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https://www.readbyqxmd.com/read/28926523/familial-mediterranean-fever-is-associated-with-increased-mortality-after-kidney-transplantation-a-19-years-single-center-experience
#1
Hefziba Green, Shelly Lichtenberg, Ruth Rahamimov, Avi Livneh, Avry Chagnac, Eytan Mor, Benaya Rozen-Zvi
BACKGROUND: Current data regarding the outcome of kidney transplantation in patients with familial Mediterranean fever (FMF) who reach end-stage renal disease (ESRD) due to reactive amyloidosis A (AA) are scarce and inconclusive. METHODS: The outcomes of 20 patients with FMF and biopsy-proven AA amyloidosis that were transplanted between 1995 and 2014 were compared with 82 control patients (32 with diabetes mellitus and 50 with nondiabetic kidney disease). Major outcome data included overall patient and graft survivals...
October 2017: Transplantation
https://www.readbyqxmd.com/read/28903572/biomarkers-in-immunoglobulin-light-chain-amyloidosis
#2
Z Kufová, T Sevcikova, K Growkova, P Vojta, J Filipová, Z Adam, L Pour, M Penka, R Rysava, P Němec, L Brozova, P Vychytilova, A Jurczyszyn, S Grosicki, A Barchnicka, M Hajdúch, M Simicek, R Hájek
Immunoglobulin light chain amyloidosis (AL amyloidosis - ALA) is a monoclonal gammopathy characterized by presence of aberrant plasma cells producing amyloidogenic immunoglobulin light chains. This leads to formation of amyloid fibrils in various organs and tissues, mainly in heart and kidney, and causes their dysfunction. As amyloid depositing in target organs is irreversible, there is a big effort to identify biomarker that could help to distinguish ALA from other monoclonal gammopathies in the early stages of disease, when amyloid deposits are not fatal yet...
2017: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
https://www.readbyqxmd.com/read/28891012/visualization-of-multiple-organ-amyloid-involvement-in-systemic-amyloidosis-using-11-c-pib-pet-imaging
#3
Naoki Ezawa, Nagaaki Katoh, Kazuhiro Oguchi, Tsuneaki Yoshinaga, Masahide Yazaki, Yoshiki Sekijima
PURPOSE: To investigate the utility of Pittsburgh compound B (PiB) positron emission tomography (PET) imaging for evaluating whole-body amyloid involvement in patients with systemic amyloidosis. METHODS: Whole-body (11)C-PiB PET was performed in seven patients with systemic immunoglobulin light-chain (AL) amyloidosis, seven patients with hereditary transthyretin (ATTRm) amyloidosis, one asymptomatic TTR mutation carrier and three healthy controls. The correlations between clinical organ involvement, radiological (11)C-PiB uptake and histopathological findings were analysed for each organ...
September 10, 2017: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28885498/patient-and-kidney-allograft-survival-in-recipients-with-end-stage-renal-disease-from-amyloidosis
#4
Deirdre Sawinski, Mary Ann Lim, Jordana C Cohen, Jayme E Locke, Brendan Weiss, Jonathan J Hogan, Laura M Dember
BACKGROUND: Outcomes following kidney transplantation for patients with amyloidosis-associated end-stage renal disease (ESRD) have not been well characterized. METHODS: We performed a retrospective propensity score matched cohort study with Cox proportional hazards modeling using data from the United Network of Organ Sharing including patients transplanted from 1987 to 2015 (N=310 629). RESULTS: Amyloidosis patients (N=576) had higher rates of death (HR 1...
September 6, 2017: Transplantation
https://www.readbyqxmd.com/read/28885307/the-treatment-of-paraprotein-related-kidney-disease
#5
Colin A Hutchison, Fei Xiong, Peter Mollee
PURPOSE OF REVIEW: Myeloma kidney and amyloid light-chain (AL) amyloidosis remain the principal kidney complications of paraproteins. In this review, we update readers to many of the recent advances which have occurred in the care and outcomes for patients with these presentations. RECENT FINDINGS: Myeloma kidney has historically caused a severe acute kidney injury with very poor outcomes. The combination of new diagnostic techniques, enabling a rapid diagnosis and novel chemotherapy agents has transformed these poor outcomes for the better...
