keyword
https://read.qxmd.com/read/38493325/amyloid-typing-in-cardiac-amyloidosis-using-western-blotting
#1
JOURNAL ARTICLE
Batia Kaplan, Rivka Goldis, Tamar Ziv, Amir Dori, Hila Magen, Amos J Simon, Alexander Volkov, Elad Maor, Michael Arad
BACKGROUND: Cardiac amyloidosis (CA) is characterized by the extracellular deposition of misfolded protein in the heart. Precise identification of the amyloid type is often challenging, but critical, since the treatment and prognosis depend on the disease form and the type of deposited amyloid. Coexistence of clinical conditions such as old age, monoclonal gammopathy, chronic inflammation, or peripheral neuropathy in a patient with cardiomyopathy creates a differential diagnosis between the major types of CA: amyloidosis light chains (AL), amyloidosis transthyretin (ATTR) and amyloidosis A (AA)...
March 2024: Israel Medical Association Journal: IMAJ
https://read.qxmd.com/read/38484868/hereditary-transthyretin-amyloidosis-impact-of-classic-and-new-treatments-on-kidney-function
#2
REVIEW
Evelyn Meléndrez-Balcázar, Karla Aranda-Vela, Alberto Cervantes-Hernández, Samuel López-Cureño
Hereditary transthyretin amyloidosis (ATTRv) is a rare, progressive, and life-threatening disease caused by misfolded transthyretin (TTR) proteins that aggregate as abnormal amyloid fibrils and accumulate throughout the body. The kidney is one of the main organs affected in amyloid light chain (AL) amyloidosis and ATTRv amyloidosis. The most common clinical presentation is proteinuria, which consists mainly of albumin; this is the first step in the natural history of ATTRv nephropathy. Not all TTR mutations are equal in terms of ATTRv kidney involvement...
March 12, 2024: American Journal of Kidney Diseases
https://read.qxmd.com/read/38453615/healthcare-resource-utilization-and-cost-of-illness-in-systemic-light-chain-al-amyloidosis-in-europe-results-from-the-real-world-retrospective-emn23-study
#3
JOURNAL ARTICLE
Arnaud Jaccard, Frank Bridoux, Wilfried Roeloffzen, Monique C Minnema, Rui Bergantim, Roman Hájek, Cristina João, M Teresa Cibeira, Giovanni Palladini, Stefan Schönland, Giampaolo Merlini, Paolo Milani, Meletios A Dimopoulos, Sriram Ravichandran, Ute Hegenbart, Hermine Agis, Blanca Gros, Aisha Asra, Valeria Magarotto, Giorgos Cheliotis, Giorgos Psarros, Pieter Sonneveld, Ashutosh Wechalekar, Efstathios Kastritis
OBJECTIVES: To report healthcare resource utilization (HCRU) and safety outcomes in systemic light chain (AL) amyloidosis from the EMN23 study. MATERIALS AND METHODS: The retrospective, observational, multinational EMN23 study included 4,480 patients initiating first-line treatment for AL amyloidosis in 2004-2018 and assessed, among other objectives, HCRU and safety outcomes. HCRU included hospitalizations, examinations, and dialysis; safety included serious adverse events (SAEs) and adverse events of special interest (AESIs)...
February 1, 2024: Clinical Lymphoma, Myeloma & Leukemia
https://read.qxmd.com/read/38437698/in-adults-with-attr-cardiac-amyloidosis-patisiran-reduced-decline-in-functional-capacity-at-12-mo
#4
JOURNAL ARTICLE
Ahmad Masri, Harriette G C Van Spall
Maurer MS, Kale P, Fontana M, et al; APOLLO-B Trial Investigators. Patisiran treatment in patients with transthyretin cardiac amyloidosis. N Engl J Med. 2023;389:1553-1565. 37888916.
