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Amyloid: the International Journal of Experimental and Clinical Investigation

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https://www.readbyqxmd.com/read/28632454/localized-amyloidogenic-immunoglobulin-light-chain-derived-amyloidosis-in-a-young-boy-and-an-adolescent-girl
#1
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
#2
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/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
#3
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/28598686/how-your-ears-can-tell-what-is-hidden-in-your-heart-wild-type-transthyretin-amyloidosis-as-potential-cause-of-sensorineural-hearing-loss-inelderly-amylodeafness-pilot-study
#4
Emilie Béquignon, Aziz Guellich, Sophie Barthier, Marc Raynal, Virginie Prulière-Escabasse, Florence Canouï-Poitrine, André Coste, Thibaud Damy
BACKGROUND: Wild-type transthyretin amyloidosis (ATTRwt) is an age-related life-threatening condition. Prognosis is mainly dependent on cardiac involvement. Other organs and tissues may be affected. Their early recognition may increase awareness of physicians and positively affects the prognosis. Presbycusis is another age-related disorder. Whether this disease is associated to ATTRwt amyloidosis is unknown. METHODS: Sixteen consecutive patients with confirmed diagnosis of ATTRwt amyloidosis at the Mondor Amyloidosis Network, France, underwent otoscopy and audiological tests including pure tone audiometry, speech reception threshold and speech discrimination score...
June 9, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28590781/cognitive-impairment-in-liver-transplanted-patients-with-transthyretin-related-hereditary-amyloid-polyneuropathy
#5
Vanda Freitas Castro, Pedro Nascimento Alves, Ana Catarina Franco, Isabel Pavão Martins, Isabel Conceição
INTRODUCTION AND AIM: Hereditary transthyretin-related amyloidosis (ATTR-FAP) is characterized by a progressive neuropathy, cardiomyopathy, nephropathy and ocular disease. More than 90% of amyloidogenic transthyretin is produced by the liver; however, this protein is also synthesized in the choroid plexus. Although some patients have transitory neurologic events, the impact on cognition is still unknown. The aim was to study the cognitive performance of ATTR-FAP V30M patients with long disease course...
June 7, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28562083/cardiac-light-chain-deposition-disease-relapsing-in-the-transplanted-heart
#6
Alberto Aimo, Giuseppe Vergaro, Angela Pucci, Sonia Bernazzali, Massimo Maccherini, Gabriele Buda, Claudio Passino, Giampaolo Merlini, Michele Emdin
No abstract text is available yet for this article.
May 31, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28553897/diagnostic-score-for-the-detection-of-cardiac-amyloidosis-in-patients-with-left-ventricular-hypertrophy-and-impact-on-prognosis
#7
Eve Cariou, Youssef Bennani Smires, Gérard Victor, Guillaume Robin, David Ribes, Pierre Pascal, Antoine Petermann, Pauline Fournier, Stanislas Faguer, Jérôme Roncalli, Hervé Rousseau, Dominique Chauveau, Didier Carrié, Isabelle Berry, Michel Galinier, Olivier Lairez
BACKGROUND: Among diagnosis associated with left ventricular hypertrophy (LVH), cardiac amyloidosis (CA) is a progressive disease with poor prognosis. Early noninvasive identification is of growing clinical importance. The objective of our study was to integrate clinical, biologic, electrocardiographic and echocardiographic parameters to build a diagnostic score in patients with LVH. METHODS AND RESULTS: One hundred and fourteen patients with LVH underwent a cardiac magnetic resonance (CMR) and a (99m)Tc-hydroxymethylene-diphosphonate scintigraphy ((99m)Tc-HMDP) allowing to discriminate three groups of diagnoses: CA (n = 50 including 31, 18 and 1 ATTR, AL and AA amyloidosis), hypertrophic cardiomyopathy (n = 19) and unspecific cardiomyopathy (n = 45)...
May 29, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28532173/cervicomedullary-compression-as-the-main-manifestation-of-wild-type-transthyretin-amyloidosis
#8
Kourosh Rezania, Peter Pytel, W Edward Highsmith, Patrik Gabikian
No abstract text is available yet for this article.
