Establishment of an induced pluripotent stem cell line from a patient with hereditary transthyretin amyloidosis carrying transthyretin (TTR) mutation p.Phe53Val
Publication date: Available online 3 August 2020Source: Stem Cell ResearchAuthor(s): He Shan, Jin Ye, Xing Hai Ping, He XinYue, Zhang ShuYang (Source: Stem Cell Research)
Source: Stem Cell Research - August 5, 2020 Category: Stem Cells Source Type: research

Cardiac involvement, morbidity and mortality in hereditary transthyretin amyloidosis because of p.Glu89Gln mutation
Conclusion Cardiac involvement is common and has significant prognostic implications in the evaluated patients with p.Glu89Gln mutation. Heart failure and rhythm disturbances are the main causes of death. An earlier identification of the disease is crucial to improve prognosis. (Source: Journal of Cardiovascular Medicine)
Source: Journal of Cardiovascular Medicine - August 4, 2020 Category: Cardiology Tags: Research articles: Heart failure Source Type: research

Nerve ultrasound in hereditary transthyretin amyloidosis: red flags and possible progression biomarkers
AbstractBackgroundDiagnostic delay of hereditary transthyretin amyloidosis (ATTRv, v for variant) prevents timely treatment and, therefore, concurs to the mortality of the disease. The aim of the present study was to explore with nerve ultrasound (US) possible red flags for early diagnosis in ATTRv patients with carpal tunnel syndrome (CTS) and/or polyneuropathy and in pre-symptomatic carriers.MethodsPatients and pre-symptomatic carriers with a TTR gene mutation were enrolled from seven Italian centers. Severity of CTS was assessed with neurophysiology and clinical evaluation. Median nerve cross-section area (CSA) was meas...
Source: Journal of Neurology - August 4, 2020 Category: Neurology Source Type: research

Tracheobronchial amyloidosis: an uncommon disease with a common presentation
We describe three cases who presented with haemoptysis, which on further evaluation were diagnosed as isolated tracheobronchial amyloidosis, and a review of literature. AbstractAmyloidosis is an uncommon heterogeneous and multi ‐systemic disease characterized by extracellular amyloid deposition. The size of proteins varies and forms a part of local disease or systemic process. Light chain amyloidosis (AL) is the most prevalent form of systemic amyloidosis which may also be seen in localized disease. Isolated tracheobronc hial amyloidosis (TBA) is rather unusual with local amyloid deposition which may pose a diagnostic di...
Source: Respirology Case Reports - August 2, 2020 Category: Respiratory Medicine Authors: Loveleen Mangla, Rohit Vadala, Shirish Kumar Kadli, Deepak Prajapat, Deepak Talwar Tags: Case Series Source Type: research

Emerging drugs for the treatment of light chain amyloidosis.
Authors: Chakraborty R, Lentzsch S Abstract INTRODUCTION: Systemic AL amyloidosis is a protein-misfolding disorder that is characterized by the deposition of insoluble amyloid fibrils derived from kinetically unstable light chains. Achieving a rapid and deep hematologic response is critical for long-term survival. AREAS COVERED: This review covers the existing and emerging treatment options for systemic AL, divided into anti-plasma cell and fibril-directed therapies. The anti-CD38 monoclonal antibody daratumumab has demonstrated an unprecedented hematologic response rate and will become the new standard-of-care...
Source: Expert Opinion on Emerging Drugs - August 1, 2020 Category: Drugs & Pharmacology Tags: Expert Opin Emerg Drugs Source Type: research

