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https://www.readbyqxmd.com/read/28340799/is-there-a-long-term-risk-for-donors-with-heterozygous-mefv-mutation-after-kidney-donation
#1
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
https://www.readbyqxmd.com/read/28339709/clinicopathologic-correlations-of-renal-pathology-in-adult-population-of-poland
#2
Agnieszka Perkowska-Ptasinska, Artur Bartczak, Malgorzata Wagrowska-Danilewicz, Agnieszka Halon, Krzysztof Okon, Aldona Wozniak, Marian Danilewicz, Henryk Karkoszka, Andrzej Marszalek, Jolanta Kowalewska, Andrzej Mroz, Agnieszka Korolczuk, Andrzej Oko, Alicja Debska-Slizien, Beata Naumnik, Zbigniew Hruby, Marian Klinger, Kazimierz Ciechanowski, Marek Myslak, Wladyslaw Sulowicz, Andrzej Rydzewski, Andrzej Wiecek, Jacek Manitius, Tadeusz Gregorczyk, Stanislaw Niemczyk, Michal Nowicki, Ryszard Gellert, Tomasz Stompor, Monika Wieliczko, Krzysztof Marczewski, Leszek Paczek, Olga Rostkowska, Dominika Deborska-Materkowska, Grazyna Bogdanowicz, Andrzej Milkowski, Magdalena Durlik
Background: This is the first report on the epidemiology of biopsy-proven kidney diseases in Poland. Methods: The Polish Registry of Renal Biopsies has collected information on all (n = 9394) native renal biopsies performed in Poland from 2009 to 2014. Patients' clinical data collected at the time of biopsy, and histopathological diagnoses were used for epidemiological and clinicopathologic analysis. Results: There was a gradual increase in the number of native renal biopsies performed per million people (PMP) per year in Poland in 2009-14, starting from 36 PMP in 2009 to 44 PMP in 2014...
February 11, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28298791/amyloidosis-and-anesthesia
#3
Zara Wani, Dev Kumar Harkawat, Meenaxi Sharma
AIM: The aim of this article is to provide a view of amyloidosis and discuss implications for the anesthetic management of patients with this condition. MATERIAL AND METHOD: Urine samples from patients with plasma cell dyscrasias were obtained from a urine bank that gathers urine samples from patients who gave research use consent for specimens that would otherwise be considered waste. RESULTS: Patients with amyloidosis may present to the anesthesiologist for procedures relating to diagnosis, surgery relating to the underlying condition (e...
January 2017: Anesthesia, Essays and Researches
https://www.readbyqxmd.com/read/28295502/assessment-of-renal-response-with-urinary-exosomes-in-patients-with-al-amyloidosis-a-proof-of-concept
#4
Marina Ramirez-Alvarado, David R Barnidge, David L Murray, Angela Dispenzieri, Marta Marin-Argany, Christopher J Dick, Shawna A Cooper, Samih H Nasr, Christopher J Ward, Surendra Dasari, Víctor H Jiménez-Zepeda, Nelson Leung
Immunoglobulin light chain (AL) amyloidosis is a fatal complication of B-cell proliferation secondary to deposition of amyloid fibrils in various organs. Urinary exosomes (UEX) are the smallest of the microvesicles excreted in the urine. Previously, we found UEX of patients with AL amyloidosis contained immunoglobulin light chain (LC) oligomers that patients with multiple myeloma did not have. To further explore the role of the LC oligomers, UEX was isolated from an AL amyloidosis patient with progressive renal disease despite achieving a complete response...
March 10, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28293126/the-risk-of-renal-disease-is-increased-in-lambda-myeloma-with-bone-marrow-amyloid-deposits
#5
Piotr Kozlowski, Scott Montgomery, Rahel Befekadu, Victoria Hahn-Strömberg
BACKGROUND: Light chain amyloidosis (AL) is a rare deposition disease and is present in 10-15% of patients with myeloma (MM). In contrast to symptomatic AL in MM, presence of bone marrow (BM) amyloid deposits (AD) in MM is not connected to kidney damage. Renal AD but not BM-AD occur mostly in MM with lambda paraprotein (lambda MM). METHODS: We investigated amyloid presence in BM clots taken at diagnosis in 84 patients with symptomatic MM and compared disease characteristics in MM with kappa paraprotein (kappa MM)/lambda MM with and without BM-AD...
