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Disease Week: Membranous Glomerulopathy

Membranous glomerulopathy: Looking forward Many excellent reviews are available that detail the current state of knowledge in membranous glomerulopathy so I will not attempt to round out this #DiseaseWeek by writing another. Instead, I would like to paint, with broad brush strokes, what I believe to be the near future of diagnostics in membranous glomerulopathy and discuss the techniques of discovery being applied today that are likely to similarly impact other types of immune complex-mediated glomerulonephritis. We live in exciting times for discovery in medicine as the tools available today are so powerful that the molecular pathogenic mechanisms of disease...

Twitter Poll (February 6, 2019)

Answer: B Minimal change disease is the most common glomerular disease associated with thymoma. Classically, the solid tumors most commonly associated with MN are lung, bronchus, and gastric cancers, followed by renal cell, prostate and thymoma. Other cancers reported with MN are colorectal, pancreatic, esophageal and hepatic carcinomas. References: Ronco PM. Paraneoplastic glomerulopathies: New insights into an old entity. Kidney Int 56: 355–377, 1999 Bacchetta J, Juillard L, et al. Paraneoplastic glomerular diseases and malignancies. Crit Rev Oncol Hematol 70: 39–58, 2009 Lefaucheur C, Stengel B, et al. Membranous nephropathy and cancer: Epidemiologic evidence and determinants of high-risk cancer association....

Arkana Employee Spotlight: Christa

One of the voices you hear when you call our office belongs to Christa Heller. She joined Arkana in 2014 and has become an integral part of our client service efforts. A core principle here at Arkana is that every call that comes in during business hours should be answered by an actual person. The job of answering the phones here is far from your typical receptionist position. These team members learn the ins and outs of every step required to turn slides into the report that gets delivered to each party involved in the patient’s case.  They are air...

Twitter Poll (January 30, 2019)

ANSWER: B The medulla is the only compartment affected by AAPOAIV amyloidosis, sometimes with large amounts of amyloid. Glomeruli and vessels are spared and not affected by this type of amyloid. AAPOAIV Amyloidosis is a systemic amyloidosis reported from wild-type apolipoprotein AIV. When the kidney is involved the patient presents with gradual loss of renal function and proteinuria. It may also have cardiac involvement. References: Sethi S, Theis JD, Shiller SM, et al. Medullary amyloidosis associated with apolipoprotein A-IV deposition. Kidney Int 81: 201–206, 2012. Dasari S, Amin S, Kurtin P, et al. Clinical, biopsy, and mass spectrometry characteristics of...

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...

Renal Oxalosis

A 25-year-old female presents to the hospital with malaise and a creatinine of 3.5. She reports that she was in her normal state of health until about 2 weeks ago when she had the “flu.” The patient has minimal proteinuria and bland urine sediment. A kidney biopsy was obtained. Figure 1 shows a normal glomerulus. Figure 2 shows severe chronicity. Figure 3 and 4 show polyhedral clear crystals within the tubular lumina. Figure 5 shows an increased number of oxalate crystals under polarized light. Renal oxalosis is characterized by excess deposition of calcium oxalate crystals within the kidney. There are...

Diagnose This (January 28, 2019)

What is your diagnosis?         ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​...

Twitter Poll (January 23, 2019)

ANSWER: A Although glomerular disease in patients with CLL/SLL is less common, the most frequent patterns of glomerular injury seen include MPGN (immune complex mediated), MCD, membranous GN, and monoclonal immunoglobulin deposition disease. The most common form of kidney involvement in patients with CLL/SLL is the presence of direct infiltration of the kidney parenchyma by leukemic cells. Although glomerular disease in patients with CLL/SLL is less common, they can be present.  The glomerular pattern of injury frequently seen in these group of patients include membranoproliferative glomerulonephritis (immune complex mediated), minimal change disease, membranous nephropathy, and monoclonal immunoglobulin deposition disease (nonamyloid...