amyloid-deposition-evident-in-kidney-biopsy-specimens-in-both-aa-and-al-amyloidosis
#13
Takeshi Kuroda, Yumi Ito, Naofumi Imai, Yukiko Nozawa, Hiroe Sato, Takeshi Nakatsue, Yoko Wada, Mitsuhiro Ueno, Masaaki Nakano, Ichiei Narita
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434302/fibrinogen-a-alpha-chain-amyloidosis-a-non-negligible-cause-of-chronic-kidney-disease-in-dialysis-patients
#14
Isabel Tavares, Luciana Moreira, Paulo Pinho Costa, Luísa Lobato
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28418359/-a-rare-concurrence-of-polymyalgia-rheumatica-and-aa-amyloidosis
#15
S G Radenska-Lopovok, O N Kotenko, N F Frolova, A I Zagrebneva, E S Stolyarevich, E V Volodina, V I Chervinko, E V Kryukov, M L Zubkin
Polymyalgia rheumatica (PMR) is a rare chronic inflammatory disease. It predominantly affects the elderly. The disease has a slow onset, pain and stiffness in the muscles of the shoulder and pelvic girdle, fever, weight loss, and a high acute-phase inflammatory response. The disease is concurrent with giant cell arteritis in a quarter of cases, which allows some authors to consider them as two different manifestations of the same pathological process. The kidneys are rarely involved. This disease is rarely complicated by AA amyloidosis...
2017: Arkhiv Patologii
https://www.readbyqxmd.com/read/28389707/the-race-of-10-synthetic-rnai-based-drugs-to-the-pharmaceutical-market
#16
REVIEW
Ricardo Titze-de-Almeida, Catherine David, Simoneide Souza Titze-de-Almeida
Ten years after Fire and Melo's Nobel Prize for discovery of gene silencing by double-stranded RNA, a remarkable progress was achieved in RNA interference (RNAi). Changes in the chemical structure of synthetic oligonucleotides make them more stable and specific, and new delivery strategies became progressively available. The attention of pharmaceutical industry rapidly turned to RNAi, as an opportunity to explore new drug targets. This review addresses nine small-interfering RNAs (siRNAs) and one unique microRNA (miRNA) inhibitor, which entered the phase 2-3 clinical trials...
April 7, 2017: Pharmaceutical Research
https://www.readbyqxmd.com/read/28359658/unrecognized-fibrinogen-a-%C3%AE-chain-amyloidosis-results-from%C3%A2-targeted-genetic-testing
#17
Isabel Tavares, João Paulo Oliveira, Ana Pinho, Luciana Moreira, Liliana Rocha, Josefina Santos, Joaquim Pinheiro, Paulo Pinho Costa, Luísa Lobato
BACKGROUND: Fibrinogen A α-chain (AFib) amyloidosis results from autosomal-dominant mutations in the gene encoding AFib (FGA). Patients with this disorder typically present with proteinuria. Isolated cases of AFib amyloidosis, carrying the FGA p.Glu545Val variant, were identified in the district of Braga, in northwest Portugal. This observation led us to hypothesize that this disorder might be an unrecognized cause of kidney disease in that region and prompted us to carry out targeted genetic testing for the p...
March 27, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28352020/profile-of-glomerular-diseases-associated-with-hepatitis-b-and-c-a-single-center-experience-from-india
#18
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/28352011/biopsy-of-small-kidneys-a-safe-and-a-useful-guide-to-potentially-treatable-kidney-disease
#19
Kamel El-Reshaid, Wael El-Reshaid, Dalal Al-Bader, Jozsef Varro, John Madda, Hosameldin Tawfik Sallam
Over the past four years, all patients with unexplained rapid progression of their renal disease were subjected to kidney biopsy, despite their small size (<9 cm), to define its etiology. Children, pregnant women, morbidly obese patients, and those with an unstable cardiovascular state, septicemia, bleeding diathesis as well as those kidney size with size <6 cm were excluded from the study. Doppler ultrasound was used to exclude renovascular/ischemic nephropathy. The procedure was performed by an interventional radiologist using a biopsy gun technique and under ultrasound guidance...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28340799/is-there-a-long-term-risk-for-donors-with-heterozygous-mefv-mutation-after-kidney-donation
#20
S Karakose, S Erdogmus, S Akturk, A Tuzuner, S Sengul, K Keven
BACKGROUND: Familial Mediterranean fever (FMF) is an autosomal-recessive autoinflammatory disorder manifested severely by systemic amyloidosis. It has been hypothesized that heterozygous carriers may also have susceptibility to certain symptoms or even diseases. Because the living kidney donors of patients with FMF are generally relatives of the kidney recipients, there is a high possibility that the donors will have a heterozygous mutation of the FMF gene. The goal of this study was to investigate the long-term kidney function of donors who are carriers of the Mediterranean fever (MEFV) gene...
April 2017: Transplantation Proceedings
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