September 6, 2017: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/28871936/xanthoma-like-skin-changes-in-an-elderly-woman-with-a-normal-lipid-profile
#6
Piotr Nockowski, Zdzisław Woźniak, Adam Reich, Joanna Maj
Dear Editor, An 83-year-old woman developed yellow-brownish infiltrates, nodules, and tumors mimicking xanthomas, mostly involving the periorbital and chest area within three months (Figure 1). She had no abnormalities in serum cholesterol or triglycerides levels. A detailed laboratory analysis revealed the presence of mild monoclonal gammopathy with a presence of immunoglobulin G (IgG) kappa light chains; however, according to hematologist consultation, it did not require medical intervention. Imaging assessment and ultrasound examination did not show any specific involvement of internal organs...
July 2017: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/28869938/mineral-and-bone-disorder-in-chronic-kidney-disease-a-case-report-from-vietnam
#7
Pham Van Bui, Nguyyen Minh Tuan, Huynh Thi Nguyen Nghia, Pham Nguyen Thanh Phuong
We report a case of calcium pyrophosphate dihydrate deposition disease (CPDD) involving a patient on maintenance hemodialysis (MHD). The 32-year-old man presented in August 2016 with a complaint of left shoulder swelling of 8 months' duration with no trauma or fever. He was diagnosed with nephrotic syndrome in 1998, which progressed to ESRD. He commenced MHD in 2012. Examination at our hospital revealed a soft nontender swelling of the left shoulder. Blood biochemistry showed elevated serum urate, phosphate, β2 microglobulin, and parathyroid hormone...
2017: Blood Purification
https://www.readbyqxmd.com/read/28865972/high-dose-melphalan-and-stem-cell-transplantation-in-patients-on-dialysis-due-to-al-amyloidosis-and-monoclonal-immunoglobulin-deposition-disease
#8
Felipe Batalini, Laura Econimo, Karen Quillen, J Mark Sloan, Shayna Sarosiek, Dina Brauneis, Andrea Havasi, Lauren Stern, Laura Dember, Vaishali Sanchorawala
The kidney is the most common organ affected by immunoglobulin light-chain (AL) amyloidosis and monoclonal immunoglobulin deposition disease (MIDD), often leading to end stage renal disease (ESRD). High-dose melphalan and stem cell transplantation (HDM/SCT) is effective for selected patients with AL amyloidosis, with high rates of complete hematologic responses and potential for improvement in organ dysfunction. Data on tolerability and response to HDM/SCT in patients with ESRD due to AL amyloidosis and MIDD are limited...
August 30, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28859624/living-kidney-transplantation-between-brothers-with-unrecognized-renal-amyloidosis-as-the-first-manifestation-of-familial-mediterranean-fever-a-case-report
#9
Ramón Peces, Sara Afonso, Carlos Peces, Julián Nevado, Rafael Selgas
BACKGROUND: Familial Mediterranean fever is an autosomal recessive disease characterized by recurrent episodes of fever and polyserositis and by the onset of reactive amyloid-associated amyloidosis. Amyloidosis due to familial Mediterranean fever can lead to end-stage renal disease, culminating in kidney transplantation for some patients. In this study, we report the clinical outcome of two brothers with familial Mediterranean fever who were the inadvertent donor and recipient, respectively, of a kidney...
August 31, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28832315/amyloidosis-and-acute-hemorrhage-of-the-kidney-ureter-and-bladder
#10
Dany N Hanna, Jason A Levy, Jonah S Marshall
Gross hematuria is a common occurrence in adults. The differential diagnosis is extensive, including: malignancy, trauma, inflammation of the urinary tract, and stones. While, urinary tract amyloidosis represents only a small percentage of causative gross hematuria, it is concerning because of its superficial resemblance to malignant processes. We report the case of an 82-year-old male with concurrent primary amyloidosis of the kidney, ureter and bladder in the setting of acute hemorrhage. Histopathological examination of several biopsied samples confirmed our diagnosis...
