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systemic amyloidosis

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https://www.readbyqxmd.com/read/28544119/common-fibril-structures-imply-systemically-conserved-protein-misfolding-pathways-in%C3%A2-vivo
#1
Karthikeyan Annamalai, Falk Liberta, Marie-Theres Vielberg, William Close, Hauke Lilie, Karl-Heinz Gührs, Angelika Schierhorn, Rolf Koehler, Andreas Schmidt, Christian Haupt, Ute Hegenbart, Stefan Schönland, Matthias Schmidt, Michael Groll, Marcus Fändrich
Systemic amyloidosis is caused by the misfolding of a circulating amyloid precursor protein and the deposition of amyloid fibrils in multiple organs. Chemical and biophysical analysis of amyloid fibrils from human AL and murine AA amyloidosis reveal the same fibril morphologies in different tissues or organs of one patient or diseased animal. The observed structural similarities concerned the fibril morphology, the fibril protein primary and secondary structures, the presence of post-translational modifications and, in case of the AL fibrils, the partially folded characteristics of the polypeptide chain within the fibril...
May 23, 2017: Angewandte Chemie
https://www.readbyqxmd.com/read/28540895/pattern-of-acute-glomerulonephritis-in-adult-population-in-dubai-a-single-center-experience
#2
Amna K Alhadari, Fakhriya J Alalawi, Ayman Aly Seddik, Kaneez Zahra, Dileep Kumar, Hussain Yousif, Hind Alnour, Michael Jansen, Mohammad J Railey
Epidemiological data of renal diseases have great geographic variability throughout the world. Due to the lack of a national renal data registry system, there is no information on the prevalence rate, clinical and pathological features of various glomerulonephritis (GN) in the United Arab Emirates (UAE). In a retrospective cross-sectional study, we analyzed 158 renal biopsies done in Dubai Hospital, UAE, between the years of 2005 and September 2014, with an aim to determine the prevalence rate and frequency of different pathological patterns of GN in adult patients who presented with proteinuria ± hematuria...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28538749/complications-of-hidradenitis-suppurativa
#3
Joyce T Yuan, Haley B Naik
Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease characterized by recurrent painful nodules and abscesses involving intertriginous areas. Repeated episodes of profound inflammation in HS can lead to a number of complications, causing significant morbidity and decreasing quality of life. Complications of HS may affect the skin alone or may have systemic impact. Cutaneous complications of HS include sinus tracts, fistulae, scarring and contractures, squamous cell carcinoma, and lymphedema...
June 2017: Seminars in Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28537855/multifocal-primary-cutaneous-nodular-amyloidosis
#4
Martha Emily Matsumoto, Mary-Katharine Collins, Anastasios Raptis, Jaroslaw Jedrych, Timothy Patton
Nodular cutaneous amyloidosis (NCA), the least common form of primary cutaneous amyloidosis, is characterized clinically by waxy, purpuric plaques and nodules and histologically by amyloid deposits in the dermis and subcutaneous tissue. We present a patient who developed multiple, non-contiguous NCA lesions over a three year period without evidence of systemic disease. We reviewed the literature and found few other cases of this unusual presentation.
May 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28536362/functionalisation-of-polyvinylpyrrolidone-on-gold-nanoparticles-enhances-its-anti-amyloidogenic-propensity-towards-hen-egg-white-lysozyme
#5
Tulika Das, Vidyalatha Kolli, Srijeeb Karmakar, Nandini Sarkar
Protein amyloids are characterized by aggregates that usually consist of fibres containing misfolded proteins and having a cross β-sheet conformation. These aggregates can eventually lead to several degenerative diseases like Alzheimer's disease, amyotrophic lateral sclerosis (ALS), Huntington's disease and Parkinson's disease. The present study describes the effect of chemically synthesized polyvinylpyrrolidone (PVP)-conjugated gold nanoparticles (PVP-AuNps) on hen egg white lysozyme (HEWL) amyloids. The synthesized nanoparticles have been characterized using various biophysical techniques like Ultraviolet-Visible (UV-Vis) Spectroscopy, Transmission electron microscopy (TEM), X-ray diffraction (XRD) analysis, dynamic light scattering (DLS), zeta-potential measurement and Fourier transform infrared spectroscopy (FTIR)...
