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systemic amyloidosis

Chantal C Clark, Mirjam M Mebius, Steven de Maat, Aloysius G M Tielens, Philip G de Groot, Rolf T Urbanus, Rob Fijnheer, Bouke P C Hazenberg, Jaap J van Hellemond, Coen Maas
ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) cleaves von Willebrand Factor (VWF) multimers to control their thrombogenicity. The fibrinolytic enzyme plasmin can cleave VWF in a similar manner. However, plasmin can also cleave ADAMTS13, which ultimately inactivates it. This leaves the overall role of plasmin in primary haemostasis uncertain.We investigated the combined molecular effects of plasmin on VWF and ADAMTS13. We first identified that plasmin destroys FRETS-VWF73 substrate by cleaving the ADAMTS13 binding region in a buffered system...
March 2018: Thrombosis and Haemostasis
Haruko Tashiro, Ryosuke Shirasaki, Masato Watanabe, Kazuo Kawasugi, Yoshihisa Takahashi, Naoki Shirafuji
We present a lymphoplasmacytic lymphoma patient with Factor X (FX) deficiency. Despite the absence of FX inhibitor, the administration of fresh frozen plasma and anti-inhibitor coagulant complex did not increase the FX level. The autopsy showed that massive amyloid depositions to multiple organs and FX existed in union with amyloidosis.
March 2018: Clinical Case Reports
Eli Muchtar, Morie A Gertz, Shaji K Kumar, Grace Lin, Barry Boilson, Alfredo Clavell, Martha Q Lacy, Francis K Buadi, Suzanne R Hayman, Prashant Kapoor, David Dingli, S Vincent Rajkumar, Angela Dispenzieri, Martha Grogan
AIM: Digoxin is considered contraindicated in light-chain (AL) amyloidosis, given reports of increased toxicity published 30-50 years ago. We sought to determine the frequency of digoxin toxicity in patients with AL. METHODS: We identified 107 patients with AL amyloidosis who received digoxin between 2000 and 2015. RESULTS: The median age was 65 and the median digoxin dose and estimated glomerular filtration rate were 0.125 mg/d and 55 ml/min/1...
March 12, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
Paolo Ruzza, Rosa Maria Vitale, Rohanah Hussain, Alessia Montini, Claudia Honisch, Alice Pozzebon, Charlotte S Hughes, Barbara Biondi, Pietro Amodeo, GianPietro Sechi, Giuliano Siligardi
BACKGROUND: Lysozyme is a widely distributed enzyme present in a variety of tissue and body fluids. Human and hen egg white lysozyme are used as validated model to study protein folding and stability and to understand protein misfolding and aggregation. We recently found that ceftriaxone, a β-lactam antibiotic able to overcome the blood-brain barrier, successfully eliminated the cellular toxic effects of misfolded proteins as Glial Fibrillary Acidic Protein and α-synuclein. To further understand the anti-amyloidogenic properties of ceftriaxone, we studied its activity towards lysozyme aggregation with the aim to investigate a possible chaperone-like activity of this molecule...
March 7, 2018: Biochimica et Biophysica Acta
Özlem Fentoğlu, Gözde Dinç, Atalay Doğru, Nermin Karahan, İlter İlhan, F Yeşim Kırzıoğlu, Mehmet Fatih Şentürk, Hikmet Orhan
BACKGROUND: There are no published studies regarding the role of the plasminogen (PLG) system in familial Mediterranean fever (FMF), FMF-associated secondary amyloidosis, or chronic periodontitis (CP), although recent limited data have focused on the association between FMF and chronic periodontitis. Therefore, the aim of this study was to evaluate the serum, salivary, and gingival tissue levels of PLG in patients with CP, FMF, and amyloidosis. METHODS: The study population included 122 patients with FMF (only FMF, and FMF and amyloidosis and 128 individuals who were systemically healthy controls...
February 21, 2018: Journal of Periodontology
Kotaro Matsumoto, Jun Kikuchi, Yuko Kaneko, Hidekata Yasuoka, Kazuko Suzuki, Hirobumi Tokuyama, Kaori Kameyama, Kunihiro Yamaoka, Tsutomu Takeuchi
RATIONALE: Dialysis-related amyloidosis (DRA) can present rheumatic manifestations in patients on long-term hemodialysis. Typical articular symptoms with DRA involve carpal-tunnel syndrome, effusion in large joints, spondyloarthropathy, or cystic bone lesions, which are usually with non-inflammatory processes. PATIENT CONCERNS: A 64-year-old man on hemodialysis for >30 years was admitted because of intermittent fever, polyarthritis, and elevated serum C-reactive protein (CRP) level, which was continuous for 2 years...