March 5, 2024: Annals of Internal Medicine
https://read.qxmd.com/read/38432865/value-of-nuclide-scintigraphy-in-the-diagnosis-and-prognosis-of-cardiac-amyloidosis
#5
JOURNAL ARTICLE
Qu Mo, Zilong Deng, Yi Xiao, Caiguang Liu, Min Zhao
Amyloidosis is a local or systemic disease caused by the deposition of misfolded proteins outside the cell, with rapid progression, and dire prognosis. Common types of cardiac amyloidosis are monoclonal immunoglobulin light chain amyloidosis (AL-CA) and transthyretin cardiac amyloidosis (ATTR-CA). Nuclear medicine examinations can be accurate, rapid, and non-invasive to help diagnose diseases and can effectively predict the prognosis of patients with CA. Technetium (99 Tcm )-labeled bisphosphonate imaging has been included in the consensus of experts and has become the first-line imaging method for the diagnosis of ATTR-CA...
November 28, 2023: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://read.qxmd.com/read/38414681/amyloid-light-chain-al-amyloidosis-of-the-trachea-associated-with-an-indolent-b-cell-neoplasm
#6
Anup Kumar Trikannad, Asis Shrestha, Sruthi Vellanki, Hira I Cheema, Tanvi H Patel, Ramya Bachu, Shobhit Sharma, Susanne K Jeffus, Sharmilan Thanendrarajan
We report the case of a 66-year-old woman who was diagnosed with localized tracheal amyloid light-chain (AL) amyloidosis caused by an underlying B-cell neoplasm. The diagnosis was confirmed through subsequent bronchoscopy and biopsies; however, she experienced a challenging episode of hypoxic respiratory failure that required intervention. Repeat bronchoscopies showed persistent subglottic stenosis and tracheobronchomalacia, which led to tracheal debulking surgery and additional interventions. The patient's treatment began with rituximab, zanubrutinib, and dexamethasone with outpatient follow-up...
January 2024: Curēus
https://read.qxmd.com/read/38399458/2023-fda-tides-peptides-and-oligonucleotides-harvest
#7
REVIEW
Danah Al Shaer, Othman Al Musaimi, Fernando Albericio, Beatriz G de la Torre
A total of nine TIDES (pepTIDES and oligonucleoTIDES) were approved by the FDA during 2023. The four approved oligonucleotides are indicated for various types of disorders, including amyotrophic lateral sclerosis, geographic atrophy, primary hyperoxaluria type 1, and polyneuropathy of hereditary transthyretin-mediated amyloidosis. All oligonucleotides show chemically modified structures to enhance their stability and therapeutic effectiveness as antisense or aptamer oligomers. Some of them demonstrate various types of conjugation to driving ligands...
February 13, 2024: Pharmaceuticals
https://read.qxmd.com/read/38365569/prognostic-value-of-native-t1-and-extracellular-volume-in-patients-with-immunoglubin-light-chain-amyloidosis
#8
JOURNAL ARTICLE
Yumeng Liu, Lingjie Wang, Jingfen Zhu, Meng Chen, Mo Zhu, Yingyu Dai, Chunhong Hu
BACKGROUND: Cardiac involvement in patients with immunoglubin light-chain amyloidosis (AL) is a major determinant of treatment choice and prognosis, and early identification of high-risk patients can initiate intensive treatment strategies to achieve better survival. This study aimed to investigate the prognostic value of native T1 and ECV in patients with AL-cardiac amyloidosis (CA). METHODS: A total of 38 patients (mean age 59 ± 11 years) with AL diagnosed histopathologically from July 2017 to October 2021 were collected consecutively...