Novel challenges in the management of immunoglobulin light chain amyloidosis: from the bench to the bedside.
Authors: Basset M, Nuvolone M, Palladini G, Merlini G Abstract INTRODUCTION: Immunoglobulin light chain (AL) amyloidosis is one of the most frequent systemic amyloidosis in Western countries. It is caused by a B-cell clone producing a misfolded light chain (LC) that deposits in organs. AREAS COVERED: The review examines recent findings on pathophysiology and clinical management of AL amyloidosis. It contains an update on the recent hot topics as novel therapeutic approaches, definition of relapse and hematologic response assessment. To review literature on AL amyloidosis, a bibliographic search was performed us...
Source: Expert Review of Hematology - July 31, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Primary systematic AL amyloidosis with upper gastrointestinal involvement: endoscopic findings of a protein deposition disorder.
PMID: 32731685 [PubMed - as supplied by publisher] (Source: The Korean Journal of Internal Medicine)
Source: The Korean Journal of Internal Medicine - July 31, 2020 Category: Internal Medicine Authors: Kiryukhin AP, Pavlov PV, Tertychnyy AS, Derinov AA, Rudenko IE Tags: Korean J Intern Med Source Type: research

A case of cardiac amyloidosis in an elderly Japanese patient with amyloidogenic transthyretin Val122Ile variant
Publication date: Available online 29 July 2020Source: Journal of Cardiology CasesAuthor(s): Ryohei Ono, Hidehisa Takahashi, Tetta Sato, Tatsuro Yamazaki, Yasuhiko Hori, Kenichi Fukushima (Source: Journal of Cardiology Cases)
Source: Journal of Cardiology Cases - July 30, 2020 Category: Cardiology Source Type: research

Amyloid A Amyloidosis After Renal Transplantation: An Important Cause of Mortality
Conclusions. AA amyloidosis is associated with higher risk of mortality after kidney transplantation. Inflammatory indicators should be monitored closely, and persistent high levels of acute-phase reactants should raise concerns about amyloid recurrence in allograft. (Source: Transplantation)
Source: Transplantation - July 30, 2020 Category: Transplant Surgery Tags: Original Clinical Science—General Source Type: research

Unveiling Outcomes in Coexisting Severe Aortic Stenosis and Transthyretin Cardiac Amyloidosis
AbstractAimsAdvances in diagnostic imaging have increased the recognition of coexisting transthyretin cardiac amyloidosis (ATTR ‐CA) and severe aortic stenosis (AS), with a reported prevalence between 8‐16%. In this prospective study, we aimed to evaluate the implications of ATTR‐CA on outcomes after TAVR.Methods and ResultsAt two academic centers, we screened patients with severe AS undergoing TAVR for ATTR ‐CA. Using Kaplan Meier analysis, we compared survival free from death and a combined endpoint of death and first heart failure (HF) hospitalization between patients with and without ATTR‐CA. Cox proportional...
Source: European Journal of Heart Failure - July 30, 2020 Category: Cardiology Authors: Hannah Rosenblum, Ahmad Masri, David L. Narotsky, Jeff Goldsmith, Nadira Hamid, Rebecca T. Hahn, Susheel Kodali, Torsten Vahl, Tamim Nazif, Omar K. Khalique, Sabahat Bokhari, Prem Soman, Jo ão L. Cavalcante, Mathew S Maurer, Adam Castaño Tags: Research Article Source Type: research

AL amyloidosis: advances in diagnosis and management.
Authors: Koh Y Abstract Light chain (AL) amyloidosis is a disease in which malignant plasma cell clones affect multiple organs including the heart and kidney. The mechanism for organ function deterioration in AL amyloidosis differs from multiple myeloma. Thus, not all agents used to treat multiple myeloma shows similar efficacy in AL amyloidosis. In AL amyloidosis, both hematologic and organ responses after treatment are important to improve the clinical outcome. Especially, improving heart function is one of the key aspects in the treatment of AL amyloidosis. With recent advances in the understanding of the pathop...
Source: Blood Research - July 29, 2020 Category: Hematology Tags: Blood Res Source Type: research

How to Identify Transthyretin Cardiac Amyloidosis at an Early Stage.
Authors: Izumiya Y, Hayashi H, Ishikawa H, Shibata A, Yoshiyama M Abstract Cardiac involvement of systemic amyloidosis is preferentially observed in patients with amyloid light chain amyloidosis or transthyretin amyloidosis (ATTR). Owing to the development of diagnostic modalities and changes in recognition by physicians, transthyretin cardiac amyloidosis (ATTR-CA) is now understood to be a more common cause of heart failure than previously thought. Recent progress in disease-modifying therapeutic interventions, such as transthyretin stabilizers, has resulted in ATTR-CA changing from an incurable disease to a curab...
Source: Internal Medicine - July 29, 2020 Category: Internal Medicine Tags: Intern Med Source Type: research

