- Cystic lung disease. [Journal Article]Radiologia (Engl Ed). 2022 Dec; 64 Suppl 3:265-276.R
- The term cystic lung disease encompasses a heterogeneous group of entities characterised by round lung lesions that correspond to cysts with fine walls, which usually contain air. The differential diagnosis of these lesions can be challenging, requiring both clinical and radiological perspectives. Entities such as pulmonary emphysema and cystic bronchiectasis can simulate cystic disease. High-res…
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- [ANMCO Position paper: Amyloidosis for the clinical cardiologist. A "clinical primer" from the ANMCO Rare Disease Working Group]. [Journal Article]G Ital Cardiol (Rome). 2023 Feb; 24(2):127-135.GI
- Cardiac amyloidosis, in the three forms of immunoglobulin light chain (AL), transthyretin (ATTR) wild type (ATTRwt) and mutated (ATTRv) amyloidosis, is an increasingly known and recognized disease in the cardiovascular setting. The first stage of the patient's journey is the clinical suspicion of the disease, which is placed, in presence of a hypertrophic phenotype, by the identification of red f…
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- [Rationale and significance of the Italian Network for Cardiac Amyloidosis]. [Journal Article]G Ital Cardiol (Rome). 2023 Feb; 24(2):93-98.GI
- The perspective on amyloidosis has changed deeply over the last 10 years following major advances in diagnosis and treatment options, especially in cardiac amyloidosis. This intrinsically heterogeneous disease exposes to the risk of fragmentation of knowledge and requires the interaction among experts of different specialties and subspecialties. Suspicion of disease, timely recognition and confir…
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- A Comprehensive Review of Dilated Cardiomyopathy in Pre-clinical Animal Models in Addition to Herbal Treatment Options and Multi-modality Imaging Strategies. [Journal Article]Cardiovasc Hematol Disord Drug Targets. 2023 Jan 23 [Online ahead of print]CH
- Dilated cardiomyopathy (DCM) is distinguished by ventricular chamber expansion, systolic dysfunction, and normal left ventricular (LV) wall thickness, and is mainly caused due to genetic or environmental factors; however, its aetiology is undetermined in the majority of patients. The focus of this work is on pathogenesis, small animal models, as well as the herbal medicinal approach, and the most…
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- Immune-mediated platelet depletion augments Alzheimer's disease neuropathological hallmarks in APP-PS1 mice. [Journal Article]Aging (Albany NY). 2023 Feb 01; 15A
- In Alzheimer's disease (AD), platelets become dysfunctional and might contribute to amyloid beta deposition. Here, we depleted platelets in one-year-old APP Swedish PS1 dE9 (APP-PS1) transgenic mice for five days, using intraperitoneal injections of an anti-CD42b antibody, and assessed changes in cerebral amyloidosis, plaque-associated neuritic dystrophy and gliosis. In APP-PS1 female mice, plate…
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- First Norwegian case of hereditary ATTR amyloidosis with a novel transthyretin variant. [Journal Article]Scand Cardiovasc J. 2023 Dec; 57(1):2174269.SC
- An earlier healthy 64-year-old man with previous surgery for bilateral carpal tunnel syndrome (CTS) in his 50s, presented with dyspnoea on exertion. Cardiac amyloidosis was suspected due to "red flag" signs and symptoms. Further investigations with scintigraphy and genetic testing confirmed the diagnosis of hereditary ATTR variant (ATTRv) amyloidosis. This is the first case report of ATTRv amyloi…
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- Renal involvement in familial Mediterranean fever in an Algerian population. [Journal Article]Clin Nephrol. 2023 Feb 03 [Online ahead of print]CN
- The objectives of this study were to investigate the clinical biological and histological renal involvement secondary to familial Mediterranean fever (FMF), the epidemiological data, genetics of our patients and their evolution under treatment. We prospectively studied 58 Algerian patients admitted in our nephrology department from January 2012 to January 2021. The diagnosis of nephropathy was su…
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- Adult-onset MELAS syndrome in a 51-year-old woman without typical clinical manifestations: a case report. [Case Reports]Eur Heart J Case Rep. 2023 Jan; 7(1):ytad028.EH
- Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome is a multi-organ disorder resulting from mitochondrial DNA (mtDNA) mutations. We report a case of suspected MELAS syndrome that progressed to left ventricular dysfunction 24 years after an initial diagnosis of atrioventricular block (AVB).