February 16, 2024: BMC Cardiovascular Disorders
https://read.qxmd.com/read/38352852/monoclonal-gammopathy-of-renal-significance-an-atypical-presentation-of-waldenstr%C3%A3-m-s-disease
#9
Pablo Rodríguez-Doyágüez, Motornaya-Morozova, Patricia Martínez-Miguel, Carolina Castillo-Torres, Óscar Toldos-González, Juan José Gil-Fernández
Waldenström's disease is a rare lymphoproliferative syndrome in the bone marrow and sometimes in lymphoid organs which secretes high amounts of monoclonal immunoglobulin M into serum. It can remain indolent for years and rarely affects the kidney, with intraglomerular rather than intratubular damage being predominant, in contrast to multiple myeloma. Different studies identified AL amyloidosis as the most frequent renal lesion, followed by cryoglobulinemic glomerulonephritis. Signs and symptoms may be unspecific, as well as renal manifestations, so collaboration between nephrologists, hematologists, and pathologists is crucial to establish the role of paraprotein in the development of renal damage...
2024: Clinical Nephrology. Case Studies
https://read.qxmd.com/read/38349861/the-role-and-efficacy-of-serial-electrobrasion-for-the-treatment-of-recalcitrant-lichenoid-amyloidosis-and-lichen-simplex-chronicus
#10
JOURNAL ARTICLE
Ilana D Breen, Bridget Myers, Zachary Kwapnoski, Theresa Canavan
No abstract text is available yet for this article.
February 13, 2024: Dermatologic Surgery: Official Publication for American Society for Dermatologic Surgery [et Al.]
https://read.qxmd.com/read/38349172/complexities-of-coexisting-cardiac-amyloidosis-and-coronary-artery-disease-a-contemporary-review-of-diagnostic-and-treatment-approaches
#11
JOURNAL ARTICLE
Yahui Lu, Geng Bai, Weiding Wang, Guangping Li
Cardiac amyloidosis (CA) represents an emerging challenge in cardiovascular medicine, with notable clinical overlaps and diagnostic complexities when coexisting with coronary artery disease (CAD). This integrative review navigates the intricate terrain of CA and CAD, elucidating epidemiology, clinical presentations, and diagnostic considerations. Examining both immunoglobulin light chain amyloidosis (AL) and transthyretin amyloidosis, we underscore their shared demographic associations, diagnostic intricacies, and potential diagnostic confounders with CAD...
February 13, 2024: Cardiology in Review
https://read.qxmd.com/read/38272520/primary-cerebral-immunoglobulin-light-chain-amyloidoma-in-a-patient-with-multiple-sclerosis
#12
JOURNAL ARTICLE
Marissa J M Traets, Krisna Chuwonpad, Roos J Leguit, Stephan T F M Frequin, Monique C Minnema
A man in his 60s, known with multiple sclerosis, presented with seizures and paresis of the left arm and leg. Brain imaging showed a white matter lesion, right parietal, which was progressive over the last 6 years and not typical for multiple sclerosis. Brain biopsy showed a B-cell infiltrate with IgA lambda monotypic plasma cell differentiation and amyloid deposits, typed as lambda immunoglobulin light chain (AL). Bone marrow biopsy and PET/CT ruled out a systemic lymphoma. Extended history taking, blood and urine testing (including cardiac biomarkers) identified no evidence of systemic amyloidosis-induced organ dysfunction...
January 24, 2024: BMJ Case Reports
https://read.qxmd.com/read/38248088/best-practices-in-nuclear-imaging-for-the-diagnosis-of-transthyretin-amyloid-cardiomyopathy-attr-cm-in-ksa-the-eagle-eyes-of-local-experts
#13
REVIEW
Abdullah Alqarni, Ahmed Aljizeeri, Aquib Mohammadidrees Bakhsh, Hossam Ahmed Maher El-Zeftawy, Hussein R Farghaly, Mukhtar Ahmed M Alqadhi, Mushref Algarni, Zain Mohammed Asiri, Ahmed Osman, Haya Haddadin, Islam Alayary, Mouaz H Al-Mallah
Transthyretin amyloid cardiomyopathy (ATTR-CM) is a complex and serious form of heart failure caused by the accumulation of transthyretin amyloid protein in the heart muscle. Variable symptoms of ATTR-CM can lead to a delayed diagnosis. Recognizing the diagnostic indicators is crucial to promptly detect this condition. A targeted literature review was conducted to examine the latest international consensus recommendations on a comprehensive diagnosis of ATTR-CM. Additionally, a panel consisting of nuclear medicine expert consultants ( n = 10) and nuclear imaging technicians ( n = 2) convened virtually from the Kingdom of Saudi Arabia (KSA) to formulate best practices for ATTR-CM diagnosis...
