For Publishers
DiscoveryMetadataPeer reviewHostingPublishing
For Researchers
JoinPublishReviewCollect
Register
Blog
About
Search
Advanced search
My ScienceOpen
Search
For Publishers
For Researchers
Blog
About
1
views
51
references
 Top references
cited by
2
    
0 reviews
 Review
0
comments
 Comment
0
recommends
+1 Recommend
0 collections
    0
    shares
      515
      similar
       All similar
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      The pathogenesis of renal injury and treatment in light chain deposition disease

      review-article

      Read this article at

      ScienceOpenPublisherPMC
      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Light chain deposition disease (LCDD) is a rare clinical disorder. The deposition of light chain immunoglobulins mainly affects the kidneys, which have different characteristics than other tissues. To date, the therapeutic approach for the treatment of LCDD has no evidence-based consensus, and clinical experience of reported cases guides current disease management strategies. The present systematic review investigates and summarizes the pathological mechanisms of renal injury and the subsequent treatments for LCDD.

          Related collections

          Most cited references51

          • Record: found
          • Abstract: found
          • Article: not found

          Renal monoclonal immunoglobulin deposition disease: a report of 64 patients from a single institution.

          Sanjeev Sethi, Nelson Leung, M Valeri … (2012)
          To better define the clinical-pathologic spectrum and prognosis of monoclonal immunoglobulin deposition disease (MIDD), this study reports the largest series. Characteristics of 64 MIDD patients who were seen at Mayo Clinic are provided. Of 64 patients with MIDD, 51 had light chain deposition disease, 7 had heavy chain deposition disease, and 6 had light and heavy chain deposition disease. The mean age at diagnosis was 56 years, and 23 patients (36%) were ≤50 years of age. Clinical evidence of dysproteinemia was present in 62 patients (97%), including multiple myeloma in 38 (59%). M-spike was detected on serum protein electrophoresis in 47 (73%). Serum free light chain ratio was abnormal in all 51 patients tested. Presentation included renal insufficiency, proteinuria, hematuria, and hypertension. Nodular mesangial sclerosis was seen in 39 patients (61%). During a median of 25 months of follow-up (range, 1-140) in 56 patients, 32 (57%) had stable/improved renal function, 2 (4%) had worsening renal function, and 22 (39%) progressed to ESRD. The mean renal and patient survivals were 64 and 90 months, respectively. The disease recurred in three of four patients who received a kidney transplant. Patients with MIDD generally present at a younger age than those with light chain amyloidosis or light chain cast nephropathy. Serum free light chain ratio is abnormal in all MIDD patients, whereas only three-quarters have abnormal serum protein electrophoresis. The prognosis for MIDD is improving compared with historical controls, likely reflecting earlier detection and improved therapies.
          Article 0 comments Cited 79 times – based on 0 reviews     Review now
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Matrix metalloproteinases: structures, evolution, and diversification.

            Irina Massova, Lakshmi Kotra, Rafael Fridman … (1998)
            A comprehensive sequence alignment of 64 members of the family of matrix metalloproteinases (MMPs) for the entire sequences, and subsequently the catalytic and the hemopexin-like domains, have been performed. The 64 MMPs were selected from plants, invertebrates, and vertebrates. The analyses disclosed that as many as 23 distinct subfamilies of these proteins are known to exist. Information from the sequence alignments was correlated with structures, both crystallographic as well as computational, of the catalytic domains for the 23 representative members of the MMP family. A survey of the metal binding sites and two loops containing variable sequences of amino acids, which are important for substrate interactions, are discussed. The collective data support the proposal that the assembly of the domains into multidomain enzymes was likely to be an early evolutionary event. This was followed by diversification, perhaps in parallel among the MMPs, in a subsequent evolutionary time scale. Analysis indicates that a retrograde structure simplification may have accounted for the evolution of MMPs with simple domain constituents, such as matrilysin, from the larger and more elaborate enzymes.
            Article 0 comments Cited 78 times – based on 0 reviews     Review now
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Transgenic mice with increased plasma levels of TGF-beta 1 develop progressive renal disease.