January 2018: Medicine (Baltimore)
Natalia Tovar, Luis Gerardo Rodríguez-Lobato, Maria Teresa Cibeira, Laura Magnano, Ignacio Isola, Laura Rosiñol, Joan Bladé, Carlos Fernández de Larrea
OBJECTIVES: Prognosis of immunoglobulin light-chain (AL) amyloidosis depends mainly on the presence of cardiac involvement and the disease burden. A higher bone marrow plasma cell (BMPC) burden has been recognized as an adverse prognostic factor. The aim of our study was to analyze the correlation between the BMPC infiltration, clinical features and outcomes in patients with AL amyloidosis. METHODS: The clinical records of 79 patients with AL amyloidosis treated at a single institution...
February 26, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
Yingjie Qi, Igor Klyubin, A Claudio Cuello, Michael J Rowan
Pro-inflammatory mechanisms have recently emerged as an important component of early Alzheimer's disease (AD) pathogenesis. A particularly attractive therapeutic strategy is to selectively prevent the disruptive effects of activation of the innate immune system in the brain at an early transitional stage by reducing the production or directly neutralizing pro-inflammatory cytokines, in particular IL-1β and TNF-α. Here we tested their in vivo effects on synaptic plasticity deficits, which provide sensitive and robust measures of synaptic failure, in a rat model of AD amyloidosis...
February 22, 2018: Neurobiology of Disease
Giorgia Brancolini, Maria Celeste Maschio, Cristina Cantarutti, Alessandra Corazza, Federico Fogolari, Vittorio Bellotti, Stefano Corni, Gennaro Esposito
Protein aggregation including the formation of dimers and multimers in solution, underlies an array of human diseases such as systemic amyloidosis which is a fatal disease caused by misfolding of native globular proteins damaging the structure and function of affected organs. Different kind of interactors can interfere with the formation of protein dimers and multimers in solution. A very special class of interactors are nanoparticles thanks to the extremely efficient extension of their interaction surface...
February 22, 2018: Nanoscale
Taru Garg, Anita Marak, Riaz Ahmed, Ram Chander, Manjula Jain
Primary localized cutaneous amyloidosis refers to a group of disorders characterized by deposition of amyloid in the dermis without any systemic involvement. It comprises the following clinical types: macular, lichenoid, nodular, and biphasic. There are also rare variants such as amyloidosis cutis dyscromica and poikiloderma-like cutaneous amyloidosis. We report a case of primary cutaneous amyloidosis in a 17-year-old boy with unusual pigmentation of various patterns (reticulate and diffuse pigmentation with mottling and rippling at places) and hypopigmented atrophic macules...
August 15, 2017: Dermatology Online Journal
Pavla Flodrova, Patrik Flodr, Tomas Pika, Jiri Vymetal, Dusan Holub, Petr Dzubak, Marian Hajduch, Vlastimil Scudla
Amyloidosis is a heterogeneous group of diseases characterised by extracellular accumulation of amyloid in various tissues and organs of the body, leading to alteration and destruction of tissues. Heart involvement is the most important prognostic factor in patients with systemic amyloidosis and the diagnosis and typing of amyloid must be made properly. The clinical picture shows congestive heart failure with predominant right-sided heart failure symptoms in fully developed disease, various types of arrhythmias and characteristic electrocardiography and echocardiography findings...
February 12, 2018: Pathology
Sanathorn Chowsilpa, Saisawat Chaiyasate, Komson Wannasai, Teerada Daroontum
Localised nasopharyngeal amyloidosis is rare. Findings on physical examination and invasive pattern on CT scan can be misleading as it can resemble nasopharyngeal carcinoma. A 64-year-old man presented with left aural fullness for 6 months. The physical examination showed straw-coloured fluid in the left middle ear and irregular reddish mass at the left side of the nasopharynx. The CT scan showed a lobulated heterogeneous mass at the left side of the nasopharynx involving the left Eustachian tube opening. Pathology report was amyloidosis, thus, surgery was done...
February 14, 2018: BMJ Case Reports
Hongli Liang, Bing Wang, Hailong Wang, Miao Yu, Lingna Zheng, Meng Wang, Aiping Zhang, Weiyue Feng
Amyloid fibrillation has been implicated in many neurodegenerations, dialysis-related amyloidosis, type II diabetes and more than 30 other amyloid-related diseases. Nanomaterials as potential inhibitors of amyloid fibrillation have attracted increasing interests. In the present study, the effects of gold nanorods (AuNRs) and nanoparticles (AuNPs) on amyloid fibrillation were investigated using hen egg white lysozyme (HEWL) as a model system. Our results indicated that AuNRs and AuNPs, especially AuNRs, present significant inhibitory effects on HEWL amyloid fibril formation during all the kinetic processes, from nucleation to elongation and equilibration stages...
