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Archive: January 2018

Glomerulomegaly

This biopsy was performed on a 26 year old muscular male with history of persistent subnephrotic proteinuria (UPCR = 2.3 g/g) and preserved renal function. Serum albumin is maintained at 4.2 gm/dl with the remaining serologic workup negative. BMI is 38.2 kg/m2. Of note, the patient has a history of premature birth and has a twin brother with similar clinical presentation. The biopsy shows marked glomerular hypertrophy (Fig 1 and 2) with no evidence of segmental glomerulosclerosis, tubulointerstitial scarring, immune complex deposition or significant foot process effacement. Glomerular hypertrophy (glomerulomegaly) is a form of adaptive structural-functional response which occurs as...

Diagnose This! (January 29, 2018)

What is your diagnosis? Immunofluorescence is negative.     ​   ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​  ...

Pushing Glass (January 26, 2018)

An otherwise asymptomatic 65-year-old man presents for medical evaluation after 15 years of not seeing a physician. He was noted to have an abnormal D-dimer test and was referred for further evaluation to the emergency department where his serum creatinine was found to be 5 mg/dL. Urinalysis showed 2+ proteinuria and 3+ blood. A renal biopsy was ordered. The images are characteristic findings of which of the following: 1. Amyloidosis 2. Fibrillary Glomerulopathy 3. Monoclonal immunoglobulin deposition disease 4. Dense Deposit Disease 5. Lupus nephritis 6. Anti-brush border antibody disease Choice 3 - Monoclonal immunoglobulin deposition disease (MIDD) is characterized...

Light Chain Proximal Tubulopathy with Crystals

The two most common morphologic variants of light chain proximal tubulopathy (LCPT) are LCPT with and without crystal formation. The histopathologic lesions of light chain proximal tubulopathy with crystal formation are shown here. (A) A case with dense crystal deposition resulting in a focally pale appearance to proximal tubules on periodic acid-Schiff stain (original magnification × 100). (B) Crystals are easily identified due to dark staining on Toluidine blue stain (original magnification × 400). (C) A light chain crystal (arrow) stains brightly fuchsinophilic on Masson trichrome stain in this case with rare crystal deposition (original magnification × 400). (D-F) Crystals can...

Fabry Disease in Female Carriers

A renal biopsy was performed on a 51 year old female with strong family history of Fabry disease and a known carrier of an alpha-glycosidase gene (GLA) pathogenic variant. At the time of the biopsy, the patient had no renal or extrarenal manifestations. The biopsy shows glomeruli with prominent visceral epithelial cells (podocytes) displaying ample vacuolated cytoplasm (Fig 1, H&E; Fig 2, Jones). Furthermore, electron microscopy shows numerous large, lamellated lipid vacuoles (myeloid bodies or zebra bodies) within the podocytes, characteristic of Fabry disease (Fig 3). Fabry disease is an X-linked disorder caused by GLA mutation and the resulting deficiency...

Client Spotlight: Cheryl Ellis

“Life’s most persistent and urgent question is, ‘What are you doing for others?’” - Martin Luther King, Jr. This month we’d like to shine the spotlight on Cheryl Ellis, Pathology Assistant at Mercy Gilbert Medical Center, in Gilbert, AZ. Late in the day, Cheryl was informed that a renal biopsy had been delayed and that their last FedEx pickup had already come and gone. Rather than risk further delay, Cheryl acted. She volunteered to drive the specimen directly to her local FedEx facility to be sure it made it to us the next day. We appreciate people like Cheryl, who work...

Diagnose This! (January 22, 2018)

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