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- A rare case of unusual scar in atrial fibrillation ablation: The "Yin-Yang" left atrium. [Journal Article]J Arrhythm. 2023 Feb; 39(1):82-83.JA
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- Cardiovascular Manifestations in Rheumatoid Arthritis. [Journal Article]Cardiol Rev. 2022 Dec 20 [Online ahead of print]CR
- Rheumatoid arthritis (RA) is a systemic inflammatory disorder that characteristically affects the joints. RA has extra-articular manifestations that can impact multiple organ systems including the heart, lungs, eyes, skin, and brain. Cardiovascular involvement is a leading cause of mortality in RA. Cardiovascular manifestations of RA include accelerated atherosclerosis, heart failure, pericarditi…
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- Inotersen to Treat Polyneuropathy Associated with Hereditary Transthyretin (hATTR) Amyloidosis. [Journal Article]
- CONCLUSIONS: hATTR is a rare, progressive, and debilitating disease. Its most common presentation is that of polyneuropathy, and it carries a very poor prognosis and a natural history conveying a median survival of < 12 years. Novel therapeutic options are groundbreaking by providing disease-modifying specific, targeted therapies against TTR production and deposition. The use of RNA interference (RNAi) opens the door to the treatment of hereditary diseases by targeting them at the genetic level.
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- Clinicopathological characteristics and disease chronicity in native kidney biopsies in Flanders. [Journal Article]
- CONCLUSIONS: The FCGG registry validates data from previous Western European registries and provides a snapshot of disease chronicity in the whole biopsied Flemish population.
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- Zinc-Epigallocatechin-3-gallate Network-Coated Nanocomposites against the Pathogenesis of Amyloid-Beta. [Journal Article]ACS Appl Mater Interfaces. 2023 Feb 01 [Online ahead of print]AA
- The aggregation of amyloid beta (Aβ) is a hallmark of Alzheimer's disease (AD), a major cause of dementia and an unmet challenge in modern medicine. In this study, we constructed a biocompatible metal-phenolic network (MPN) comprising a polyphenol epigallocatechin gallate (EGCG) scaffold coordinated by physiological Zn(II). Upon adsorption onto gold nanoparticles, the MPN@AuNP nanoconstruct elici…
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- Association of Tafamidis With Health Status in Patients With ATTR Cardiac Amyloidosis: A Post Hoc Analysis of the ATTR-ACT Randomized Clinical Trial. [Journal Article]JAMA Cardiol. 2023 Feb 01 [Online ahead of print]JC
- CONCLUSIONS: In ATTR-ACT, although patients with baseline NYHA class III symptoms had worse overall outcomes, treatment with tafamidis yielded better health status compared with placebo.
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- All-trans retinoic acid works synergistically with the γ-secretase inhibitor crenigacestat to augment BCMA on multiple myeloma and the efficacy of BCMA-CAR T cells. [Journal Article]Haematologica. 2023 02 01; 108(2):568-580.H
- B-cell maturation antigen (BCMA) is the lead antigen for chimeric antigen receptor (CAR) T-cell therapy in multiple myeloma (MM). A challenge is inter- and intra-patient heterogeneity in BCMA expression on MM cells and BCMA downmodulation under therapeutic pressure. Accordingly, there is a desire to augment and sustain BCMA expression on MM cells in patients that receive BCMA-CAR T-cell therapy. …
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- Automated Inline Myocardial Segmentation of Joint T1 and T2 Mapping Using Deep Learning. [Journal Article]
- CONCLUSIONS: Automated inline analysis of joint T1 and T2 mapping allows accurate segment-wise tissue characterization, with performance equivalent to that of human experts.Keywords: MRI, Neural Networks, Cardiac, Heart Supplemental material is available for this article. © RSNA, 2022.
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- A quick glance at selected topics in this issue. [Review]J Nucl Cardiol. 2023 Jan 31 [Online ahead of print]JN
- "A quick glance at selected topics in this issue" aims to highlight contents of the Journal and provide a quick review to the readers.
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- Early autonomic biomarkers in ATTRv carriers. [Editorial]Clin Auton Res. 2023 Jan 31 [Online ahead of print]CA
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- Factors affecting the accuracy of amyloidosis identification and referral to a specialty centre. [Journal Article]Amyloid. 2023 Jan 31 [Online ahead of print]A
- CONCLUSIONS: Recognising these potential sources of diagnostic error in the workup of amyloidosis can improve patient care. Referral to a centre of excellence for amyloidosis helps confirm an accurate diagnosis and avoid mistreatment.
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- Decreased ratios of matrix metalloproteinases to tissue-type inhibitors in cerebrospinal fluid in sporadic and hereditary cerebral amyloid angiopathy. [Journal Article]
- CONCLUSIONS: CSF MMP-2/TIMP-2 and MMP-14/TIMP-2 were consistently decreased in sCAA, compared to controls. Additionally, MMP-14/TIMP-2 levels were also decreased in symptomatic D-CAA, compared to both pre-symptomatic D-CAA and controls, and can therefore be considered a biomarker for sporadic and late-stage hereditary forms of CAA.
