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https://www.readbyqxmd.com/read/28639774/-discovery-of-left-ventricular-hypertrophy-during-adult-echocardiography
#1
Nicolas B Dayal, Hajo Müller
Left ventricular hypertrophy is a common finding during echocardiography. A precise evaluation of the left ventricular wall thickness, ventricular mass and distribution of hypertrophy is crucial both for diagnostic workup, follow-up and for prognostic evaluation. The differential diagnosis of left ventricular hypertrophy includes hypertrophic cardiomyopathies, hypertrophy secondary to abnormal left ventricular filling conditions, hypertrophy linked to intense physical training and the isolated basal septal hypertrophy of the elderly...
May 24, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28637682/cellular-mechanism-of-fibril-formation-from-serum-amyloid-a1-protein
#2
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/28637503/expression-of-endogenous-mouse-app-modulates-%C3%AE-amyloid-deposition-in-happ-transgenic-mice
#3
Johannes Steffen, Markus Krohn, Christina Schwitlick, Thomas Brüning, Kristin Paarmann, Claus U Pietrzik, Henrik Biverstål, Baiba Jansone, Oliver Langer, Jens Pahnke
Amyloid-β (Aβ) deposition is one of the hallmarks of the amyloid hypothesis in Alzheimer's disease (AD). Mouse models using APP-transgene overexpression to generate amyloid plaques have shown to model only certain parts of the disease. The extent to which the data from mice can be transferred to man remains controversial. Several studies have shown convincing treatment results in reducing Aβ and enhancing cognition in mice but failed totally in human. One model-dependent factor has so far been almost completely neglected: the endogenous expression of mouse APP and its effects on the transgenic models and the readout for therapeutic approaches...
June 20, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28637305/left-atrial-structure-and-function-in-cardiac-amyloidosis
#4
Kotaro Nochioka, Candida Cristina Quarta, Brian Claggett, Gabriela Querejeta Roca, Claudio Rapezzi, Rodney H Falk, Scott D Solomon
Aims: Although cardiac amyloidosis (CA) is characterized by significant left atrial (LA) dilatation, the characteristics of LA function remain to be fully investigated. Methods and results: We assessed LA function by speckle-tracking echocardiography in 124 patients with CA and sinus rhythm: 68 with light chain (AL), 29 with mutant (ATTRm), 27 with wild-type (ATTRwt) transthyretin amyloidosis. Conventional and strain-derived parameters, including LA peak longitudinal strain (LS) and strain rate (peak LSR: reservoir function; early LSR: conduit function; late LSR: active function), were assessed compared between CA patients and 20 healthy controls of similar age and gender...
June 16, 2017: European Heart Journal Cardiovascular Imaging
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/28635949/non-coding-variants-contribute-to-the-clinical-heterogeneity-of-ttr-amyloidosis
#6
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/28634774/learning-from-synthetic-models-of-extracellular-matrix-differential-binding-of-wild-type-and-amyloidogenic-human-apolipoprotein-a-i-to-hydrogels-formed-from-molecules-having-charges-similar-to-those-found-in-natural-gags
#7
Silvana A Rosú, Leandro Toledo, Bruno F Urbano, Susana A Sanchez, Graciela C Calabrese, M Alejandra Tricerri
Among other components of the extracellular matrix (ECM), glycoproteins and glycosaminoglycans (GAGs) have been strongly associated to the retention or misfolding of different proteins inducing the formation of deposits in amyloid diseases. The composition of these molecules is highly diverse and a key issue seems to be the equilibrium between physiological and pathological events. In order to have a model in which the composition of the matrix could be finely controlled, we designed and synthesized crosslinked hydrophilic polymers, the so-called hydrogels varying the amounts of negative charges and hydroxyl groups that are prevalent in GAGs...
June 20, 2017: Protein Journal
https://www.readbyqxmd.com/read/28632471/lenalidomide-as-a-treatment-for-relapsed-al-amyloidosis-in-an-hiv-positive-patient
#8
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/28632454/localized-amyloidogenic-immunoglobulin-light-chain-derived-amyloidosis-in-a-young-boy-and-an-adolescent-girl
#9
Shu-Ichi Ikeda
No abstract text is available yet for this article.
June 20, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
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
#10
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/28630445/myocardial-deformation-in-cardiac-amyloid-light-chain-amyloidosis-assessed-with-3t-cardiovascular-magnetic-resonance-feature-tracking
#11
Rui Li, Zhi-Gang Yang, Hua-Yan Xu, Ke Shi, Xi Liu, Kai-Yue Diao, Ying-Kun Guo
Clinically, assessment of myocardial function is essential in patients with amyloid light-chain cardiac amyloidosis (AL-CA) to predict outcome and determine therapeutic approach. The aim of this study was to investigate the feasibility of cardiovascular magnetic resonance (CMR)-derived feature tracking algorithm for assessing left ventricular (LV) myocardial deformation in AL-CA, and to determine if these abnormal myocardial deformation parameters are correlated to impaired LV myocardial microvascular dysfunction...
