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systemic amyloidosis

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https://www.readbyqxmd.com/read/28079687/periocular-amyloidosis-manifesting-as-pseudopemphigoid-treated-with-mitomycin-c
#1
Kaveh Vahdani, Vladimir T Thaller, Giorgio Albanese, Andrew F Dean
PURPOSE: To describe an unusual cicatrizing manifestation of periocular amyloid and the utility of adjuvant antimetabolite therapy. METHODS: This is a case report of a 49-year old woman with progressive bilateral upper lid ptosis, who was found to have bilateral inferior forniceal masses with thickening of the conjunctiva. Conjunctival and eyelid biopsies showed evidence of amyloid deposition. Systemic evaluation did not reveal any evidence of systemic amyloidosis...
January 11, 2017: Cornea
https://www.readbyqxmd.com/read/28078835/pathological-characteristics-of-fifty-patients-with-renal-amyloidosis-in-sri-lanka
#2
D L Dasanayaka, S Wijetunge, N V Ratnatunga
Renal involvement is the most important cause of morbidity and mortality in systemic amyloidosis. This retrospective analysis was conducted to analyse the clinico-pathological characteristics of renal amyloidosis in a group of Sri Lankan patients undergoing renal biopsy. Renal amyloidosis was observed in 50/ 9712 (0.5%) renal biopsies. The underlying cause for amyloidosis was not known in most. Of the known causes multiple myeloma was the commonest. Nephrotic range proteinuria was the most common clinical outcome and most had grade I to III chronic kidney disease at the time of diagnosis...
30, 2016: Ceylon Medical Journal
https://www.readbyqxmd.com/read/28076605/isolated-primary-amyloidosis-of-the-inferior-rectus-muscle-mimicking-graves-orbitopathy
#3
Mário Luiz Ribeiro Monteiro, Allan Christian Pieroni Gonçalves, Alanna Mara Pinheiro Sobreira Bezerra
The diagnosis of Graves' orbitopathy is usually straightforward. However, orbital diseases that mimick some clinical signs of Graves' orbitopathy may cause diagnostic confusion, particularly when associated to some form of thyroid dysfunction. This report describes the rare occurrence of localized inferior rectus muscle amyloidosis in a patient with autoimmune hypothyroidism, who was misdiagnosed as Graves' orbitopathy. A 48-year-old man complained of painless progressive proptosis on the left side and intermittent vertical diplopia for 6 months...
October 2016: Einstein
https://www.readbyqxmd.com/read/28073516/early-diagnosis-of-systemic-amyloidosis-by-means-of-a-transverse-carpal-ligament-biopsy-carried-out-during-carpal-tunnel-syndrome-surgery
#4
Judit Fernández Fuertes, Óscar Rodríguez Vicente, Sergio Sánchez Herráez, Luis Rafael Ramos Pascua
INTRODUCTION AND OBJECTIVE: The systematic analysis of a carpal transverse ligament (CTL) sample obtained during routine carpal tunnel syndrome (CTS) surgery may constitute a method of early diagnosis for systemic amyloidosis. MATERIAL AND METHODS: Prospective study carried out on 147 consecutive CTL samples collected from patients intervened for CTS at the University Hospital of León from April 2006 to May 2007. In those cases in which amyloid deposition was observed in the CTL sample, the study was completed with a fine needle aspiration biopsy (FNAB) of the subcutaneous fascia, using the Red Congo stain in both cases...
January 7, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28056871/lung-adenocarcinoma-expressing-receptor-for-advanced-glycation-end-products-with-primary-systemic-al-amyloidosis-a-case-report-and-literature-review
#5
Shouichi Okamoto, Shinsaku Togo, Ichiro Nagata, Kazue Shimizu, Yoshika Koinuma, Yukiko Namba, Jun Ito, Toshimasa Uekusa, Kazuhisa Takahashi
BACKGROUND: Receptor for advanced glycation end-products (RAGE), a receptor for amyloids, is constitutively expressed in lungs and generally observed to be downregulated in lung cancer tissues. However, increasing levels of RAGE or serum amyloids is associated with poor outcome in lung cancer patients. We report a rare case of primary systemic amyloid light-chain (AL) amyloidosis in biopsy-proven multiple organs with early-stage non-small cell lung cancer (NSCLC) that displayed strong staining for RAGE in the tumour tissue...
