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systematic amyloidosis

Stéphane Epelbaum, Rémy Genthon, Enrica Cavedo, Marie Odile Habert, Foudil Lamari, Geoffroy Gagliardi, Simone Lista, Marc Teichmann, Hovagim Bakardjian, Harald Hampel, Bruno Dubois
Preclinical Alzheimer's disease (AD) is a relatively recent concept describing an entity characterized by the presence of a pathophysiological biomarker signature characteristic for AD in the absence of specific clinical symptoms. There is rising interest in the scientific community to define such an early target population mainly because of failures of all recent clinical trials despite evidence of biological effects on brain amyloidosis for some compounds. A conceptual framework has recently been proposed for this preclinical phase of AD...
February 7, 2017: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
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
Chun-Lan Zhang, Jun Feng, Xin-Xin Cao, Cong-Li Zhang, Kai-Ni Shen, Xu-Fei Huang, Lu Zhang, Dao-Bin Zhou, Jian Li
Objective To evaluate the sensitivities of various biopsy methods for the diagnosis of systematic amyloidosis (SA). Methods The clinical data and biopsy results of 194 SA patients who were treated in Peking Union Medical College Hospital from January 2009 to June 2015 were retrospectively analyzed. Results The highest sensitivity was achieved by biopsy of affected organs,with renal biopsy 97.4%,heart biopsy 95.0% and liver biopsy 87.5%. Among non-invasive biopsy methods,tongue biopsy was found to be 75% sensitive,followed by gingiva biopsy at 57%,abdominal fat pad aspiration at 57%,rectum biopsy at 16%,and bone marrow examination at 8%...
December 20, 2016: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
Kathrin Andrich, Ute Hegenbart, Christoph Kimmich, Niraja Kedia, H Robert Bergen, Stefan Schönland, Erich 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 do 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...
February 10, 2017: Journal of Biological Chemistry
Samira Alinejad, Kazem Ghaemi, Mohammad Abdollahi, Omid Mehrpour
BACKGROUND: Methadone is commonly administered for chronic pain relief and treatment of opioid dependence. Concurrent with its increased consumption, toxicities and fatalities have increased. One of the adverse effects of opioid analgesics, including methadone, is that of nephrotoxicity. Opioids can have an effect on renal function through several different mechanisms. METHODS: We searched common bibliographical databases for the terms methadone, toxicity, poisoning, kidney, renal, and nephrotoxicity and summarize our findings in this review...
2016: SpringerPlus
Sentaro Imamura, Shintaro Narita, Ryuta Nishikomori, Hiroshi Tsuruta, Kazuyuki Numakura, Atsushi Maeno, Mitsuru Saito, Takamitsu Inoue, Norihiko Tsuchiya, Hiroshi Nanjo, Toshio Heike, Shigeru Satoh, Tomonori Habuchi
BACKGROUND: Secondary bladder amyloidosis is an extremely rare disease, resulting from a chronic systematic inflammatory disorder associated with amyloid deposits. Although uncommon in Japan, familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease characterized by recurrent episodes of fever of short duration and serositis and is frequently associated with systemic amyloidosis. Here, we present a case of a Japanese patient complaining of fever and macroscopic hematuria after a living donor renal transplantation...
October 19, 2016: BMC Research Notes
Sameer Raina, Shelly Y Lensing, Ramez S Nairooz, Naga Venkata K Pothineni, Abdul Hakeem, Sabha Bhatti, Tarun Pandey
OBJECTIVES: The aim of this study was to access the prognostic implication of late gadolinium enhancement (LGE) in patients with systemic amyloidosis undergoing cardiac magnetic resonance (CMR). BACKGROUND: Cardiac amyloidosis confers significantly worse prognosis in patients with systemic amyloidosis. CMR imaging has emerged as an attractive noninvasive modality to diagnose cardiac involvement in patients with systemic amyloidosis. We performed a systemic review and meta-analysis to evaluate the prognostic role of LGE-CMR imaging in patients with systemic amyloidosis...
November 2016: JACC. Cardiovascular Imaging
Lara Ordoñez-Gutierrez, Ivan Fernandez-Perez, Jose Luis Herrera, Marta Anton, Irene Benito-Cuesta, Francisco Wandosell
Cerebellar pathology has been related to presenilin 1 mutations in certain pedigrees of familial Alzheimer's disease. However, cerebellum tissue has not been intensively analyzed in transgenic models of mutant presenilins. Furthermore, the effect of the sex of the mice was not systematically analyzed, despite the fact that important gender differences in the evolution of the disease in the human population have been described. We analyzed whether the progression of amyloidosis in a double transgenic mouse, AβPP/PS1, is susceptible to aging and differentially affects males and females...
