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systemic amyloidosis

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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/28723285/case-244-systemic-amyloidosis-a-complication-of-waldenstr%C3%A3-m-macroglobulinemia
#2
Maja Hrabak-Paar, Marko Kralik
History A 68-year-old man was admitted to the hospital for work-up because of generalized fatigue, anorexia, chronic diarrhea, and weight loss. Laboratory work-up revealed an erythrocyte sedimentation rate of 58 mm/h (reference range, 3-23 mm/h), a hemoglobin level of 14.1 g/dL (reference range, 13.8-17.5 g/dL), a leukocyte count of 8.1 × 10(9)/L (reference range, [3.4-9.7] × 10(9)/L), a platelet count of 223 × 10(9)/L (reference range, [158-424] × 10(9)/L), an alkaline phosphatase level of 85 U/L (1.42 μkat/L) (normal level, <142 U/L [2...
August 2017: Radiology
https://www.readbyqxmd.com/read/28719236/applying-an-artificial-neural-network-model-for-developing-a-severity-score-for-patients-with-hereditary-amyloid-polyneuropathy
#3
Shenia Novis, Felipe Machado, Victor B Costa, Debora Foguel, Marcia W Cruz, José Manoel de Seixas
Hereditary (familial) amyloid polyneuropathy (FAP) is a systemic disease that includes a sensorimotor polyneuropathy related to transthyretin (TTR) mutations. So far, a scale designed to classify the severity of this disease has not yet been validated. This work proposes the implementation of an artificial neural network (ANN) in order to develop a severity scale for monitoring the disease progression in FAP patients. In order to achieve this goal, relevant symptoms and laboratory findings were collected from 98 Brazilian patients included in THAOS - the Transthyretin Amyloidosis Outcomes Survey...
July 18, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28705053/cardiac-amyloidosis-pathogenesis-clinical-context-diagnosis-and-management-options
#4
Hassan Alkhawam, Darshan Patel, James Nguyen, Saumya Mariam Easaw, Mohammed Al-Sadawi, Umer Syed, Feras Zaiem, Maher Homsi, Timothy J Vittorio
Amyloidosis covers a group of disorders that can manifest in virtually any organ system in the body and is thought to be secondary to misfolding of extracellular proteins with subsequent deposition in tissues. The precursor protein that is produced in excess defines the specific amyloid type. This requires histopathological confirmation using Congo-red dye with its characteristic demonstration of green birefringence under cross-polarized light. There are three main types of amyloidosis associated with cardiac involvement: light-chain (AL), familial or senile (ATTR), and secondary (AA) amyloidosis...
July 14, 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28701686/congestive-heart-failure-with-preserved-ejection-fraction-in-a-patient-with-light-chain-al-amyloidosis-and-multiple-myeloma
#5
Edgar Francisco Carrizales-Sepúlveda, Alejandro Ordáz-Farías, Raymundo Vera-Pineda, Mario Alberto Benavides-González, Ramiro Flores-Ramírez
BACKGROUND Amyloidosis is characterized by tissue deposition of insoluble fibrillar proteins and it affects almost every organ; there are many types and the heart can be affected in all of them. CASE REPORT Our report describes a middle-aged man who presented to the Emergency Department with congestive heart failure. Clinical, electrocardiographic, and echocardiographic findings suggested the presence of an infiltrative disease, so an abdominal fat tissue biopsy was performed. A final diagnosis of systemic amyloidosis with heart involvement was made...
July 13, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28699793/predictors-of-early-treatment-failure-following-initial-therapy-for-systemic-immunoglobulin-light-chain-amyloidosis
#6
Nidhi Tandon, Surbhi Sidana, S Vincent Rajkumar, Angela Dispenzieri, Morie A Gertz, Martha Q Lacy, Robert A Kyle, Francis K Buadi, David Dingli, Suzanne R Hayman, Amie L Fonder, Miriam A Hobbs, Wilson I Gonsalves, Prashant Kapoor, Yi Lisa Hwa, Nelson Leung, Ronald S Go, John A Lust, Stephen J Russell, Steven R Zeldenrust, Shaji K Kumar
We analysed factors predicting early treatment failure (ETF), after first-line therapy for light-chain amyloidosis (AL). AL amyloidosis patients seen at Mayo Clinic within 90 days of diagnosis, from 2006 to 2015, excluding those who died within 3 months of initial therapy, were analysed retrospectively. ETF was defined as progression requiring treatment change or death within 12 (ETF12) or 24 (ETF24) months of first-line treatment. Non-ETF included those with a follow-up of more than 12 or 24 months who had progression beyond 12 or 24 months...