May 23, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28494620/genetic-testing-improves-identification-of-transthyretin-amyloid-attr-subtype-in-cardiac-amyloidosis
#9
Emily E Brown, Yi Zhen Joan Lee, Marc K Halushka, Charles Steenbergen, Nicole M Johnson, Johana Almansa, Ryan J Tedford, Oscar Cingolani, Stuart D Russell, Kavita Sharma, Daniel P Judge
Amyloidosis is a group of conditions characterized by the accumulation of amyloid deposits in various tissues. Among these disorders, ATTR amyloidosis occurs either with or without a TTR pathogenic variant. Treatment for amyloidosis depends on the subtype, which is often identified through a tissue biopsy followed by liquid chromatography tandem mass spectrometry (LC-MS/MS). Genetic testing may be done to confirm these results for patients with ATTR amyloidosis; however, the necessity of genetic testing after LC-MS/MS has not been evaluated...
May 11, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28488941/a-v-block-as-presentation-of-cardiac-amyloid-prominent-infiltration-of-conduction-tissue-revealed-by-endomyocardial-biopsy
#10
Andrea Frustaci, Claudio Letizia, Francesco Adamo, Claudia Grande, Romina Verardo, Cristina Chimenti
No abstract text is available yet for this article.
May 10, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28485639/cathepsin-k-as-a-novel-amyloid-fibril-protein-in-humans
#11
Reinhold P Linke, Louise C Serpell, Friedrich Lottspeich, Mitsuyasu Toyoda
No abstract text is available yet for this article.
May 9, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28475415/transthyretin-amyloidosis-a-phenocopy-of-hypertrophic-cardiomyopathy
#12
Alexa M C Vermeer, Anneloes Janssen, Peter C Boorsma, Marcel M A M Mannens, Arthur A M Wilde, Imke Christiaans
OBJECTIVES: Hypertrophic cardiomyopathy (HCM) is an inherited cardiac disorder that affects over one in 500 persons worldwide. The autosomal dominant transmission of HCM implies that many relatives are at risk for HCM associated morbidity and mortality, therefore genetic testing and counselling is of great importance. However, in only 50-60% of the patients a mutation is found, which hampers predictive genetic testing in relatives. In HCM patients in whom the causal mutation has not been identified (yet), phenocopies of HCM - i...
May 5, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434267/amyloid-in-bone-marrow-smears-in-systemic-light-chain-amyloidosis
#13
Christoph Kimmich, Stefan Schönland, Sandra Kräker, Mindaugas Andrulis, Anthony D Ho, Gudrun Mayer, Tobias Dittrich, Michael Hundemer, Ute Hegenbart
We performed a prospective sensitivity analysis to detect amyloid in bone marrow (BM) smears stained with Congo red (CR) and according to Pappenheim of patients with systemic light-chain (AL) amyloidosis. Results were directly compared to routine BM histology and fat aspiration. We analysed 198 BM smears from patients with the diagnosis or suspicion of systemic AL amyloidosis. Ultimately, the diagnosis could be established for 168 patients. Amyloid was detected on BM smears with CR in 33% (56/168). All patients suspicious for amyloid on Pappenheim staining (n = 39) showed substantial amyloid infiltration on CR...
April 23, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434259/establishing-and-validating-the-fluorescent-amyloid-ligand-h-ftaa-heptamer-formyl-thiophene-acetic-acid-to-identify-transthyretin-amyloid-deposits-in-carpal-tunnel-syndrome
#14
Katharina Hahn, K Peter R Nilsson, Per Hammarström, Peter Urban, Rolf Rüdiger Meliss, Hans-Michael Behrens, Sandra Krüger, Christoph Röcken
Transthyretin-derived (ATTR) amyloidosis is a frequent finding in carpal tunnel syndrome. We tested the following hypotheses: the novel fluorescent amyloid ligand heptameric formic thiophene acetic acid (h-FTAA) has a superior sensitivity for the detection of amyloid compared with Congo red-staining; Amyloid load correlates with patient gender and/or patient age. We retrieved 208 resection specimens obtained from 184 patients with ATTR amyloid in the carpal tunnel. Serial sections were stained with Congo red, h-FTAA and an antibody directed against transthyretin (TTR)...
