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Archive: June 2021

Acquired Etiologies of Myopathy with Lobulated Muscle Fibers

Myopathy with Lobulated Muscle Fibers
An elderly male patient presents with a chief complaint of “dull achy pain.” The patient relates that the pain is predominantly in his thighs and hip girdle region, most intense in his left lateral thigh. He says the pain has ongoing and progressive for several months but has significantly worsened over the last few days.  The patient had been recently admitted for “rhabdomyolysis” thought due to his statin medication, which was discontinued; however, no muscle biopsy was performed at that time and his muscle enzymes showed CPK 934.  Other past medical history includes diabetes mellitus, hyperlipidemia, hypertension, hypercalcemia, and vitamin...

Diagnose This (June 14, 2021)

sickled cell within a glomerular capillary
What would be your diagnosis for these findings in a 30-year-old African American male with CKD stage III and proteinuria?     ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​...

Twitter Poll (June 9, 2021)

Which of the following is considered a primary podocytopathy and classically shows an excellent response to steroids? A) Minimal Change B) HSP C) Post-strep GN D) C3 GN Answer: A) Minimal change disease Minimal change disease is classically seen in young children and usually is identified by a sudden onset of nephrotic syndrome characterized by proteinuria > 3.5 g/24 hr, hypoalbuminemia, and edema. Histologically, the biopsy demonstrates minimal to no significant glomerular changes while proximal tubules show increased protein resorption droplets. The major finding however is seen by electron microscopy which demonstrates widespread (complete) podocyte foot process effacement by electron...

C9orf72 Gene

C9orf72 gene, neuronotes, neropathology
The patient is a middle-aged Caucasian male who presents with chief complaint of progressive muscle weakness and muscle cramps of insidious onset. He also reported muscle mass loss and muscle twitching. No muscle pain, no skin rash, or myotoxic medications are reported.  Myositis panel is negative.  CPK levels over the last few months have trended slightly upward, and the most recent CPK is ~800.  EMG/NCS shows denervation-type changes.  No family history of  neuromuscular disorder is reported.  A muscle biopsy of the thigh was pursued to aid in diagnostic work-up, and confirms denervation-type changes that are active and chronic.  A clinical...

U1-RNP-Related Myositis/Myopathy

U1-RNP-Related Myositis/Myopathy
Hematoxylin and eosin (H&E)-stained section of frozen skeletal muscle demonstrates occasional slightly basophilic regenerating muscle fibers and occasional pale eosinophilic necrotic muscle fibers with a subset infiltrated by mononuclear inflammatory cells, consistent with macrophages (i.e. active myophagocytosis is observed).  In addition, there is sparse perivascular inflammation of perimysial vessels without evidence of vessel wall injury.    The patient is an elderly female who presents with chief complaint of progressive symmetric proximal muscle weakness over the past one to two months. She also complains of dysphagia, as well as neck and low back pain. The patient’s medication list includes a statin. ...

Diagnose This (May 31, 2021)

intravascular large B-cell lymphoma (LBCL) in renal biopsy at Arkana Laboratories
This is a CD20 immunohistochemical stain. Knowing that these cells are mostly within peritubular capillaries, what is your diagnosis?     ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​...