Results 41 to 50 of about 19,748 (182)

The impact of limited healthcare access among patients with light chain and transthyretin amyloidosis: real-world survey during COVID-19 lockdown period in France

Orphanet Journal of Rare Diseases
Background The containment strategies during the COVID-19 pandemic between December 2019 and 2022 significantly disrupted the healthcare system. Cardiac amyloidosis has a poor prognosis and requires frequent follow-up in reference centres.
D. Guijarro, A. Jobbe-Duval, S. Aguilhon, F. Bauer, E. Donal, J. C. Eicher, J. Jeanneteau, B. Gellen, D. Kenizou, O. Lairez, B. Lequeux, D. Legallois, P. Réant, M. Salvat, M. F. Seronde, M. Kharoubi, A. Whereat, A. Farrugia, C. Taieb, A. Zaroui, T. Damy   +20 more
doaj   +1 more source

The diagnosis and characteristics of renal heavy-chain and heavy/light-chain amyloidosis and their comparison with renal light-chain amyloidosis [PDF]

Kidney International, 2013
Little is known about the rare entities of heavy- and light-chain amyloidosis (AHL) and heavy-chain amyloidosis (AH). Here, we report the renal and hematological characteristics, pathology, and outcome of 16 patients with renal AH/AHL (5 with AH and 11 with AHL) and compare them with 202 patients with renal light-chain amyloidosis (AL) diagnosed during
Nasr, Samih H., Said, Samar M., Valeri, Anthony M., Sethi, Sanjeev, Fidler, Mary E., Cornell, Lynn D., Gertz, Morie A., Dispenzieri, Angela, Buadi, Francis K., Vrana, Julie A., Theis, Jason D., Dogan, Ahmet, Leung, Nelson   +12 more
openaire   +2 more sources

The Spectrum of Biopsy‐Proven Renal Diseases in Chinese Elderly Patients and Its Temporal Shift Over 20 Years: A Retrospective Cohort Study

AGING MEDICINE, EarlyView.
This 20‐year retrospective study reveals a significant increase in renal biopsy utilization and a pivotal shift in biopsy‐proven renal disease among Chinese elderly patients. Secondary glomerular nephropathy (particularly diabetic nephropathy and hypertensive nephropathy) has surpassed primary glomerular nephropathy as the leading biopsy‐proven ...
Shasha Han, Lengnan Xu, Ying Sun, Fang Fang, Xin Liu   +4 more
wiley   +1 more source

Determinants of Long‐Term Benefit From High Dose Melphalan With Autologous Stem Cell Transplant in AL Amyloidosis

American Journal of Hematology, EarlyView.
ABSTRACT High dose melphalan (HDM) with autologous stem cell transplant is an established treatment for systemic light chain amyloidosis, but its incremental benefit in the era of effective standard intensity therapy is unknown. We retrospectively analyzed 475 transplant‐eligible patients who completed standard intensity treatment with or without HDM ...
Maximilian J. Steinhardt, Ute Hegenbart, Tamer Hellou, Sara Oubari, Murielle Roussel, Rahel Schwotzer, Francis Buadi, David Dingli, Moritz Binder, Taxiarchis Kourelis, Saurabh S. Zanwar, Max J. Rieger, Caroline Morbach, Vladimir Cejka, Bernhard Gerber, Stefan Störk, Hermann Einsele, Shaji Kumar, Morie Gertz, Alexander Carpinteiro, K. Martin Kortum, Eli Muchtar, Angela Dispenzieri, Stefan Schönland   +23 more
wiley   +1 more source

Cardiac Amyloidosis: Clinical Features, Pathogenesis, Diagnosis, and Treatment [PDF]

Türk Patoloji Dergisi
Cardiac amyloidosis is a type of amyloidosis that deserves special attention as organ involvement significantly worsens the prognosis. Cardiac amyloidosis can be grouped under three main headings: immunoglobulin light chain (AL) amyloidosis that is ...
Asuman ARGON, Deniz NART, Funda YILMAZ BARBET   +2 more
doaj   +1 more source

Nonviral Gold Nanoparticle‐Mediated Delivery of CRISPR‐Cas9 Ribonucleoprotein and Long DNA Transgenes Into Primary Blood Cells

Advanced NanoBiomed Research, EarlyView.
CRISPR/Cas9 has revolutionized the field of gene therapy, but delivery remains an outstanding issue. We propose a nonviral gold‐nanoparticle platform for co‐delivery of CRISPR/Cas9 ribonucleoprotein and long 2.1 kilobase dsDNA transgene constructs. This CRISPR‐AuNP is inexpensive to produce and mediate gene editing and DNA delivery in T cells and CD34+
Rachel A. Cunningham, Karthikeya S. V. Gottimukkala, Daniel D. Lane, Katrina Poljakov, Patricia Lipson, Mark R. Enstrom, Alessandro Rizzi, Aude G. Chapuis, Jennifer E. Adair   +8 more
wiley   +1 more source

A Multifaceted Interplay Among Hemophagocytosis, Interleukin‐18, and Type I Interferon Distinguishes Still Disease From Other Autoinflammatory Diseases

Arthritis &Rheumatology, EarlyView.
Objective The unknown pathophysiology and the lack of specific features for systemic juvenile idiopathic arthritis and adult‐onset Still disease (collectively known as Still disease; SD) delay diagnosis and appropriate treatment. The goal of this study was to identify features and mechanisms that distinguish SD from other systemic autoinflammatory ...
Yvonne M. Mueller, Charlotte Girard, Harmen J. G. van de Werken, Stefan J. Erkeland, Dwin G. B. Grashof, Laurie Vaillant, Renske J. H. den Dekker, Iris van Zuijen, Sharon Veenbergen, Fanny Coffin, Eric Bindels, Yvan Jamilloux, Katerina Laskari, Vassili Soumelis, Bruno Fautrel, Cem Gabay, Peter D. Katsikis   +16 more
wiley   +1 more source

Diagnosing Smoldering Multiple Myeloma and Light Chain Amyloidosis in the Setting of Severe Transthyretin Amyloid Cardiomyopathy and Extracardiac Sarcoidosis

Annals of Internal Medicine: Clinical Cases
Light chain (AL) amyloidosis involves deposition of monoclonal immunoglobulin light chain-derived amyloid fibrils, whereas ATTR amyloidosis involves deposition of insoluble antiparallel beta-pleated protein within tissues, leading to organ-specific ...
Robert A. Churchill, Andrew Rosenbaum
doaj   +1 more source

Extensive amyloid deposits in bone marrow aspirate smears

Clinical Case Reports, 2020
Amyloid light‐chain (AL) amyloidosis is the most common form of systemic amyloidosis. It can cause progressive organ dysfunction and eventually death, mainly due to cardiac involvement. Amyloidosis may rarely present as extensive amorphous, purplish‐blue
Ting Hon Stanford Li, Kit Fai Wong, Wai Shan Wong   +2 more
doaj   +1 more source

Skeletal Myopathy as the Initial Manifestation of Light Chain Multiple Myeloma

European Journal of Case Reports in Internal Medicine, 2020
Monoclonal gammopathies due to plasma cell dyscrasias can cause various rare neuromuscular disorders. The peripheral nervous system is most commonly affected, while muscle diseases associated with monoclonal gammopathies are rare. Skeletal myopathy, as a
Mohamed Reda Belkhribchia, Sara Moukhlis, Tarik Bentaoune, Najat Chourkani, Mohamed Zaidani, Mehdi Karkouri   +5 more
doaj   +1 more source