September 6, 2016: Journal of Alzheimer's Disease: JAD
David Hamon, Vincent Algalarrondo, Estelle Gandjbakhch, Fabrice Extramiana, Eloi Marijon, Nathalie Elbaz, Dounia Selhane, Jean-Luc Dubois-Rande, Emmanuel Teiger, Violaine Plante-Bordeneuve, Thibaud Damy, Nicolas Lellouche
BACKGROUND: Cardiac amyloidosis (CA) is associated with a poor prognosis with the proposed mechanism of sudden cardiac death in the majority of patients being pulseless electrical activity. However, the incidence of ventricular arrhythmias (VA) and implantable cardioverter-defibrillator (ICD) indications in CA patients are unclear. We performed a detailed evaluation of our CA population undergoing ICD implantation and assessed appropriate ICD therapy and survival predictors. METHODS: We included consecutive patients from June 2008 to November 2014 in five centers...
November 1, 2016: International Journal of Cardiology
Adam J Loavenbruck, Wolfgang Singer, Michelle L Mauermann, Paola Sandroni, P James B Dyck, Morie Gertz, Christopher J Klein, Phillip A Low
OBJECTIVE: To systematically compare transthyretin with primary amyloid neuropathy to define their natural history and the underlying mechanisms for differences in phenotype and natural history. METHODS: All patients with defined amyloid subtype and peripheral neuropathy who completed autonomic testing and electromyography at Mayo Clinic Rochester between 1993 and 2013 were included. Medical records were reviewed for time of onset of defined clinical features. The degree of autonomic impairment was quantified using the composite autonomic severity scale...
September 2016: Annals of Neurology
Kari Hemminki, Asta Försti, Raimo Tuuminen, Otto Hemminki, Hartmut Goldschmidt, Kristina Sundquist, Jan Sundquist, Xinjun Li
Plasma cell dyscrasias, including monoclonal gammopathy of undetermined significance (MGUS), multiple myeloma (MM), Waldenström macroglobulinemia (WM) and light chain AL amyloidosis, are characterized by clonal expansion of plasma cells which produce a vast amount of an immunoglobulin-derived M-protein. We noted that MGUS diagnosis often coincided with diagnoses of senile cataract and glaucoma and tested the associations of MGUS, MM, WM and AL amyloidosis with subsequent eye diseases identified from the Swedish patient registers between 1997 and 2012...
2016: Scientific Reports
Lei Zhao, Zhuang Tian, Quan Fang
BACKGROUND: This study is a systematic review and meta-analysis of the diagnostic value of cardiovascular magnetic resonance (CMR) in cardiac amyloidosis (CA). METHODS: A wide variety of electronic databases were searched for studies of CMR that reported the diagnostic accuracy in patients with suspected CA. Research manuscripts were subjected to further systematic review and meta-analysis. Methodological evaluation was performed under the guidance of the Quality Assessment of Diagnostic Accuracy Studies -2 (QUADAS-2)...
2016: BMC Cardiovascular Disorders
E Monge, N Coolen-Allou, P Mascarel, V Gazaille
INTRODUCTION: Primary pulmonary lymphoma is a rare disease; diagnosis is often delayed because of atypical clinical presentation and slow progression. OBSERVATION: A 42-year-old woman consulted because of haemoptysis. Chest CT-scan showed multiple nodular calcified masses. A lung biopsy led to the diagnosis of pulmonary amyloidosis with pulmonary MALT lymphoma (mucosa-associated lymphoid tissue). The patient developed two paraneoplastic syndromes: a hypertrophic osteoarthropathy and mucinosis...
November 2016: Revue des Maladies Respiratoires
Jeroen Ch van der Hilst, Michel Moutschen, Peter E Messiaen, Bernard R Lauwerys, Steven Vanderschueren
INTRODUCTION: In 5%-10% of patients with familial Mediterranean fever (FMF), colchicine is not effective in preventing inflammatory attacks. Another 5%-10% of patients are intolerant to effective doses of colchicine and experience serious side effects. Treatment with anti-interleukin-1 (IL-1) drugs may be an alternative for these patients, although it is not reimbursed for this indication in many countries. METHODS: We systematically searched PubMed, Web of Science, and Scopus for reports of anti-IL-1 treatment in FMF patients...
