Publications by authors named "Mendelson L"

Background: Circulating cardiac troponin-I (cTnI) plays a crucial role in biomarker staging systems, offering important information for prognostification and risk stratification of patients with AL amyloidosis. High-sensitivity cTnI (HS-cTnI) assays have been introduced in practice; however, the data on the concordance between conventional and HS-cTnI and the utility of HS-cTnI in cardiac biomarker staging are lacking.

Methods: Seventy-eight consecutive patients with AL amyloidosis who were prospectively evaluated at the Boston University Amyloidosis Center from October 2022 through March 2023 were included.

View Article and Find Full Text PDF
Article Synopsis
  • - Patients with AL amyloidosis can still experience organ dysfunction even after achieving a complete hematological response for at least 6 months
  • - Among 143 patients studied, non-response rates varied by organ: 34% for kidneys, 28% for hearts, and 18% for livers
  • - Specific factors contributed to non-response for each organ, with kidney issues linked to more severe dysfunction at diagnosis and heart issues associated with certain light chains; many patients also had residual clonal disease impacting recovery
View Article and Find Full Text PDF
Article Synopsis
  • Immunoglobulin light chain (AL) amyloidosis is a complex disease with diverse treatment options, requiring better strategies to predict patient outcomes.
  • The study used machine learning to analyze data from 2067 patients, identifying three risk groups (low, intermediate, and high) that correlate with significantly different survival rates over time.
  • The research also developed a classifier using accessible clinical data that showed high accuracy in predicting patient risk, representing progress in tailoring treatment strategies for AL amyloidosis.
View Article and Find Full Text PDF

Background: Clostridium autoethanogenum is an acetogenic bacterium that autotrophically converts carbon monoxide (CO) and carbon dioxide (CO) gases into bioproducts and fuels via the Wood-Ljungdahl pathway (WLP). To facilitate overall carbon capture efficiency, the reaction stoichiometry requires supplementation of hydrogen at an increased ratio of H:CO to maximize CO utilization; however, the molecular details and thus the ability to understand the mechanism of this supplementation are largely unknown.

Results: In order to elucidate the microbial physiology and fermentation where at least 75% of the carbon in ethanol comes from CO, we established controlled chemostats that facilitated a novel and high (11:1) H:CO uptake ratio.

View Article and Find Full Text PDF
Article Synopsis
  • * Key factors linked to shorter survival include a delay of over 6 months from symptom onset to diagnosis, high bone marrow plasmacytosis, elevated troponin levels, and advanced heart failure classifications.
  • * Early responses to treatment—both hematologic and cardiac—are crucial for improving survival rates, with significant boosts in overall survival observed for patients achieving these responses within specific timeframes.
View Article and Find Full Text PDF
Article Synopsis
  • Hereditary gelsolin (AGel) amyloidosis is a systemic condition that affects multiple organs with significant neurological symptoms being a prominent feature.
  • A study involving 15 patients highlighted that a majority experienced cranial neuropathy (93%), as well as peripheral and autonomic neuropathy (57%), and carpal tunnel syndrome (73%).
  • Understanding these neurological manifestations is crucial for early diagnosis and can guide the development of better treatments for AGel amyloidosis.
View Article and Find Full Text PDF

Objective: Diagnostic algorithms for amyloidosis have evolved over the past decade, particularly with the incorporation of imaging-based techniques to detect amyloid cardiomyopathy. We sought to identify the key sources of amyloidosis misidentification in the community, which lead to false positive referrals to a tertiary centre.

Methods: We conducted a retrospective review of all referrals to the Amyloidosis Centre from 2010 to 2021 and identified cases lacking amyloid pathology upon final adjudication after extensive assessment at the centre.

View Article and Find Full Text PDF
Article Synopsis
  • A study analyzed 378 patients suspected of having transthyretin amyloid cardiomyopathy (ATTR-CM) who were referred for noninvasive technetium-99m pyrophosphate (PYP) imaging, focusing on the prevalence of light chain abnormalities.
  • Out of these, 26% showed abnormal plasma cell dyscrasia (PCD) markers, and after excluding other conditions, 82 patients were assessed, with 76% having positive PYP scans.
  • The findings indicated that 69% of confirmed ATTR-CM cases exhibited a free light chain (FLC) ratio between 1.65 and 3.1, suggesting that ATTR-CM may be diagnosed without needing a cardiac biopsy when
View Article and Find Full Text PDF

Background:  Primary systemic light-chain (AL) amyloidosis is a rare clonal plasma cell disorder characterized by the production of abnormal immunoglobulin fragments, which form insoluble fibrils that aggregate as amyloid deposits in organs and tissues, leading to organ dysfunction and death.

Objectives:  The aim of this literature review is to increase awareness of AL amyloidosis and educate nurses on the care of this patient population.

Methods:  This overview is based on a literature search of AL amyloidosis, including its pathogenesis, prognosis, and presentation.

