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10.1002/mus.24495

http://scihub22266oqcxt.onion/10.1002/mus.24495
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C4577279!4577279!25363903
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suck abstract from ncbi


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pmid25363903      Muscle+Nerve 2015 ; 51 (3): 449-54
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  • Progressive polyradiculoneuropathy due to intraneural oxalate deposition in type 1 primary hyperoxaluria #MMPMID25363903
  • Berini SE; Tracy JA; Engelstad JK; Lorenz EC; Milliner DS; Dyck PJ
  • Muscle Nerve 2015[Mar]; 51 (3): 449-54 PMID25363903show ga
  • Introduction: A 24 year old man with primary hyperoxaluria type 1 (PH1) presented with a rapidly progressive axonal and demyelinating sensorimotor polyradiculoneuropathy shortly after the onset of end stage renal disease. His plasma oxalate level was markedly elevated at 107 µmol/L (normal: <1.8 µmol/L). Methods: A sural nerve biopsy was performed. Teased fiber, paraffin and epoxy sections, and morphometric procedures were performed on this sample and on an archived sample from a 22 year old man as an age- and gender-matched control. Embedded teased fiber electron microscopy was also performed. Results: The biopsy revealed secondary demyelination and axonal degeneration. Under polarizing light, multiple bright hexagonal, rectangular, and starburst inclusions typical of calcium oxalate monohydrate crystals were seen1,2,3. Discussion: Proposed mechanisms of nerve damage include disruption of axonal transport due to crystal deposition, toxic effect of oxalate, or nerve ischemia related to vessel occlusion from oxalate crystal deposition.
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