2017: Journal of Blood Medicine
https://www.readbyqxmd.com/read/28290973/total-artificial-heart-implantation-as-a-bridge-to-heart-transplantation-in-an-active-duty-service-member-with-amyloid-cardiomyopathy
#6
Michael S Scully, Dylan E Wessman, James M McKee, Gregory M Francisco, Keshav R Nayak, Jon A Kobashigawa
INTRODUCTION: Cardiac involvement by light-chain (AL) amyloid occurs in up to 50% of patients with primary AL amyloidosis. The prognosis of amyloid heart disease is poor with 1-year survival rates of 35 to 40%. Historically, heart transplantation was considered controversial for patients with AL amyloid cardiomyopathy (CM) given the systemic nature of the disease and poor survival. We present a case report of an active duty service member diagnosed with advanced cardiac amyloid who underwent total artificial heart transplant as a bridge to heart transplant and eventual autologous stem cell transplant...
March 2017: Military Medicine
https://www.readbyqxmd.com/read/28272196/transthyretin-related-hereditary-amyloidosis-with-recurrent-vomiting-and-renal-insufficiency-as-the-initial-presentation-a-case-report
#7
Jing Xu, Meng Yang, Xiaoxia Pan, Xialian Yu, Jingyuan Xie, Hong Ren, Xiao Li, Nan Chen
RATIONALE: Hereditary amyloidosis is diagnosed worldwidely with an increasing incidence. As the most common form, transthyretin-related hereditary amyloidosis (ATTR amyloidosis) is an autosomal dominant inherited disease due to mutations of TTR. Over the past several decades, more than 130 mutations have been reported. Previous studies suggested that ATTR amyloidosis initially showed polyneuropathy and autonomic dysfunction but later involving many visceral organs, such as kidney. PATIENT CONCERNS: A young proband carrying TTR p...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28260482/late-diagnosis-of-e148q-mutation-positive-familial-mediterranean-fever-in-a-kidney-transplant-patient-with-fever-of-unknown-origin-a-case-report
#8
Erhan Tatar, Adam Uslu, Cenk Simsek, Ahmet Aykas, Giray Bozkaya, Cetin Imamoglu
Fever of unknown origin is a rare condition after solid organ transplant and is generally associated with atypical infections (eg, tuberculosis, fungal infections) and/or lymphoproliferative disorders. Here, we present a kidney transplant patient with a late diagnosis of E148Q mutation-positive familial Mediterranean fever as the cause of fever of unknown origin. A 22-year-old female patient with a previous history of 4 years of hemodialysis and unknown primary renal disease received a deceased-donor kidney transplant at our center 5 years previously...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28260476/infections-after-renal-transplant-in-recipients-with-familial-mediterranean-fever-a-life-threatening-issue
#9
Erhan Tatar, Murat Karatas, Ahmet Aykas, Gokalp Okut, Giray Bozkaya, Adam Uslu
OBJECTIVES: We evaluated long-term results and infections requiring hospitalization in kidney transplant patients with Familial Mediterranean Fever (associated amyloidosis-type). MATERIALS AND METHODS: We retrospectively reviewed medical records of patients with familial Mediterranean fever with at least 1-year posttransplant follow-up. Kidney transplant recipients with primary glomerulonephritis and equivalent demography, immunity status, and follow-up comprised the control group...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28255922/the-clinical-and-pathological-characteristics-of-nephropathies-in-connective-tissue-diseases-in-the-japan-renal-biopsy-registry-j-rbr
#10
Kazunobu Ichikawa, Tsuneo Konta, Hiroshi Sato, Yoshihiko Ueda, Hitoshi Yokoyama
BACKGROUND: In connective tissue diseases, a wide variety of glomerular, tubulointerstitial, and vascular lesions of the kidney are observed. Nonetheless, recent information is limited regarding renal lesions in connective tissue diseases, except in systemic lupus erythematosus (SLE). METHODS: In this study, we used a nationwide database of biopsy-confirmed renal diseases in Japan (J-RBR) (UMIN000000618). In total, 20,523 registered patients underwent biopsy between 2007 and 2013; from 110 patients with connective tissue diseases except SLE, we extracted data regarding the clinico-pathological characteristics of the renal biopsy...
