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Primary amyloidosis

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https://www.readbyqxmd.com/read/28197412/bronchoscopic-diagnosis-and-treatment-of-primary-tracheobronchial-amyloidosis-a-retrospective-analysis-from-china
#1
Xiaoxiao Lu, Bixiu He, Ge Wang, Baimei He, Lijing Wang, Qiong Chen
Objective. To assess the value of bronchoscopy in the diagnosis and treatment of primary tracheobronchial amyloidosis (TBA), in order to reduce misdiagnosis rates and improve prognosis. Methods. Clinical data of 107 patients with TBA reported from 1981 to 2015 in China were retrospectively analyzed for clinical features, bronchoscopic manifestations, pathologies, treatments, and outcomes. Results. 105 of 107 TBA patients were pathologically confirmed by bronchoscopy. Main bronchoscopic manifestations of TBA were single or multiple nodules and masses within tracheobronchial lumens; local or diffuse luminal stenosis and obstruction; luminal wall thickening and rigidity; rough or uneven inner luminal walls; congestion and edema of mucosa, which was friable and prone to bleeding upon touch; and so forth...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28188164/peritoneal-amyloidosis-with-myopathy-in-primary-systemic-al-amyloidosis
#2
Ali Al-Adhami, Kate Steiner, Spencer Ellis
No abstract text is available yet for this article.
February 10, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28168952/primary-amyloidosis
#3
A M Arnáiz-García, M E Arnáiz-García, J Arnáiz
No abstract text is available yet for this article.
February 3, 2017: Revista Clínica Española
https://www.readbyqxmd.com/read/28145535/amyloid-cardiomyopathy
#4
Lucie Karafiatova, Tomas Pika
Amyloidosis is a heterogeneous group of diseases characterized by the deposition of amyloid. It is caused by extracellular deposition of insoluble fibrils with beta-pleated sheet configuration. The protein misfolding abnormalities result in amyloid fibrils and may manifest as primary, secondary, or familial amyloidosis. Amyloid deposition can occur in multiple organs (eg, heart, liver, kidney, skin, eyes, lungs, nervous system) resulting in a variety of clinical manifestations. Cardiac involvement can occur as part of a systemic disease or as a localized phenomenon...
February 1, 2017: Biomedical Papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
https://www.readbyqxmd.com/read/28134587/amyloidosis-of-the-lung
#5
Andras Khoor, Thomas V Colby
CONTEXT: -Amyloidosis is a heterogeneous group of diseases characterized by the deposition of congophilic amyloid fibrils in the extracellular matrix of tissues and organs. To date, 31 fibril proteins have been identified in humans, and it is now recommended that amyloidoses be named after these fibril proteins. Based on this classification scheme, the most common forms of amyloidosis include systemic AL (formerly primary), systemic AA (formerly secondary), systemic wild-type ATTR (formerly age-related or senile systemic), and systemic hereditary ATTR amyloidosis (formerly familial amyloid polyneuropathy)...
February 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28130214/the-amyloidogenic-light-chain-is-a-stressor-that-sensitizes-plasma-cells-to-proteasome-inhibitor-toxicity
#6
Laura Oliva, Ugo Orfanelli, Massimo Resnati, Andrea Raimondi, Andrea Orsi, Enrico Milan, Giovanni Palladini, Paolo Milani, Fulvia Cerruti, Paolo Cascio, Simona Casarini, Paola Rognoni, Thierry Touvier, Magda Marcatti, Fabio Ciceri, Silvia Mangiacavalli, Alessandro Corso, Giampaolo Merlini, Simone Cenci
Systemic light chain (AL) amyloidosis is caused by the clonal production of an unstable immunoglobulin light chain (LC), which affects organ function systemically. While pathogenic LCs have been characterized biochemically, little is known about the biology of amyloidogenic PCs. Intrigued by the unique response rates of AL amyloidosis patients to the first-in-class proteasome inhibitor (PI) bortezomib, we purified and investigated patient-derived AL PCs, in comparison with primary multiple myeloma (MM) PCs, the prototypical PI-responsive cells...