July 12, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28697378/changes-in-phagocytosis-and-potassium-channel-activity-in-microglia-of-5xfad-mice-indicate-alterations-in-purinergic-signaling-in-a-mouse-model-of-alzheimer-s-disease
#7
Stefan Wendt, Meron Maricos, Natascha Vana, Niklas Meyer, Dilansu Guneykaya, Marcus Semtner, Helmut Kettenmann
As the immunocompetent cells of the central nervous system, microglia accumulate at amyloid beta plaques in Alzheimer's disease (AD) and acquire a morphological phenotype of activated microglia. Recent functional studies, however, indicate that in mouse models of amyloidosis and AD, these cells are rather dysfunctional indicated by a reduced phagocytic activity. Here, we report that this reduction in phagocytic activity is associated with perturbed purinergic receptor signaling, since phagocytosis could be stimulated by P2Y6 receptor activation in control, but not in 5xFAD transgenic animals, an animal model of amyloid deposition...
June 8, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28696431/synaptic-activity-protects-against-ad-and-ftd-like-pathology-via-autophagic-lysosomal-degradation
#8
Y Akwa, E Gondard, A Mann, E Capetillo-Zarate, E Alberdi, C Matute, S Marty, T Vaccari, A M Lozano, E E Baulieu, D Tampellini
Changes in synaptic excitability and reduced brain metabolism are among the earliest detectable alterations associated with the development of Alzheimer's disease (AD). Stimulation of synaptic activity has been shown to be protective in models of AD beta-amyloidosis. Remarkably, deep brain stimulation (DBS) provides beneficial effects in AD patients, and represents an important therapeutic approach against AD and other forms of dementia. While several studies have explored the effect of synaptic activation on beta-amyloid, little is known about Tau protein...
July 11, 2017: Molecular Psychiatry
https://www.readbyqxmd.com/read/28686088/changing-epidemiology-of-aa-amyloidosis-clinical-observations-over-25-years-at-a-single-national-referral-centre
#9
Thirusha Lane, Jennifer H Pinney, Janet A Gilbertson, David F Hutt, Dorota M Rowczenio, Shameem Mahmood, Sajitha Sachchithanantham, Marianna Fontana, Taryn Youngstein, Candida C Quarta, Ashutosh D Wechalekar, Julian D Gillmore, Philip N Hawkins, Helen J Lachmann
OBJECTIVE: Systemic AA amyloidosis is a serious complication of chronic inflammation; however, there are relatively few published data on its incidence. We investigated the changing epidemiology of AA amyloidosis over a 25-year period at a single national referral centre. METHODS: We conducted a retrospective study of all patients diagnosed with AA amyloidosis who had attended the centre between 1990 and 2014 inclusive. Six hundred and twenty-five patients were studied in three cohorts: C1: 1990-1997; C2: 1998-2006; C3: 2007-2014...
July 7, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28680784/urethral-amyloidosis-in-a-patient-with-iga-nephropathy-after-renal-transplant
#10
Nicholas Ojile, Mona Brake
Urethral amyloidosis is a rare condition that can cause hematuria and urinary obstruction symptoms. While there is no established association between immunoglobulin A (IgA) nephropathy and secondary amyloidosis, which is typically found in chronic inflammatory conditions, it is hypothesized that IgA nephropathy may be a systemic condition with inflammatory mediators. We present a case of urethral amyloidosis in a patient with IgA nephropathy who previously received a renal transplant.
May 30, 2017: Curēus
https://www.readbyqxmd.com/read/28670093/pathognomonic-stew-visible-clues-to-systemic-amyloidosis
#11
EDITORIAL
Herbert L Fred, Hendrik A van Dijk
No abstract text is available yet for this article.