May 3, 2017: Biomedicines
https://www.readbyqxmd.com/read/28532139/systemic-amyloidosis-in-a-teenage-boy-with-inflammatory-bowel-disease
#6
Farzaneh Motamed, Rita Bagherian, Gholamhossin Adalat, Mohammad Hassan Moradinejad, Nilofar Hajizadeh, Mohammad Vasei, Mehrzad Mehdizadeh, Nima Rezaei
Systemic amyloidosis is a very rare complication of inflammatory bowel disease (IBD). The reported cases of secondary amyloidosis in children with IBD are much fewer than those reported in adults. Herein, a teenage boy with Crohn's disease is presented who developed nephrotic syndrome due to renal involvement secondary to amyloidosis, whereas the patient was under treatment with corticosteroid and 6-mercaptopurine. To our best knowledge, this is the first reported case of secondary amyloidosis in a teenage Iranian boy with Crohn's disease...
April 2017: Acta Medica Iranica
https://www.readbyqxmd.com/read/28528458/cardiac-amyloidosis-diagnosis-and-treatment-strategies
#7
REVIEW
Mirela Tuzovic, Eric H Yang, Arnold S Baas, Eugene C Depasquale, Mario C Deng, Daniel Cruz, Gabriel Vorobiof
Cardiac amyloidosis in the United States is most often due to myocardial infiltration by immunoglobulin protein, such as in AL amyloidosis, or by the protein transthyretin, such as in hereditary and senile amyloidosis. Cardiac amyloidosis often portends a poor prognosis especially in patients with systemic AL amyloidosis. Despite better understanding of the pathophysiology of amyloid, many patients are still diagnosed late in the disease course. This review investigates the current understanding and new research on the diagnosis and treatment strategies in patients with cardiac amyloidosis...
July 2017: Current Oncology Reports
https://www.readbyqxmd.com/read/28523319/amyloidosis-a-rare-cause-of-refractory-heart-failure-in-a-young-female
#8
Irina Iuliana Costache, Claudia Florida Costea, Mihai Danciu, Victor Vlad Costan, Viviana Aursulesei, Gabriela FlorenŢa Dumitrescu, Mihaela Dana Turliuc, Anca Sava
Cardiac amyloidosis may occur in any type of systemic amyloidosis. The clinical picture is often characterized by restrictive cardiomyopathy. We report the case of a 41-year-old female patient admitted to the Department of Cardiology with clinical signs of right heart failure: congested jugular veins, hepatomegaly, peripheral edema, ascites associated with atrial fibrillation, low values of arterial blood pressure and oliguria. Echocardiographic findings were helpful for the diagnosis of cardiac amyloidosis: enlarged atrial cavities, normal size ventricles, thickened ventricular septum and posterior left ventricle wall with normal left ventricular ejection fraction, mitral and tricuspid regurgitation...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/28516795/a-comprehensive-guide-to-telocytes-and-their-great-potential-in-cardiovascular-system
#9
I Kucybala, P Janas, S Ciuk, W Cholopiak, W Klimek-Piotrowska, M K Holda
Telocytes, a recently discovered type of interstitial cells, have a very distinctive morphology - the small cell body with long extensions, named telopodes. In our review, apart from introducing general aspects of telocytes, we focus on properties, functions and future potential of those cells in cardiovascular system. However, physiological functions of telocytes in cardiovascular system are still regarded as quite enigmatic. Previous studies claim that they play a role in organogenesis and regeneration, bioelectrical signalling, mechanoelectrical coupling, anti-oxidative protection, angiogenesis and regulation of blood flow...
2017: Bratislavské Lekárske Listy
https://www.readbyqxmd.com/read/28505007/al-amyloidoma-of-the-skin-subcutis-cutaneous-amyloidosis-plasma-cell-dyscrasia-or-a-manifestation-of-primary-cutaneous-marginal-zone-lymphoma
#10
Noreen M Walsh, Ian Marie Lano, Peter Green, Christopher Gallant, Sylvia Pasternak, Thai Yen Ly, Luis Requena, Heinz Kutzner, Andreas Chott, Lorenzo Cerroni
It is unclear whether AL amyloidoma of the skin/subcutis represents a distinct entity, an indolent precursor of systemic amyloidosis, or a manifestation of cutaneous marginal zone lymphoma (cMZL). We collected 10 cases of cutaneous AL amyloidoma in order to better characterize the clinicopathologic features of this elusive entity (M:F=4:6; median age: 62.5 y, range: 31 to 82 y). Nine patients had a solitary nodule or plaque on the lower extremity (n=7), upper extremity (n=1), or chin (n=1). One patient had an AL amyloidoma on the right thigh and a second lesion on the right arm showing histopathologic features of cMZL without amyloid deposits...
