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Art of Medicine: Light Chain Proximal Tubulopathy

The painting above shows light chain proximal tubulopathy with crystals, which is one type of a monoclonal gammopathy of renal significance (MGRS).  MGRS, as defined by the International Kidney and Monoclonal Gammopathy Research Group, is a “clonal proliferative disorder that produces a nephrotoxic monoclonal immunoglobulin” (Leung N et al, 2019), and indicates end-organ damage resulting from a monoclonal gammopathy.   The most common MGRS manifestations include AL amyloidosis, monoclonal immunoglobulin deposition disease, light chain cast nephropathy, and light chain proximal tubulopathy.   In light chain proximal tubulopathy, a nephrotoxic monoclonal light chain induces acute tubular injury or proximal tubular dysfunction.  Proximal tubular...

Art of Medicine: Fabry

The image above shows myelinosomes, also known as “zebra bodies” for their appearance on tangential sectioning.  Myelinosomes are seen in Fabry disease, a rare x-linked recessive genetic disorder caused by the defective activity of the enzyme alpha-galactosidase A.   Renal, cardiac, and cerebrovascular involvement is common and can be fatal by the 4th decade without enzyme replacement therapy.   Alpha galactosidase A deficiency results in accumulation of globotriaosylceramide within podocytes of glomeruli, tubules, myocytes, and endothelium.  Globotriaosylceramide accumulation results in the formation of myelinosomes. Since myelinosomes are lipid-derived, they are maintained on osmium fixation, but are lost with exposure to xylenes in...

Art of Medicine: Membranous Glomerulopathy

The painting above depicts membranous glomerulopathy.   A single glomerular capillary loop with confluent subepithelial and intramembranous electron dense deposits along the glomerular basement membrane is shown.  Podocytes are showing foot process effacement and microvillous transformation, which results in loss of the filtration barrier leading to nephrotic syndrome.  An electron micrograph from a patient with membranous glomerulopathy is shown below. Membranous glomerulopathy is the second most common cause of nephrotic syndrome in adults.  A majority of cases are considered primary with autoantibodies directed against the podocyte antigens phospholipase A2 receptor (PLA2R, ~70% of cases) or thrombospondin type 1 domain containing 7A...

Art of Medicine: Uromodulin

Welcome to our first post in the Art of Medicine series! In the hustle and bustle of busy clinics, labs, and hospitals we are often focused on the practical aspects of the practice of medicine. Dr. Tiffany Caza is the content creator for this series as well as being a renal pathologist and artist. Her art focuses on her interpretations of the histologic, cellular, and sub-cellular milieu where a dazzling variety of chemical and structural forces inherent to all life exist. In this series, she will be sharing her art (and occasionally others) and providing correlates to the science represented...