July 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28655092/-evaluation-of-clinical-characteristics-myd88-l265p-mutation-cxcr4-whim-mutation-and-prognosis-in-waldenstr%C3%A3-m-macroglobulinemia-a-single-center-retrospective-study-of-93-patients
#12
X X Cao, Q Meng, H Cai, Y Y Mao, M H Duan, T N Zhu, W Zhang, B Han, J L Zhuang, H C Cai, M Chen, J Feng, X Han, Y Zhang, C Yang, L Zhang, D B Zhou, J Li
Objective: To evaluate the clinical characteristics, MYD88(L265P) mutation, CXCR4(W)HIM mutation and prognosis in patients with Waldenström macroglobulinemia (WM). Methods: The clinical characteristics, International Prognostic Scoring System for symptomatic WM (WPSS) , and overall survival (OS) were retrospectively assayed in 93 patients with newly diagnosed WM at Peking Union Medical College Hospital during January 2000 to August 2016. The MYD88(L265P) mutation and CXCR4(W)HIM mutation were tested among 34 patients...
June 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28654386/dual-energy-spect-and-the-development-of-peptide-p5-14-for-imaging-amyloidosis
#13
Jonathan S Wall, Stephen J Kennel, Emily B Martin
Amyloidosis is associated with a number of rare diseases and is characterized by the deposition, in abdominothoracic organs and peripheral nerves, of extracellular protein fibrils, which leads to dysfunction and severe morbidity. Effective clinical evaluation and management of patients with systemic amyloidosis are hampered by the lack of a noninvasive, quantitative method for detecting whole-body amyloid load. We have used a battery of assays including dual-energy SPECT imaging and comparative effectiveness studies in support of translation of a synthetic polybasic peptide, p5+14, as a novel radiotracer for visualization of amyloidosis by molecular imaging...
January 1, 2017: Molecular Imaging
https://www.readbyqxmd.com/read/28648672/prognostic-significance-of-interventricular-septal-thickness-in-patients-with-al-amyloidosis
#14
Hyunsoo Cho, Soo-Jeong Kim, Chi Young Shim, Geu-Ru Hong, Jong-Won Ha, Yu Ri Kim, Woo Ick Yang, Haerim Chung, Ji Eun Jang, June-Won Cheong, Yoo Hong Min, Jin Seok Kim
The major prognostic determinant of immunoglobulin light chain (AL) amyloidosis is cardiac involvement. However, the role of interventricular septal thickness (IVST), which reflects the extent of cardiac involvement, remains unclear. Therefore, we analyzed 77 patients with newly diagnosed AL amyloidosis and evaluated the prognostic role of IVST. Fifty patients (64.9%) had cardiac involvement and 17 patients (22.1%) showed IVST >15mm. Among all patients, the revised Mayo Clinic Stage III-IV and IVST >15mm were independently associated with inferior overall survival (OS) in a multivariable analysis...
June 16, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28647518/increasing-the-accuracy-of-proteomic-typing-by-decellularisation-of-amyloid-tissue-biopsies
#15
P Patrizia Mangione, Giuseppe Mazza, Janet A Gilbertson, Nigel B Rendell, Diana Canetti, Sofia Giorgetti, Luca Frenguelli, Marco Curti, Tamer Rezk, Sara Raimondi, Mark B Pepys, Philip N Hawkins, Julian D Gillmore, Graham W Taylor, Massimo Pinzani, Vittorio Bellotti
Diagnosis and treatment of systemic amyloidosis depend on accurate identification of the specific amyloid fibril protein forming the tissue deposits. Confirmation of monoclonal immunoglobulin light chain amyloidosis (AL), requiring cytotoxic chemotherapy, and avoidance of such treatment in non-AL amyloidosis, are particularly important. Proteomic analysis characterises amyloid proteins directly. It complements immunohistochemical staining of amyloid to identify fibril proteins and gene sequencing to identify mutations in the fibril precursors...
