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1 results

  • Chapter 4 - Haematuric Glomerular Diseases (Collagenopathies)

  • from Section 2 - Glomerular Diseases
  • Edited by Helen Liapis, Ludwig Maximilian University, Nephrology Center, Munich, Adjunct Professor and Washington University St Louis, Department of Pathology and Immunology, Retired Professor
  • Book:
    Pediatric Nephropathology & Childhood Kidney Tumors
    Published online:
    10 August 2023
    Print publication:
    09 March 2023, pp 56-86

  • Summary

    This chapter will present clinical and histopathologic findings, pathophysiology and molecular genetics of glomerular basement membrane (GBM) diseases presenting primarily with hematuria. The GBM is a complex structure mainly composed of collagen IV in its mature form, but also other components such as nidogen, agrin and perlecan. Diseases of collagen IV disorders include Alport syndrome and thin basement membrane disease, Pierson syndrome, caused by mutations in the LAMB2 gene, nail–patella syndrome caused by LMX1B mutations with collagen type III accumulation that requires unmasking, and type III collagenopathy characterised by increased type III collagen in the GBM and the glomerular mesangium. Current and future therapies will also be discussed