Cover Image

Systemic immunoglobulin light chain amyloidosis

Systemic immunoglobulin light chain amyloidosis is a protein misfolding disease caused by the conversion of immunoglobulin light chains from their soluble functional states into highly organized amyloid fibrillar aggregates that lead to organ dysfunction. The disease is progressive and, accordingly,...

Full description

Saved in:
Bibliographic Details
Main Authors: Merlini, Giampaolo (Author) , Dispenzieri, Angela (Author) , Sanchorawala, Vaishali (Author) , Schönland, Stefan (Author) , Palladini, Giovanni (Author) , Hawkins, Philip N. (Author) , Gertz, Morie A. (Author)
Format: Article (Journal)
Language:English
Published: 25 October 2018
In: Nature reviews. Disease Primers
Year: 2018, Volume: 4
ISSN:2056-676X
DOI:10.1038/s41572-018-0034-3
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1038/s41572-018-0034-3
Get full text
Author Notes:Giampaolo Merlini, Angela Dispenzieri, Vaishali Sanchorawala, Stefan O. Schönland, Giovanni Palladini, Philip N. Hawkins, Morie A. Gertz

MARC

LEADER 00000caa a2200000 c 4500
001 1694129039
003 DE-627
005 20230427100442.0
007 cr uuu---uuuuu
008 200406s2018 xx |||||o 00| ||eng c
024 7 |a 10.1038/s41572-018-0034-3  |2 doi 
035 |a (DE-627)1694129039 
035 |a (DE-599)KXP1694129039 
035 |a (OCoLC)1341313805 
040 |a DE-627  |b ger  |c DE-627  |e rda 
041 |a eng 
084 |a 33  |2 sdnb 
100 1 |a Merlini, Giampaolo  |e VerfasserIn  |0 (DE-588)1207790745  |0 (DE-627)1694130657  |4 aut 
245 1 0 |a Systemic immunoglobulin light chain amyloidosis  |c Giampaolo Merlini, Angela Dispenzieri, Vaishali Sanchorawala, Stefan O. Schönland, Giovanni Palladini, Philip N. Hawkins, Morie A. Gertz 
264 1 |c 25 October 2018 
300 |a 19 
336 |a Text  |b txt  |2 rdacontent 
337 |a Computermedien  |b c  |2 rdamedia 
338 |a Online-Ressource  |b cr  |2 rdacarrier 
500 |a Gesehen am 06.04.2020 
520 |a Systemic immunoglobulin light chain amyloidosis is a protein misfolding disease caused by the conversion of immunoglobulin light chains from their soluble functional states into highly organized amyloid fibrillar aggregates that lead to organ dysfunction. The disease is progressive and, accordingly, early diagnosis is vital to prevent irreversible organ damage, of which cardiac damage and renal damage predominate. The development of novel sensitive biomarkers and imaging technologies for the detection and quantification of organ involvement and damage is facilitating earlier diagnosis and improved evaluation of the efficacy of new and existing therapies. Treatment is guided by risk assessment, which is based on levels of cardiac biomarkers; close monitoring of clonal and organ responses guides duration of therapy and changes in regimen. Several new classes of drugs, such as proteasome inhibitors and immunomodulatory drugs, along with high-dose chemotherapy and autologous haematopoietic stem cell transplantation, have led to rapid and deep suppression of amyloid light chain production in the majority of patients. However, effective therapies for patients with advanced cardiac involvement are an unmet need. Passive immunotherapies targeting clonal plasma cells and directly accelerating removal of amyloid deposits promise to further improve the overall outlook of this increasingly treatable disease. 
650 4 |a Angiotensin-Converting Enzyme Inhibitors 
650 4 |a Anti-Bacterial Agents 