January 5, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28049394/coexistence-of-amyloidosis-and-primary-sj%C3%A3-gren-s-syndrome-an-overview-amyloidosis-and-sj%C3%A3-gren-s-syndrome
#6
Gabriela Hernandez-Molina, David Faz-Muñoz, Miguel Astudillo-Angel, Alejandra Iturralde-Chávez, Edgardo Reyes
BACKGROUND: The association of primary Sjögren´s syndrome (SS) and amyloidosis has been recognized but scarcely assessed. OBJECTIVE: Herein we analyzed the literature regarding this association in order to describe a SS phenotype prone to both conditions. METHODS: PubMed and CINAHL databases were searched from inception until April 2016 for the key words: amyloidosis, amyloid, Sjögren's syndrome and their combinations. RESULTS: Most of the cases corresponded to localized amyloidosis mainly at skin and lung, whereas only three cases were systemic...
January 3, 2017: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/28042785/anterior-aortic-plane-systolic-excursion-a-novel-indicator-of-transplant-free-survival-in-systemic-light-chain-amyloidosis
#7
Marco M Ochs, Johannes Riffel, Arnt V Kristen, Ute Hegenbart, Stefan Schönland, Stefan E Hardt, Hugo A Katus, Derliz Mereles, Sebastian J Buss
BACKGROUND: Anterior aortic plane systolic excursion (AAPSE) was evaluated in the present pilot study as a novel echocardiographic indicator of transplant-free survival in patients with systemic light-chain amyloidosis. METHODS: Eighty-nine patients with light-chain amyloidosis were included in the post-hoc analysis. A subgroup of 54 patients with biopsy-proven cardiac amyloid infiltration were compared with 41 healthy individuals to evaluate the discriminative ability of echocardiographic findings...
December 2016: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/28035639/mycobacterium-infection-from-a-cardiopulmonary-bypass-heater-cooler-unit-in-a-patient-with-steroid-induced-immunosuppression
#8
Yi Cai, Kevin Landolfo, Johnathan R Renew
PURPOSE: To present a case of mycobacterium infection transmitted through a heater-cooler unit during cardiac bypass surgery. CLINICAL FEATURES: A 63-yr-old woman with a past medical history of aortic coarctation repair in 1963 and a mechanical aortic valve replacement in 2010 was prescribed antibiotics and steroids at an outpatient care facility in September 2015 for symptoms of an upper respiratory tract infection. Four months later, she developed malaise and intermittent fever with anemia and acute kidney dysfunction...
December 29, 2016: Canadian Journal of Anaesthesia, Journal Canadien D'anesthésie
https://www.readbyqxmd.com/read/28033255/primarily-isolated-hepatic-involvement-of-amyloidosis-a-case-report-and-overview
#9
Lei Ye, Hui Shi, Hui-Min Wu, Fang-Yu Wang
BACKGROUND: Amyloidosis is particularly difficult to diagnose because the signs and symptoms are subtle. Additionally, there are no specific imaging or laboratory tests, except histopathology. Although it is considered to be a systemic disorder, a small portion of cases may be localized. INTRODUCTION OF THE CASE: A 54-year-old man presented with nonspecific symptoms (jaundice and back pruritus). Biochemical tests showed a high level of bilirubin and elevated serum tumor markers (CA19-9 and CA125)...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28031465/aggregation-of-full-length-immunoglobulin-light-chains-from-al-amyloidosis-patients-is-remodeled-by-epigallocatechin-3-gallate
#10
Kathrin Andrich, Ute Hegenbart, Christoph Kimmich, Niraja Kedia, H Robert Bergen, Stefan Schönland, Erich E Wanker, Jan Bieschke
Intervention into amyloid deposition with anti-amyloid agents like the polyphenol Epigallocatechin-3-gallate (EGCG) is emerging as an experimental secondary treatment strategy in systemic light chain amyloidosis (AL). In both AL and Multiple Myeloma (MM), soluble immunoglobulin light chains (LC) are produced by clonal plasma cells, but only in AL they form amyloid deposits in vivo. We investigated the amyloid formation of patient-derived LC and their susceptibility to EGCG in vitro to probe commonalities and systematic differences in their assembly mechanisms...