Non-invasive detection and differentiation of cardiac amyloidosis using 99mTc-pyrophosphate scintigraphy and 11C-Pittsburgh compound B PET imaging
. (Source: Amyloid: The Journal of Protein Folding Disorders)
Source: Amyloid: The Journal of Protein Folding Disorders - July 29, 2020 Category: Biochemistry Authors: Ken Takasone Nagaaki Katoh Yusuke Takahashi Ryuta Abe Naoki Ezawa Tsuneaki Yoshinaga Shin Yanagisawa Masahide Yazaki Kazuhiro Oguchi Jun Koyama Yoshiki Sekijima Source Type: research

Primary Prevention Implantable Cardioverter ‐Defibrillators in Transthyretin Cardiac Amyloidosis
ConclusionPrimary prevention ICDs do not prolong survival in patients with ATTR ‐CA and a reduced EF. Our findings are observational and will need to be validated in future prospective studies.This article is protected by copyright. All rights reserved (Source: Pacing and Clinical Electrophysiology : PACE)
Source: Pacing and Clinical Electrophysiology : PACE - July 29, 2020 Category: Cardiology Authors: Eoin Donnellan, Oussama M Wazni, Mazen Hanna, Walid Saliba, Wael Jaber, Mohamed Kanj Tags: BRIEF COMMUNICATION Source Type: research

Etanercept in the treatment of ankylosing spondylitis and nephrotic syndrome in the context of AA amyloidosis: A 48-month follow-up.
Authors: Domingos A, Vidinha J, Osório R, Jerónimo T, Ribeiro C, Mendonça C, Góis M, Neves PL PMID: 32703646 [PubMed - as supplied by publisher] (Source: Nefrologia : publicacion oficial de la Sociedad Espanola Nefrologia)
Source: Nefrologia : publicacion oficial de la Sociedad Espanola Nefrologia - July 28, 2020 Category: Urology & Nephrology Tags: Nefrologia Source Type: research

Complexities and Pitfalls in Cardiac Amyloidosis.
PMID: 32718247 [PubMed - in process] (Source: Circulation)
Source: Circulation - July 28, 2020 Category: Cardiology Authors: Cuddy SAM, Dorbala S, Falk RH Tags: Circulation Source Type: research

Types of M protein and clinicopathological profiles in patients with monoclonal gammopathy of renal significance.
CONCLUSION: The clinicopathological features were distinct in patients with different types of M protein. Integration of the types of M protein and renal pathologic findings may shed light on individual management of patients with MGRS. PMID: 32725498 [PubMed - as supplied by publisher] (Source: Journal of Nephrology)
Source: Journal of Nephrology - July 28, 2020 Category: Urology & Nephrology Authors: Liang D, Liu J, Liang S, Xu F, Cheng Z, Huang X, Zeng C, Liu Z Tags: J Nephrol Source Type: research

Clinical evaluation of infiltrative cardiomyopathies resulting in heart failure with preserved ejection fraction.
Authors: Madan N, Kalra D Abstract Heart failure with preserved ejection fraction is a very common clinical problem. Its prevalence is increasing with aging of the population. A diverse group of risk factors and etiologies comprise the HFpEF syndrome. No specific therapies have been shown to improve survival for the vast majority of HFpEF cases. Restrictive cardiomyopathies account for a significant portion of HFpEF patients and are characterized by diastolic dysfunction due to infiltration of the myocardium or ventricular hypertrophy. Many of these restrictive diseases occur in the context of myocardial infiltrati...
Source: Reviews in Cardiovascular Medicine - July 27, 2020 Category: Cardiology Tags: Rev Cardiovasc Med Source Type: research