January 27, 2017: Blood
https://www.readbyqxmd.com/read/28129067/radiologic-pathologic-correlation-of-primary-and-secondary-cardiomyopathies-mr-imaging-and-histopathologic-findings-in-hearts-from-autopsy-and-transplantation
#7
Hiromi Hashimura, Fumiko Kimura, Hatsue Ishibashi-Ueda, Yoshiaki Morita, Masahiro Higashi, Shintaro Nakano, Atsushi Iguchi, Kensuke Uotani, Kazuro Sugimura, Hiroaki Naito
Cardiac magnetic resonance (MR) imaging with late gadolinium enhancement (LGE) is used to detect and assess the myocardial damage seen with a variety of cardiomyopathies. Gadolinium-based contrast material accumulates in the expanded interstitial space of the myocardium. Areas with LGE correspond to replacement fibrosis, fibrofatty change, epithelioid granuloma, inflammatory cell infiltration, cardiomyocyte necrosis, and amyloid deposition-conditions that represent a focal increase in interstitial space. Areas without LGE correspond to interstitial or plexiform fibrosis, mildly degenerated cardiomyocytes, inflammatory cell infiltration, and diffuse amyloid deposition-conditions that represent diffuse increases in interstitial space...
January 27, 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28117074/light-chain-cardiac-amyloidosis
#8
REVIEW
Anit K Mankad, Isata Sesay, Keyur B Shah
Cardiac amyloidosis is an underrecognized condition, in which delays to diagnosis have great implications on management options, prognosis, and morbidity. Once cardiac tissue is infiltrated by amyloid fibrils, there is a cascade of pathologic changes that can display an array of clinical manifestations, from impaired relaxation of the ventricular myocardium to severe restrictive disease or even progressive systolic heart failure. Management is guided not only by recognizing the subtype of amyloidosis (primary, hereditary, and wild-type transthyretin amyloidosis), but also the clinical stage of the disease...
November 17, 2016: Current Problems in Cancer
https://www.readbyqxmd.com/read/28103719/pegylated-nanoliposomal-clusterin-for-amyloidogenic-light-chain-induced-endothelial-dysfunction
#9
Diana Guzman-Villanueva, Raymond Q Migrino, Seth Truran, Nina Karamanova, Daniel A Franco, Camelia Burciu, Subhadip Senapati, Dobrin Nedelkov, Parameswaran Hari, Volkmar Weissig
Light chain (AL) amyloidosis is a disease associated with significant morbidity and mortality arising from multi-organ injury induced by amyloidogenic light chain proteins (LC). There is no available treatment to reverse the toxicity of LC. We previously showed that chaperone glycoprotein clusterin (CLU) and nanoliposomes (NL), separately, restore human microvascular endothelial function impaired by LC. In this work, we aim to prepare PEGylated-nanoliposomal clusterin (NL-CLU) formulations that could allow combined benefit against LC while potentially enabling efficient delivery to microvascular tissue, and test efficacy on human arteriole endothelial function...
January 20, 2017: Journal of Liposome Research
https://www.readbyqxmd.com/read/28087478/renal-involvement-in-lysinuric-protein-intolerance-contribution-of-pathology-to-assessment-of-heterogeneity-of-renal-lesions
#10
Emmanuel Estève, Pauline Krug, Aurélie Hummel, Jean-Baptiste Arnoux, Olivia Boyer, Anais Brassier, Pascale de Lonlay, Vincent Vuiblet, Stéphanie Gobin, Rémi Salomon, Christine Piètrement, Jean-Paul Bonnefont, Aude Servais, Louise Galmiche
Lysinuric protein intolerance (LPI) is a rare autosomal recessive disease caused by mutations in the SLC7A7 gene encoding the light subunit of a cationic amino acid transporter. Symptoms mimic primary urea cycle defects but dysimmune symptoms are also described. Renal involvement in LPI was first described in the 1980's. In 2007, it appeared that it could concern as much as 75% of LPI patients and could lead to end stage renal disease. The most common feature is proximal tubular dysfunction and nephrocalcinosis but glomerular lesions are also reported...