December 28, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28026881/intrinsic-factors-adrenal-gland-morphology-and-disease-burden-in-captive-cheetahs-acinonyx-jubatus-in-south-africa
#11
Nina Gillis-Germitsch, Pamela-Rose Vybiral, Daryl Codron, Marcus Clauss, Antoinette Kotze, Emily P Mitchell
Adrenal gland weight (AW) and corticomedullary ratio (ACMR) are used as indicators of stress in animals. Captive cheetahs (Acinonyx jubatus) have higher ACMRs than free-ranging ones and stress has been linked to gastritis, amyloidosis, glomerulosclerosis, and myocardial fibrosis. We reviewed age, sex, body weight (BW), kidney weight (KW), and left AW and ACMR with necropsy findings in 51 South African captive cheetahs. Eleven common histopathologic lesions were counted for each animal as measure of its disease burden...
December 27, 2016: Zoo Biology
https://www.readbyqxmd.com/read/28024929/kidney-involvement-in-systemic-calcitonin-amyloidosis-associated-with-medullary-thyroid-carcinoma
#12
Timco Koopman, Cindy Niedlich-den Herder, Coen A Stegeman, Thera P Links, Johan Bijzet, Bouke P C Hazenberg, Arjan Diepstra
A 52-year-old woman with widely disseminated medullary thyroid carcinoma developed nephrotic syndrome and slowly decreasing kidney function. A kidney biopsy was performed to differentiate between malignancy-associated membranous glomerulopathy and tyrosine kinase inhibitor-induced focal segmental glomerulosclerosis. Surprisingly, the biopsy specimen revealed diffuse glomerular deposition of amyloid that was proved to be derived from the calcitonin hormone (Acal), produced by the medullary thyroid carcinoma...
December 23, 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28002971/new-and-developing-therapies-for-al-amyloidosis
#13
Giulia Zumbo, Omid Sadeghi-Alavijeh, Philip N Hawkins, Marianna Fontana
Systemic light-chain (AL) amyloidosis is an infiltrative disorder associated with an underlying plasma cells dyscrasia, in which monoclonal immunoglobulin light chains accumulate in an abnormal misfolded form as amyloid fibrils in the extracellular space. Symptoms and prognosis are governed by which organs are affected, and cardiac involvement is the major determinant of survival. Diagnosis requires demonstration of amyloid deposition and confirmation of the fibril protein type. Areas covered: This review will focus on the available treatments for systemic AL amyloidosis and on new drug targets and therapeutic approaches...
December 29, 2016: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/27974983/ectropion-and-conjunctival-mass-in-a-patient-with-primary-bilateral-conjunctival-amyloidosis
#14
Alessandro Meduri, Miguel Rechichi, Cosimo Mazzotta, Sergio Zaccharia Scalinci, Mahmoud O Jaroudi
Background. Amyloidosis is a group of disorders characterized by deposition of an extracellular protein, known as amyloid, in an abnormal fibrillar form with highly characteristic histopathologic staining properties. The clinical presentation can vary from a focal, localized lesion where amyloidosis has minor clinical consequences to extensive systemic disease that can involve any organ system of the body. Ocular amyloidosis can occur as a localized lesion or as a part of a systemic disorder. Conjunctival amyloidosis is an uncommon condition that is rarely associated with systemic disease...
2016: Case Reports in Ophthalmological Medicine
https://www.readbyqxmd.com/read/27972560/healthcare-cost-and-utilization-among-u-s-patients-with-relapsed-refractory-systemic-light-chain-amyloidosis-rral
#15
H M Lin, C V Asche, J Ren, C Yong, D Faller, B Seal
No abstract text is available yet for this article.