June 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28627988/pharyngeal-amyloidomas-variable-appearance-on-imaging
#12
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
https://www.readbyqxmd.com/read/28626633/mediastinal-hemangioendothelioma-case-report-and-review-of-the-literature
#13
Davide Patrini, Laura Scolamiero, Reena Khiroya, David Lawrence, Elaine Borg, Martin Hayward, Nikolaos Panagiotopoulos
BACKGROUND: Epithelioid haemangioendothelioma (EHE) is a rare low-grade vascular neoplasm that can arise in the lung, liver, soft tissues or, less commonly, bone. Due to its low prevalence of less than one in a million and its non-specific clinical features, EHE is often misdiagnosed and managed inappropriately. Here we discuss the case of a 58 year-old gentleman with mediastinal EHE and review existing literature on pulmonary EHE (PEH). CASE HISTORY: A 58 year-old gentleman presented to our outpatient Clinic with chest discomfort and palpitations...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28626447/autoimmune-thyroiditis-and-glomerulopathies
#14
REVIEW
Domenico Santoro, Carmela Vadalà, Rossella Siligato, Michele Buemi, Salvatore Benvenga
Autoimmune thyroiditis (AIT) is generally associated with hypothyroidism. It affects ~2% of the female population and 0.2% of the male population. The evidence of thyroid function- and thyroid autoantibody-unrelated microproteinuria in almost half of patients with AIT and sometimes heavy proteinuria as in the nephrotic syndrome point to a link of AIT with renal disease. The most common renal diseases observed in AIT are membranous nephropathy, membranoproliferative glomerulonephritis, minimal change disease, IgA nephropathy, focal segmental glomerulosclerosis, antineutrophil cytoplasmic autoantibody (ANCA) vasculitis, and amyloidosis...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28625707/-algorithms-based-on-medico-administrative-data-in-the-field-of-endocrine-nutritional-and-metabolic-diseases-especially-diabetes
#15
S Fosse-Edorh, A Rigou, S Morin, L Fezeu, L Mandereau-Bruno, A Fagot-Campagna
BACKGROUND: Medico-administrative databases represent a very interesting source of information in the field of endocrine, nutritional and metabolic diseases. The objective of this article is to describe the early works of the Redsiam working group in this field. METHODS: Algorithms developed in France in the field of diabetes, the treatment of dyslipidemia, precocious puberty, and bariatric surgery based on the National Inter-schema Information System on Health Insurance (SNIIRAM) data were identified and described...
June 15, 2017: Revue D'épidémiologie et de Santé Publique
https://www.readbyqxmd.com/read/28624931/familial-mediterranean-fever-review-of-the-literature
#16
Mansour Alghamdi
Familial Mediterranean fever (FMF) is the most common monogenic periodic fever syndrome and characterized by recurrent episodes of fever, serositis, arthritis, dermal manifestations, and long-term renal complications. The MEFV gene was described in 1997 as the gene responsible for FMF and is inherited in autosomal recessive manner. It encodes mutated protein pyrin, an important player in the innate immune system and the component of inflammasome which leads to exaggerated inflammatory response through uncontrolled production of interleukin-1...
June 18, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28622303/addition-of-cyclophosphamide-and-higher-doses-of-dexamethasone-do-not-improve-outcomes-of-patients-with-al-amyloidosis-treated-with-bortezomib
#17
E Kastritis, M Gavriatopoulou, M Roussou, D Fotiou, D C Ziogas, M Migkou, E Eleutherakis-Papaiakovou, I Panagiotidis, N Kanellias, E Psimenou, E Papadopoulou, C Pamboucas, E Manios, H Gakiopoulou, A Ntalianis, A Tasidou, S Giannouli, E Terpos, M A Dimopoulos
Bortezomib, in combination with dexamethasone (VD) or with the addition of cyclophosphamide (VCD), is highly effective in patients with amyloid light-chain (AL) amyloidosis. Currently, VCD is considered as a primary regimen for patients with AL, but it is not clear whether the addition of cyclophosphamide to VD further and significantly improves efficacy, given the substantial activity of bortezomib itself. We retrospectively compared the outcomes of 101 patients with AL amyloidosis who received VD (n=59) or VCD (n=42) in two consecutive periods...
June 16, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/28616786/laryngotracheal-involvement-in-systemic-light-chain-amyloidosis
#18
Daniel T Ginat, Jefree Schulte, Louis Portugal, Nicole A Cipriani
Laryngotracheal amyloid deposition is an uncommon manifestation of systemic light chain amyloidosis. Diagnostic imaging, such as CT, is useful for suggesting the possibility of amyloidosis and delineating the extent of the lesions for surgical management; however, the diagnosis is confirmed with the histologic finding of amorphous eosinophilic material which stains positively for Congo red and may show apple green birefringence on polarization. These features are exemplified in this sine qua non radiology-pathology correlation article...
June 14, 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/28616564/regression-of-cardiac-amyloidosis-following-stem-cell-transplantation-a-comparison-between-echocardiography-and-cardiac-magnetic-resonance-imaging-in-long-term-survivors
#19
Benjamin Thomas Fitzgerald, John Bashford, Katrina Newbigin, Gregory Malcolm Scalia
BACKGROUND: AL amyloidosis and multiple myeloma result in extracellular deposition of insoluble fibrillary protein in tissue and organs. Untreated median survival is very poor, and even worse with cardiac involvement. Chemotherapy and peripheral blood stem cell transplantation (PBSCT) have been shown to dramatically improve survival, with hematologic remission documented. Regression of cardiac changes has previously been shown, as assessed by echocardiography (TTE) and cardiac magnetic resonance imaging (CMR)...
March 2017: IJC Heart & Vasculature
https://www.readbyqxmd.com/read/28615223/daratumumab-yields-rapid-and-deep-hematologic-responses-in-patients-with-heavily-pretreated-al-amyloidosis
#20
Gregory P Kaufman, Stanley L Schrier, Richard A Lafayette, Sally Arai, Ronald M Witteles, Michaela Liedtke
The majority of patients with immunoglobulin light chain amyloidosis (AL) fail to achieve a complete response (CR) to standard light chain suppressive chemotherapy, and almost all patients eventually experience hematologic relapse and progression of organ involvement; additional well tolerated treatment options are therefore needed. We present our retrospective experience of 25 consecutive previously treated AL patients who received daratumumab, a CD38-directed monoclonal antibody approved for the treatment of multiple myeloma...
June 14, 2017: Blood
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