March 2, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28254515/hereditary-cerebral-small-vessel-disease-and-stroke
#11
REVIEW
Christian Baastrup Søndergaard, Jørgen Erik Nielsen, Christine Krarup Hansen, Hanne Christensen
Cerebral small vessel disease is considered hereditary in about 5% of patients and is characterized by lacunar infarcts and white matter hyperintensities on MRI. Several monogenic hereditary diseases causing cerebral small vessel disease and stroke have been identified. The purpose of this systematic review is to provide a guide for determining when to consider molecular genetic testing in patients presenting with small vessel disease and stroke. CADASIL, CARASIL, collagen type IV mutations (including PADMAL), retinal vasculopathy with cerebral leukodystrophy, Fabry disease, hereditary cerebral hemorrhage with amyloidosis, and forkhead box C1 mutations are described in terms of genetics, pathology, clinical manifestation, imaging, and diagnosis...
April 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28229334/specific-amyloid-binding-of-polybasic-peptides-in-vivo-is-retained-by-%C3%AE-sheet-conformers-but-lost-in-the-disrupted-coil-and-all-d-amino-acid-variants
#12
Jonathan S Wall, Angela Williams, Tina Richey, Alan Stuckey, Craig Wooliver, J Christopher Scott, Robert Donnell, Emily B Martin, Stephen J Kennel
PURPOSE: The heparin-reactive, helical peptide p5 is an effective amyloid imaging agent in mice with systemic amyloidosis. Analogs of p5 with modified secondary structure characteristics exhibited altered binding to heparin, synthetic amyloid fibrils, and amyloid extracts in vitro. Herein, we further study the effects of peptide helicity and chirality on specific amyloid binding using a mouse model of systemic inflammation-associated (AA) amyloidosis. PROCEDURES: Peptides with disrupted helical structure [p5(coil) and p5(Pro3)], with an extended sheet conformation [p5(sheet)] or an all-D enantiomer [p5(D)], were chemically synthesized, radioiodinated, and their biodistribution studied in WT mice as well as transgenic animals with severe systemic AA amyloidosis...
February 22, 2017: Molecular Imaging and Biology: MIB: the Official Publication of the Academy of Molecular Imaging
https://www.readbyqxmd.com/read/28153807/amyloidosis-a-cancer-derived-paraproteinemia-and-kidney-involvement
#13
REVIEW
Jolanta Małyszko, Klaudia Kozłowska, Jacek Stanisław Małyszko
Amyloidosis is the general term describing the extracellular tissue deposition of fibrils composed of low molecular weight subunits of a variety of proteins. There are multiple different human protein precursors of amyloid fibrils. Amyloid deposits are stained using Congo Red and show typical apple-green birefringence in polarized microscopy. Nowadays, a novel technique LMD/MS technique or laser microdissection combined with mass spectrometry help to diagnose amyloidosis. Amyloidosis of the kidney is typically classified as being either one of two types: AL or AA...
January 30, 2017: Advances in Medical Sciences
https://www.readbyqxmd.com/read/28145535/amyloid-cardiomyopathy
#14
Lucie Karafiatova, Tomas Pika
Amyloidosis is a heterogeneous group of diseases characterized by the deposition of amyloid. It is caused by extracellular deposition of insoluble fibrils with beta-pleated sheet configuration. The protein misfolding abnormalities result in amyloid fibrils and may manifest as primary, secondary, or familial amyloidosis. Amyloid deposition can occur in multiple organs (eg, heart, liver, kidney, skin, eyes, lungs, nervous system) resulting in a variety of clinical manifestations. Cardiac involvement can occur as part of a systemic disease or as a localized phenomenon...
February 1, 2017: Biomedical Papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
https://www.readbyqxmd.com/read/28139293/clinical-biopsy-and-mass-spectrometry-findings-of-renal-gelsolin-amyloidosis
#15
Sanjeev Sethi, Surendra Dasari, Md Shahrier Amin, Julie A Vrana, Jason D Theis, Mariam P Alexander, Paul J Kurtin
Gelsolin amyloidosis is a rare type of amyloidosis typically involving the cranial and peripheral nerves, but rarely the kidney. Here we report the clinical, kidney biopsy, and mass spectrometry findings in 12 cases of renal gelsolin amyloidosis. Of the 12 patients, five were men and seven were women with mean age at diagnosis of 63.8 years. Gelsolin amyloidosis was most common in Caucasians (six patients) and Asians (four patients), and included one each African-American and Hispanic patients. Nephrotic syndrome was the most common cause of biopsy, although most patients also had progressive loss of kidney function...