Timing and impact of a deep response in the outcome of patients with systemic light chain (AL) amyloidosis
. (Source: Amyloid: The Journal of Protein Folding Disorders)
Source: Amyloid: The Journal of Protein Folding Disorders - July 27, 2020 Category: Biochemistry Authors: Efstathios Kastritis Despina Fotiou Foteini Theodorakakou Ioanna Dialoupi Magdalini Migkou Maria Roussou Eleni A. Karatrasoglou Maria Irini Tselegkidi Argyrios Ntalianis Nikolaos Kanellias Evangelos Eleutherakis-Papaiakovou Ioannis Ntanasis-Stathopoulos C Source Type: research

Coexistent transthyretin amyloid cardiomyopathy and monoclonal gammopathy: Diagnostic challenges and prognostic implications
AbstractEstablishing an accurate diagnosis of amyloid subtype in patients with coexistent cardiac amyloidosis and monoclonal gammopathy is crucial due to treatment and prognostic implications. Here, we discuss a case of coexistent diagnoses of transthyretin amyloid cardiomyopathy and smoldering multiple myeloma, highlighting the challenges associated with the possibility of several disease combinations and the limitations of diagnostic testing. In addition, the importance of clinical clues such as disease course and progression, patient preference, and multidisciplinary collaboration should not be discounted in the diagnos...
Source: Journal of Nuclear Cardiology - July 27, 2020 Category: Nuclear Medicine Source Type: research

Natural History and Disease Progression of Early Cardiac Amyloidosis Evaluated by Echocardiography
Since the diagnosis of cardiac amyloidosis (CA) is often delayed, echocardiographic findings are frequently indicative of advanced cardiomyopathy. We aimed to describe early echocardiographic features in patients subsequently diagnosed with CA. Pre-amyloid diagnosis echocardiographic studies were screened for structural and functional parameters and stratified according to the pathogenetic subtype (immunoglobulin light-chain (AL) or amyloid transthyretin (ATTR)). Abnormalities were defined based on published guidelines. (Source: The American Journal of Cardiology)
Source: The American Journal of Cardiology - July 27, 2020 Category: Cardiology Authors: Osnat Itzhaki Ben Zadok, Alon Eisen, Yaron Shapira, Daniel Monakier, Zaza Iakobishvili, Shmuel Schwarzenberg, Aryeh Abelow, Hadass Ofek, Shirit Kazum, Binyamin Ben-Avraham, Ashraf Hamdan, Tamir Bental, Alik Sagie, Ran Kornowski, Mordehay Vaturi Source Type: research

Quantitation of myocardial 99m Tc-HMDP uptake with new SPECT/CT cadmium zinc telluride (CZT) camera in patients with transthyretin-related cardiac amyloidosis: Ready for clinical use?
AbstractBackgroundThe aim of this study was to investigate the feasibility of assessing absolute myocardial 99mTc-HMDP uptake in patients with suspected cardiac ATTR using SUV with a whole-body CZT SPECT-CT camera (DNM670CZT).MethodsFifteen patients with suspected cardiac ATTR (Perugini ≥ 2) underwent a conventional 99mTc-HMDP planar imaging and a thoracic SPECT/CT using a DNM 670CZT. A control group consisted of 15 patients with negative scintigraphy (Perugini
Source: Journal of Nuclear Cardiology - July 26, 2020 Category: Nuclear Medicine Source Type: research

Amyloidosis inhibition, a new frontier of the protein corona
Publication date: December 2020Source: Nano Today, Volume 35Author(s): Pengyu Chen, Feng Ding, Rong Cai, Ibrahim Javed, Wen Yang, Zhenzhen Zhang, Yuhuan Li, Thomas P. Davis, Pu Chun Ke, Chunying Chen (Source: Nano Today)
Source: Nano Today - July 24, 2020 Category: Nanotechnology Source Type: research