January 18, 2024: Diagnostics
https://read.qxmd.com/read/38245144/a-case-of-disappearing-amyloid-on-technetium-pyrophosphate-scan
#14
JOURNAL ARTICLE
Andy Wang, Uzair Mahmood, Xiaoyu Tang, Diwakar Jain, Stephen Pan
Technetium-99mm pyrophosphate (Tc-PYP) scintigraphy is a highly accurate non-invasive method for the diagnosis of transthyretin (ATTR) cardiac amyloidosis. Prognosis for this disease is improved following treatment with the transthyretin (TTR) stabilizer tafamidis. Although tafamidis slows disease progression, its effects on myocardial amyloid and Tc-PYP uptake remain unclear. We present a patient with ATTR cardiac amyloidosis who had a strongly positive initial Tc-PYP scan, with a dramatic decrease in Tc-PYP uptake on repeat scan after 3 years of tafamidis treatment...
October 2023: Journal of Nuclear Cardiology
https://read.qxmd.com/read/38229422/successful-treatment-of-systemic-light-chain-amyloidosis-with-liver-involvement-using-low-frequency-daratumumab-a-case-report
#15
JOURNAL ARTICLE
Xinyi Meng, Jingsong He, Fei Cheng, Hui Yan, Chunting Zhu, Xing Guo, Yi Li, Zhen Cai, Donghua He
BACKGROUND Systemic light chain (AL) amyloidosis is a disease characterized by the deposition of amyloid fibrils throughout tissues due to the production of misfolded immunoglobulin light chains by clonally expanded populations of CD38+ plasma cells. Some patients can have liver involvement, which typically presents with nonspecific symptoms. Daratumumab, a human CD38-targeting antibody, has shown efficacy in improving hematological parameters and organ function in patients with AL amyloidosis. Low-frequency daratumumab can reduce financial burden, but whether it is effective for patients with liver involvement has not been reported...
January 17, 2024: American Journal of Case Reports
https://read.qxmd.com/read/38204273/cardiac-toxicity-in-the-treatment-of-light-chain-amyloidosis-systematic-review-of-clinical-studies
#16
JOURNAL ARTICLE
Jairo Javier Jattin-Balcázar, Paula Andrea Quiroga-Ramírez
BACKGROUND: Light chain amyloidosis (AL) is a progressive and a fatal disease that primarily affects cardiac tissue. Although the current approach to anti-amyloidosis treatments has managed to reduce amyloidosis morbimortality, the dynamics of cardiac adverse events are unknown. OBJECTIVE: to provide evidence about reported cardiac toxicity during treatment of AL amyloidosis through a systematic review of the literature. METHODS: A search was performed for registered clinical trials on ClinicalTrials...
January 9, 2024: Current Drug Safety
https://read.qxmd.com/read/38202290/safety-and-tolerability-of-sglt2-inhibitors-in-cardiac-amyloidosis-a-clinical-feasibility-study
#17
JOURNAL ARTICLE
Maximilian J Steinhardt, Vladimir Cejka, Mengmeng Chen, Sabrina Bäuerlein, Julia Schäfer, Ali Adrah, Sandra M Ihne-Schubert, Aikaterini Papagianni, K Martin Kortüm, Caroline Morbach, Stefan Störk
Sodium-glucose transport protein 2 inhibitors (SGLT2i) slow the progression of renal dysfunction and improve the prognosis of patients with heart failure. Amyloidosis constitutes an important subgroup for which evidence is lacking. Amyloidotic fibrils originating from misfolded transthyretin and light chains are the causal agents in ATTR and AL amyloidosis. In these most frequent subtypes, cardiac involvement is the most common organ manifestation. Because cardiac and renal function frequently deteriorate over time, even under best available treatment, SGLT2i emerge as a promising treatment option due to their reno- and cardioprotective properties...
