Results 61 to 70 of about 32,786 (220)

A Case of Primary Amyloidosis Involving Liver, Stomach, Intestines, and Heart without Evident Kidney Involvement

Российский журнал гастроэнтерологии, гепатологии, колопроктологии, 2022
Aim. Systemic amyloidosis caused by the synthesis and deposition of immunoglobulin light chains (AL amyloidosis) is a relatively rare disease that involves heart, kidneys, peripheral nervous system, gastrointestinal tract, and has a large number of ...
L. S. Kholopov, N. B. Gubegrits, O. A. Dyadyk, Yu. E. Chirkov, Yu. V. Tsohoyeva   +4 more
doaj   +1 more source

Lineage tree analysis of immunoglobulin variable-region gene mutations in autoimmune diseases: chronic activation, normal selection [PDF]

, 2006
Autoimmune diseases show high diversity in the affected organs, clinical manifestations and disease dynamics. Yet they all share common features, such as the ectopic germinal centers found in many affected tissues.
Neta S. Zuckerman, Zuckerman, N.S., Michal Barak, Roshini S. Abraham, Ramit Mehr, Mehr, Ramit, Steiman-Shimony, Avital, David I. Stott, Edelman, H., Hutzler, A., Edelman, Hanna, Zuckerman, Neta S., Stott, David I., Shahaf, G., Deborah Dunn-Walters, Abraham, Roshim S., Shahaf, Gitit, Stott, D.I., Gitit Shahaf, Dunn-Walters, D., Avital Steiman-Shimony, Hutzler, Anat, Barak, M., Steiman-Shimony, A., Barak, Michal, Mehr, R., Dunn-Walters, Deborah, Anat Hutzler, Hanna Edelman, Abraham, R.S.   +29 more
core   +1 more source

Senile Systemic Amyloidosis: Clinical Features at Presentation and Outcome

, 2013
Background Cardiac amyloidosis is a fatal disease whose prognosis and treatment rely on identification of the amyloid type. In our aging population transthyretin amyloidosis (ATTRwt) is common and must be differentiated from other amyloid types.
Banypersad, SM, Whelan, CJ, Lachmann, HJ, Gillmore, JD, Gibbs, SD, Hawkins, PN, McCarthy, CA, Sattianayagam, P, Rowczenio, D, Wechalekar, A, Venner, CP, Petrie, A, Dungu, J, Wassef, N, Pinney, JH, Gilbertson, JA   +15 more
core   +1 more source

Concurrent light chain amyloidosis and proximal tubulopathy: Insights into different aggregation behavior—A case report

eJHaem, 2022
Due to differences in the protein folding mechanisms, it is exceedingly rare for amyloid light chain (AL) amyloidosis and monoclonal gammopathy of renal significance (MGRS) to coexist.
Simone Feurstein, Julian Zoller, Constantin Schwab, Sarah Schreiner, Heiko Mundt, Iris Breitkreutz, Brigitte Schneider, Jörg Beimler, Martin Zeier, Rüdiger Waldherr, Stefan Gröschel, Carsten Müller‐Tidow, Stefan O. Schönland, Ute Hegenbart   +13 more
doaj   +1 more source

Producing amyloid fibrils in vitro: A tool for studying AL amyloidosis

Biochemistry and Biophysics Reports, 2023
Amyloid light-chain (AL) amyloidosis is the second most common form of systemic amyloidosis which is characterized by a high level of mortality and no effective treatment to remove fibril deposition.
Daria V. Sizova, Steve Raiker, Deaneira Lakheram, Vishwanatha Rao, Andrew Proffitt, Yazen Jmeian, Walter Voegtli, Melissa Batonick   +7 more
doaj   +1 more source

Teclistamab in relapsed/refractory light chain amyloidosis: A retrospective multicenter study by the German Society for Amyloid Diseases. [PDF]

Hemasphere
Abstract Systemic light chain (AL) amyloidosis is a rare, acquired protein misfolding disorder characterized by extracellular deposition of misfolded immunoglobulin light chain fibrils, resulting in organ damage. Treatment is based on anti‐plasma cell regimens derived from multiple myeloma therapy.
Carpinteiro A, Kimmich C, Trajanova D, Hegenbart U, Hansen T, Holtick U, von Landenberg-Roberg V, Bohl SR, Teipel R, von Metzler I, Engelhardt M, Shumilov E, Steinhardt M, Reinhardt HC, Oubari S, Schönland S.   +15 more
europepmc   +2 more sources

A prospective study of nutritional status in immunoglobulin light chain amyloidosis

Haematologica, 2013
Weight loss is common in systemic immunoglobulin light chain amyloidosis but there are limited data on the impact of nutritional status on outcome.
Prayman T. Sattianayagam, Thirusha Lane, Zoe Fox, Aviva Petrie, Simon D.J. Gibbs, Jennifer H. Pinney, Signe S. Risom, Dorota M. Rowczenio, Ashutosh D. Wechalekar, Helen J. Lachmann, Janet A. Gilbertson, Philip N. Hawkins, Julian D. Gillmore   +12 more
doaj   +1 more source

A stepwise data interpretation process for renal amyloidosis typing by LMD-MS

BMC Nephrology, 2022
Backgrounds Systemic amyloidosis is classified according to the deposited amyloid fibril protein (AFP), which determines its best therapeutic scheme. The most common type of AFP found are immunoglobulin light chains.
Ming Ke, Xin Li, Lin Wang, Shuling Yue, Beibei Zhao   +4 more
doaj   +1 more source

Chronic myopathy due to immunoglobulin light chain amyloidosis [PDF]

Molecular Genetics and Metabolism, 2013
Amyloid myopathy associated with a plasma cell dyscrasia is a rare cause of muscle hypertrophy. It can be a challenging diagnosis, since pathological findings are often elusive. In addition, the mechanism by which immunoglobulin light-chain deposition stimulates muscle overgrowth remains poorly understood. We present a 53-year old female with a 10-year
Manoli, Irini, Kwan, Justin Y, Wang, Qian, Rushing, Elisabeth J, Tsokos, Maria, Arai, Andrew E, Burch, Warner M, Dispenzieri, Angela, McPherron, Alexandra C, Gahl, William A   +9 more
openaire   +4 more sources

Immunoglobulin light chain amyloidosis: 2018 Update on diagnosis, prognosis, and treatment

American journal of hematology/oncology, 2018
Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues.
M. Gertz
semanticscholar   +1 more source