Plasma Cell Myeloma Presenting With Amyloid-Laden Crystal-Negative Histiocytosis.
CONCLUSIONS: We describe a case of crystal-negative histiocytosis associated with PCM. CSH is a rare disorder associated with paraprotein-producing conditions in which immunoglobulins aggregate as intracellular crystals in the lysosomes of organ-specific phagocytic macrophages. Light chain tropism in PCM can also lead to the development of amyloid deposition in organs and, in rare cases, is associated with light chain aggregation as intracellular crystals in macrophages. PMID: 32705137 [PubMed - as supplied by publisher] (Source: American Journal of Clinical Pathology)
Source: American Journal of Clinical Pathology - July 24, 2020 Category: Pathology Authors: Braunstein MJ, Petrova-Drus K, Rosenbaum CA, Jayabalan DS, Rossi AC, Salvatore S, Rech K, Pearse RN, Hassane DC, Postley J, Jhanwar YS, Geyer JT, Niesvizky R Tags: Am J Clin Pathol Source Type: research

Near tetrapoloid karyotype with translocation t(11;14) in a Moroccan patient with amyloid light-chain amyloidosis and multiple myeloma
Publication date: Available online 22 July 2020Source: Leukemia Research ReportsAuthor(s): Hasna Hamdaoui, Abdelhafid Natiq, Oumaima Benlarroubia, Thomas Liehr, Hind Dehbi, Latifa Loukhmas, Fatima Chegdani (Source: Leukemia Research Reports)
Source: Leukemia Research Reports - July 23, 2020 Category: Hematology Source Type: research

Clinical Analysis of Cardiac Involvement in 53 Patients With Multiple Myeloma Coexistent With Light Chain Amyloidosis
Publication date: August 2020Source: Clinical Lymphoma Myeloma and Leukemia, Volume 20, Issue 8Author(s): Yuanyuan Yu, Zhongxia Huang, Wanli Hu, Xin Li, Man Shen, Jiajia Zhang, Ran Tang, Shilun Chen, Wenming Chen (Source: Clinical Lymphoma Myeloma and Leukemia)
Source: Clinical Lymphoma Myeloma and Leukemia - July 23, 2020 Category: Cancer & Oncology Source Type: research

A study from The Mayo Clinic evaluated long-term outcomes of kidney transplantation in patients with immunoglobulin light chain amyloidosis.
Longer survival using modern therapies has increased the number of patients with immunoglobulin light-chain amyloidosis receiving kidney transplantation. We evaluated 60 patients with immunoglobulin light chain amyloidosis who underwent kidney transplantation based on their hematologic response for outcomes of death, graft failure, and complications. Patient hematologic responses (light-chain in blood or urine) prior to kidney transplantation were three patients had no response, five had a partial response, six had a very good partial response, 37 had a complete response, and nine were treatment-naive patients (never treat...
Source: Kidney International - July 23, 2020 Category: Urology & Nephrology Authors: Cihan Heybeli, Andrew Bentall, Jiqiu Wen, Mariam Priya Alexander, Francis K. Buadi, Fernando Cosio, Patrick G. Dean, Angela Dispenzieri, David Dingli, Mireille El Ters, Morie A. Gertz, Amer Hatem, Prashant Kapoor, Hasan Khamash, Taxiarchis Kourelis, Shaji Tags: clinical investigation Source Type: research

Digoxin Use in Cardiac Amyloidosis
We present our experience with digoxin use in patients with CA and report the event rate of suspected digoxin-related arrhythmias and toxicity. This is a retrospective study of patients with CA seen at our institution between November 1995 and October 2018. Patients were screened for a history of ≥7 days of continuous digoxin use and stratified based on amyloid precursor protein – transthyretin (ATTR) and immunoglobulin light chain (AL). (Source: The American Journal of Cardiology)
Source: The American Journal of Cardiology - July 23, 2020 Category: Cardiology Authors: Joseph P. Donnelly, Brett W. Sperry, Andrej Gabrovsek, Asad Ikram, W.H. Wilson Tang, Jerry Estep, Mazen Hanna Source Type: research

Successful Treatment of Rheumatoid Arthritis-Associated Renal AA Amyloidosis with Tocilizumab.
Authors: Kovács A, Cserenyecz A, Baksay B, Kemény É, Szekanecz Z PMID: 32692504 [PubMed - as supplied by publisher] (Source: The Israel Medical Association Journal)
Source: The Israel Medical Association Journal - July 22, 2020 Category: General Medicine Tags: Isr Med Assoc J Source Type: research