January 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/28076605/isolated-primary-amyloidosis-of-the-inferior-rectus-muscle-mimicking-graves-orbitopathy
#11
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/28056871/lung-adenocarcinoma-expressing-receptor-for-advanced-glycation-end-products-with-primary-systemic-al-amyloidosis-a-case-report-and-literature-review
#12
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
#13
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
#14
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/27995877/-the-diagnostic-and-prognostic-values-of-serum-free-light-chain-in-patients-with-primary-light-chain-amyloidosis
#15
C L Zhang, J Feng, K N Shen, W Su, C L Zhang, X F Huang, X X Cao, L Zhang, D B Zhou, J Li
Objective: To investigate the diagnostic and prognostic values of serum free light chain (sFLC) in patients with primary light chain amyloidosis (pAL). Methods: Patients diagnosed with pAL between January 2009 and June 2015 at Peking Union Medical College Hospital were included in this study to retrospectively evaluate the clinical data, sFLC, treatment and survival. Results: In total, 126 newly diagnosed pAL patients with complete sFLC data were included in this study. The median age was 57 years old (range, 37-81 years) and male to female ratio was 1...
November 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/27974983/ectropion-and-conjunctival-mass-in-a-patient-with-primary-bilateral-conjunctival-amyloidosis
#16
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/27957427/a-rare-case-of-hematuria-primary-amyloidosis-of-the-bladder-neck
#17
Sotonye Tolofari, Asif Ansari, Ross J Knight
Primary and localized amyloidosis of the urinary tract is considered to be a rare clinical entity with approximately 160 cases documented in the literature. Here we present a case of a 77 year old gentleman with painless visible hematuria. Flexible cystoscopy revealed abnormal and polypoidal bladder mucosa. Transurethral resection of these lesions revealed histology with congo-red staining and positive green-apple birefringence, these findings are consistent with amyloidosis. There has so far been no sign of recurrence in this gentleman, with routine cystoscopic surveillance...
January 2017: Urology Case Reports
https://www.readbyqxmd.com/read/27957424/bullous-skin-lesions-in-a-patient-with-end-stage-renal-disease-combined-with-myeloma-and-primary-amyloidosis
#18
Jong Hwan Jung
No abstract text is available yet for this article.
December 2016: Kidney Research and Clinical Practice
https://www.readbyqxmd.com/read/27940040/geniposide-ameliorates-cognitive-deficits-by-attenuating-the-cholinergic-defect-and-amyloidosis-in-middle-aged-alzheimer-model-mice
#19
Chunhui Zhao, Haijing Zhang, Hang Li, Cui Lv, Xiaoli Liu, Zhi Li, Wenfeng Xin, Yongyan Wang, Wensheng Zhang
Alzheimer's disease (AD) is a progressive neurodegenerative disorder characterized by memory deficits and cognitive decline. Amyloid-β (Aβ) deposition and cholinergic defect are widely thought to be the underlying mechanism of learning and memory impairment. Geniposide, which is the main active component of the traditional Chinese herbal Gardenia jasminoides Ellis, elicits neuroprotective effects by alleviating inflammation responses and oxidative damages. In this study, we investigated the protective effect of geniposide on levels of cholinergic markers, RAGE, RAGE-dependent signalling pathways and amyloid accumulation in the APPswe/PS1dE9 AD model mouse...
December 6, 2016: Neuropharmacology
https://www.readbyqxmd.com/read/27879490/intense-uptake-in-amyloidosis-of-the-seminal-vesicles-on-68ga-psma-pet-mimicking-locally-advanced-prostate-cancer
#20
Maximilian Stephens, David Insoo Kim, Benjamin Shepherd, Sonja Gustafson, Paul Thomas
We report a case of benign senile seminal vesicle amyloidosis demonstrating intense Ga-prostate-specific membrane antigen (PSMA) uptake on PET/CT. A 68-year-old man underwent staging PSMA PET/CT and MRI for biopsy-proven prostate adenocarcinoma. There was an intense focus of Ga-PSMA uptake in the primary malignancy, as well as symmetrical intense uptake in the seminal vesicles bilaterally that was reported as multifocal disease with local invasion. Final histology after radical prostatectomy showed amyloidosis of the seminal vesicles without any evidence of prostate cancer...
February 2017: Clinical Nuclear Medicine
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