August 2017: Canadian Journal of Urology
https://www.readbyqxmd.com/read/28828707/pathology-and-diagnosis-of-renal-non-al-amyloidosis
#11
REVIEW
Sanjeev Sethi, Jason D Theis
Renal amyloidosis is characterized by acellular Congo red positive deposits in the glomeruli, interstitium and/or arteries. Light chain restriction on immunofluorescence studies is present in AL-amyloidosis, the most common type of amyloidosis involving the kidney. The detection of Congo red positive deposits coupled with negative immunofluorescence studies is highly suggestive of non-AL amyloidosis. Some of the non-AL amyloidosis are common while others are relatively rare. The clinical features, laboratory and renal pathology findings are helpful in the diagnosis and typing of non-AL amyloidosis...
August 21, 2017: Journal of Nephrology
https://www.readbyqxmd.com/read/28827166/hemin-is-able-to-disaggregate-lysozyme-amyloid-fibrils-into-monomers
#12
Sumalata Sonavane, Syeda Zehra Haider, Anil Kumar, Basir Ahmad
Lysozyme amyloidosis (ALys) is a disease of the gastrointestinal tract, liver and kidneys, which is caused by the accumulation of insoluble fibrils of lysozyme in the tissues of above organs. The ALys can be cured by disintegration and clearance of the fibrils from the affected tissues and organs. It is thought that protein fibrils are extremely stable. Consequently, small molecule-induced dissociation of fibrils under physiological conditions is really challenging. Here, we report kinetic and thermodynamic analyses of hemin-induced dissociation of hen egg white lysozyme amyloid fibrils...
August 18, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28808991/light-chain-al-amyloidosis-the-journey-to-diagnosis
#13
Kristen L McCausland, Michelle K White, Spencer D Guthrie, Tiffany Quock, Muriel Finkel, Isabelle Lousada, Martha S Bayliss
BACKGROUND: Light chain (AL) amyloidosis is a rare, complex disease associated with significant morbidity and mortality. Delays in diagnosis are common and may have detrimental consequences on patients' prognosis. Too little is known regarding the patient journey to diagnosis. OBJECTIVE: The objective of this study was to describe the patient-reported journey to a correct diagnosis for AL amyloidosis. METHODS: Using a mixed-methods approach, data were collected from clinician (n = 4) and patient (n = 10) interviews and a survey of community-based patients with AL amyloidosis (n = 341)...
August 14, 2017: Patient
https://www.readbyqxmd.com/read/28803538/pharmacokinetics-of-tafamidis-a-transthyretin-amyloidosis-drug-in-rats
#14
Kyeong-Ryoon Lee, Jong-Woo Jeong, Hun-Chan Hyun, Jang Eunseo, Sunjoo Ahn Ahn, Sungwook Choi, Sang Hoon Joo, Sung-Sub Kim, Tae-Sung Koo
1. We characterized the pharmacokinetics of tafamidis, a novel drug to treat transthyretin-related amyloidosis, in rats after intravenous and oral administration at doses of 0.3-3 mg/kg. In vitro Caco-2 cell permeability and liver microsomal stability, as well as in vivo tissue distribution and plasma protein binding were also examined. 2. After intravenous injection, systemic clearance (CL), volumes of distribution at steady state (Vss), and half-life (T½) remained unaltered as a function of dose, with values in the ranges of 6...
August 14, 2017: Xenobiotica; the Fate of Foreign Compounds in Biological Systems
https://www.readbyqxmd.com/read/28802308/vitreous-amyloidosis-with-autonomic-neuropathy-of-the-digestive-tract-associated-with-a-novel-transthyretin-p-gly87arg-variant-in-a-bangladeshi-patient-a-case-report
#15
Benjamin Terrier, Magali Colombat, Caroline Beugnet, Astrid Quéant, Jonathan London, Jean-Baptiste Daudin, Claire Le Jeunne, Luc Mouthon, Dominique Monnet, Cécile Cauquil, Catherine Lacroix, David Adams, Antoine Brézin, Sophie Valleix
BACKGROUND: Hereditary transthyretin amyloidosis is an autosomal dominant inherited disorder, first described in families with sensorimotor and autonomic neuropathy. Since its first description, more than 120 amyloidogenic transthyretin mutations have been reported with various geographic distributions and associated with a wide range of phenotypes involving the peripheral nerve, the heart, the gastrointestinal tract, the eyes, the central nervous system, or the kidneys. In some cases of transthyretin amyloidosis, the first clinical manifestation is vitreous opacity...