May 12, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28501163/on-line-immunoaffinity-solid-phase-extraction-capillary-electrophoresis-mass-spectrometry-using-fab%C3%A2-antibody-fragments-for-the-analysis-of-serum-transthyretin
#11
Laura Pont, Fernando Benavente, José Barbosa, Victoria Sanz-Nebot
This paper describes an on-line immunoaffinity solid-phase extraction capillary electrophoresis mass spectrometry (IA-SPE-CE-MS) method using an immunoaffinity sorbent with Fab' antibody fragments (Fab'-IA) for the analysis of serum transthyretin (TTR), a homotetrameric protein (Mr~56,000) involved in different types of amyloidosis. The IA sorbent was prepared by covalent attachment of Fab' fragments obtained from a polyclonal IgG antibody against TTR to succinimidyl silica particles. The Fab'-IA-SPE-CE-MS methodology was first established analyzing TTR standard solutions...
August 1, 2017: Talanta
https://www.readbyqxmd.com/read/28499374/clinical-improvement-of-renal-amyloidosis-in-a-patient-with-systemic-onset-juvenile-idiopathic-arthritis-who-received-tocilizumab-treatment-a-case-report-and-literature-review
#12
Songkiat Chantarogh, Soamarat Vilaiyuk, Thipwimol Tim-Aroon, Suchin Worawichawong
BACKGROUND: Juvenile idiopathic arthritis (JIA) is a common rheumatic disease in children and adolescents. Although JIA may cause secondary amyloidosis, this is a rare complication in patients with JIA and other rheumatic diseases. Many previous studies have revealed that common heterozygous or homozygous mutations in the MEFV gene are associated with systemic-onset JIA (SJIA). CASE PRESENTATION: We herein report a case involving a 19-year-old female patient with difficult-to-control SJIA...
May 12, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28498108/imaging-for-abdominal-involvement-in-amyloidosis
#13
H Nursun Özcan, Mithat Haliloğlu, Cenk Sökmensüer, Deniz Akata, Mustafa Özmen, Muşturay Karçaaltıncaba
Involvement of the abdominal organs has variable presentations mostly without specific findings. The objective of this pictorial was to illustrate the computed tomography (CT) and magnetic resonance imaging (MRI) findings of abdominal involvement in systemic amyloidosis. Heterogeneous appearance of the liver, periportal involvement, diffuse low signal intensity of spleen on T2-weighted MRI, and thickened bowel wall may be helpful imaging findings when accompanied by presence or history of chronic inflammatory disease and clinical suspicion for amyloidosis...
May 12, 2017: Diagnostic and Interventional Radiology: Official Journal of the Turkish Society of Radiology
https://www.readbyqxmd.com/read/28490298/raccoon-eyes-in-systemic-light-chain-amyloidosis
#14
Cecily J Forsyth, Campbell R Tiley
No abstract text is available yet for this article.
May 15, 2017: Medical Journal of Australia
https://www.readbyqxmd.com/read/28485623/inhibition-of-amyloid-oligomerization-into-different-supramolecular-architectures-by-small-molecules-mechanistic-insights-and-design-rules
#15
Sayanti Brahmachari, Ashim Paul, Daniel Segal, Ehud Gazit
Protein misfolding and aggregation have been associated with several human disorders, including Alzheimer's, Parkinson's and Huntington's diseases, as well as senile systemic amyloidosis and Type II diabetes. However, there is no current disease-modifying therapy available for the treatment of these disorders. In spite of extensive academic, pharmaceutical, medicinal and clinical research, a complete mechanistic model for this family of diseases is still lacking. In this review, we primarily discuss the different types of small molecular entities which have been used for the inhibition of the aggregation process of different amyloidogenic proteins under diseased conditions...