April 23, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28413910/phenotypic-characterization-of-late-onset-cardiac-amyloidosis-caused-by-the-transthyretin-mutation-ttra45s-p-ala65ser
#15
Katrine M Müllertz, Steen Baerentzen, Henrik K Jensen, Henning Mølgaard
No abstract text is available yet for this article.
April 16, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28413892/amyloid-detection-in-the-transverse-carpal-ligament-of-patients-with-hereditary-attr-v30m-amyloidosis-and-carpal-tunnel-syndrome
#16
Raquel Samões, Ricardo Taipa, Kátia Valdrez, Isabel Gonçalves, Manuel Melo Pires, Ana Martins da Silva, Teresa Coelho
INTRODUCTION: Carpal tunnel syndrome (CTS) is a nonspecific manifestation of hereditary ATTR amyloidosis (ATTRm). Amyloid deposition of wild-type TTR (WT-ATTR) has been found in transverse carpal ligament (TCL) in idiopathic CTS. We retrospectively studied a group of patients with ATTRm and CTS submitted to carpal tunnel release surgery (CTRS). METHODS: From the nerve conduction studies performed in our Clinical Unit dedicated to hereditary amyloidosis between July 2009 and October 2013, we selected patients who fulfilled neurophysiological criteria for CTS, had been submitted to CTRS and whose TCL was available for pathology...
April 16, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28393636/tocilizumab-postpones-the-start-of-hemodialysis-compared-to-conventional-oral-treatment-in-amyloid-a-amyloidosis-patients-with-advanced-renal-insufficiency-by-suppressing-serum-saa-levels
#17
Hiroshi Uda, Osamu Saiki
No abstract text is available yet for this article.
April 10, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28393633/csp-1103-chf5074-stabilizes-human-transthyretin-in-healthy-human-subjects
#18
Lixia Qiang, Yanxia Guan, Xiangshun Li, Li Liu, Yanshuang Mu, Aki Sugano, Yutaka Takaoka, Toshiyuki Sakaeda, Bruno P Imbimbo, Ken-Ichi Yamamura, Shoude Jin, Zhenghua Li
Hereditary amyloid polyneuropathy is a type of protein misfolding disease. Transthyretin (TTR) is a homotetrameric serum protein and TTR tetramer dissociation is the limiting step in amyloid fibril formation. Thus, prevention of TTR dissociation is a promising therapeutic approach and some TTR stabilizers have been approved for the treatment of TTR amyloidosis. CSP-1103 (CHF5074) is a non-steroidal anti-inflammatory derivative that lacks cyclooxygenase inhibitory activity. In vitro, CSP-1103 stabilizes the TTR tetramer by binding to the thyroxine (T4) binding site...
April 10, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28393574/transbronchial-biopsies-safely-diagnose-amyloid-lung-disease
#19
Praveen Govender, Colleen M Keyes, Elizabeth A Hankinson, Carl J O'Hara, Vaishali Sanchorawala, John L Berk
BACKGROUND: Autopsy identifies lung involvement in 58-92% of patients with the most prevalent forms of systemic amyloidoses. In the absence of lung biopsies, amyloid lung disease often goes unrecognized. Report of a death following transbronchial biopsies in a patient with systemic amyloidosis cautioned against the procedure in this patient cohort. We reviewed our experience with transbronchial biopsies in patients with amyloidosis to determine the safety and utility of bronchoscopic lung biopsies...
April 10, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28393570/tafamidis-delays-disease-progression-in-patients-with-early-stage-transthyretin-familial-amyloid-polyneuropathy-additional-supportive-analyses-from-the-pivotal-trial
#20
Denis Keohane, Jeffrey Schwartz, Balarama Gundapaneni, Michelle Stewart, Leslie Amass
BACKGROUND: Tafamidis, a non-NSAID highly specific transthyretin stabilizer, delayed neurologic disease progression as measured by Neuropathy Impairment Score-Lower Limbs (NIS-LL) in an 18-month, double-blind, placebo-controlled randomized trial in 128 patients with early-stage transthyretin V30M familial amyloid polyneuropathy (ATTRV30M-FAP). The current post hoc analyses aimed to further evaluate the effects of tafamidis in delaying ATTRV30M-FAP progression in this trial. METHODS: Pre-specified, repeated-measures analysis of change from baseline in NIS-LL in this trial (ClinicalTrials...
April 10, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
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