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Efficacy and safety of daratumumab plus bortezomib and dexamethasone in newly diagnosed Mayo 2004 stage IIIA or IIIB light-chain amyloidosis: a prospective phase II study
Haematologica. 2024 Mar 28. doi: 10.3324/haematol.2024.285145. Online ahead of print.ABSTRACTNot available.PMID:38546676 | DOI:10.3324/haematol.2024.285145 (Source: Haematologica)
Source: Haematologica - March 28, 2024 Category: Hematology Authors: Kai-Ni Shen Ya-Juan Gao Long Chang Lu Zhang Xin-Xin Cao Zhuang Tian Yi-Ning Wang Dao-Bin Zhou Jian Li Source Type: research

Patisiran, an RNAi therapeutic for hereditary transthyretin-mediated amyloidosis: Sub-analysis in Taiwanese patients from the APOLLO study
CONCLUSIONS: This analysis suggests that patisiran is well tolerated and may provide a substantial clinical benefit for Taiwanese patients with hATTR amyloidosis with polyneuropathy.TRIAL REGISTRATION INFORMATION: The studies were registered on the ClinicalTrials.gov. The APOLLO study ClinicalTrials.gov identifier is NCT01960348 (https://clinicaltrials.gov/ct2/show/NCT01960348), with the registration date of October 10, 2013, and the first patient was enrolled on December 13, 2013. For the global OLE, the ClinicalTrials.gov identifier is NCT02510261 (https://clinicaltrials.gov/ct2/show/NCT02510261) with the registration da...
Source: J Formos Med Assoc - March 28, 2024 Category: General Medicine Authors: Kon-Ping Lin Chih-Chao Yang Yi-Chung Lee Ming-Jen Lee John Vest Marianne T Sweetser Matthew T White Prajakta Badri Sung-Tsang Hsieh Chi-Chao Chao Source Type: research

Case report: Systemic lupus erythematosus combined with myocardial hypertrophy
CONCLUSION: Myocardial hypertrophy may be an important sign of active and prognostic assessment in SLE diagnosis and management. Similarly, when encountering cases of myocardial hypertrophy, the possibility of autoimmune disease should be considered in addition to common causes.PMID:38533913 | DOI:10.1002/iid3.1214 (Source: Atherosclerosis)
Source: Atherosclerosis - March 27, 2024 Category: Cardiology Authors: Shanshan Wang Xinfeng Wei Wenqing Yang Dan Zhang Source Type: research

Case report: Systemic lupus erythematosus combined with myocardial hypertrophy
ConclusionMyocardial hypertrophy may be an important sign of active and prognostic assessment in SLE diagnosis and management. Similarly, when encountering cases of myocardial hypertrophy, the possibility of autoimmune disease should be considered in addition to common causes. (Source: Immunity, Inflammation and Disease)
Source: Immunity, Inflammation and Disease - March 27, 2024 Category: Allergy & Immunology Authors: Shanshan Wang, Xinfeng Wei, Wenqing Yang, Dan Zhang Tags: SHORT REPORT Source Type: research

Laryngeal Amyloidosis
Amyloidosis is a disease characterized by abnormal extracellular deposition of fibrillar proteins in tissues that creates a chronic inflammatory response leading to local tissue injury. The larynx is estimated to be involved in up to 15% of all amyloidosis cases.1 Symptoms of amyloidosis are nonspecific, and the diagnosis is determined by the severity and laryngeal site involved. It typically presents with dysphonia, but patients can also have dyspnea, stridor, odynophagia, cough, and hemoptysis. (Source: Mayo Clinic Proceedings)
Source: Mayo Clinic Proceedings - March 27, 2024 Category: Internal Medicine Authors: Renata M. Knoll, Peter M. Sadow, Matthew R. Naunheim Tags: Medical image Source Type: research

Efficacy of Computed Tomography-Based Evaluation of Myocardial Extracellular Volume Combined With Red Flags for Early Screening of Concealed Cardiac Amyloidosis in Patients With Atrial Fibrillation
CONCLUSIONS: CT-ECV combined with red flags could contribute to the systematic early screening of concealed ATTR-CM in AF patients undergoing catheter ablation.PMID:38522901 | DOI:10.1253/circj.CJ-23-0948 (Source: Circulation Journal)
Source: Circulation Journal - March 24, 2024 Category: Cardiology Authors: Hirochika Yamasaki Hidekazu Kondo Tomoaki Shiroo Naohiro Iwata Teruaki Masuda Taiki Makita Yuki Iwabuchi Kota Tanazawa Masaki Takahashi Yuma Ono Naoko Ogawa Taisuke Harada Kazuki Mitarai Shuichiro Yamauchi Masayuki Takano Nozomi Kodama Kei Hirota Miho Miy Source Type: research

Detection of cardiac amyloidosis on routine bone scintigraphy: an important gatekeeper role for the nuclear medicine physician
The objective was to evaluate whether all CA cases are detected and what happens during follow-up. Al l routine BS performed at the Maastricht University Medical Center (May 2012–August 2020) were screened for the presence of CA. Scans performed for suspected CA were excluded. A Perugini stage ≥1 was classified as positive necessitating further examination. The electronic medical record system w as evaluated for any contact with cardiology or other specialists until 2021. Of the 2738 BS evaluated, 40 scans (1.46%; median age 73.5 [IQR: 65.8–79.5], 82.5% male) were positive (Perugini grade 1: 31/77.5%, grade 2: 6/15%,...
Source: The International Journal of Cardiovascular Imaging - March 23, 2024 Category: Radiology Source Type: research

Real-World Effectiveness of High-Dose Tafamidis on Neurologic Disease Progression in Mixed-Phenotype Variant Transthyretin Amyloid Cardiomyopathy
ConclusionThis is the first real-world study to demonstrate the potential value of high-dose tafamidis for delaying neurologic disease progression in patients with mixed-phenotype ATTRv-CM. The findings underscore the importance of multidisciplinary assessment for patients with ATTR amyloidosis.Trial registrationClinicalTrials.gov: NCT05139680. (Source: Cardiology and Therapy)
Source: Cardiology and Therapy - March 23, 2024 Category: Cardiology Source Type: research

Impact of Baseline Neuropathy Severity on Vutrisiran Treatment Response in the Phase 3 HELIOS-A Study
ConclusionsVutrisiran demonstrated benefit in neurologic function and other key efficacy measures versus external placebo across all four baseline neuropathy severity quartiles. Patients initiating vutrisiran earlier in their disease course retained the highest neurologic function level after 18 months, highlighting the importance of early diagnosis and treatment.Trial Registration NumberClinicalTrials.gov: NCT03759379. (Source: Neurology and Therapy)
Source: Neurology and Therapy - March 21, 2024 Category: Neurology Source Type: research

Bullous Amyloidosis as a cutaneous feature of Familial Lysozyme Amyloidosis
Australas J Dermatol. 2024 Mar 19. doi: 10.1111/ajd.14250. Online ahead of print.NO ABSTRACTPMID:38504439 | DOI:10.1111/ajd.14250 (Source: The Australasian Journal of Dermatology)
Source: The Australasian Journal of Dermatology - March 20, 2024 Category: Dermatology Authors: R Islam L Abbott A Potter C Thomas Source Type: research