January 27, 2017: Kidney International
https://www.readbyqxmd.com/read/28108533/noncerebral-amyloidoses-aspects-on-seeding-cross-seeding-and-transmission
#16
Gunilla T Westermark, Marcus Fändrich, Katarzyna Lundmark, Per Westermark
More than 30 proteins form amyloid in humans, most of them outside of the brain. Deposition of amyloid in extracerebral tissues is very common and seems inevitable for an aging person. Most deposits are localized, small, and probably without consequence, but in some instances, they are associated with diseases such as type 2 diabetes. Other extracerebral amyloidoses are systemic, with life-threatening effects on the heart, kidneys, and other organs. Here, we review how amyloid may spread through seeding and whether transmission of amyloid diseases may occur between humans...
January 20, 2017: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/28098102/evaluation-of-renal-lesions-and-clinicopathologic-correlation-in-rheumatoid-arthritis
#17
Periyasamy Muthukumar, Jeyachandran Dhanapriya, Natarajan Gopalakrishnan, Thanigachalam Dineshkumar, Ramanathan Sakthirajan, T Balasubramaniyan
The most common causes of renal disease in rheumatoid arthritis (RA) are glomerulonephritis (GN), amyloidosis, tubulo-interstitial nephritis, and drug toxicity. Our aim was to evaluate the clinicopathologic correlation of renal lesions and to assess the course and prognosis of renal disease in patients with RA. We conducted a prospective observational study in all adult patients with RA between July 2010 and June 2015. The total number of patients studied was 90, with a female:male ratio of 2.3:1. Mean follow-up duration was 30 ± 6...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28087478/renal-involvement-in-lysinuric-protein-intolerance-contribution-of-pathology-to-assessment-of-heterogeneity-of-renal-lesions
#18
Emmanuel Estève, Pauline Krug, Aurélie Hummel, Jean-Baptiste Arnoux, Olivia Boyer, Anais Brassier, Pascale de Lonlay, Vincent Vuiblet, Stéphanie Gobin, Rémi Salomon, Christine Piètrement, Jean-Paul Bonnefont, Aude Servais, Louise Galmiche
Lysinuric protein intolerance (LPI) is a rare autosomal recessive disease caused by mutations in the SLC7A7 gene encoding the light subunit of a cationic amino acid transporter. Symptoms mimic primary urea cycle defects but dysimmune symptoms are also described. Renal involvement in LPI was first described in the 1980's. In 2007, it appeared that it could concern as much as 75% of LPI patients and could lead to end stage renal disease. The most common feature is proximal tubular dysfunction and nephrocalcinosis but glomerular lesions are also reported...
January 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/28078835/pathological-characteristics-of-fifty-patients-with-renal-amyloidosis-in-sri-lanka
#19
D L Dasanayaka, S Wijetunge, N V Ratnatunga
Renal involvement is the most important cause of morbidity and mortality in systemic amyloidosis. This retrospective analysis was conducted to analyse the clinico-pathological characteristics of renal amyloidosis in a group of Sri Lankan patients undergoing renal biopsy. Renal amyloidosis was observed in 50/ 9712 (0.5%) renal biopsies. The underlying cause for amyloidosis was not known in most. Of the known causes multiple myeloma was the commonest. Nephrotic range proteinuria was the most common clinical outcome and most had grade I to III chronic kidney disease at the time of diagnosis...
December 30, 2016: Ceylon Medical Journal
https://www.readbyqxmd.com/read/28049394/coexistence-of-amyloidosis-and-primary-sj%C3%A3-gren-s-syndrome-an-overview-amyloidosis-and-sj%C3%A3-gren-s-syndrome
#20
Gabriela Hernandez-Molina, David Faz-Muñoz, Miguel Astudillo-Angel, Alejandra Iturralde-Chávez, Edgardo Reyes
BACKGROUND: The association of primary Sjögren´s syndrome (SS) and amyloidosis has been recognized but scarcely assessed. OBJECTIVE: Herein we analyzed the literature regarding this association in order to describe a SS phenotype prone to both conditions. METHODS: PubMed and CINAHL databases were searched from inception until April 2016 for the key words: amyloidosis, amyloid, Sjögren's syndrome and their combinations. RESULTS: Most of the cases corresponded to localized amyloidosis mainly at skin and lung, whereas only three cases were systemic...
January 3, 2017: Current Rheumatology Reviews
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