Context: In plasma cell dyscrasias, involvement of the distal tubules is frequent and well characterized. In contrast, proximal tubules have only rarely been reported to show diagnostic pathology such as intracytoplasmic crystals.
Objective: To look for additional morphologic features that might be helpful in the diagnosis of proximal tubulopathy associated with an underlying plasma cell dyscrasia.
Design: We examined patients presenting with nonspecific renal symptoms who were found to have light chain restriction limited to proximal tubular epithelium by immunofluorescence. We correlated these results with light microscopy, electron microscopy, and the clinical findings.
Results: By immunofluorescence, 5 patients had light chain restriction in proximal tubular epithelium. By light microscopy, only 1 patient had focal rhomboid crystals in the proximal tubular epithelium; all other biopsies failed to show any discernible pathology within the proximal tubules or elsewhere in the kidney. By electron microscopy, proximal tubules from 2 patients showed crystals with a latticelike structure, whereas the remaining 3 patients had only prominent phagolysosomes. However, by immunoelectron microscopy, the lysosomal content showed light chain restriction (in 2 cases studied). Post-kidney biopsy, all patients were diagnosed with multiple myeloma or plasma cell dyscrasia. One patient developed renal failure and had recurrence of crystals in the allograft.
Conclusions: Light chain proximal tubulopathy may be associated with the presence of crystals or with the presence of phagolysosomes with light chain restriction as the sole abnormality. Both kappa and lambda light chains may be involved. The prognosis is variable and the pathology may recur in transplants.
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http://dx.doi.org/10.5858/2007-131-1368-ETPSOI | DOI Listing |
Clin Chim Acta
January 2025
Department of Clinical Biochemistry, Aarhus University Hospital, Palle Juul Jensens Boulevard 99 8200 Aarhus N, Denmark; Department of Clinical Medicine, Aarhus University 8000 Aarhus C, Denmark.
Objective: This exploratory study investigates if neurofilament light chain (NfL) is excreted in the urine and whether this depends on plasma NfL (pNfL) levels and kidney function in terms of eGFR and U-albumin-creatinine ratio (uACR).
Methods: Using a computer algorithm, we identified excess urine and plasma from routine testing of uACR and eGFR in patients 45-50 years old. Up to 17 paired urine-plasma samples in each of six categories of kidney function defined by uACR and eGFR were analysed for NfL, and the urinary NfL-creatinine ratio (uNCR) was calculated to correct for urine dilution.
Int J Biol Macromol
January 2025
College of Biological Engineering, Sichuan University of Science & Engineering, Yibin 643000, China; Sichuan Province Engineering Technology Research Center of Liquor-Making Grains, Yibin 643000, China.
Glutinous and japonica sorghum can be applied to different production processes by their amylopectin content and starch structure. However, the differences in the fine structure and physiochemistry properties of their starches, as well as their fermentation properties remain unclear. Compared with japonica sorghum, glutinous sorghum has a higher amylopectin content, short amylose chain content, relative crystallinity, and ∆H, but lower setback (SB), and starch granule size.
View Article and Find Full Text PDFAngew Chem Int Ed Engl
January 2025
Southeast University, Institute of Advanced Materials and School of Chemistry and Chemical Engineering, Institute of Advanced Materials and School of Chemistry and Chemical Engineering, 211189, Nanjing, CHINA.
In nature, organisms adapt to environmental changes through training to learn new abilities, offering valuable insights for developing intelligent materials. However, replicating this adaptive learning in synthetic materials presents a significant challenge. This study introduces a feasible approach to train liquid crystal elastomers (LCEs) by integrating a mechanophore tetraarylsuccinonitrile (TASN) into their main chain, addressing the challenge of enabling synthetic materials to exchange substances with their environment.
View Article and Find Full Text PDFAnn Clin Transl Neurol
January 2025
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
Defining the CSF cytokine/chemokine and injury biomarker signature of glial fibrillary acidic protein (GFAP) autoimmunity can inform immunopathogenesis. CSF GFAP-IgG-positive samples (N = 98) were tested for 17 cytokines/chemokines, neurofilament light chain (NfL), and GFAP (ELLA, Bio-Techne). Controls included non-inflammatory (N = 42), AQP4-IgG-positive (N = 83), CNS infections (N = 13), and neurosarcoidosis (N = 32).
View Article and Find Full Text PDFPlant J
January 2025
Systems Biotechnology Group, Department Microbial Biotechnology, Helmholtz Centre for Environmental Research - UFZ, Leipzig, 04318, Germany.
Biophotovoltaics offers a promising low-carbon footprint approach to utilize solar energy. It aims to couple natural oxygenic photosynthetic electrons to an external electron sink. This lays the foundation for a potentially high light-to-energy efficiency of the Biophotovoltaic process.
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