January 4, 2024: Journal of Clinical Medicine
https://read.qxmd.com/read/38202228/cardiac-amyloidosis-and-valvular-heart-disease
#18
REVIEW
Franz Duca, Christina Kronberger, Robin Willixhofer, Philipp E Bartko, Jutta Bergler-Klein, Christian Nitsche
Growing interest has accrued in the co-existence of cardiac amyloidosis and valvular heart disease. Amyloid infiltration from either transthyretin (ATTR) or of light chain (AL) origin may affect any structure of the heart, including the valves. The recent literature has mainly focused on aortic stenosis and cardiac amyloidosis, improving our understanding of the epidemiology, diagnosis, treatment and prognosis of this dual pathology. Despite being of high clinical relevance, data on mitral/tricuspid regurgitation and cardiac amyloidosis are rather scarce and mostly limited to case reports and small cases series...
December 30, 2023: Journal of Clinical Medicine
https://read.qxmd.com/read/38194690/complete-responses-in-al-amyloidosis-are-unequal-the-impact-of-free-light-chain-mass-spectrometry-in-al-amyloidosis
#19
JOURNAL ARTICLE
Joshua Bomsztyk, Sriram Ravichandran, Hannah Victoria Giles, Nicola Jane Wright, Oscar Berlanga, Jahanzaib Khwaja, Shameem Mahmood, Brendan Wisniowski, Oliver C Cohen, Darren Foard, Janet Gilbertson, Muhammad Umaid Rauf, Neasa Starr, Ana Martinez-Naharro, Lucia Venneri, Carol Whelan, Marianna Fontana, Philip N Hawkins, Julian D Gillmore, Helen J Lachmann, Stephen Harding, Guy Pratt, Ashutosh D Wechalekar
Amyloidogenic serum free light chains (sFLC) drive disease progression in AL amyloidosis. Matrix-assisted laser desorption/ionization-time of flight mass spectrometry-based free light chain assay (FLC-MS) has greater sensitivity than conventional sFLC assays allowing for the detection of serological residual disease. We report the utility of FLC-MS in a large series of patients with AL amyloidosis assessing the impact of FLC-MS negativity after treatment on overall survival and organ response rates. Serum samples were analysed using FLC-MS at diagnosis and at 6-, and 12months post-treatment...
January 9, 2024: Blood
https://read.qxmd.com/read/38148411/risk-factors-treatments-and-outcomes-of-patients-with-light-chain-amyloidosis-who-relapse-after-autologous-stem-cell-transplantation
#20
JOURNAL ARTICLE
Yuanyuan Zhang, Jinzhou Guo, Wencui Chen, Liang Zhao, Xianghua Huang
Relapse after ASCT is an important factor affecting the long-term prognosis of patients with AL amyloidosis. However, the risk factors of relapse are unknown and there are limited studies on treatment outcomes of these patients. We retrospectively reviewed 170 patients with AL amyloidosis who underwent ASCT between 2010 and 2021. Seventy-six patients confirmed as relapse and the median time from ASCT to relapse was 39 months. On multivariate analysis of variables before and after ASCT, lambda restricted, dFLC >30 mg/L pre ASCT, reduced dose melphalan and dFLC >10 mg/L at 6 months after ASCT were independent risk factors for relapse, and achieving CR after induction therapy and renal response after ASCT were protective factors...
December 26, 2023: Bone Marrow Transplantation
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