August 13, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28794296/recurrent-aa-amyloidosis-combined-with-chronic-active-antibody-mediated-rejection-after-kidney-transplantation
#16
Min-Kyung Yeo, Young Rok Ham, Song-Yi Choi, Yong-Moon Lee, Moon Hyang Park, Kwang-Sun Suh
Kidney transplantation for amyloidosis remains a contentious issue. Recurrence of amyloidosis is one of the risks of transplantation. Chronic active antibody-mediated rejection is an important cause of chronic allograft dysfunction. A 47-year-old woman underwent kidney transplantation due to renal AA amyloidosis with unknown etiology. Six years posttransplantation, a kidney biopsy showed AA amyloidosis with chronic active antibody-mediated rejection. Donor-specific antibody class II was positive. The patient underwent intravenous plasmapheresis and treatment with rituximab and colchicine...
July 2017: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/28790241/-amyloidoma-adjacent-to-the-thoracic-vertebral-body-developed-into-systemic-amyloid-light-chain-amyloidosis
#17
Kyoko Tanaka, Hideto Goto, Shuhei Ikeda, Nami Masumoto, Katsuya Watanabe, Motofumi Tsubakihara
Amyloidoma is very rare case of amyloidosis, and seldom develop into systemic disease. We report a case of an 82-year-old man who was referred to our hospital because of an oppressive feeling in the upper chest. Chest computed tomography showed a tumor on thoracic vertebral body. Percutaneous needle biopsy showed pathological findings of AL amyloidosis. No obvious systemic finding was confirmed, and the tumor was diagnosed as amyloidoma. After a 1-year observation, heart amyloidosis was appeared, then 6 months later he died...
August 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28758811/novel-pathologic-scoring-tools-predict-end-stage-kidney-disease-in-light-chain-al-amyloidosis
#18
Samuel Rubinstein, Robert F Cornell, Liping Du, Beatrice Concepcion, Stacey Goodman, Shelton Harrell, Sara Horst, Daniel Lenihan, David Slosky, Agnes Fogo, Anthony Langone
BACKGROUND AND OBJECTIVES: Light chain (AL) amyloidosis frequently involves the kidney, causing significant morbidity and mortality. A pathologic scoring system with prognostic utility has not been developed. We hypothesized that the extent of amyloid deposition and degree of scarring injury on kidney biopsy, could provide prognostic value, and aimed to develop pathologic scoring tools based on these features. METHODS: This is a case-control study of 39 patients treated for AL amyloidosis with biopsy-proven kidney involvement at a large academic medical center...
July 31, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28745684/-autoinflammatory-diseases-and-kidney-involvement
#19
EDITORIAL
N A Mukhin, M V Bogdanova, V V Rameev, L V Kozlovskaya
Autoinflammatory disease (AID) is a new concept formulated from the results of studying the pathogenesis of familial periodic fevers, a heterogeneous group of genetically determined diseases characterized by causelessly recurrent exacerbations of the inflammatory process due to genetically determined disorders of innate immunity and accompanied by uncontrolled hypersecretion of interleukin-1 (IL-1). These mechanisms were a basic model for understanding a wide range of rheumatologic and other inflammatory diseases of the internal organs...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28739313/cardiac-amyloidosis-an-update-on-pathophysiology-diagnosis-and-treatment
#20
REVIEW
Omar K Siddiqi, Frederick L Ruberg
The amyloidoses are a group of systemic diseases characterized by organ deposition of misfolded protein fragments of diverse origins. The natural history of the disease, involvement of other organs, and treatment options vary significantly based on the protein of origin. In AL amyloidosis, amyloid protein is derived from immunoglobulin light chains, and most often involves the kidneys and the heart. ATTR amyloidosis is categorized as mutant or wild-type depending on the genetic sequence of the transthyretin (TTR) protein produced by the liver...
July 13, 2017: Trends in Cardiovascular Medicine
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