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Twitter Poll (September 16, 2020)

Fibrillary glomerulonephritis, arkana laboratories, twitter poll
Answer: C Fibrillary glomerulonephritis is a rare disease affecting less than 1% of all native kidney biopsies and is due to fibrillary deposits of immunoglobulin that by electron microscopy are randomly arranged fibrils of approximately 18-20 nm in diameter. Clinically patients will present with proteinuria and hematuria. Histologically it can show several patterns within glomeruli including mesangial proliferative, membranoproliferative, or even a crescentic pattern of glomerular injury. Positive staining with the DNAJB9 stain is extremely useful in identifying this disease as it is around 98% sensitive and 99% specific for Fibrillary GN. Associations with Fibrillary GN include autoimmune disease, infection...

Disease Week: Alport Syndrome

alport syndrome, disease week, arkana laboratories
Monday In 1927, Dr. Cecil Alport published a series on "hereditary familial congenital haemorrhagic nephritis" where he described its association with deafness and the gender differences in disease severity.          Interestingly, Dr Alport initially believed the etiology of the disorder was an individual susceptibility to a toxin of an unknown organism, probably belonging to the streptococcal group.   The pathogenesis of the disorder known as Alport syndrome remained unknown until early 1970's, when advances in electron microscopy allowed the identification of characteristic abnormalities in GBMs.   Dr Curtis Atkin, who suffered from Alport syndrome...

Art of Medicine: ANCA-Associated Glomerulonephritis

anca-associated-glomerulonephritis, art of medicine
The painting above shows a crescentic glomerulonephritis and necrotizing arteritis.  A mixed interstitial inflammatory infiltrate is shown in the top left corner, and tubules are dilated with a few red blood cell casts.  This cluster of findings can occur in ANCA-associated disease.  Renal biopsy images representing manifestations of ANCA-associated glomerulonephritis are shown below.  The main differential diagnosis for crescents within glomeruli includes immune complex glomerulonephritides, anti-GBM nephritis, and ANCA-associated glomerulonephritis (pauci-immune), although nearly any glomerulonephritis with mesangial and/or endocapillary proliferation can produce crescents (examples – infection-associated glomerulonephritis, fibrillary glomerulonephritis, and others).  Correlation with ANCA serologies is recommended in any case...

Digging Deeper, or How We Never Quit

Alport Syndrome, Digging Deeper, Arkana laboratories, renal pathology, kidney pathology, nephropathology
We received the biopsy from a 25-year-old female who presented for evaluation of nephrotic range proteinuria.  Lab evaluation reveals a creatinine of 0.55 and 24-hour urine protein is 3.6 g.  Serologies were negative or normal for ANA, hepatitis B, hepatitis C, HIV, and complement levels.  There was no history of hypertension or diabetes. A biopsy was performed to evaluate the source of proteinuria. Two cores of renal tissue were sampled on light microscopy evaluation.  They consisted mostly of medulla, and only two glomeruli were seen in multiple sections. The glomeruli have capillary loops with attenuated contours. The capillary loops appeared...

Lupus Nephritis (Class IV)

Lupus Nephritis, arkana laboratories, teaching point, nephropathology
A 20-year-old female presents with hematuria, proteinuria, and a creatinine of 1.2 mg/dl. Serologies for ANA are positive. Complement levels are decreased. She states that auto-immune disease runs in her family, but is unsure about any specific diagnosis. Serologies for dsDNA, SSA, SSB, rheumatoid factor, hepatitis B, hepatitis C, and HIV are pending. Figure 1 shows mesangial and endocapillary hypercellularity with prominent "hyaline thrombi." Figure 2 shows no significant interstitial fibrosis. Figure 3 shows "wire loops" and no "spikes" and "holes." Figure 4, Figure 5, and Figure 6 shows mesangial and capillary staining with IgG, kappa, and lambda, respectively. Figure...

Diagnose This (January 28, 2019)

GBM, glomerular basement membranes, diagnose this, arkana laboratories
What is your diagnosis?         ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​...

Diagnose This (January 21, 2019)

A limited sample showed this lesion and negative staining on paraffin-retrieved IF tissue. What is your diagnosis?     ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​...

Digging Deeper – Here, There, and Everywhere

digging deeper, Dr. Messias, Arkana Laboratories, pathology diseases
This biopsy came from an elderly gentleman in his 80s, who presented with acute renal failure.  His serum creatinine had increased from a baseline of 1.3 mg/dL up to 6.6 mg/dL.  And in addition to that, urinalysis was positive for proteinuria and blood.  His medical history included pulmonary embolism, BPH, gastrointestinal hemorrhage and GERD.  Multiple serologies were ordered upon presentation, and among these, pANCA and MPO were markedly positive.  A biopsy was performed to investigate the possibility of an ANCA-mediated crescentic glomerulonephritis. Unfortunately, the material obtained for biopsy was small in size, and the longest core, submitted to light microscopy,...