May 1, 2018: Journal of Nanoscience and Nanotechnology
Isabel H Salas, Akila Weerasekera, Tariq Ahmed, Zsuzsanna Callaerts-Vegh, Uwe Himmelreich, Rudi D'Hooge, Detlef Balschun, Takaomi C Saido, Bart De Strooper, Carlos G Dotti
Type 2 diabetes (T2DM) and obesity might increase the risk for AD by 2-fold. Different attempts to model the effect of diet-induced diabetes on AD pathology in transgenic animal models, resulted in opposite conclusions. Here, we used a novel knock-in mouse model for AD, which, differently from other models, does not overexpress any proteins. Long-term high fat diet treatment triggers a reduction in hippocampal N-acetyl-aspartate/myo-inositol metabolites ratio and impairs long term potentiation in hippocampal acute slices...
February 7, 2018: Neurobiology of Disease
Pooja Phull, Vaishali Sanchorawala, Lawreen H Connors, Gheorghe Doros, Frederick L Ruberg, John L Berk, Shayna Sarosiek
OBJECTIVE: To identify the prevalence of monoclonal gammopathy of undetermined significance (MGUS) in patients with transthyretin (ATTR) amyloidosis. PATIENTS AND METHODS: We performed a retrospective analysis of patients with biopsy-proven ATTRwt (wild-type transthyretin amyloid protein) and genopositive ATTR V122I (valine-to-isoleucine substitution at position 122 of the TTR gene) amyloidosis evaluated at the Amyloidosis Center at Boston University and Boston Medical Center between 1 January 2003 and 31 December 2016...
February 9, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
Kazunori Takemura, Ryuta Nakamura, Kazuhito Shimazu, Youichiro Sugimoto, Tetsuro Takase, Minamimoto Ryogo, Michiaki Hiroe
A 52-year-old male visited our hospital with abnormal electrocardiogram and exertional fatigue. The electrocardiogram showed first-degree atrioventricular block, complete right bundle branch block, and inverted T waves in Leads II, III, aVF, V3, and V4. Echocardiography showed biventricular wall thickening involving granular sparkling of the interventricular septum. Late gadolinium enhancement on cardiovascular magnetic resonance (CMR) was found at the circumferential right ventricular wall and patchy regions of the left ventricle...
February 5, 2018: ESC Heart Failure
Matthew Mold, Manpreet Kumar, Ambreen Mirza, Emma Shardlow, Christopher Exley
Alzheimer's disease is a debilitating neurodegenerative condition that progressively causes synaptic loss and major neuronal damage. Immunotherapy utilising Aβ as an active immunogen or via passive treatment utilising antibodies raised to amyloid have shown therapeutic promise. The migratory properties of peripheral blood-borne monocytes and their ability to enter the central nervous system, suggests a beneficial role in mediating tissue damage and neuroinflammation. However, the intrinsic phagocytic properties of such cells have pre-disposed them to internalise misfolded amyloidogenic peptides that could act as seeds capable of nucleating amyloid formation in the brain...
February 5, 2018: Scientific Reports
Mario Merlini, Tunahan Kirabali, Luka Kulic, Roger M Nitsch, Maria Teresa Ferretti
BACKGROUND: Strong genetic and epidemiological evidence points to a crucial role of the immune system in the development of Alzheimer disease (AD). CD3+ T lymphocytes have been described in brains of postmortem AD patients and in transgenic models of AD-like cerebral amyloidosis and tau pathology. However, the occurrence of T cells in AD brains is still controversial; furthermore, the relationship between T cells and hallmarks of AD pathology (amyloid plaques and neurofibrillary tangles) remains to be established...
February 7, 2018: Neuro-degenerative Diseases
Anum Bilal, Paul Der Mesropian, Franklin Lam, Gulvahid Shaikh
Amyloidosis is a systemic illness characterized by the extracellular deposition of abnormal proteins in body tissues and organs. In addition to renal involvement, amyloidosis can also present with a variety of skin manifestations, though rarely with alopecia. Sixteen cases of alopecia secondary to systemic amyloidosis are reported. There is one reported case that presented with alopecia universalis. We report a case of a 68-year-old woman presenting with alopecia universalis, rapid decline in kidney function, and nephrotic syndrome who was found to have multiple myeloma-associated AL amyloidosis (immunoglobulin light chain)...
January 2018: Journal of Investigative Medicine High Impact Case Reports
Linge Sun, Lei Zhang, Wenlu Hu, Tian-Fang Li, Shengyun Liu
INTRODUCTION: Amyloid light chain (AL) results from the deposition of immunoglobulin light chain fragments, and can affect multiple organs/systems. Our patient was diagnosed as scleroderma repeatedly because of extensive skin thickening and hardening, but the treatment was not effective. We did extensive laboratory examinations including serum/urine protein electrophoresis and flow cytometry assay of bone marrow aspiration. CONCLUSION: A diagnosis of primary AL amyloidosis was established...
December 2017: Medicine (Baltimore)
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