May 2017: Future Medicinal Chemistry
https://www.readbyqxmd.com/read/28483281/preclinical-data-support-leveraging-cs1-chimeric-antigen-receptor-t-cell-therapy-for-systemic-light-chain-amyloidosis
#16
Michael Rosenzweig, Ryan Urak, Miriam Walter, Laura Lim, James F Sanchez, Amrita Krishnan, Stephen Forman, Xiuli Wang
BACKGROUND AIMS: Light chain amyloidosis (AL) is a protein deposition disorder that is a result of a plasma cell dyscrasia, similar to multiple myeloma (MM). Immunotherapy is an attractive approach because of the low burden of disease, but the optimal target for AL is unclear. CS1 and B-cell maturation antigen (BCMA) are two potential targets because they are expressed on normal plasma cells and MM cells. METHODS: We performed a prospective study evaluating bone marrow specimens of 20 patients with plasma cell diseases, 10 with AL and 10 with MM...
May 5, 2017: Cytotherapy
https://www.readbyqxmd.com/read/28483059/presentation-and-outcomes-of-localized-immunoglobulin-light-chain-amyloidosis-the-mayo-clinic-experience
#17
Taxiarchis V Kourelis, Robert A Kyle, David Dingli, Francis K Buadi, Shaji K Kumar, Morie A Gertz, Martha Q Lacy, Prashant Kapoor, Ronald S Go, Wilson I Gonsalves, Rahma Warsame, John A Lust, Suzanne R Hayman, S Vincent Rajkumar, Steven R Zeldenrust, Stephen J Russell, Yi Lin, Nelson Leung, Angela Dispenzieri
OBJECTIVE: To describe treatment types, outcomes, and relapse patterns in patients with localized immunoglobulin light chain amyloidosis (ALL). PATIENTS AND METHODS: We included all patients with ALL seen at Mayo Clinic in Rochester, Minnesota, from January 1, 1968, through June 30, 2014. The diagnosis of ALL was predicated on the presence of a Congo red-positive biopsy specimen and negative serum and urine immunofixation. Treatment response categories were response, stability, and progression...
May 5, 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28482636/intrahippocampal-pathways-involved-in-learning-memory-mechanisms-are-affected-by-intracerebral-infusions-of-amyloid-%C3%AE-25-35-peptide-and-hydrated-fullerene-c60-in-rats
#18
Rita Gordon, Igor Podolski, Ekaterina Makarova, Alexander Deev, Ekaterina Mugantseva, Sergey Khutsyan, Frank Sengpiel, Arkady Murashev, Vasily Vorobyov
Primary memory impairments associated with increased level of amyloid-β (Aβ) in the brain have been shown to be linked, partially, with early pathological changes in the entorhinal cortex (EC) which spread on the whole limbic system. While the hippocampus is known to play a key role in learning and memory mechanisms, it is as yet unclear how its structures are involved in the EC pathology. In this study, changes in memory and neuronal morphology in male Wistar rats intrahippocampally injected with Aβ25-35 were correlated on days 14 and 45 after the injection to reveal specific cognitive-structural associations...
May 2, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28476949/angiotensin-iv-receptors-mediate-the-cognitive-and-cerebrovascular-benefits-of-losartan-in-a-mouse-model-of-alzheimer-s-disease
#19
Jessika Royea, Luqing Zhang, Xin-Kang Tong, Edith Hamel
The use of angiotensin receptor blockers (ARBs) correlates with reduced onset and progression of Alzheimer's disease (AD). The mechanism depicting how ARBs, such as losartan, restore cerebrovascular and cognitive deficits in AD is unknown. Here, we propose a mechanism underlying losartan's benefits by selectively blocking the effects of Angiotensin IV (AngIV) at its receptor (AT4R) with divalinal in mice overexpressing the AD-related Swedish and Indiana mutations of the human amyloid precursor protein (APP mice) and wild-type mice...
May 5, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28462876/-aa-amyloidosis
#20
Katia Stankovic Stojanovic, Sophie Georgin-Lavialle, Gilles Grateau
AA amyloidosis remains one of the three main types of systemic amyloidosis with AL and ATTR. Its incidence has been however decreasing recently in Western countries. Chronic inflammatory diseases are currently the first cause of AA amyloidosis, including rheumatoid arthritis, spondyloarthritis and autoinflammatory diseases. Castleman's disease is a specific cause of AA amyloidosis that can be cured by surgery. A chronic inflammatory response is required to develop amyloidosis. Other genetic and environmental factors are also involved...
April 24, 2017: Néphrologie & Thérapeutique
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