Left Ventricular Pressure Strain –Derived Myocardial Work at Rest and during Exercise in Patients with Cardiac Amyloidosis
In a recent paper, Clemmensen et  al.1 have applied left ventricular pressure strain–derived myocardial work (LVMW) indices in a cohort of cardiac amyloidosis (CA) patients and healthy controls, both at rest and during semisupine exercise.1 The authors report that CA patients have significantly lower rest longitudinal strain (LS ) along with lower LVMW and myocardial work efficiency compared with controls. In addition, during exercise, they present a lower improvement of LVMW mainly driven by apical regions and a significantly reduced energy exploitation (based on LS evaluation). (Source: Journal of the American...
Source: Journal of the American Society of Echocardiography - July 22, 2020 Category: Cardiology Authors: Efstathios D. Pagourelias, Vassilios P. Vassilikos, Jens-Uwe Voigt Tags: Letters to the Editor Source Type: research

ATTRv amyloidosis Italian Registry: clinical and epidemiological data
. (Source: Amyloid: The Journal of Protein Folding Disorders)
Source: Amyloid: The Journal of Protein Folding Disorders - July 22, 2020 Category: Biochemistry Authors: Massimo Russo Laura Obici Ilaria Bartolomei Francesco Cappelli Marco Luigetti Silvia Fenu Tiziana Cavallaro Maria Grazia Chiappini Chiara Gemelli Luca Guglielmo Pradotto Fiore Manganelli Luca Leonardi Filomena My Simone Sampaolo Chiara Briani Luca Gentile Source Type: research

Histopathological Study of Benign Tumours of the Larynx: A Descriptive Study in Coastal Karnataka
AbstractA laryngoscopically benign lesion with minimal hoarseness may turn out to be mimickers of malignancy or malignancy itself. Histopathological study is mandatory in such cases to rule out malignancy. A descriptive study of histopathological and laryngoscopic findings of benign laryngeal lesions were studies over a period of 4.5  years. Among the thirty-six patients studied, the mean age of the patients was 42 years. The most common presenting complaint was hoarseness of voice seen in 87.5% of cases. Twelve cases of vocal cord polyps were diagnosed based on clinical, laryngoscopical and histopathological fea...
Source: Indian Journal of Otolaryngology and Head and Neck Surgery - July 21, 2020 Category: ENT & OMF Source Type: research

Wild-type transthyretin cardiac amyloidosis: When is a rare disease no longer a rare disease?
(Source: Journal of Nuclear Cardiology)
Source: Journal of Nuclear Cardiology - July 19, 2020 Category: Nuclear Medicine Source Type: research

Colonic Amyloidosis Concomitant with Systemic Lupus Erythematosus.
Authors: Ikuta K, Hatachi S, Fujimori T, Shio S PMID: 32669495 [PubMed - as supplied by publisher] (Source: Internal Medicine)
Source: Internal Medicine - July 18, 2020 Category: Internal Medicine Tags: Intern Med Source Type: research

Towards a novel target therapy for renal diseases related to plasma cell dyscrasias: The example of AL amyloidosis
Publication date: Available online 12 July 2020Source: Autoimmunity ReviewsAuthor(s): Dario Roccatello, Roberta Fenoglio, Simone Baldovino, Carla Naretto, Michela Ferro, Antonella Barreca, Daniela Rossi, Savino Sciascia (Source: Autoimmunity Reviews)
Source: Autoimmunity Reviews - July 17, 2020 Category: Allergy & Immunology Source Type: research

RF - Diagnostic biopsy for amyloidosis: subcutaneous fat or minor salivary gland?
Publication date: Available online 15 July 2020Source: Actas Dermo-Sifiliográficas (English Edition)Author(s): T. Ródenas-Herranz, L. Linares-Gonzalez, R. Ruiz-Villaverde (Source: Actas Dermo-Sifiliograficas)
Source: Actas Dermo-Sifiliograficas - July 16, 2020 Category: Dermatology Source Type: research

Tc-99m-PYP imaging for cardiac amyloidosis: Defining the best protocol before the flood gates burst
(Source: Journal of Nuclear Cardiology)
Source: Journal of Nuclear Cardiology - July 16, 2020 Category: Nuclear Medicine Source Type: research