650 4 |a Doxycycline 
650 4 |a Heart 
650 4 |a Humans 
650 4 |a Immunoglobulin Light-chain Amyloidosis 
650 4 |a Kidney 
650 4 |a Quality of Life 
650 4 |a Risk Factors 
700 1 |a Dispenzieri, Angela  |e VerfasserIn  |4 aut 
700 1 |a Sanchorawala, Vaishali  |e VerfasserIn  |4 aut 
700 1 |a Schönland, Stefan  |d 1969-  |e VerfasserIn  |0 (DE-588)122405226  |0 (DE-627)705896137  |0 (DE-576)293255792  |4 aut 
700 1 |a Palladini, Giovanni  |e VerfasserIn  |4 aut 
700 1 |a Hawkins, Philip N.  |e VerfasserIn  |4 aut 
700 1 |a Gertz, Morie A.  |e VerfasserIn  |4 aut 
773 0 8 |i Enthalten in  |t Nature reviews. Disease Primers  |d Basingstoke : Nature Publishing Group, 2015  |g 4(2018) Artikel-Nummer 38, 19 Seiten  |h Online-Ressource  |w (DE-627)824452755  |w (DE-600)2819522-X  |w (DE-576)429990839  |x 2056-676X  |7 nnas  |a Systemic immunoglobulin light chain amyloidosis 
773 1 8 |g volume:4  |g year:2018  |g extent:19  |a Systemic immunoglobulin light chain amyloidosis 
856 4 0 |u https://doi.org/10.1038/s41572-018-0034-3  |x Verlag  |x Resolving-System  |z lizenzpflichtig  |3 Volltext 
951 |a AR 
992 |a 20200406 
993 |a Article 
994 |a 2018 
998 |g 122405226  |a Schönland, Stefan  |m 122405226:Schönland, Stefan  |d 910000  |d 910100  |e 910000PS122405226  |e 910100PS122405226  |k 0/910000/  |k 1/910000/910100/  |p 4 
999 |a KXP-PPN1694129039  |e 361911112X 
BIB |a Y 
SER |a journal 
JSO |a {"language":["eng"],"title":[{"title":"Systemic immunoglobulin light chain amyloidosis","title_sort":"Systemic immunoglobulin light chain amyloidosis"}],"recId":"1694129039","origin":[{"dateIssuedDisp":"25 October 2018","dateIssuedKey":"2018"}],"type":{"bibl":"article-journal","media":"Online-Ressource"},"note":["Gesehen am 06.04.2020"],"relHost":[{"titleAlt":[{"title":"Nature reviews / Disease Primers"}],"type":{"bibl":"periodical","media":"Online-Ressource"},"recId":"824452755","origin":[{"publisherPlace":"Basingstoke","dateIssuedDisp":"[2015]-","publisher":"Nature Publishing Group"}],"note":["Gesehen am 30.08.22"],"id":{"zdb":["2819522-X"],"issn":["2056-676X"],"eki":["824452755"]},"disp":"Systemic immunoglobulin light chain amyloidosisNature reviews. Disease Primers","physDesc":[{"extent":"Online-Ressource"}],"pubHistory":["2015 -"],"part":{"volume":"4","text":"4(2018) Artikel-Nummer 38, 19 Seiten","year":"2018","extent":"19"},"language":["eng"],"title":[{"title":"Nature reviews","partname":"Disease Primers","title_sort":"Nature reviews"}]}],"id":{"doi":["10.1038/s41572-018-0034-3"],"eki":["1694129039"]},"physDesc":[{"extent":"19 S."}],"name":{"displayForm":["Giampaolo Merlini, Angela Dispenzieri, Vaishali Sanchorawala, Stefan O. Schönland, Giovanni Palladini, Philip N. Hawkins, Morie A. Gertz"]},"person":[{"family":"Merlini","display":"Merlini, Giampaolo","role":"aut","given":"Giampaolo"},{"family":"Dispenzieri","display":"Dispenzieri, Angela","given":"Angela","role":"aut"},{"given":"Vaishali","role":"aut","display":"Sanchorawala, Vaishali","family":"Sanchorawala"},{"family":"Schönland","role":"aut","given":"Stefan","display":"Schönland, Stefan"},{"display":"Palladini, Giovanni","role":"aut","given":"Giovanni","family":"Palladini"},{"family":"Hawkins","role":"aut","given":"Philip N.","display":"Hawkins, Philip N."},{"family":"Gertz","given":"Morie A.","role":"aut","display":"Gertz, Morie A."}]} 
SRT |a MERLINIGIASYSTEMICIM2520 

Similar Items