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Archive: August 2017

PLA2R

Autoantibody formation directed against PLA2R1 is the underlying etiology in most cases of primary membranous glomerulopathy. This new understanding of the pathogenesis of primary membranous has rapidly transformed diagnosis and monitoring of this disease. Proteinuria can take months to resolve after a patient has undergone serologic remission and, as a result, is not an ideal biomarker of disease activity. Testing for levels of serum PLA2R autoantibodies gives a more accurate picture of the patient’s current disease status. Ideally, serum PLA2R testing would be performed immediately after biopsy diagnosis in cases with positive PLA2R staining on biopsy. At this point in...

Lead Toxicity

The arrow in this image points to a cluster of tubular epithelial cells whose nuclei contain eosinophilic intranuclear inclusions characteristic of acute lead nephrotoxicity. In addition to inclusions, acute nephrotoxicity from lead and other heavy metals (e.g. platinum, mercury, gold, copper, iron, and lithium) is usually associated with morphologic features of acute tubular injury. As with all suspected cases of heavy metal toxicity, clinical correlation to assess for recent or past heavy metal exposure is required for an accurate diagnosis. In cases of chronic lead exposure, especially to low lead levels, the relationship between exposure and chronic kidney disease or...

Museum of Discovery Visit

We enjoyed showing our laboratories to our friends at the Museum of Discovery today. We're excited to continually build partnerships with other science-based organizations.

Chronic Active Tubulointerstitial Nephritis

Chronic tubulointerstitial nephritis in children may be secondary to a number of possible etiologies such as drug and other exogenous toxin exposure, infections, lower urinary tract obstruction, autoimmune diseases, nephronophthisis and genetic disorders such as autosomal dominant tubulointerstitial kidney disease due to MUC1, UMOD, REN or HNF1β. Figures 1-3 are representative images from the kidney biopsy of a 15 month old male with a history of prune belly syndrome, status post multiple lower urinary tract procedures and recurrent urinary tract infections. There is severe tubulointerstitial scarring which is disproportionate to the degree of glomerulosclerosis, with a moderate, predominantly chronic, interstitial...

Diagnose This! (August 28, 2017)

What is this finding and what is the most likely etiology?     ​   ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​...

Lace Up

Bringing a pair of sneakers to work is a good reason to get up from your desk and get some fresh air!

Focal Segmental Glomerulosclerosis Not Otherwise Specified

Focal segmental glomerulosclerosis (FSGS) is a nonspecific pattern of glomerular scarring in which a subset of the glomeruli show partial involvement of the glomerular tuft. It should not be confused with a specific disease. The differential diagnosis is too long to include here but includes genetic etiologies, primary injury to podocytes, and adaptive responses to diseases such as hypertension and obesity. Clinicopathologic correlation can be useful to identify patients with primary FSGS. Specifically, primary FSGS is more likely if >80% foot process effacement is present by electron microscopy and the clinical evaluation reveals nephrotic syndrome (not just nephrotic-range proteinuria) including...

Light Chain Proximal Tubulopathy Crystals

This image shows the prominent intracytoplasmic tubular epithelial crystals characteristic of a subset of light chain proximal tubulopathy cases associated with Fanconi syndrome (normoglycemic glycosuria, aminoaciduria, uricosuria, hyperphosphaturia with hypophosphatemia). The crystals represent precipitated nephrotoxic monoclonal light chains, and unlike cases without crystals, pronase-digested immunofluorescence may be helpful in proving light chain restriction when routine immunofluorescence is negative. Light chain proximal tubulopathy with crystals indicates an underlying clonal plasma cell proliferation, and may be seen in patients with monoclonal gammopathy of undetermined significance (MGUS), multiple myeloma, or lymphoma. Evidence of light chain cast nephropathy and/or monoclonal immunoglobulin deposition disease may also...

Pushing Glass (August 22, 2017)

An 85-year-old gentleman presents to the clinic with nephrotic range proteinuria and a creatinine of 10.0 mg/dl. He reports a 20 lb weight loss over the last 2 months and says that he is having trouble keeping down anything solid. A SPEP and UPEP show a monoclonal kappa band. What is the best diagnosis? a. Light Chain Cast Nephropathy b. Light Chain Deposition Disease c. Light Chain Tubulopathy d. AL-Type Amyloidosis The correct answer is a (light chain cast nephropathy). The biopsy shows several fractured casts with a cell reaction. The casts are PAS-negative and “chatter” marks are identified. The...