June 21, 2017: Journal of Proteomics
https://www.readbyqxmd.com/read/28637682/cellular-mechanism-of-fibril-formation-from-serum-amyloid-a1-protein
#16
Stephanie Claus, Katrin Meinhardt, Tobias Aumüller, Ioana Puscalau-Girtu, Julia Linder, Christian Haupt, Paul Walther, Tatiana Syrovets, Thomas Simmet, Marcus Fändrich
Serum amyloid A1 (SAA1) is an apolipoprotein that binds to the high-density lipoprotein (HDL) fraction of the serum and constitutes the fibril precursor protein in systemic AA amyloidosis. We here show that HDL binding blocks fibril formation from soluble SAA1 protein, whereas internalization into mononuclear phagocytes leads to the formation of amyloid. SAA1 aggregation in the cell model disturbs the integrity of vesicular membranes and leads to lysosomal leakage and apoptotic death. The formed amyloid becomes deposited outside the cell where it can seed the fibrillation of extracellular SAA1...
June 21, 2017: EMBO Reports
https://www.readbyqxmd.com/read/28635949/non-coding-variants-contribute-to-the-clinical-heterogeneity-of-ttr-amyloidosis
#17
Andrea Iorio, Antonella De Lillo, Flavio De Angelis, Marco Di Girolamo, Marco Luigetti, Mario Sabatelli, Luca Pradotto, Alessandro Mauro, Anna Mazzeo, Claudia Stancanelli, Federico Perfetto, Sabrina Frusconi, Filomena My, Dario Manfellotto, Maria Fuciarelli, Renato Polimanti
Coding mutations in TTR gene cause a rare hereditary form of systemic amyloidosis, which has a complex genotype-phenotype correlation. We investigated the role of non-coding variants in regulating TTR gene expression and consequently amyloidosis symptoms. We evaluated the genotype-phenotype correlation considering the clinical information of 129 Italian patients with TTR amyloidosis. Then, we conducted a re-sequencing of TTR gene to investigate how non-coding variants affect TTR expression and, consequently, phenotypic presentation in carriers of amyloidogenic mutations...
June 21, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28632471/lenalidomide-as-a-treatment-for-relapsed-al-amyloidosis-in-an-hiv-positive-patient
#18
Johanna Denman, Kaveh Manavi, Mark Cook
We present a case of an HIV-positive man with systemic immunoglobulin light chain (AL) amyloid with cardiac involvement. At relapse, he was treated with lenalidomide and dexamethasone having previously developed autonomic neuropathy with bortezomib-based chemotherapy. The patient achieved a serological complete response with symptomatic improvement. After 11 cycles, lenalidomide was discontinued due to extensive ischaemia of the gastrointestinal tract. The patient remains symptomatically stable with normal levels of serum-free light chains 11 months after the treatment was discontinued...
January 1, 2017: International Journal of STD & AIDS
https://www.readbyqxmd.com/read/28632419/in-vitro-co-expression-of-human-amyloidogenic-immunoglobulin-light-and-heavy-chain-proteins-a-relevant-cell-based-model-of-al-amyloidosis
#19
Elena S Klimtchuk, Tatiana B Prokaeva, Brian H Spencer, Olga Gursky, Lawreen H Connors
Immunoglobulin (Ig) light chain (LC) amyloidosis (AL) is characterized by the overproduction and tissue deposition of monoclonal LC in various organs and tissues. The plasma circulating monoclonal LC is believed to be the precursor of the deposited protein and in vitro studies aimed at understanding AL pathobiology have mainly focused on LC and its variable domain. While 33% of patients have free circulating monoclonal LC, ∼40% feature LC complexed to heavy chain (HC) forming a monoclonal intact Ig; the significance of free vs...
June 20, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28627988/pharyngeal-amyloidomas-variable-appearance-on-imaging
#20
C A Prause, Q Zhai, S M Weindling
Amyloidomas are rare tumor-like depositions of abnormally folded, insoluble proteins that may be seen in the setting of systemic amyloidosis or as isolated tumoral deposits. Focal, isolated amyloidomas carry an excellent prognosis whereas systemic amyloidoses do not. The ability to identify or suggest amyloidoma on imaging studies may help direct laboratory testing and eventual diagnosis. Amyloidomas involving the head and neck have been variably described from homogeneously T2 hypointense to iso-slightly hyperintense relative to skeletal muscle...
June 2017: Neuroradiology Journal
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