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- New challenges in the management of patients with transthyretin cardiac amyloidosis: beyond oral anticoagulation. [Journal Article]Rev Esp Cardiol (Engl Ed). 2023 Jan 27 [Online ahead of print]RE
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- Differential effects of DTT on HEWL amyloid fibrillation and fibril morphology at different pH. [Journal Article]Biophys Chem. 2023 Jan 25; 294:106962.BC
- Proteins can transform from their native state to a state having fibrillar aggregates characterized by cross β sheet structure. The fibrillar aggregates are known as amyloid and have been linked to several disorders. Disulfide bonds in proteins are one of the important factors that determine the propensity of aggregation. Hen Egg White Lysozyme (HEWL) was used by us as a model protein to decipher…
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- Long-term survival in people with transthyretin amyloid cardiomyopathy who took tafamidis: A Plain Language Summary. [Review]Future Cardiol. 2023 Jan 30 [Online ahead of print]FC
- This summary presents the results from an ongoing, long-term extension study that followed an earlier study called ATTR-ACT. People who took part in this extension study and ATTR-ACT have a type of heart disease known as transthyretin amyloid cardiomyopathy (ATTR-CM for short), which causes heart failure and death. In ATTR-ACT, people took either a medicine called tafamidis or a placebo (a pill t…
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- Treatment attrition rates and relevant risk factors in multiple myeloma: A real-world study in China. [Journal Article]
- Background: For multiple myeloma (MM), the proportions of patients reaching the subsequent line of therapy (LOT) decline gradually and real-world data describing the attrition rates of LOT in Chinese MM were limited. Herein, we investigated the attrition rates by subsequent LOTs and their relevant risk factors in MM patients in China. Methods: MM patients who had been hospitalized and received at…
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- Functional connectivity favors hyperactivity leading to synapse loss in amyloidosis. [Preprint]bioRxiv. 2023 Jan 05B
- Hyperactivity is observed in early Alzheimer's disease (AD) in multiple brain regions, including the visual cortex. We recently found that the postsynaptic structures favor visual cortex hyperactivity, which disrupts functional connectivity and leads to visual recognition memory deficits in a mouse AD model. It is unclear whether presynaptic structures also favor hyperactivity and whether hyperac…
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- Outcomes in patients with cardiac amyloidosis undergoing heart transplantation: the eurotransplant experience. [Journal Article]J Heart Lung Transplant. 2023 Jan 12 [Online ahead of print]JH
- CONCLUSIONS: HTX is a viable treatment option for patients at an advanced stage of cardiac amyloidosis as overall survival after transplantation has improved in the modern age. Patients at a very advanced stage of the disease, indicated by low serum albumin and blood pressure, show worse outcomes following HTX. Optimal timing and careful patient selection may therefore be particularly important to further improve post-HTX survival in amyloidosis patients.
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- Localized Amyloidosis of Bilateral Palatine Tonsils Mimicking Tonsillolithiasis. [Journal Article]Ear Nose Throat J. 2023 Jan 29 [Online ahead of print]EN
- A rare disease called localized tonsillar amyloidosis can cause serious problems with airway patency in severe cases. This was the case with an elderly man who experienced difficulty breathing and swallowing due to enlarged palatine tonsils. The physical and imaging findings suggested tonsillolithiasis, and the patient underwent bilateral tonsillectomy. The diagnosis of amyloidosis was confirmed …
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- Linking changes in quality of life to haematologic response and survival in systemic immunoglobulin light-chain amyloidosis. [Journal Article]Br J Haematol. 2023 Jan 29 [Online ahead of print]BJ
- This study reports health-related quality of life (HRQL) among newly-diagnosed immunoglobulin light-chain (AL) patients (n = 914) treated with a bortezomib-based regimen and its association with response depth and survival. Haematologic response/HRQL were assessed over 24 months in an ongoing, prospective study. HRQL change was calculated across haematologic/cardiac response levels. The relations…
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- Budget impact analysis of daratumumab for light chain amyloidosis in Cyprus. [Journal Article]Expert Rev Pharmacoecon Outcomes Res. 2023 Jan 28 [Online ahead of print]ER
- CONCLUSIONS: The introduction of daratumumab for AL amyloidosis with a 90% annual uptake over 5 years, leads to substantial budget impact. Managed entry agreement schemes can be considered in order to mitigate the impact.
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- Quality of Life and Symptoms Among Patients With Relapsed/Refractory AL Amyloidosis Treated With Ixazomib-Dexamethasone Versus Physician's Choice. [Journal Article]Am J Hematol. 2023 Jan 28 [Online ahead of print]AJ
- Patient-reported outcomes in AL amyloidosis have not been well studied. We analyzed health-related quality of life (HRQOL) and AL amyloidosis symptoms data from the phase 3 TOURMALINE-AL1 trial (NCT01659658) (ixazomib-dexamethasone, n=85; physician's choice of chemotherapy [PC], n=83). HRQOL and symptom burden were measured with the SF-36v2, Functional Assessment of Cancer Therapy/Gynecologic Onc…
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