View Article and Find Full Text PDF
Article Synopsis
  • High-dose melphalan and stem cell transplantation (HDM/SCT) shows long-term effectiveness in treating AL amyloidosis, with 39% of patients achieving complete hematologic response (CR).
  • The median CR duration is 12.3 years, while overall survival (OS) for patients with CR is impressive, reaching up to 15 years for some.
  • Factors such as elevated dFLC levels and plasma cell percentages are linked to shorter event-free survival (EFS), allowing the development of a risk score to better predict outcomes.
View Article and Find Full Text PDF

Rationale & Objective: Test the feasibility of replacing 24-hour urine collection with a single voided urinary protein-creatinine ratio (UPCR) in patients with amyloid light-chain (AL) amyloidosis.

Study Design: Retrospective study examining the correlation between a 24-hour urine measurement and UPCR at various proteinuria levels using a linear regression analysis with Pearson's correlation coefficient (r). We assessed how using these 2 different measurements would alter the diagnosis, staging, and kidney response assessment in patients with AL amyloidosis.

View Article and Find Full Text PDF

The recent decades have ushered in considerable advancements in the diagnosis and treatment of systemic light chain (AL) amyloidosis. As disease outcomes improve, AL amyloidosis-unrelated factors may impact mortality. In this study, we evaluated survival trends and primary causes of death among 2337 individuals with AL amyloidosis referred to the Boston University Amyloidosis Center.

View Article and Find Full Text PDF

Aquatic organisms jumping for aerial prey require high-performance propulsion, accurate aim, and trajectory control to succeed. Archer fish, capable of jumping up to twice their body length out of the water, address these considerations through multifaceted fin and body kinematics. In this study, we utilized 3D synthetic aperture particle image velocimetry to visualize the wakes of archer fish throughout the jumping process.

View Article and Find Full Text PDF

Introduction: Symptomology of AL amyloidosis can be vague, with a broad range of manifestations and potential etiologies. We sought to determine whether time from initial patient-reported symptom onset to diagnosis was associated with survival.

Methods: The Boston University Amyloidosis Patient Database was queried for patients with AL amyloidosis who presented to the Center for initial evaluation from 2010 to 2015.

View Article and Find Full Text PDF

Background: Peanut oral immunotherapy is an effective treatment for desensitizing peanut-allergic patients, but the frequency of adverse reactions has limited its widespread use.

Objective: To review the frequency of adverse reactions that patients on peanut oral immunotherapy experience during build-up and maintenance phases and explore factors that may contribute to adverse events.

Methods: A retrospective chart review of children and adults with peanut allergy undergoing peanut oral immunotherapy at the New England Food Allergy Treatment Center in West Hartford, Conn was performed.

View Article and Find Full Text PDF

Gastrointestinal cancers are some of the most common malignancies worldwide. Traditional chemotherapy has been disappointing in improving overall survival in patients with unresectable or metastatic disease. The dawn of immunotherapy has led to emerging strategies in incorporating immune checkpoint inhibition either as single agents or in combination when treating gastrointestinal cancers.

View Article and Find Full Text PDF
Article Synopsis
  • * The proportion of systemic immunoglobulin light-chain (AL) amyloidosis cases decreased from 77% to 50%, while ATTR amyloidosis cases rose from 12% to 29%, mainly due to the identification of ATTRwt and ATTRV122I forms.
  • * The findings indicate growing awareness and diagnosis of amyloidosis across the nation, influenced by better diagnostic methods and educational initiatives, despite demographic differences among patients remaining evident.
View Article and Find Full Text PDF
Article Synopsis
  • Systemic immunoglobulin light-chain (AL) amyloidosis is a rare disorder caused by misfolded proteins from plasma cells, resulting in deposits that affect various organs.
  • The symptoms are often vague and can resemble other common diseases, leading to delays in diagnosis, which negatively impacts patient outcomes, especially in cases involving the heart.
  • Increased awareness among healthcare practitioners of the key symptoms and the latest diagnostic tests can help identify AL amyloidosis earlier, ultimately improving patient care and outcomes.
View Article and Find Full Text PDF

Background: Ten percent of the population claims an allergy to penicillin, but 90% of these individuals are not allergic. Patients labeled as penicillin-allergic have higher medical costs, longer hospital stays, are more likely to be treated with broad-spectrum antibiotics, and develop drug-resistant bacterial infections. Most penicillin skin test reagents are not approved by the Food and drug Administration or readily available to evaluate patients labeled penicillin-allergic.

View Article and Find Full Text PDF

Objective: To review the history of the penicillin minor determinants and evaluate their relevance for current diagnosis.

Data Sources: Skin testing to detect immunoglobulin E (IgE) sensitivity to penicillins in patients with a history of penicillin allergy has been the subject of more than 55 years of published research involving tens of thousands of patients.

Study Selections: Selection of data was based on its relevance to the objective of this article.

View Article and Find Full Text PDF
Article Synopsis
  • Many beta-lactam antibiotics are linked to adverse reactions that often result in recorded allergies, though most of these are not clinically significant.
  • Unconfirmed beta-lactam allergies pose a public health risk as patients may avoid effective penicillins and cephalosporins, leading to worse outcomes and increased healthcare challenges.
  • There is a need for standardized testing protocols to confirm allergy status and improve patient safety, as well as further research to understand the regional differences in allergy reporting and beta-lactam usage.
View Article and Find Full Text PDF