November 2016: Value in Health: the Journal of the International Society for Pharmacoeconomics and Outcomes Research
https://www.readbyqxmd.com/read/27959636/amyloidosis-insights-from-proteomics
#16
Ahmet Dogan
Amyloidoses are a spectrum of disorders caused by abnormal folding and extracellular deposition of proteins. The deposits lead to tissue damage and organ dysfunction, particularly in the heart, kidneys, and nerves. There are at least 30 different proteins that can cause amyloidosis. The clinical management depends entirely on the type of protein deposited, and thus on the underlying pathogenesis, and often requires high-risk therapeutic intervention. Application of mass spectrometry-based proteomic technologies for analysis of amyloid plaques has transformed the way amyloidosis is diagnosed and classified...
December 5, 2016: Annual Review of Pathology
https://www.readbyqxmd.com/read/27951538/colchicine-in-renal-medicine-new-virtues-of-an-ancient-friend
#17
Yalcin Solak, Dimitrie Siriopol, Abdulmecit Yildiz, Mahmut Ilker Yilmaz, Alberto Ortiz, Adrian Covic, Mehmet Kanbay
Colchicine is a plant-derived alkaloid that disrupts the cell microtubule system and accumulates in neutrophils, inhibiting neutrophil adhesion and recruitment. Colchicine has been used extensively in the prevention and treatment of gouty arthritis attacks, familial Mediterranean fever attacks and resultant AA amyloidosis, and recurrent pericarditis. Colchicine also disrupts the intracellular traffic of additional inflammatory and fibrosis mediators. Renal fibrosis is the final common pathway of chronic renal disease...
December 13, 2016: Blood Purification
https://www.readbyqxmd.com/read/27933393/prevalence-and-prognostic-impact-of-left-sided-valve-thickening-in-systemic-light-chain-amyloidosis
#18
Dania Mohty, Sarah Pradel, Julien Magne, Bahaa Fadel, Cyrille Boulogne, Vincent Petitalot, Safaa Raboukhi, Nicole Darodes, Thibaud Damy, Victor Aboyans, Arnaud Jaccard
BACKGROUND: Left heart valve thickening (LVT) was described in patients with light-chain amyloidosis (AL). This phenomenon reflects likely infiltration of the valve by amyloid proteins. However, the prevalence of LVT and its prognostic value have not been investigated in patients with AL. METHODS AND RESULTS: Comprehensive transthoracic echocardiography was performed at baseline in 150 patients [median age 68 (33-87) years; 59% male] with confirmed AL. The presence of abnormal mitral and/or aortic valve thickening (>3 mm) was assessed in all included patients...
December 8, 2016: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
https://www.readbyqxmd.com/read/27931749/-hereditary-epidermolysis-bullosa-french-national-guidelines-pnds-for-diagnosis-and-treatment
#19
C Chiaverini, E Bourrat, J Mazereeuw-Hautier, S Hadj-Rabia, C Bodemer, J-P Lacour
Hereditary epidermolysis bullosa (EB) is a heterogeneous group of rare genetic diseases characterized by fragile skin and/or mucous membrane, and it may be either local or generalized. It is caused by mutations in genes encoding different proteins involved mainly in the structure and function of the dermal-epidermal junction. Nineteen genes have so far been identified. They are classified by level of skin cleavage (from top to bottom) into four groups: EB simplex, junctional EB, dystrophic EB and Kindler syndrome...
December 5, 2016: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/27927236/chronic-infantile-neurological-cutaneous-and-articular-cinca-syndrome-a-review
#20
REVIEW
Martina Finetti, Alessia Omenetti, Silvia Federici, Roberta Caorsi, Marco Gattorno
INTRODUCTION: The Chronic Infantile Neurological Cutaneous and Articular (CINCA, or Neonatal-onset multisystem inflammatory disease NOMID) is a rare autoinflammatory disease identified in 1987 by Prieur et al., typically characterized by the triad of skin rash, arthropathy and central nervous system manifestations. It represents the most severe phenotype of the cryopyrin-associated periodic syndrome (CAPS). CLINICAL DESCRIPTION AND ETIOLOGY: The syndrome is due to autosomal dominant gain of function mutations in NLRP3, which encodes a key component of the innate immunity that regulates the activation and secretion of interleukin (IL)-1β...
December 7, 2016: Orphanet Journal of Rare Diseases
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