2016: Biologics: Targets & Therapy
Joan Tosca Cuquerella, Marta Maia Bosca-Watts, Rosario Anton Ausejo, Sandra Tejedor Alonso, Francisco Mora De Miguel, Miguel Minguez Perez
BACKGROUND AND AIMS: Amyloidosis is a rare complication of inflammatory bowel disease [IBD]; its low prevalence has hindered both descriptive and therapeutic studies. The aim of this study was to estimate the prevalence of amyloidosis in IBD and the risk factors associated with this complication. METHODS: This paper presents an observational study, followed by a systematic review of the epidemiological and clinical characteristics of the disease and a review of the diagnostic and therapeutic options...
October 2016: Journal of Crohn's & Colitis
Jessica Oliboni Scapineli, Lucieli Ceolin, Márcia Khaled Puñales, José Miguel Dora, Ana Luiza Maia
Multiple endocrine neoplasia type 2A (MEN2A) may be rarely associated with cutaneous lichen amyloidosis (CLA), a skin lesion located in the interescapular region. Here, we describe 3 MEN2A-related CLA kindred and perform a systematic review (SR) of the literature on clinical, biochemical and molecular characteristics of MEN2A-related CLA patients. Thirty-eight patients with MEN2A-related CLA followed at our institution were evaluated. The median age at MEN2A diagnosis in our cohort was 25 (13-41) years, 68 % were women and all harbored codon 634 RET mutations...
October 2016: Familial Cancer
Guy Touchard, Frank Bridoux, Jean-Michel Goujon
The spectrum of glomerular disorders with organized immunoglobulin (Ig) deposits is heterogeneous. It encompasses 2 mains categories: glomerulopathies with fibrillary deposits are mostly represented by immunoglobulinic amyloidosis (most commonly AL amyloidosis, characterized by monoclonal light chain deposits often of the lambda isotype), and pseudo-amyloid fibrillary glomerulonephritis in which deposits predominantly contain polyclonal IgG4. Glomerulopathies with microtubular deposits include cryoglobulinemic glomerulonephritis (type I and type II, with or without detectable serum cryoglobulin) and glomerulonephritis with organized microtubular monoclonal Ig deposits (GOMMID) also referred to as immunotactoid glomerulopathy...
February 2016: Néphrologie & Thérapeutique
P Chandrasekaran, R Rajasekaran
Beta-2 microglobulin (β2m) is an amyloidogenic protein belongs to the immunoglobulin superfamily, responsible for the dialysis-related amyloidosis (DRA). Misfolding of β2m is a prerequisite to the formation of systemic amyloidosis that has an effect on the structure and function of the affected organ. The aim of our present study is to intensively explore the structural characterization of amyloid aggregation propensity of recently identified natural mutation D76N by applying the classical molecular dynamics (MD) approach...
March 2016: Molecular BioSystems
Laura Obici, Jan B Kuks, Juan Buades, David Adams, Ole B Suhr, Teresa Coelho, Theodore Kyriakides
PURPOSE OF REVIEW: These recommendations highlight recent experience in genetic counselling for the severe autosomal-dominant, late-onset transthyretin familial amyloid polyneuropathy (TTR-FAP) disease, and present a structured approach towards identification and monitoring of asymptomatic carriers of the mutated gene. RECENT FINDINGS: The effectiveness of current treatment options is still limited in patients with TTR-FAP beyond stage I. Diagnosis in the early stages of TTR-FAP is essential to prevent or delay the progression of disease...
February 2016: Current Opinion in Neurology
Burak Erer, Erkan Demirkaya, Seza Ozen, Tilmann Kallinich
The most dreaded complication of familial Mediterranean fever (FMF) is amyloidosis; controversy exists as to what acute phase reactant (APR) should be monitored in these patients. To analyze the best acute phase reactant for FMF follow-up to help guide physicians to decide on what APR parameter to use, we also attempted to define the best APR in predicting the complications of FMF, specifically the development of amyloidosis. Systematic review based on a sensitive search to capture studies that: (1) included FMF patients; (2) measured serum amyloid A (SAA), CRP (C-reactive protein), proteinuria, or ESR (erythrocyte sedimentation rate); (3) amyloidosis were the outcome measure; (4) sensitivity, specificity, predictive value, and other performance parameters could be calculated; and (5) had a longitudinal design...
April 2016: Rheumatology International
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