Worldwide Perspectives of Amyloidosis
Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - July 15, 2020 Category: Hematology Source Type: research

Doxycycline decreases amyloidogenic light chain-induced autophagy in isolated primary cardiac myocytes
Immunoglobulin light chain (AL) cardiac amyloidosis is characterized by extracellular deposition of amyloid fibrils in the heart and is potentially fatal. Untreated, it manifests clinically as heart failure with a precipitous decline and a median survival of (Source: International Journal of Cardiology)
Source: International Journal of Cardiology - July 15, 2020 Category: Cardiology Authors: Mar ía Valero-Muñoz, Richard M. Wilson, Rosa Bretón-Romero, Dominique Croteau, David C. Seldin, Flora Sam Tags: Short communication Source Type: research

Worldwide Perspectives of Amyloidosis.
PMID: 32668431 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - July 15, 2020 Category: Hematology Authors: Vaxman I, Gertz MA Tags: Acta Haematol Source Type: research

Diagnostic performance of PET for detection of cardiac amyloidosis: A systematic review and meta-analysis.
CONCLUSION: Amyloid PET has a high sensitivity and specificity for the detection of CA. However, F-18 NaF PET showed relatively low sensitivity with high specificity. At present, the literature regarding the use of amyloid and F-18 NaF PET for diagnosis of CA is still limited; thus, further large multicenter studies would be necessary to substantiate the diagnostic accuracy of amyloid and F-18 NaF PET for detection of CA. PMID: 32682627 [PubMed - as supplied by publisher] (Source: Journal of Cardiology)
Source: Journal of Cardiology - July 15, 2020 Category: Cardiology Authors: Kim SH, Kim YS, Kim SJ Tags: J Cardiol Source Type: research

Late Acute Cellular Rejection After Anakinra Treatment in a Kidney Transplant Patient, Is It a Coincidence?
Authors: Yeter HH, Yetkin N, Akcay O, Derici U, Arinsoy T Abstract Familial mediterranean fever (FMF) is an autosomal recessive auto-inflammatory disorder, which could lead to secondary (AA) amyloidosis. Anakinra is an IL-1 receptor blocker and a treatment option for patients with FMF. There is no reported rejection episode associated with the use of Anakinra in the literature. A fortynine years old woman with a history of kidney transplantation is described here. Anakinra was initiated in the patients whose FMF attacks were exacerbated, and the inflammation could not be controlled under the colchicine treatment. A...
Source: Iranian Journal of Kidney Diseases - July 14, 2020 Category: Urology & Nephrology Tags: Iran J Kidney Dis Source Type: research

Protective Effects of PACAP in Peripheral Organs
Pituitary adenylate cyclase activating polypeptide (PACAP) is a neuropeptide widely distributed in the nervous system, where it exerts strong neuroprotective effects. PACAP is also expressed in peripheral organs but its peripheral protective effects have not been summarized so far. Therefore, the aim of the present paper is to review the existing literature regarding the cytoprotective effects of PACAP in non-neuronal cell types, peripheral tissues, and organs. Among others, PACAP has widespread expression in the digestive system, where it shows protective effects in various intestinal pathologies, such as duodenal ulcer, ...
Source: Frontiers in Endocrinology - July 14, 2020 Category: Endocrinology Source Type: research

Intramural Coronary Light Chain Amyloidosis and Left Ventricular Assist Device Implantation
Light chain amyloidosis (AL) with cardiac involvement is largely due to interstitial amyloid infiltration resulting in a thickened left ventricle (LV) and restrictive hemodynamics. However, amyloid protein can be deposited mainly in the coronary microvasculature, with resultant ischemia and cardiogenic shock (1, 2). Left ventricular assist devices (LVAD) are not often considered in patients with AL cardiac amyloidosis due to a small LV size and ongoing chemotherapy. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - July 13, 2020 Category: Transplant Surgery Authors: Forum Kamdar, Carmela D. Tan, E. Rene Rodriguez, Nader Moazami, Mazen Hanna Tags: Case Anecdote Source Type: research