              M Mozes, P. Nagy, P Klotman … (1996)
              Several lines of evidence suggest that local production of transforming growth factor-beta (TGF-beta) contributes to renal disease, particularly to the accumulation of the extracellular matrix protein that characterizes glomerulosclerosis and interstitial fibrosis. We have examined whether elevated levels of circulating TGF-beta adversely affect the kidney. We have studied mice that are transgenic for an active form of TGF-beta 1 under the control of murine albumin promoter and enhancer DNA sequences. These mice express the transgene exclusively in the liver and have elevated plasma concentrations of TGF-beta 1. Renal disease was seen in two of three lines of Alb/TGF-beta 1 transgenic mice; these two lines had the highest levels of hepatic transgene expression and the highest plasma TGF-beta 1 levels. Histologic abnormalities, which included mesangial expansion and thickened capillary loops, were noted in the glomeruli by 3 weeks of age. Interstitial fibrosis and tubular atrophy appeared subsequently. Mice from Line 25, the line with highest levels of TGF-beta 1, developed proteinuria by 5 weeks of age. These mice subsequently manifested nephrotic syndrome with ascites and progressive azotemia; uremic death occurred in more than 25% of the mice by 15 weeks of age. The glomeruli contained immune deposits in subendothelial and mesangial locations, but complement deposition was infrequent. Ultrastructural examination revealed an increase in extracellular matrix material, including collagen fibrils, in subendothelial and mesangial locations. Increased levels of circulating TGF-beta 1 induced progressive renal disease that was characterized by mesangial expansion, accumulation of glomerular immune deposits and matrix proteins, and interstitial fibrosis in this transgenic mouse model. These data suggest that chronically elevated circulating levels of TGF-beta 1 induce progressive glomerulosclerosis.
              Article 0 comments Cited 64 times – based on 0 reviews     Review now
                Bookmark
                All references

                Author and article information

                Contributors
                Gaosi Xu: gaosixu@163.com
                Journal
                Journal ID (nlm-ta): J Transl Med
                Journal ID (iso-abbrev): J Transl Med
                Title: Journal of Translational Medicine
                Publisher: BioMed Central (London )
                ISSN (Electronic): 1479-5876
                Publication date (Electronic): 25 November 2019
                Publication date PMC-release: 25 November 2019
                Publication date Collection: 2019
                Volume: 17
                Electronic Location Identifier: 387
                Affiliations
                [1 ]GRID grid.412455.3, Department of Nephrology, , The Second Affiliated Hospital of Nanchang University, ; No. 1, Minde Road, Donghu District, Nanchang, Zip Code: 330006 People’s Republic of China
                [2 ]GRID grid.260463.5, ISNI 0000 0001 2182 8825, Medical Center of the Graduate School, , Nanchang University, ; Nanchang, China
                [3 ]Department of Nephrology, People’s Hospital of Xinyu City, No. 369, Xinxin North Avenue, High-tech District, Xinyu, People’s Republic of China
                Article
                Publisher ID: 2147
                DOI: 10.1186/s12967-019-02147-4
                PMC ID: 6878616
                PubMed ID: 31767034
                SO-VID: 6434b3a5-43ee-4c77-90ff-5bb4df515679
                Copyright © © The Author(s) 2019
                License:

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                Date received : 19 May 2019
                Date accepted : 19 November 2019
                Funding
                Funded by: the Supporting Project for the Foregoers of Main Disciplines of Jiangxi Province
                Award ID: 20162BCB22023
                Award Recipient :
                Funded by: National Natural Science Foundation of China
                Award ID: 81970583
                Award ID: 81560132
                Categories
                Subject: Review
                Custom metadata
                issue-copyright-statement © The Author(s) 2019

                ScienceOpen disciplines: Medicine
                Keywords: light chain deposition disease,renal injury,biopsy,chemotherapy,treatment
                Data availability:
                ScienceOpen disciplines: Medicine
                Keywords: light chain deposition disease, renal injury, biopsy, chemotherapy, treatment

                Comments

                Comment on this article

                scite_

                Similar content515

                See all similar

                Cited by2

                See all cited by

                Most referenced authors537

                See all reference authors