Monoclonal gammopathy of undetermined significance (MGUS): where is the hidden danger? Definition and work-up
SummaryMonoclonal gammopathy of undetermined significance (MGUS) is a  premalignant hematological condition arising from B‑cells, characterized by the presence of monoclonal immunoglobulin production, also known as paraprotein. It is found in up to 3% of individuals over the age of 50 years. The overall progression rate is low at around 1% per year, with most pati ents progressing to multiple myeloma (MM). Other diseases that may arise from MGUS include non-Hodgkin lymphomas, Waldenström macroglobulinemia, amyloid light-chain (AL) amyloidosis, POEMS (polyneuropathy, organomegaly, endocrinopathy, M‑protei...
Source: Memo - Magazine of European Medical Oncology - July 13, 2020 Category: Cancer & Oncology Source Type: research

Cardiac amyloidosis CT sign
(Source: Clinical Research in Cardiology)
Source: Clinical Research in Cardiology - July 13, 2020 Category: Cardiology Source Type: research

Intramural coronary light-chain amyloidosis and left ventricular assist device implantation
Light-chain (AL) amyloidosis with cardiac involvement is largely because of interstitial amyloid infiltration resulting in a thickened left ventricle (LV) and restrictive hemodynamics. However, amyloid protein can be deposited mainly in the coronary microvasculature, with resultant ischemia and cardiogenic shock.1,2 Left ventricular assist devices (LVADs) are not often considered in patients with AL cardiac amyloidosis because of a small LV size and ongoing chemotherapy. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - July 13, 2020 Category: Transplant Surgery Authors: Forum Kamdar, Carmela D. Tan, E. Rene Rodriguez, Nader Moazami, Mazen Hanna Tags: CASE ANECDOTES, COMMENTS AND OPINIONS Source Type: research

Towards a novel target therapy for renal diseases related to a plasma cell dyscrasia: The example of AL amyloidosis
Publication date: Available online 12 July 2020Source: Autoimmunity ReviewsAuthor(s): Dario Roccatello, Roberta Fenoglio, Simone Baldovino, Carla Naretto, Michela Ferro, Antonella Barreca, Daniela Rossi, Savino Sciascia (Source: Autoimmunity Reviews)
Source: Autoimmunity Reviews - July 12, 2020 Category: Allergy & Immunology Source Type: research

Accumulation of amyloid beta in human glioblastomas.
cute;guez R, Inyushin M Abstract Many cancer types are intrinsically associated with specific types of amyloidosis, in which amyloidis accumulated locally inside tumors or systemically. Usually, this condition relates to the hyper-production of specific amylogenic proteins. Recently, we found that the accumulation of amyloid beta (Aβ) peptide immunofluorescence is linked to glioma cells in mouse tumors. Here we report that amyloid-specific histochemical dyes reveal amyloid accumulation in all human glioma samples. Application of two different antibodies against Aβ peptide (a polyclonal antibody against h...
Source: Clinical and Developmental Immunology - July 12, 2020 Category: Allergy & Immunology Authors: Zayas-Santiago A, Díaz-García A, Nuñez-Rodríguez R, Inyushin M Tags: Clin Exp Immunol Source Type: research

Organ Transplantation in Hereditary Fibrinogen A α-Chain Amyloidosis: A Case Series of French Patients
Publication date: Available online 10 July 2020Source: American Journal of Kidney DiseasesAuthor(s): Lara Meyer, Marc Ulrich, Didier Ducloux, Valérie Garrigue, Cécile Vigneau, Dominique Nochy, Guillaume Bobrie, Sophie Ferlicot, Magalie Colombat, Jean-Jacques Boffa, Karine Clabault, Jeannette Mansour, Christiane Mousson, Raymond Azar, Jean-Louis Bacri, Antoine Dürrbach, Christian Duvic, Khalil El Karoui, Maxime Hoffmann, Arnaud Lionet (Source: American Journal of Kidney Diseases)
Source: American Journal of Kidney Diseases - July 11, 2020 Category: Urology & Nephrology Source Type: research